Abstracts - IANCON 2024

Abstract ID: 3: Impairment in Higher-Order Vision Predicts Cognitive Impairment and Severity in Patients with Parkinsonian Disorders

Lesley Ponraj, Appaswamy Thirumal Prabhakar, Shaik Atif Iqbal ahamed

Christian Medical College, Vellore, Tamil Nadu, India

Background and Aim: Visual processing impairment is a phenomenon described in parkinsonian disorders (PD). Tests used in this study are dot motion test (DMT), biological motion test (BMT), and noise pareidolia test (NPT). It still remains unknown whether these tests can predict cognitive impairment and whether it will be different in various parkinson’s disease subtypes. Our aim was to see if abnormalities in the higher-order visual impairment tests predicted severity, cognitive impairment, psychosis, and subtype of parkinsonism.

Methodology: This was a retrospective electronic medical record-based study done in 109 parkinsonian patients who underwent higher-order visual testing in a tertiary hospital.

Results: Performance on NPT was associated with vascular PD (F = 6.128, P = 0.015). Impairment in BMT was associated with the Hoehn & Yahr (HNY) stage (F = 10.049, P = 0.002). Poor performances on DMT (F = 7.221, P = 0.008) and BMT (F = 26.541, P < 0.001) were associated with low Montreal Cognitive Assessment scores predicting cognitive impairment. However, receiver operating characteristic curves of the tests did not show any significant results to predict the subtype of PD.

Conclusions: Higher-order visual testing, namely BMT and DMT, can predict the severity and cognitive impairment in patients with PD. NPT can be a tool to identify vascular contributions to PD.

Abstract ID: 4: Linezolid-Induced Peripheral Neuropathy in Multidrug-Resistant Tuberculosis – A Prospective Observational Study

Gaurav Chaudhary, Pawan T. Ojha, Akash Chheda, Kamlesh A. Jagiasi, Rakesh Singh, Arjun G. Shah, Anuradha P. Mahto, Aamna Maniyar, Shashank Nagendra, Billia K Appu, Karthik Gaud, Harshil Bagadia

Grant Medical College and JJ Hospital, Mumbai, Maharashtra, India

Background and Aim: Linezolid is used for treatment of multidrug-resistant tuberculosis (MDR TB). One of the common adverse effects associated with the treatment is peripheral neuropathy. Dose modification or interruption at the onset of peripheral neuropathy can prevent the development of irreversible deficits. We aim to study the clinical features, grade the severity of linezolid-induced peripheral neuropathy, and determine the effects of dose modification to avoid long-term deficits.

Methodology: MDR TB patients on linezolid treatment who developed peripheral neuropathy were studied. National Cancer Institute Neurosensory Scale was used to grade patients into mild, moderate, and severe neuropathy. Patients with mild neuropathy were continued on linezolid 600 mg. In patients with moderate neuropathy, the dose was reduced to 300 mg, and linezolid was withdrawn for severe neuropathy patients. Follow-up was done regularly and dose modified accordingly. End result was analyzed at 18 months of tuberculosis treatment completion.

Results: Eighty-four patients were included in the study. On presentation, 31 (36.9%) patients had mild neuropathy, 33 (39.28%) had moderate neuropathy, and 20 (23.8%) had severe peripheral neuropathies. Linezolid was stopped early in 41.6% patients. Improvement or stabilization of symptoms from onset was observed in 67.7% of patients in mild, 60.6% of patients in moderate, and 55% of patients in severe group. Moreover, 16.6% patients had persistent severe deficits at 18 months.

Conclusions: This study highlights the importance of grading linezolid-induced peripheral neuropathy and the need to provide standard guidelines for dose modification to prevent irreversible severe deficits.

Abstract ID: 5: The Utility of Clock Drawing Test and Cube Construction in Diagnosis of Early Cognitive Impairment – A Critical Analysis

Sayooja Sachithanandan

Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Thiruvananthapuram, Kerala, India

Background and Aim: Clock drawing test (CDT) and cube construction test (CCT) are simple-to-administer tasks which require multiple cognitive domains. This study was aimed to find out the utility of these tests in relation to global tools for screening early cognitive impairment (ECI).

Methodology: All subjects meeting the inclusion and exclusion criteria were categorized into cognitively normal (CN), mild cognitive impairment (MCI), and early dementia (ED) using standard clinical criteria. The mean CDT and CCT scores as well as types of qualitative errors were compared among the three groups. Spearman’s rank correlation test was used to study the relationship of various factors with CDT and CCT, and receiver operating characteristic curve was plotted to determine the utility of these tests as a screening tool in diagnosing MCI and ED from CN.

Results: A total of 228 subjects with a mean age of 69 ± 6.7 years were categorized into three groups. A cut-off score of 14 for CDT and 25 for CCT had optimal sensitivity and specificity to diagnose MCI from CN. Similar cut-off scores were obtained to diagnose ED from CN (12 for CDT and 22 for CCT). Age was negatively correlated and level of education was positively correlated to CDT and CCT score among CN subjects, but not in ECI subjects. Qualitative errors patterns of CDT were distinct among MCI and ED.

Conclusions: CDT and CCT were found to be performing better in diagnosing ED from CN than MCI from CN. CDT scored better as a screening tool than CCT possibly due to better objectivity in the scoring system.

Abstract ID: 6: Effect of Viewing the Event on Psychologic Outcomes in Patients with Psychogenic Nonepileptic Events (PNEE): A Randomized Controlled Trial

Lakshmi Priya, Ashalatha Radhakrishnan, Ramshekhar N. Menon

Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Thiruvananthapuram, Kerala, India

Background and Aim: Psychogenic nonepileptic events (PNEE) is one of the common disorders that feigns a true epilepsy or movement disorder, yet there are no current treatment guidelines to manage it even after setting up the diagnosis. This study aimed to determine the effect of viewing one’s own event as a novel treatment strategy in PNEE.

Methodology: This was a single-center, double-blinded, randomized controlled trial, where patients with at least one habitual psychogenic event recorded in the epilepsy monitoring unit were recruited. The participants were randomized (1:1) into two arms and all were given standard psychotherapy, while the intervention arm patients were shown their own recorded event in addition. The psychologic status (primary outcome), measured using standard questionnaires Hospital Anxiety and Depression Scale (HADS) and Quality of Life In Epilepsy-31 (QoLIE- 31) and event frequency (secondary outcome), was compared between baseline and follow-up at 3 months. Wilcoxon singed-rank test and Chi-square test were used to compare both the arms. Trial Registration information: CTRI/2021/04/033220

Results: Sixty-five participants (31 in the intervention /34 in the non-intervention arm) with a mean age of 32.3 years in the intervention arm and 29.2 years in the non-intervention arm were recruited. QoLIE-31 scores in the intervention group showed significant improvement in seizure worry (52.5, 95% confidence interval [CI] [32.5–60.0] to 44.0, 95% CI [33.0–52.0], P = 0.011), overall quality of life (52.5, 95% CI [32.5–60.0] to 65.0, 95% CI [52.5–72.5], P = 0.001), and emotional well-being (60.0, 95% CI [52.0–64.0] to 52.0, 95% CI [48.0–56.0], P = 0.004) compared to the scores in the non-intervention group. HADS-A, HADS-D, and event frequency improved favourably in both the arms.

Conclusions: Event viewing improves psychologic status in PNEE, especially regarding seizure worry and emotional well-being, without adverse outcomes.

Abstract ID: 7: Utility of OCT-RNFL in Disease Phenotyping, Prospective Differentiation, and Monitoring Treatment Responses in Idiopathic Intracranial Hypertension

Gomathy Krithiga, Angel Miraclin T, A T Prabhakar, Sanjith Aaron, Vivek Mathew, Ajith Sivadasan, Anupama Arthur

Christian Medical College, Vellore, Tamil Nadu, India

Background and Aim: Idiopathic intracranial hypertension (IIH) may cause vision loss with severe morbidity, thus necessitating the need for noninvasive biomarkers, such as optical coherence tomography (OCT), for deciding management strategies. Our study aimed to determine the association of OCT–retinal nerve fiber layer (RNFL) thickness with clinical, radiologic features, cerebrospinal fluid (CSF) opening pressure, and visual impairment and its potential to predict improvement in disease status.

Methodology: Our study was a retrospective cohort study of 66 patients with IIH hospitalized between 2019 and 2024. Visual outcomes included visual acuity, fields, OCT–RNFL measurements, optic nerve, and optic nerve sheath diameter (ONSD). Patients were classified based on the degree of vision impairment as normal, mild, and severe. Statistical analysis was performed using Statistical Package for the Social Sciences version-25. P -values were significant at <0.05.

Results: In our study, 92% were females; the median age of patients was 31 years. The median follow-up was 7 months. Disease cohorts included those with normal vision (52.3%), those with mild impairment (34.4%), and those with severe impairment (14.3%). 1. Patients with severe vision impairment had higher CSF opening pressures compared to patients with normal vision ( P =0.001 * ) and patients with mild vision impairment ( P = 0.007 * ). 2. OCT–RNFL showed significant correlation with grades of papilledema and degree of vision impairment in the right eye in all quadrants with early atrophy changes in the temporal quadrant ( P –=0.026 * ) and thickening more prominent in the inferior quadrant ( P = 0.032 * ). Change in OCT–RNFL thickness and baseline optic nerve diameter correlated with vision recovery. 3. ONSD had a strong positive correlation with CSF opening pressures (R: P = 0.002 * , L: P = 0.001 * ).

Conclusions: The multi-quadrant RNFL values, along with optic nerve and sheath diameter values could help in better characterization of disease status and serve as a biomarker for prognostication.

Abstract ID: 8: Transcranial Doppler in Detection of Intracranial Vasculopathy and Prognosis in Patients with Tuberculous Meningitis

Sachidananda Swain, Sanjeev Kumar Bhoi

All India Institute of Medical Sciences, Bhubaneswar (AIIMS, Bhubaneswar), Odisha, India

Background and Aim: Cerebral infarct due to intracranial vasculopathy is the most severe complication of tuberculous meningitis (TBM). Transcranial Doppler (TCD) allows early detection and monitoring of cerebral hemodynamics. The aims and objectives of the study were to 1) detect cerebral vasculopathy in adult TBM patients by using TCD, 2) correlate TCD (Lindgaard ratio) with cerebrospinal fluid (CSF) parameters, 3) study prognosis of the disease by serial TCD monitoring (comparing Modified Rankin Scale (mRS) with Lindegaard ratio (LR)), and 4) correlate TCD with neuroimaging.

Methodology: Consecutive TBM patients admitted in the Department of Neurology were evaluated. Serial TCD was performed two times during hospital stay and thereafter at 1- and 3-month follow-up after obtaining written informed consent. TCD findings (LR) were evaluated and then correlated with various parameters (CSF cells and protein, mRS, neuroimaging) by using Statistical Package for the Social Sciences version 26.

Results: Out of 33 TBM patients (22 male) who underwent TCD imaging, 23 patients (69%) showed high Lindegaard ratio (>3) at admission. Fifteen out of 16 patients of stage 3 and eight out of 13 patients of stage 2 had LR >3. There was statistically significant correlation between CSF cells and protein with LR. Twenty-one patients (58%) showed good mRS (0–2) with appropriate treatment after 1 month. On comparing mRS with TCD parameters, we found there was positive correlation at different time periods (at admission, at 1 month, and at 3 months).

Conclusions: Serial TCD can be used as a reliable modality for early diagnosis of intracranial vasculopathy in tubercular meningitis patients.

Abstract ID: 9: Does Classical Semiology Predict the Postoperative Outcome in Patients with Temporal Lobe Epilepsy?

Bharanidharan G, Ramshekhar N Menon, George C. Vilanilam, Mathew Abraham, Chandrasekara kesavadas, Bejoy Thomas, Ashalatha Radhakrishnan

Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Thiruvananthapuram, Kerala, India

Background and Aim: Temporal lobe epilepsy (TLE) accounts for about 50%–73% of cases being referred for epilepsy surgery. The objective is to find whether the classical semiology in TLE has a role in predicting the postoperative outcome in patients with refractory epilepsy.

Methodology: We included 684 patients who underwent a detailed clinical evaluation, neuropsychologic assessment, 1.5 T/3 T magnetic resonance imaging, and video electroencephalogram (VEEG) monitoring, followed by a standard anterior temporal lobectomy from 1995 to 2008. They were followed up at 3 months, 12 months, and yearly with electroencephalogram. The outcome was classified into good if they did not have either seizure or aura, irrespective of the drugs used, and bad even if a single seizure occurred postoperatively.

Results: Of the 684 patients, 93.7% had behavioral arrest; 88.6% had automatisms, 86.7% had amnesia for the events, 74.6% had auras, and 63.6% had antecedents. All the classical semiology features were present in 37.7% of the whole cohort. In addition, 47.8% of the study population had good outcome till the last follow-up. The mean follow-up period was 75.6 months with a maximum follow-up of 14 years. Cases with antecedents had lesser probability of seizures at the last follow-up ( P = 0.009). Febrile seizures as a separate variable were found protective ( P = 0.021), and encephalitis was a risk factor ( P = 0.026). Behavioral arrest was associated with poor seizure outcome following surgery ( P = 0.006). No significant association with outcome was found for the presence of auras, automatisms, or amnesia.

Conclusions: Antecedents, especially febrile seizures and behavioral arrest had a favorable and poor surgical outcome, respectively. The combinations of different semiological features had no correlation with surgical outcome.

Abstract ID: 10: Empowering Schools Empowering Children with Epilepsy: A Public Health Perspective

Sulena Sulena, Gagandeep Singh

Baba Farid University of Health Sciences, Faridkot, Punjab

Background and Aim: Epilepsy, as global health concern, disproportionately affects children in India. School-based data is crucial for identifying these children and fostering inclusive environments. However, limited awareness often hinders optimal care. This study evaluates a longitudinal school-based educational intervention program designed to enhance epilepsy detection and management within India’s national health program (Rashtriya Bal Swasthya Karyakram [RBSK]).

Methodology: Conducted in a district of India, the study assessed an epilepsy education program in 406 government schools, enrolling over 63,000 children and 4169 teachers. A mixed methods approach employed a pre-post intervention design within schools. Stage 1 assessed baseline epilepsy coverage under RBSK. Stage 2 implemented a longitudinal educational intervention based on the World Health Organization and International League Against Epilepsy (ILAE) guidelines. Intervention: This 180-min program, delivered in the local language, targeted diverse stakeholders: teachers, health-care providers, and parents. It included lectures, role-plays, and discussions on epilepsy knowledge, management, and stigma reduction. The study employed mixed methods. Quantitative assessment reviewed RBSK child health registers to identify children referred to and confirmed for epilepsy before and after the intervention. Qualitative assessment used focus group discussions to explore program effectiveness.

Results: This school-based educational intervention demonstrated effectiveness and reached a large proportion of stakeholders. Epilepsy screening coverage and detection in schoolchildren significantly increased (baseline: 40/100,000 [95% confidence interval {CI}: 24–55]; year 3: 74/100,000 [95% CI: 54–95]). Stakeholders displayed improved knowledge and attitudes toward epilepsy and valued the program as appropriate, acceptable, feasible, and effective in improving epilepsy care.

Conclusions: This school-based educational intervention significantly improved epilepsy screening and detection in children. Stakeholders across schools and health care reported increased knowledge and confidence in managing epilepsy. With its positive impact on identification and support, this scalable program offers a promising approach to improve the care of children with epilepsy within national health initiatives.

Abstract ID: 11: Surgical Outcome and Prognostic Factors of Epilepsy Surgery in Tuberous Sclerosis Patients

Zubin Shah, Sandeep Patil, Sujit Jagtap, Nilesh Kurwale

Deenanath Mangeshkar Hospital and Research Center, Pune, Maharashtra, India

Background and Aim: Tuberous sclerosis complex (TSC) is a multisystem, autosomal dominant neurocutaneous syndrome. Epilepsy occurs in 80%–90% of patients, and over 50% of patients suffer from drug-resistant epilepsy (DRE). We aimed to determine the predictive factors in surgical outcomes of TSC patients.

Methodology: This retrospective observational study included 43 patients of TSC admitted to the Epilepsy Monitoring Unit between 2015 and 2023. Patients’ clinical details, developmental status, magnetic resonance imaging, and 1-year postsurgical outcome status were recorded.

Results: Median age at presentation, median seizure onset age, and median epilepsy duration at presentation were 4 years, 6 months, and 3.2 years, respectively. Seizure onset below 2 years of age predicted delayed development. Depending on predominant seizure type, out of 43 patients 24 had focal seizures, four had spasms, six had tonic, and four had drop attacks. Out of 39 patients, six had polymorphic seizures in the form of diffuse-onset ictal rhythm in addition to focal onset, 14 had diffuse onset, and 19 had focal-onset ictal rhythm. Twenty-three patients underwent surgery, of whom 20 had tuber resection and three had corpus callosotomy. Engel class Ia was seen in 56.5% (13/23) of patients. All the patients with Engel Ia outcome had unifocal ictal rhythm on electroencephalogram (EEG). Engle class II was seen in 13% of patients (3/23). Seven patients (30.4%) had Engle class III and IV outcome. Out of these seven patients, five had spasms and four patients had Lennox-Gastaut syndrome (LGS). On EEG, three patients had multifocal and 4 had generalized interictal epileptiform discharge (IEDs), while six patients had diffuse-onset ictal rhythm.

Conclusions: Presence of spasms, LGS, multifocal and generalized IEDs, and diffuse-onset ictal rhythms was associated with a poor postoperative seizure outcome.

Abstract ID: 12: Sexual Health in Men with Epilepsy – A Case–Control Study

Sumant Balgandi, Sanjib Sinha, L. G. Viswanathan, Ajay Asranna, Nandakumar D.N., E. Sinu

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Sexual dysfunction in men with epilepsy (MWE) significantly affects the quality of life, but remains understudied. This research explores the factors contributing to sexual dysfunction and psychiatric comorbidities in MWE to enhance personalized care.

Methodology: A prospective study was conducted with 226 married men (150 MWE, 76 controls) from a neurology hospital in southern India between December 2021 and December 2023. The MWE group included 79 on monotherapy and 71 on polytherapy. Inclusion criteria were married men aged 21–45 years with epilepsy and on treatment for at least a year. Controls were healthy married men in the same age range. Exclusions included conditions affecting sexual health, substance abuse, and psychiatric medication use. Demographic and clinical data were collected. Sexual dysfunction was assessed using the Arizona Sexual Experience Scale (ASEX) and the Premature Ejaculation Diagnostic Tool (PEDT). Psychiatric symptoms were evaluated using the Depression, Anxiety and Stress Scale -21 (DASS-21) questionnaire. Data was statistically analyzed.

Results: Demographic characteristics were similar across groups. The polytherapy group had a significantly lower median age of epilepsy onset, longer epilepsy treatment duration, and higher seizure frequency. Significant sexual dysfunction (ASEX >18) was found in 19% of MWE, with 22.6% having definite premature ejaculation (PEDT >10). Diminished sexual drive was the most common (26%). Polytherapy had the highest ASEX scores, correlating with epilepsy, treatment duration, and seizure frequency. Psychiatric symptoms were highest in the polytherapy group. Valproate use significantly predicted sexual dysfunction ( P = 0.007).

Conclusions: This large-scale study in India provides insights into the interplay between epilepsy, antiseizure medications, psychiatric comorbidities, and sexual dysfunction in men, informing improved holistic care.

Abstract ID: 13: The Clinical and Economic Yield of Continuous Video EEG in Neonatal ICU of a Developing Health-Care System

Madhugandha Wahane, Ajay Goenka Goenka

Mahatma Gandhi Medical college, Rajasthan, Jaipur

Background and Aim: Seizure burden in neonates can be very high, majority of which do not have any clinical correlate. Its accurate diagnosis requires continuous video electroencephalogram monitoring (CVEM). The primary objective of this study was to precisely diagnose the frequency of electroclinical and electrographic seizures in a neonatal intensive care unit. The secondary objective was to assess the real-world implications in terms of the duration of hospital stay.

Methodology: A prospective analysis of CVEM in 140 patients between the ages of day 1 and day 60 of life was performed at a community hospital in India. The patients were divided into Group A patients with paroxysmal episodes and treated as electroclinical seizures, Group B patients with high risk of electrographic seizures but no electroclinical seizures, and a control group of 140 patients with similar clinical presentation before initiation of CVEM.

Results: CVEM was performed in 40 patients of Group A and 100 patients of Group B. The median monitoring duration was 18 h. A total of 652 seizures were captured, of which only 80 (12.2%) had clinical events. Among the 40 patients with paroxysmal events, 50% were confirmed to be electroclinical seizures. CVEM assisted in the medical management of 43% of infants. In the high-risk group ( n = 100), electrographic seizures were recorded in 35% of patients. Twelve of these 35 (34%) patients were found to be in status epilepticus. The duration of hospital stay decreased from 15.4 to 11.5 days ( P = 0.125) and from 18.3 to 14.1 days ( P < 0.001) in Group A and Group B, respectively, compared to the control group.

Conclusions: The study highlights CVEM’s crucial role in promptly identifying and treating neonatal seizures, while also significantly reducing hospital stays, making CVEM a cost-effective tool.

Abstract ID: 14: A Study of the NLRP-3 Inflammasome Activity in Guillain–Barré Syndrome

Srinath Rajeevan, Pradeepkumar B, Sandipan Mondal, Saikat Dey, Vamsi Chalasani, Gopika Suresh Babu, Aritrani Sarkar, Lakshminarayanapuram G Viswanathan, Doniparthi V. Seshagiri, Binu V S, Madhu Nagappa, Monojit Debnath

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS), Karnataka, India

Background and Aim: Autoimmunity and inflammation play important roles in the pathobiology of Guillain–Barré syndrome (GBS). The role of the NLRP-3 inflammasome, which acts as immune “sensors” and is the key determinant of inflammatory responses, has not been evaluated in GBS. This study aimed to study the gene expression levels of the NLRP-3 inflammasome components in GBS and correlate them with the clinical and electrophysiologic profile.

Methodology: Fifty-one patients with “typical” GBS and 54 healthy controls were prospectively recruited from a single neurology unit after obtaining informed consent from May 2021 to March 2023. Gene expression levels of the NLRP-3 inflammasome components (Nlrp3, Casp1, and Il1b) were quantified by quantitative polymerase chain reaction using the TaqMan chemistry. The Institutional Ethics Committee approved the study.

Results: The gene expression levels of Casp1 and Nlrp3 were significantly increased in GBS compared to controls ( P = 0.006 and P = 0.035, respectively). No correlations were noted between the gene expression levels and the clinical severity or electrophysiologic subtypes.

Conclusions: An elevated NRLP-3 inflammasome activity adds a new dimension to the inflammatory origin of GBS. Longitudinal studies and comparison with chronic inflammatory demyelinating polyradiculopathy, Multiple Sclerosis, and Neuromyelitis Optica Spectrum Disorder (NMOSD) of these parameters may provide better insights into their differential alteration and their roles in the pathogenesis of these central and peripheral nervous system demyelinating disorders.

Abstract ID: 15: A Family-Centered Multimodal Tele-rehabilitation Intervention for Individuals with Dementia: A Randomized Controlled Trial for Manastik Mobile App

Pushkraj Marne

Manastik Technology, Pune, Maharashtra, India

Background and Aim: Cognitive impairment and dementia present significant challenges globally, necessitating innovative solutions for care delivery. Tele-rehabilitation interventions, such as the Manastik mobile application, offer a promising avenue for addressing the needs of individuals with mild cognitive impairment or dementia and their caregivers, especially in remote settings.

Methodology: This prospective randomized controlled trial aims to evaluate Manastik as a family-centered multimodal tele-rehabilitation intervention for individuals aged 50 and above. Collaborating with health-care providers, the study will recruit participants from health-care hospitals, clinics, and rehabilitation centers randomly assigned to the intervention group (Manastik) or control group (usual care).

Results: The trial will demonstrate that the Manastik intervention significantly improves physical abilities, cognitive function, and psychological well-being in poststroke patients, compared to usual care. Participants in the intervention group will also show higher engagement and adherence to therapy, with improved caregiver health outcomes observed over 12 weeks.

Conclusions: This trial aims to provide robust evidence on the effectiveness of Manastik in enhancing the well-being of individuals with dementia, potentially informing the development of a tele-rehabilitation program for this vulnerable population.

Abstract ID: 16: Biological Correlates of Sleep Abnormalities in Children with Autism Spectrum Disorder

Madisetty Krishna, Hansashree Padmanabha, Monojith Debnath, Madhu Nagappa, John Vijay sagar, Saikat Dey, Gopika Suresh

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS), Karnataka, India

Background and Aim: Sleep disturbances are commonly reported in autism spectrum disorder (ASD) with a prevalence of 40%–80% compared to typically developing children. Sleep, immune, and circadian dysfunction are interlinked and probably play a crucial role in ASD pathophysiology. Our aim was to correlate the impact of gene expression changes in inflammatory, circadian, and sleep homeostatic pathway-related genes on sleep dysfunction in ASD children and healthy controls (HCs).

Methodology: A prospective case–control study was conducted in departments of neurology, child and adolescent psychiatry, and human genetics at National Institute of Mental Health and Neuro-Sciences, Bengaluru between June 2022 and May 2024 after obtaining ethical approval. Sleep disturbances were screened using Children’s Sleep Habits Questionnaire. Plasma samples from 57 ASD children and 56 HCs aged 2–10 years were collected with informed consent. Gene expression of five genes (circadian- BMAL1 and CLOCK , sleep homeostasis- Orexin, inflammatory- STAT3 and IL-6 ) was quantified using quantitative polymerase chain reaction and droplet digital polymerase chain reaction (ddPCR) ( IL-6 ). Statistical analysis was performed by the biostatistics department.

Results: This study found sleep dysfunction in 66.7% of ASD children and 14.3% of HCs ( P < 0.001). BMAL1 gene expression significantly differed between ASD children and controls ( P = 0.02), with BMAL1 correlating positively with CLOCK ( P = 0.046) and Orexin ( P = 0.022). Orexin also correlated with CLOCK ( P = 0.006) and IL-6 ( P < 0.001). No significant differences were found for CLOCK , Orexin, IL-6 , and STAT3 gene expressions; in addition, no correlations between gene expressions and sleep dysfunction were found in either group.

Conclusions: BMAL1 gene has a significant role in ASD’s molecular mechanisms; its correlation with other genes implies the potential therapeutic targets for sleep disturbances in ASD.

Abstract ID: 17: Development of an Artificial Intelligence-Based Prognostication Model to Predict Functional Outcomes in Survivors of Acute Ischemic Stroke

Dulari Gupta, Shankar Prasad Gorthi, Sreehari Nil Dinesh, Sumit Nil Kharat, Chetna Nil Patil, Priscilla Nil Joshi, Vivek Nil Murumkar, Dhiraj Nil Dhane, Aditya Nil Ghadge, Soham Nil Sant

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim: Acute ischemic stroke (AIS) is a leading cause of morbidity and mortality in the world. Many outcome prediction scales have been devised, but are not frequently used in clinical practice. This study aims to harness the power of artificial intelligence (AI) in prediction of stroke outcomes at 3 months using stroke volume and clinical features.

Methodology: A single-center observational analytical study from a medical college in western India was conducted from Oct 2022 to May 2024. Data sets of patients with anterior circulation AIS were collected, including demographic data, risk factors, laboratory parameters, treatment, and outcomes at 3 months by modified Rankin Scale (mRS; dichotomized into good outcomes mRS <2 and bad outcomes mRS >3). Stroke volume was calculated using semi-automated segmentation on 3-D Slicer. Deep neural network was used for supervised model building.

Results: A total of 179 patients were studied, of whom 118 (65.9%) were male; their mean age was 59.36 ± 14.51 (range 25–92) years. Mean stroke volume was 24.64 ± 38 ml (range 0.05–249.6 ml). Of the patients, 118 (65.9%) had mRS at 3 months with good outcomes (mRS <2) and 61 (34.1%) had poor outcomes (mRS >3). Random forest model emerged as the best overall performer with high accuracy (0.89), sensitivity (0.96), and Jaccard Index (0.85), complemented by a robust receiver operating characteristic area under the curve of 0.93.

Conclusions: We have developed an AI stroke prediction model which can predict outcomes of stroke at 3 months with an accuracy of 89%. Such AI-based prognostication models can assist clinicians in counselling patients and relatives about the prognosis and functional outcomes after AIS.

Abstract ID: 18: Comparative Efficacy of Endovascular and Thrombolytic Interventions for Successful Recanalization in Acute Ischemic Stroke: A Network Meta-analysis of 184 studies

Pradeep Kumar, Manabesh Nath, Annu Gulia, Manabesh Nath

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Acute ischemic stroke is a leading cause of morbidity and mortality worldwide. Various endovascular and thrombolytic interventions are employed to achieve successful recanalization, yet their comparative efficacy remains unclear. This study aims to comprehensively compare these treatments through a network meta-analysis.

Methodology: We conducted a network meta-analysis of published studies encompassing a broad range of interventions, including Solitaire, TREVO, MERCI, Penumbra, intravenous thrombolysis (IVT), intra-arterial thrombolysis (IAT), standard treatment (ST), Mix-Mechanical Thrombectomy (MT)-Devices, MT + IVT, and MT + IAT. Risk ratio (RR) with 95% confidence intervals (CIs) was calculated to assess the efficacy of each treatment in achieving successful recanalization (thrombolysis in cerebral infarction (TICI) or thrombolysis in myocardial infarction (TIMI) flow)

Results: A total of 184 studies (40 randomized controlled trials and 144 cohorts) were included in our network meta-analysis. Comparison between MERCI and TREVO demonstrated a significantly higher risk associated with MERCI devices (RR = 2.42; 95% CI 1.15–5.09). Conversely, comparing Penumbra versus MERCI indicated a considerably lower risk with Penumbra (RR 0.69, 95% CI 0.52–0.92). In addition, MT + IVT was found to have a significantly lower risk compared to Mix-MT-Devices (RR 0.56, 95% CI 0.40–0.77). ST versus MT + IVT highlighted a significantly higher risk with ST (RR 5.94, 95% CI 2.64–13.30). TREVO versus Solitaire and IVT versus Penumbra showed no significant differences. The network plot suggests that Mix-MT-Devices, MT + IVT, and MT + IAT emerged as pivotal interventions, supported by substantial comparative data.

Conclusions: Future studies should focus on filling these gaps to optimize treatment strategies and improve patient outcomes in acute ischemic stroke.

Abstract ID: 19: Effect of Anodal Transcranial Direct Current Stimulation on Naming in Aphasic Patients with Acute Ischemic Stroke – A Randomized, Double-Blind, Sham-Controlled Study

Devavrata Sahu, Arnesh Bhattacharya, Milan Mori, Jyoti Garg, Ashish Duggal, B. K. Bajaj, Jyoti Garg, Ashish K. Duggal

ABVIMS and Dr RML Hospital, New Delhi, India

Background and Aim: Studies on non-invasive brain stimulation techniques such as transcranial magnetic stimulation (TMS) and transcranial direct current stimulation (tDCS) have shown positive results in rehabilitating chronic aphasia patients after a stroke. However, their effects on acute ischemic stroke patients with aphasia have not been well studied.

Methodology: This was a prospective, double-blinded, randomized trial. Using predefined inclusion and exclusion criteria, 28 patients were enrolled and randomized into two 14-patient groups. One group received 5 days of anodal tDCS to the Broca’s area, whereas the other received sham stimulation. The outcomes were a change in the accuracy, reaction time, and picture naming test (PNT) scores on the Indian Council of Medical Research PNT at 4 weeks after the last session of anodal tDCS.

Results: Both groups had similar baseline characteristics. Naming Accuracy results at 4 weeks after tDCS compared to baseline were statistically significant in the anodal tDCS group (Friedman test, P = 0.002). Reaction Time results at 4 weeks after tDCS compared to baseline were statistically significant in the anodal tDCS group (Friedman test, P = 0.001). PNT score results at 4 weeks after tDCS compared to baseline were statistically significant in the anodal tDCS group (Friedman test, P = 0.002). No such results were obtained in the sham stimulation group ( P = 0.992). A mixed analysis of variance comparing the anodal tDCS and sham stimulation groups showed a significant effect of both time and stimulation on both groups, eliminating time as a confounding factor (F (4, 104) = 4.27, P = 0.003 for Accuracy, F (4, 104) = 4.47869, P = 0.004 for Reaction Time, and F (4, 104) = 4.42, P = .002 for PNT scores).

Conclusions: tDCS is helpful in patients with aphasia after acute stroke, but larger studies are needed to confirm these findings.

Abstract ID: 20: Predictors of Good Functional Recovery in Patients with Acute Ischemic Stroke at 3 Months of the Acute Ischemic Event – A Prospective Cohort Study

Sohini Chakraborty, Awadh Pandit, M. V. Padma Srivastava, Rohit Bhatia, Manjari Tripathi, Achal Kumar Srivastava, Deepti Vibha, Vishnu V. Y., Rajesh Kumar Singh, Ajay Garg, B. D. Charan, Ashish Upadhyaya

All India Institute of Medical Sciences, New Delhi (AIIMS), India

Background and Aim: Stroke is the fourth leading cause of death and fifth leading cause of disability in India. Our aim was to assess predictors of good functional outcome (modified Rankin Scale 0–2) at 3 months and early neurologic improvement (ENI) - National Institutes of Health Stroke Scale (NIHSS) reduction by 8 or NIHSS of 0/1 at 24 h in acute ischemic stroke (AIS) patients.

Methodology: A single-center prospective cohort study recruiting 311 adult AIS patients with pre-stroke mRS 1, within 72 h of ictus, fulfilling the inclusion and exclusion criteria was conducted from February 2022 till November 2023.

Results: Of the patients, 66% were males, having a mean age of 56 years (±16.18); 53% were hypertensive, 35% were diabetic, 33% had dyslipidemia, 38% consumed tobacco, and 23% consumed alcohol. Results also showed that 22%, 31%, 27%, 5%, and 14% had small vessel disease (SVD), large artery atherosclerosis, cardioembolic stroke, stroke of other determined etiology, and stroke of undetermined etiology, respectively. Mean systolic blood pressure, diastolic blood pressure, Glasgow Coma Scale (GCS), NIHSS at presentation, Computed tomography - Alberta Stroke Program Early CT Score (CT-ASPECTS), collateral score, time to hospital arrival, time to thrombolysis, and time to thrombectomy were 147 mmHg (±28.58), 86 mmHg (±16.4), 6 (±1), 11 (6–17), 6 (±3), 3 (±1), 6.5 h (3.5–12.5), 5.5 h (3.5–9), and 3.5 h (2.5–4.5), respectively. In addition, 52% had large vessel occlusion (LVO) and 38% achieved ENI. On univariate analysis, younger age of ictus, lower NIHSS at 0, 24, and 72 h, higher GCS at presentation, higher CT-ASPECTS, absence of LVO, higher multiphase computed tomography angiography (mTCA) collateral score, Trial of ORG 10172 in Acute Stroke Treatment (TOAST) of SVD, ENI, absence of cardiac chamber enlargement on echocardiography, absence of arrhythmia diagnosed by either echocardiogram/electrocardiogram/Holter, intake of dual antiplatelet, those who did not undergo decompression surgery, higher serum albumin, lower C-reactive protein, lower D-dimer, lesser time from ictus to hospital, and reduced duration of hospital stay, all were prognostic factors for good outcome at 3 months. On performing multivariate regression, NIHSS at presentation, age, achieving ENI, intake of dual antiplatelet therapy (DAPT), and absence of arrythmia were significant independent predictors of good outcome at 3 months after adjusting for confounding factors. With the increase in age and NIHSS at 0 h, chances of good outcome significantly decreased with adjusted odds ratio of 0.943 (0.889–0.999, P = 0.049) and 0.791 (0.656–0.954, P = 0.014), respectively. Achieving ENI, usage of DAPT, and absence of arrythmia had higher odds of achieving good outcome with odds ratio (OR) of 68.688 (28.127–167.742), 0.268 (0.096–0.749), and 0.296 (0.091–0.962), respectively. On performing multivariate regression, lower admission NIHSS, intravenous thrombolysis (IVT), use of DAPT, absence of arrythmia, and right hemispheric stroke were independent predictors of ENI. With the increase in NIHSS at 0 h, chances of ENI significantly decreased with adjusted odds ratio of 0.795 (0.717–0.881, P < 0.0001). With IVT, use of DAPT, absence of arrythmia, and right hemispheric stroke, odds of ENI increased with OR of 4.312 (2.258–8.233), 0.077 (0.015–0.395), 0.226 (0.112–0.456), and 0.153 (0.058–0.406), respectively.

Conclusions: Improving our knowledge about predictive factors might enhance rehabilitative procedures in AIS patients.

Abstract ID: 21: Clinical Features and 3-Month Outcome of Stroke After Solid Organ Transplant – A Retrospective Study of 35 Cases from a Comprehensive Stroke Center

Samatha Mukkamalla, Vivek Nambiar, Snigdha Komakula, Rajesh Nair, Sudhindran S

Amrita School of Medicine, Kochi, Kerala, India

Background and Aim: Post-transplant stroke incidence usually ranges from 1.1% to 8% in kidney transplant recipients and from 1.8% to 4% in liver transplant recipients. Our aim was to assess the clinical features and 3-month outcome of stroke after solid organ transplant in a study of 35 cases from a comprehensive stroke center. We tried to investigate the etiologies and characteristics of stroke occurring after transplantation.

Methodology: We conducted a retrospective analysis of patients who underwent solid organ transplantation (liver, kidney with pancreas) at Amrita Hospital from 2009 to 2023. Patients with stroke post-transplantation were included. Patient demographics, comorbidities, National institutes of health stroke scale (NIHSS), modified Rankin Scale, type of stroke (ischemic or hemorrhagic), TOAST classification of ischemic stroke, immunotherapy, and stroke treatment were recorded. Data were retrieved from electronic medical records.

Results: Out of 2050 patients, 35 experienced a stroke, post-transplantation: 17 were liver transplant recipients and 18 were kidney transplant recipients. Among these 35 patients, 17 had ischemic strokes, 16 had hemorrhagic strokes, and two had transient ischemic attacks. The mortality rate was 1.02% in liver transplant group (six patients succumbed) and 1.8% in kidney transplant group (10 patients succumbed). Unfortunately, 16 of the 35 patients succumbed to their conditions, with hemorrhagic stroke (10 patients) being the most common cause of death, regardless of their comorbidities.

Conclusions: Our study underscores the critical need for vigilant monitoring and management of stroke risk factors in post-transplant patients, particularly within a few months of perioperative period for liver transplant recipients and throughout life for kidney transplant recipients.

Abstract ID: 22: Clinico-etiological Profile of Movement Disorders in Children: A Prospective Study in a Tertiary Care Hospital

Suprabha Shukla, Ashok Kumar Mallick, Dillip Kumar Das, Kali Prasanna Swain

Shriram Chandra Bhanja Medical College and Hospital Cuttack, Odisha, India

Background and Aim: Movement disorders (MDs) are characterized by impaired voluntary movement, the presence of involuntary movements, or both. Children more commonly demonstrate hyperkinetic MDs, such as tics, chorea, ballismus, dystonia, myoclonus, stereotypies, and tremor, than hypokinetic disorder. Determining their etiology is important for specific treatment and prognostication. This study aimed to find out the clinical pattern of various MDs in children and to establish their etiology and outcome.

Methodology: A prospective observational study was conducted from September 2022 to October 2023 in a tertiary care hospital in eastern India. Children from 6 months to 15 years of age presenting with involuntary movements to the outpatient department and wards of neurology and pediatrics were included. History and clinical examination were carried out with a predesigned proforma. A diagnostic workup was done, and results were analyzed to find the pattern of MDs and their etiology. Patients were followed up over a period of 3 months for the outcome.

Results: Out of 84 patients, isolated MDs were seen in 58 (69%) and mixed MDs in 26 (31%). Among isolated MDs, dystonia ( n = 32, 55%), tremor ( n = 9, 15%), and chorea ( n = 6, 10%) were common, whereas among mixed type MDs, majority were dystonia plus chorea ( n = 18, 69%).

Conclusions: Etiologies of MDs specific to this part of India are infection-related, genetic/metabolic, and cerebral palsy. A significant number of children had more than one type of MD. Early recognition and specific diagnosis are required for better outcome.

Abstract ID: 23: Clinical Presentation and Evolution of Isolated Focal and Segmental Dystonia in Adults – 20-Year Experience from a Botulinum Toxin Clinic

Asish Vijayaraghavan, Syam Krishnan, Divya K P, Sambha Murthy Krishna Mohan Mavuru, Patel Khushboo Subash

Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Thiruvananthapuram, Kerala, India

Background and Aim: We aimed to describe the clinical profile and evolution of adult patients with isolated focal and segmental dystonia presenting for treatment in our Botulinum toxin (BoNT) clinic, and the response to treatment, over 20 years.

Methodology: We reviewed the prospectively maintained electronic medical records of patients with isolated focal and segmental dystonia who had at least two visits to our BoNT clinic. We analyzed the clinical features at presentation and during follow-up, the muscles injected for each type of dystonia, the self-reported benefit with BoNT, and adverse effects.

Results: We had 578 patients with isolated dystonia who were injected and had at least one more follow-up. Five hundred and seventeen (89.4%) of them had focal dystonia, and 61 (10.6%) were segmental. Cervical dystonia was the most common type. Spread to other parts affecting more regions was seen in 81 (14%) patients. Those with blepharospasm as the initial presentation tended to have the highest occurrence (27%) of spread to other parts. The mean response to BoNT was around 70%. The presence of sensory trick and absence of tremor were found to be predictors of good outcomes with BoNT.

Conclusions: Our study provides evidence for the response to BoNT therapy in a real-life scenario, outside of a clinical trial setting. The study also observed that the first site of onset in isolated focal dystonia is associated with the risk of spreading to other body regions later.

Abstract ID: 24: Nonmotor Symptoms, Sleep Disturbances, and Genetic Insights in Young-Onset Parkinson’s Disease: A Prospective Descriptive Study

Hareen Veerapaneni, Ajit Prasad Mishra, Lulup Kumar Sahoo, Srimant Pattnaik, Srikanta Kumar Sahoo

IMS and SUM hospital Kalinga Nagar, Bhubaneswar Odisha, India

Background and Aim: Young-onset Parkinson’s disease (YOPD) refers to patients who develop Parkinson’s disease earlier in life, significantly impacting social, occupational, and familial dynamics. This study aims to assess the incidence of nonmotor symptoms and sleep dysfunction in YOPD patients, alongside genetic analysis.

Methodology: This prospective descriptive study, approved by the institutional ethics committee, included patients diagnosed with Parkinson’s disease by the Movement Disorder Society Clinical Diagnostic Criteria for Parkinson’s Disease, with an age at onset between 21 and 45 years. Patients underwent Modified Hoehn and Yahr scale (H & Y) staging, and nonmotor symptom assessment using Movement Disorder Society Non-motor Rating Scale (MDS-NMS), Non-Motor Symptoms Questionnaire (NMSQ), and Pittsburgh Sleep Quality Index (PSQI) after obtaining their informed consent. Whole exome sequencing was performed on willing patients after detailed discussions on its implications with patients and caregivers. Statistical analysis was conducted by the presenting author.

Results: A total of 27 patients were enrolled in the study, consisting of 20 males and seven females. Mean age of onset was 37.5 years with the median duration of illness being 5 years. Median modified H & Y stage is 2. Genetic analysis was performed in 21 of the 27 patients, where 12 patients had genes suggestive of Parkinson’s disease. Among the nonmotor symptoms, the highest prevalence was seen for anxiety (in 24 patients), depressive symptoms (in 20 patients), sexual dysfunction (seen in 20 patients), and gastrointestinal symptoms (seen in 14 patients). Psychosis and impulse control disorders were relatively rare with 11 and seven patients, respectively, showing them. Sleep disturbances in patients were common, and only six patients rated their sleep as very good.

Conclusions: YOPD patients experience significant nonmotor symptoms and sleep disturbances, with a notable genetic component. Comprehensive management and an individualized approach are necessary to address these challenges.

Abstract ID: 25: Prevalence and Clinical Characteristics of Non-parkinsonian Tremor in a Tertiary Care Hospital in a Rural Medical Teaching Hospital in Gujarat: A Cross-Sectional Study

Sachin Udwani

Shree Krishna Hospital, Pramukhswami Medical College Karamsad, Anand, Gujarat, India

Background and Aim: Non-parkinsonian tremors (NPTs) are a heterogeneous group of tremor disorders, with essential tremor (ET) being the most common type. The revised 2017 Movement Disorder Society (MDS) classification system has provided a new framework for categorizing these disorders. This study aimed to assess the prevalence, clinical characteristics, and severity of NPT in a tertiary care hospital in western India using the 2017 MDS classification.

Methodology: This cross-sectional study enrolled 202 patients aged more than or equal to 18 years with NPT attending the movement disorder clinic at a rural tertiary care hospital in Gujarat, India, over 14 months. Patients underwent a comprehensive clinical evaluation, including the 2017 MDS Consensus Classification system for tremors and the Fahn–Tolosa–Marín Tremor Rating Scale for tremor severity assessment.

Results: The prevalence of NPT was 2.34% in the neurology outpatient clinics and 28.25% in the movement disorder clinic. Idiopathic tremor disorder was the most prevalent subtype (71.78%), followed by acquired tremor disorders (26.7%) and genetically determined syndromes (1.98%). ET, ET-plus, and dystonic tremors had similar prevalence, each constituting nearly one-fifth of the study population. Significant differences were observed in age of onset, gender distribution, family history, and tremor severity among tremor subtypes.

Conclusions: This study provides a comprehensive assessment of NPT prevalence, clinical characteristics, and severity in a rural western Indian population using the 2017 MDS classification. The findings highlight the heterogeneity of NPT disorders, the impact of the ET-plus category, and the importance of personalized diagnostic and management approaches. Further research is needed to optimize patient care and outcomes in the Indian context.

Abstract ID: 26: Genotype–Phenotype Correlates of Monogenic Focal Epilepsies in an Indian Cohort

Manisha Karamala Yalapalli, Nandana Jayakumari, Alfiya Fasaludeen, Ashalatha Radhakrishnan, Ramshekhar N. Menon

SCTIMST, Thiruvananthapuram, Kerala, India

Background and Aim: Focal epilepsies constitute about 50%–60% of all epilepsies and are regarded as acquired disorders with underlying structural etiology. Due to the general emphasis on developmental–epileptic encephalopathies, sparse data exists regarding the diagnostic yield of genetic testing in focal epilepsies. We aimed to study the genotype–phenotype correlates of monogenic focal epilepsies and their impact on the understanding of pathophysiologic mechanisms and outcome variables.

Methodology: We conducted a single-center retrospective study of prospectively maintained records with data of all monogenic epilepsies between 2019 and 2023. The probands fulfilling the criteria for focal epilepsy with underlying genetic etiology were included, and the electroclinical data was collected. Variant interpretation was stratified as per American College of Medical Genetics and Genomics(ACMG) guidelines. The probands were further divided based on underlying pathophysiologic mechanism into channelopathies and non-channelopathies, and the electroclinical and outcome data were compared between them. Predictors of seizure freedom were analyzed.

Results: Of the 620 probands who underwent genetic testing between 2019 and 2023, 142 patients were included. Variants involving ion channels 38 [34.9%], receptors (5 [4.6%]), transporters (3 [2.8%]), synaptopathies (5 [4.6%]), cell proliferation (3 [2.8%]), metabolic (10 [9.2%]), and other multiple function proteins (44 [41.3%]) were included. Comparison analysis between channelopathies and non-channelopathies showed predominant infantile-onset ( P = 0.023), predominant focal seizures plus (added semiology) ( P = 0.029), atypical febrile seizures ( P = 0.028), nocturnal predominant seizures ( P = 0.021), and poor seizure outcome ( P = 0.037) in the latter. The factors associated with the absence of seizure freedom were the presence of myoclonic jerks in addition to focal semiology ( P = 0.009), global developmental delay ( P = 0.015), psychomotor regression ( P = 0.044), presence of antecedent febrile seizures ( P = 0.006), bihemispheric interictal epileptiform discharges (IEDs) ( P = 0.045), absence of sleep structures ( P = 0.045), and channelopathies ( P = 0.037).

Conclusions: Identification of genetic focal epilepsy syndromes has significant implications in predicting surgical outcome, accurate prognostication, and potentially altering management strategies.

Abstract ID: 27: Genetics Analysis of a Case Series of Myotonia Congenita in a Tertiary Hospital

Shaik Meera Sharief, Arjuman Parveen Shaik, Garuda Garuda Butchiraju

Rangaraya Medical College, Kakinada, Andhra Pradesh

Background and Aim: Myotonia congenita is a rare non-dystrophic myotonic disorder caused by mutations in the skeletal muscle chloride channel gene ( CLCN1 ). The clinical manifestations are muscle stiffness, pain, weakness, and fatigue. We aimed to study the genetics of myotonia cases and to know the common genetic mutations prevailing in our area and describe variable manifestations of the same mutation.

Methodology: This is an observational study including 14 cases attending the outpatient and inpatient departments of a government hospital over a period of 5 years. Genetic analysis was conducted in 14 cases by clinical exome sequencing by Interproteomics and MedGenome labs.

Results: Genetic tests showed four different CLCN1 mutations in 13 patients and one novel mutation.

Conclusions: This is a largest genetic case series of myotonia congenita in India, and one novel mutation was detected. It was found that patients with the same mutation can present with variable clinical manifestations and inheritance patterns. Patients can be misdiagnosed as myotonic dystrophy clinically without genetic testing and may miss appropriate treatment.

Abstract ID: 28: Diagnostic Yield of Whole Exome Testing in Neurology Patients Presenting to a Tertiary Care Center

Aditya Gudhate, Mayur Thakkar, Aishwarya Jirvankar, Neeraj Jain, Sangeeta Ravat, Mayur Thakkar, Sangeeta Ravat

Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

Background and Aim: Several neurologic diseases present with complex phenotypes caused by genetic mutations. These are labelled “neurogenetic syndromes” and are diagnosed by appropriate genetic testing. As these tests become increasingly accessible and affordable in our country, neurologists must be familiar with their benefits and limitations. We present here a retrospective, cross-sectional study examining the yield of whole exome sequencing (WES) in suspected neurogenetic diseases.

Methodology: We included 205 patients suspected of having neurogenetic diseases and visiting the neurology department of a tertiary care center between January 2018 and January 2024 for this study. WES was performed in these patients due to high suspicion of genetic etiology as per the clinical guidelines after a case conference. Patients with variant of unknown significance (VUS) were offered further testing to reclassify the variant.

Results: Ninety-one patients showed “pathogenic” mutations, giving a diagnostic yield of 44%. Seventy patients (34%) showed VUS and 44 (22%) patients did not show any mutation. Yield increased to 55% after testing in the VUS group. Subgroup analysis was performed for the following: “neuromuscular,” “epilepsy,” “movement disorder,” and “others.” Higher yield was seen in neuromuscular diseases (58%). Yield in movement disorders was 31%, followed by epilepsy at 19%, which is higher than in previously published studies. Moreover, 34% of patients experienced significant change in treatment plan after testing.

Conclusions: This study should inform future clinical practice when it comes to diagnosis, prognosis, surveillance, family planning, disability rights, academic research, drug development, and treatment of neurology patients seen in clinical practice.

Abstract ID: 29: Multimodal Profile of Inflammatory Myositis and Longitudinal Outcomes

Prashanth Poulose, Nalini Atchayaram, Seena Vengalil, Deepak Menon, Saraswati Nashi, Ravi Girikematha Shankar, Karthik K, Anita Mahadevan

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS), Karnataka, India

Background and Aim: To assess the longitudinal trends of evolution in idiopathic inflammatory myopathies (IIM) and look at multimodal parameters that predict treatment responses.

Methodology: Ninety-two (M: F = 38:54) patients with a clinical and immunologic diagnosis of IIM according to the American College of Rheumatology (ACR)/ European League Against Rheumatism (EULAR) criterion 2017 and evaluated between 2014 and 2020 were included in this retrospective analytic study after obtaining approval from the institutional ethics committee. Statistical analysis was done as univariate association analysis and logistic regression using appropriate statistical tools.

Results: The 92 patients had a mean follow-up duration of 24 ± 6.2 months. The mean age of onset was 37.9 ± 10.12 years. The clinical phenotypes were: dermatomyositis 37% ( n = 34), overlap myositis 38% ( n = 35), necrotizing myositis 18.5% ( n = 17), and antisynthetase syndrome 6.5% ( n = 6). On immune profiling myositis-specific antibodies (MSA) = 68.5% (63) and myositis-associated autoantibodies (MAA) = 62% (57). Predominant antibody subclasses were MSA (54.3%), MAA (3%), and both MSA and MAA (7.6% each). Muscle biopsy (35.9%), necrosis (27.2%), inflammatory infiltrates (26.1%), peri-fascicular atrophy (10.9%) & fibrosis (22.8%). Longitudinal follow-up showed the following: best treatment responders = 35.9% (33), satisfactory responders = 39.1% (36), unsatisfactory responders = 13% (12), and nonresponders = 12% (11). Profile of disease outcomes was as follows: monocyclic = 55.4% ( n = 51), chronic continuous = 27.2% ( n = 25), and relapse = 22.8% ( n = 21). Multivariate logistic regression showed that a lower score of Manual Muscle Test 8 (MMT-8) at baseline ( P < 0.01) and a higher number of relapses ( P < 0.05) were independent predictors of nonresponse. These parameters could predict nonresponse with a sensitivity of 30.4% and specificity of 91.2% with area under the curve (receiver operating characteristic- 0.775).

Conclusions: This study paves the way for creating predictive models in IIM, which can aid in early prognostication and identifying potential candidates who would benefit from early more aggressive immunomodulatory strategies with antibody-depleting agents. A highly specific model was another major highlight which aligns with precision diagnostic frameworks in tertiary care settings.

Abstract ID: 30: A Comparative Study of Clinicoradiologic Features and Outcome of Longitudinally Extensive Transverse Myelitis With or Without AQP4/Mog Antibody – A Retrospective Observational Study

Anuradha Mahto, Kamlesh Jagiasi, Pawan Ojha, Bilia Kizhekekudy Aipu, Gaurav Chaudhary, Rakesh Singh, Akash Chheda, Arjun Shah, Karthik Goud, Harshil Bagadia, Shashank Nagendra

Grant Medical College & JJ Hospital, Mumbai, Maharashtra, India

Background and Aim: Longitudinally extensive transverse myelitis (LETM) is often associated with neuromyelitis optica spectrum disorders or myelin oligodendrocyte glycoprotein (MOG)-Ab–associated disease. We aimed to study the difference in the clinical and radiologic features, treatment response, and recurrence of seronegative LETM when compared to antibody-positive patients.

Methodology: A retrospective observational study was conducted in the inpatient department of neurology in a tertiary care center from April 2022 to April 2024. Written informed consent was taken from patients, and approval from the Institutional Ethics Committee was obtained.

Results: Forty-three patients who presented with LETM (20 seronegative, 14 anti-AQP4 Ab, and nine anti-MOG positive) were included. A cervical and cervicodorsal cord with central grey matter involvement was present in antibody-positive LETM (52.3% vs. 45% and 95.6% vs. 70%, respectively). In addition, 58.1% of cases had normal brain magnetic resonance imaging. On follow-up, both the groups exhibited a significant reduction in the modified Rankin Scale (mRS) score. Eleven patients experienced at least one relapse (six (26.1%) seropositive and five (25%) seronegative).

Conclusions: Patients with seronegative LETM had clinical and radiologic differences when compared to those with seropositive LETM. However, the response to early immunotherapy is beneficial and prevents relapses and disability.

Abstract ID: 31: Autoimmune Thyroiditis and Newly Detected Hypothyroidism – A Predictor of Acute Migraine Attacks and its Severity?

Suranjana Basak, Sudesh Prabhakar, Harpreet Mann, Jeenendra Singhvi, Amit Shankar Singh

Fortis Hospital Sector 62, Mohali 1662

Background and Aim: Migraine is an intermittent painful neurologic headache disorder with an estimated 1-year prevalence of 15%–18%. Migraine has been linked to several comorbidities, including psychiatric, cardiovascular, hormonal, and thyroid diseases. Previous studies found a bidirectional relationship between migraine and hypothyroidism, suggesting that both diseases can be considered to be comorbid. As prolactin is a crucial regulator of the hypothalamus-pituitary–gonadal axis, it might contribute to signaling mechanisms underlying migraine. However, the nature of the relationship remains unclear. This study aimed to understand the correlation of prolactin and thyroid hormone levels in assessing the severity of acute attacks of migraine and to investigate the role of autoimmune thyroiditis influencing migraine.

Methodology: This prospective observational cohort study involved consecutive migraine patients who consulted at xxx from November 2022 to October 2023. Clinical profile, serological–radiologic investigations, and Headache Impact Test -6 (HIT-6) were assessed, and results were calculated in statistically appropriate tests.

Results: Females were more affected by migraine, more so episodic with a mean duration of >1 year of disease onset. Headache was primarily holocranial, occipital, and frontal sequentially. Attacks were associated with psychologic features followed by blurring, nausea, and giddiness, with sunlight being a crucial trigger. Eighty percent of patients had remission in the past 3 years. Eighty-one out of 120 migraine patients were those with newly detected hypothyroidism, with a significantly elevated anti-TPO antibodies found in 69 patients ( P < 0.001). There was twice as much as the baseline prolactin level in patients during an episode of acute migraine attack ( P = 0.3). It was found that these elevations were associated with a very severe impact on HIT-6 during an acute attack.

Conclusions: Newly detected hypothyroidism and autoimmune thyroiditis are closely related to newly detected migraine. Serum prolactin could be used as a predictor for acute migraine attacks.

Abstract ID: 32: Clinical Spectrum of Anti-GQ1B Antibody Syndrome

Chinmay Kumbhar, Rahul Vitthal Kulkarni, Shripad Suryakant Pujari, Vishal Vijay Deshpande

Deenanath Mangeshkar Hospital and Research Center, Pune, Maharashtra, India

Background and Aim: To study the clinical spectrum of anti-GQ1b antibody syndrome with respect to preceding illness, key presenting complaints, cranial nerve involvement, magnetic resonance imaging findings, nerve conduction velocity (NCV) findings, cerebrospinal fluid (CSF) routine findings, limb weakness, treatment modality, as well as the clinical outcome.

Methodology: Eighteen anti-GQ1b antibody-positive patients between 2019 and 2024 were included in the study.

Results: Sixty-one percent of patients had an antecedent illness. Results showed that 28% had diarrhoea and 33% of patients had upper respiratory tract infection (URTI). In addition, 83% had ophthalmoplegia, 72% had ataxia, 66% patients had areflexia, and 5% had hyperreflexia. Sixteen percent had isolated ophthalmoplegia, 48% had anti-GQ1b spectrum disorder; 5% had Bickerstaff brainstem encephalitis, 15% had incomplete anti-GQ1b syndrome, and 16% had Miller Fisher syndrome (MFS). Sixty-one percent had ocular motor nerve involvement, 55% had lower cranial nerve involvement, and 33% had bifacial involvement. Seven (39%) patients had reduced motor power. CSF routine microscopy was abnormal in 27% of patients. NCV was abnormal in 28% of patients.

Conclusions: Around 60%–70% of patients can have antecedent illness and URTI more commonly than gastroenteritis. Partial and overlap GQ1b antibody syndrome is more common than classic Miller Fisher/Bickerstaff encephalitis. Ophthalmoplegia is the most common presenting symptom and sign. Limb weakness can be present in 40% and bulbar palsy in 40%–70% of patients. CSF can be abnormal in 27%–40% and NCV can be abnormal in 28%–80%. 80%–95% patients show good recovery with intravenous immunoglobulin.

Abstract ID: 33: The NIMS-ICGNMD Study – Spectrum of Inherited Neuromuscular Disorders and Their Genetic Profile

Keerthi M, Sireesha Yareeda, Suryaprabha Turaga

Nizam’s Institute of Medical Sciences, Hyderabad (NIMS), Telangana, India

Background and Aim: Most of the neuromuscular disorders that we encounter in clinical practice mimic each other. They require a thorough understanding of the clinical phenotype, electrophysiology, followed by genetic analysis to decipher the diagnosis. The cost of genetic sequencing is a major hurdle. Sometimes, sequencing might still not be able to underpin a diagnosis, making it much more challenging. The NIMS-ICGNMD project facilitated establishment of the registry and clinical phenotyping and ongoing sampling to establish the final diagnosis.

Methodology: A study led from June 2021 to May 2024. All patients presenting to xxxx who were diagnosed as inherited neuromuscular disorder through clinical evaluation and ancillary investigations were selected. Relevant demographics and clinical phenotype were obtained through a structured proforma. Further workup including blood tests, electrodiagnostic studies, and muscle biopsies was considered on case basis. Genetic analysis was done by collecting the patient’s blood sample and subjecting it to clinical exome sequencing, after taking informed consent from patients. The samples were sent to CDFD, Hyderabad, for processing, and the data was sent to UCL for analysis.

Results: Our findings revealed a large cohort of patients with motor neuron disease (32%), followed by limb-girdle muscular dystrophy (LGMD) (21%). Duchenne muscular dystrophy (DMD) occupied 16% of the cohort and Beckers (1%). Others were spinal muscular atrophy (6%), myotonic dystrophy (2%), congenital muscular dystrophy (2%), congenital myopathy (4%), congenital myasthenic syndrome (3%), metabolic myopathy (4%), Hirayama disease (1%), hereditary motor and sensory neuropathy (HMSN) (3%), and distal myopathy (2%).

Conclusions: This is the largest cohort reported so far showing the entire spectrum of neuromuscular diseases. The study also highlights the importance of having a precise genetic diagnosis, providing the opportunity for a future therapeutic direction.

Abstract ID: 34: Spectrum of Young-Onset Motor Neuron Disorders in Indian Cohort

Farsana Mustafa, William L. Macken, M. V. Padma Srivastava, Christopher J. Record, Ashwin Dalal, Nimita Rani, Tanveer Ahamed, Rohit Bhatia, Robert D. S. Pitceathly, Thankaraj K, Mary M. Reilly, Michael G. Hanna, Venugopalan Y. Vishnu

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Young-onset motor neuron disorders (age <45 years) present with mixed upper motor neuron (UMN) and lower motor neuron (LMN) features or with LMN or UMN features in isolation with a high likelihood of genetic etiology. We aim to describe the clinical, electrophysiologic, radiologic, and genetic profile of a clinically suspected cohort of young-onset MND.

Methodology: All clinically suspected patients with young-onset MNDs were prospectively recruited into the MRC-funded International Centre for Genomic Medicine in Neuromuscular Diseases cohort from a tertiary care hospital in North India. After taking informed consent, all patients underwent clinical examination, deep phenotyping, NCS, electromyography (EMG), and magnetic resonance imaging (brain/spine). Genetic testing included triplet-primed polymerase chain reaction (PCR) and short PCR for C90rf72 and whole exome sequencing (WES).

Results: We included 95 patients (M: F – 70:25) with a mean age of disease onset at 27 years. Four patients had a positive family history. Classic phenotype (UMN plus LMN in three segments) was observed in 48 (50%) patients. Other clinical phenotypes included flail arm syndrome in 11 (12%), flail leg syndrome in 10 (11%), bulbar onset in 17 (18%), Mills variant in three (3%), distal hereditary motor neuropathy in five (5%), and frontotemporal dementia (FTD)-associated phenotype in one (1%) patient. Sixty-five patients underwent electrophysiological study (EPS). EMG showed involvement of four segments in 12 (18%), three segments in 11 (17%), two segments in 21 (32%), and one segment in 21 (32%) patients. Management included riluzole in 85 patients (89%), either alone or in combination with edaravone. Twenty-five (26%) took both edaravone and riluzole, 60 (63%) took riluzole alone, and three (3%) succumbed. WES was available for 28 patients and C9orf72 for 28 patients. Two patients had pathogenic SOD1 mutation, one each had pathogenic ATP13A2 , POLG , and AARS1 gene mutation, one each had a variant of unknown significance for SOD1, SLCA7, GDAP 1, ANXA11, ALS2, BSCL2, ACTA1, TBK1, UNC13A, DCTN1, NEK1, and TRP4.

Conclusions: We describe young MND patients with their genetic characterization.

Abstract ID: 35: Role of Prazosin in Patients with Guillain–Barré Syndrome with Sympathetic Overactivity: A Prospective Cohort Study

Mritunjai Singh, Abhishek Guin, Anu Singh, Rajni Singh, Ashutosh Tiwari

All India Institute of Medical Sciences, Rishikesh (AIIMS, Rishikesh), Uttarakhand, India

Background and Aim: In Guillain–Barré (GBS) patients with dysautonomia, the sympathovagal balance is shifted toward sympathetic overactivity (SO). This study assessed the role of prazosin (1-blocker) in the management of SO.

Methodology: This prospective cohort study was conducted during Jan 2022 and Sept 2023. Thirty-two GBS patients with dysautonomia received prazosin (2.5–10 mg three times a day) (prazosin arm). For comparison, we also included 33 age- and disability-matched GBS patients with dysautonomia (control group), who did not receive prazosin, from a prospectively maintained GBS registry and admitted during Feb 2018–Dec 2021. The primary outcome was fluctuations during the 24-h period in systolic blood pressure (SBP; maximum SBP – minimum SBP, in mmHg) and diastolic blood pressure (DBP; maximum DBP – minimum DBP) at 15 days. Secondary endpoints were days to resolution of dysautonomia, duration of hospital stay, in-hospital mortality, adverse events, and 3- month disability.

Results: The median age was 36 (interquartile range 25–49) years. The demographic and clinical parameters were comparable. The mean fluctuations in SBP (30 ± 15.8 vs. 44 ± 17.6 mmHg, P = 0.002) and DBP (21 ± 10.6 vs. 28 ± 9.3 mmHg, P = 0.02) at 15 days were significantly lower in the prazosin group. Prazosin resulted in significantly earlier normalization of dysautonomia compared to the control group (median 15 vs. 20 days, P = 0.01). However, duration of hospital stay (30 vs. 27 days, P = 0.54) and good recovery at 3 months (71.9% vs. 63.6%, P = 0.48) were comparable. Three (9.3%) patients in the prazosin group developed hypotension, while two patients in the no-prazosin group developed posterior reversible encephalopathy syndrome (PRES).

Conclusions: Prazosin resulted in earlier normalization of dysautonomia and lower incidence of PRES, but did not affect 3-month outcome and duration of hospital stay.

Abstract ID: 36: Does Thymus Pathology and Timing of Thymectomy Influence the Long-Term Clinical Outcomes in “Thymomatous” Myasthenia Gravis?

Venkateswar Reddy Kandula, Aditya Nair, Santosh Benjamin, Roy Gnanamuthu, Thomas Alex, Prabhakar AT, Vivek Mathew, Sanjith Aaron, Ajith Sivadasan

Christian Medical College, Vellore (CMC, Vellore), Tamil Nadu, India

Background and Aim: Thymectomy is an integral part of treatment strategies for both thymomatous and non-thymomatous myasthenia gravis (MG). Both subtypes differ in terms of their clinical profile, treatment response, and natural history. There is scarce literature regarding the long-term outcomes in thymomatous MG, especially with regards to the predictive role of thymus pathology and the timing of thymectomy.

Methodology: This was an observational study of MG patients admitted in a quaternary referral hospital, who underwent thymectomy and had at least 6 months follow-up. Clinical profile, thymus pathologies, and outcomes at the last follow-up were recorded.

Results: Eighty-two patients (42 females, 40 males) were included in the study. Their mean age was 43.58 years (standard deviation 12.76). Majority (95%) had generalized MG. The median time to thymectomy from the onset of symptoms was 8 months (interquartile range [IQR] 13.5–84 months). Median follow-up time was 45 months (IQR 13.5–84). Thymoma grades were B2 (33.7%), AB (26.5%), B1 (18.1%), and B3 (15.7%). Masaoka stages were stage 1 (33.7%), IIA (17.1%), IIB (30.5%), and III–IV (18.7%). Timing of thymectomy did not significantly influence the overall outcome ( P = 0.529). Coexistent thymic hyperplasia was more common in patients with thymic histology B2/B3 (52.5%) compared to other histologic grades (21.4%) ( P < 0.0001*) and was associated with active MG at follow-up (40% vs. 15.4%, P =0.012*). Good outcomes in 73 (89%) and poor outcomes in nine (11%). Masaoka stage III–IV patients had a higher mortality rate (33% vs. 4.5%, P =0.003)

Conclusions: Majority of the patients had good outcomes. Masaoka staging was significantly associated with overall survival. There was no significant association between tumour pathology and MG course. Coexistent thymic hyperplasia was associated with a higher chance of active MG at follow-up, which is attributable to changes in secondary lymphoid organs. Meticulous tumour surveillance is paramount for good long-term outcomes.

Abstract ID: 37: Therapeutic Observations on 22 Patients with Anti-neurofascin 186 Neuropathy

Jharna Mahajan, Satish V. Khadilkar, Varsha A. Patil

Bombay Hospital, Mumbai, Maharashtra, India

Background and Aim: Neurofascins belong to the family of cell adhesion molecules and are critical for the maintenance of nodes of Ranvier. Ample research is available for anti-Nf 155–related neuropathies. Only few case reports and case series have reported the clinical features of anti-NF 186–related neuropathy, with very limited evidence on detailed treatment response. The main aim of this study is to evaluate the therapeutic observations on 22 patients with anti-neurofascin 186 neuropathy.

Methodology: In this retrospective observational study, patients with acquired inflammatory neuropathies with positive antibodies against NF186 antigen, presenting to our tertiary care center over 5 years (January 2019 to December 2023) were included. We documented the treatment outcomes pre- and post-treatment based on inflammatory neuropathy cause and treatment (INCAT), inflammatory rasch-built overall disability scale (IRODS), and expanded disability status scale (EDSS) scores.

Results: Among the 22 recruited patients, we observed 16 patients with acute/subacute illnesses (like acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN)/acute motor sensory axonal neuropathy (AMSAN)/recurrent Guillain–Barré) and chronic inflammatory neuropathy. The remainder of the six patients had atypical clinical features. The mean duration of treatment was 11 months (6–58 months). Response to intravenous immunoglobulin (IVIG) and corticosteroids was 41.6% and 71%, respectively, as first-line therapies. Sixteen/22 patients (72.7%) required more than two immunomodulatory therapies, and rituximab was the most common third-line therapy used in this cohort for refractory cases. Response rates of rituximab in refractory cases were observed to be 64.3%. Four patients were refractory to all the therapies.

Conclusions: This study provides early information regarding therapy outcomes in this group of uncommon neuropathies. In particular, the benefits derived from the use of rituximab in refractive cases are encouraging.

Abstract ID: 38: Postural Instability in Idiopathic Parkinson’s Disease – Determination of VEP, BAER, and SSEP Cut-off Values for an Early Screening of Fall

Joydeep Mukherjee, Manoj Kumar Roy, Jasodhara Chaudhuri, Kartik Chandra Ghosh

NRS Medical College & Hospital, Kolkata, India

Background and Aim: Idiopathic Parkinson’s disease (IPD) patients have progressively increased slowness, rest tremors, rigidity, and postural instability. Postural stability depends on sensory inputs from visual, auditory, and somatosensory modalities. We tried to find out important cut-off values of visual evoked potential (VEP), brainstem auditory evoked response (BAER), and short-latency somatosensory evoked potentials (SSEP) which determine the postural stability in IPD patients.

Methodology: Fifty IPD patients were recruited in a cross-sectional observational study. “Pull test” was used to determine postural stability. Patients were sub grouped into tremor dominant variant (TV; n = 37) and postural instability and gait disorder (PIGD; n = 13). We generated receiver operating characteristic curves to classify patients into posturally stable and unstable and measured VEP, BAER, and SSEP cut-off values. Area under the curve >0.8 was taken as significant.

Results: Significant VEP N75, P100, and N145 cut-off values were noted in IPD and its subgroups (TV and PIGD) bilaterally. Except for wave I, the latency of all other BAER waves showed significant cut-off values bilaterally in IPD and subgroups (TV and PIGD). Most BAER cut-off values in the IPD and TV subgroups reached 100% specificity. No significant SSEP values were noted.

Conclusions: We found many evoked potential significant cut-off values determining the postural stability in IPD and its subgroups (TV and PIGD). A lesser vestibular compensatory support in PIGD led them to a more severe phenotype than TV.

Abstract ID: 39: Effect of Repetitive Bihemispheric Anodal Transcranial Direct Current Stimulation on Motor Function of Patients with Parkinson’s Disease – A Double-Blind, Randomized, Sham-Controlled Study

Milan Mori, Ashish Duggal, Jyoti Garg, B. K. Bajaj, Ashish Kumar Duggal

ABVIMS& Dr RML Hospital, New Delhi, India

Background and Aim: Parkinson’s disease (PD) is characterized by progressive loss of response to dopaminergic therapy, thereby posing a significant therapeutic challenge. Deep brain stimulation has proven efficacy, but carries risks and is not possible in all patients. Non-invasive brain stimulation like transcranial direct current stimulation (tDCS), which modulates cortical activity, has shown promising results and may provide an alternative therapeutic modality.

Methodology: This was a prospective, double-blinded, randomized trial. Thirty patients were enrolled and randomized to receive either true or sham stimulation. The true stimulation group received two or three sessions of bihemispheric anodal tDCS per week for a total of 10 sessions, whereas the other group received sham stimulation in the same settings. The primary outcome was to compare the changes in motor function and gait assessed by Movement Disorder Society-Unified Parkinson’s Disease Rating Scale (MDS UPDRS) Part-III and TUG (Timed up and Go) score, respectively, at 1 week after the repetitive stimulation compared to that of sham group of tDCS. A mixed-model analysis of variance was used to compare the UPDRS scores and TUG as dependent variables between the two groups and stimulation, time, and interaction between stimulation and time as fixed variables.

Results: Both groups had similar baseline characteristics. The study found no significant interaction between stimulation and time in TUG (F = 2.29, P = 0.11) and UPDRS Part III scores (F = 2.1, P = 0.12). However, subgroup analysis of bradykinesia scores in UPDRS III revealed a significant interaction between time and type of stimulation (F = 4.328, P = 0.018), indicating that tDCS may have a beneficial effect on bradykinesia.

Conclusions: tDCS of the motor and prefrontal cortices may have therapeutic potential in PD with some improvement in bradykinesia over time. However, optimized stimulation parameters and protocols need to be established to make the technique clinically viable with larger studies.

Abstract ID: 40: Long-Term Surgical Outcome and its Predictors after Hemispherotomy for Drug-Resistant Unihemispheric Epilepsy

Nandana J, Manisha K Y, Ramshekhar N Menon, George Vilanilam, Mathew Abraham, Chandrahekharan Kesavadas, Bejoy Thomas, Ashalatha Radhakrishnan

SCTIMST, Thiruvananthapuram, Kerala, India

Background and Aim: Hemispherotomy is an effective surgical approach for drug-resistant epilepsy patients with hemispheric epileptogenic lesions. We aimed to determine the surgical outcome as well as its predictors in patients undergoing hemispherotomy for drug-resistant epilepsy and to compare the outcome between the pediatric age group and above.

Methodology: This is a single-center retrospective study. Using logistic regression, we evaluated the correlation between clinical, demographic, electroencephalography (EEG; ictal, interictal, and postoperative EEG), magnetic resonance imaging (MRI) of the brain variables, and the postsurgical outcome. We grouped patients based on age, pathology, EEG findings and evaluated the outcome in terms of postoperative seizure outcome.

Results: One hundred and fifty patients’ mean age at surgery was 11.21 + 6.39 years, and their mean follow-up was 16.79 + 8.38 years. Eighty-nine patients (59.3%) were of age less than 12 years (pediatric group). Most common pathology was porencephalic cyst (33 patients, 30%), and MRI showed contralateral pathology in 15 patients (10%). EEG showed lateralized IEDs in 59 patients (39.3%) and ipsilateral ictal onset in 66 patients (44%). Moreover, 67.3% were seizure free and 82.7% had Engel class I outcome at the last follow-up with a mean follow-up of 5.65 years. Presence of aura (odds ratio [OR] 4.9, confidence interval [CI] 1.09–22.01; P -value: 0.016), acute postoperative seizures (OR 2.3, CI 1.59–12.01; P -value: 0.043), and contralateral slowing in postoperative EEG at 1 year (OR 6.63, CI 1.34–13.73; P -value: 0.018) were independently predictive of poor seizure outcome. At 5-year follow-up, the seizure outcome was better in the adolescent–adult age group (51.7% vs. 35.2%; P -value: 0.048).

Conclusions: Knowledge about independent determinants of surgical outcome following hemispherotomy is crucial for the counselling of patients and families.

Abstract ID: 41: Role of Supination, External Rotation, Abduction (SEA) Posture in Prevention of Development of Upper Limb Spasticity in Acute Stroke Patients: A Randomized, Open-Label, Controlled Trial

Varun Singh, Sanchit Shailendra Chowksey, Akansha Jain, Rameshwar Nath Chaurasia, Abhishek Pathak, Anand Kumar, Vijaya Nath Mishra, Deepika Joshi

Institute of Medical Sciences, Varanasi (IMS, Varanasi), Uttar Pradesh, India

Background and Aim: Post-stroke spasticity is a common complication of stroke. The study evaluated the efficacy of a posture opposite to the typical spastic posture, that is, Supinated forearm, Extended fingers and elbow, and Abducted and externally rotated shoulder (SEA), with the wrist in neutral position in acute stroke patients with flaccid weakness, in prevention of development of spasticity.

Methodology: Hemodynamically stable acute stroke patients between 18 and 80 years of age with flaccid upper limb weakness presenting within 72 h were included. This is a prospective, single-center, randomized, open-label trial with blinded endpoint assessment.

Results: Of 170 acute stroke patients, 76 met the inclusion criteria. Thirty-eight patients were randomized in each splint (Group 1) and control arm (Group 2). Ten patients were lost to follow-up. Of the remaining 66 patients (34 in splint and 32 in control), the median age of patients was 55 years. Forty-two (64%) patients were male. There was no significant difference in baseline demographic, clinical, radiologic, and biochemical parameters between the two groups. At 3 and 6 months, there was significant difference ( P < 0.05) in modified Ashworth upper limb spasticity score at the elbow, forearm (pronation and supination), wrist, and fingers between the two groups. Barthel Index for Activities of Daily Living was also significantly better in group 1 at 3 and 6 months. However, there was no difference in modified Rankin Scale during follow-up.

Conclusions: SEA posture, if applied early in acute stroke patients, is effective in reducing the grade of upper limb spasticity.

Abstract ID: 42: Brown–Vialetto–Van-Laere Syndrome: A Close Mimic of Madras Motor Neuron Disease but Potentially Treatable Syndrome – Experience from a South Indian Center

Dipti Baskar, Sai Bhargava Sanka, Seena Vengalil, Saraswati Nashi, Kiran Polavarapu, Veeramani Preethish Kumar, Ananthapadmanabha K, Yamini Harikishen, Priya Treesa Thomas, Gautham Arunchal, Atchayaram Nalini

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Mutations in the genes SLC52A2 and SLC52A3 , which encode the riboflavin transporters RFVT2 and RFVT3, respectively, cause Brown–Vialetto–Van-Laere (BVVL) syndrome. Typically, patients present with sensorineural deafness and progressive pontobulbar palsy. Other features include sensorimotor axonal neuropathy, optic atrophy, and respiratory compromise. Here, we describe the clinical and genetic spectrum of eight genetically confirmed BVVL syndrome patients, which closely mimics the elusive Madras motor neuron disease (MMND).

Methodology: This was a retrospective observational study on eight patients with BVVL syndrome and corresponding genetic variants. Baseline demographic, clinical, and neurophysiology details were recorded, and genetic analysis was done by next generation sequencing.

Results: The mean age at disease onset was 11.25 (range: 7–16) years. All had varying illness durations, with a mean duration of 6.75 (range: 3–12) years. All had severe sensorineural hearing loss, dysarthria and severe hypophonia, tongue atrophy and fasciculations, impaired tandem gait, and sensory ataxia. Weakness and atrophy of limb muscles were noted in five, facial weakness in four, sensory symptoms in three, and cranial nerve palsy was observed in 9–11 patients (5). Of the eight patients, five novel variations (two homozygous and two heterozygous variants in the SLC52A3 gene and one heterozygous variant in the SLC52A2 gene) and previously reported one homozygous and one heterozygous variant in SLC52A3 were noted. Six of eight patients reported significant clinical improvement after 3–6 months of high-dose riboflavin therapy (600 mg/day).

Conclusions: This report highlights the importance of genetic confirmation of all patients suspected to have MMND/BVVL syndrome and not mislabel cases as MMND, for which still further advanced genetic testing is warranted.

Abstract ID: 43: Comparison of Long-Term Outcomes in Spontaneous Supratentorial Intracerebral Hemorrhage Patients Treated With and Without Surgical Intervention

Ayush., Agrata Sharma, Ajay Garg, Venugopalan Y. Vishnu, Rohit Bhatia, M. V. Padma Srivastava

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Intracerebral hemorrhage (ICH) is associated with high mortality and morbidity. Uncertainty still exists regarding benefit of surgery in the management of spontaneous supratentorial ICH (sICH), especially of the basal ganglia and thalamus. Studies have not shown clinical benefit of early surgical management compared to best medical management plus delayed surgery, when necessary. Our aim was to compare the efficacy of different neurosurgical interventions and best medical management with best medical management alone.

Methodology: We conducted a single-center, retrospective study in sICH patients at a tertiary care center in India between January 2015 and December 2022. The primary outcome was functional disability evaluated by the modified Rankin Scale (mRS) at 3, 6, and 12 months. Time-to-event outcomes were compared using the Kaplan–Meier curve.

Results: Among 2600 stroke patients screened, 661 had sICH. Median age was 55 years, and 250 patients (37.8%) underwent neurosurgical intervention. The most common intervention was craniotomy and hematoma evacuation. The median mRS at discharge and follow-up at 3, 6, and 12 months was lower in the conservatively manged group (4, 3, 3, 3) compared to the surgical intervention group (5, 5, 5, 4). However, the ICH score at admission was lower in the conservatively managed group and after adjustment for ICH score, there was no statistically significant difference between the two. Among the interventions, patients undergoing decompression craniectomy had the best functional outcome.

Conclusions: Neurosurgical intervention was not associated with better functional outcome when compared to conservative management.

Abstract ID: 44: Clinical Features, Etiology, and Outcome of New-Onset Refractory Status Epilepticus (NORSE) in Neurointensive Care Unit – A Cohort Study

Pachipala Sudheer, Sita Jayalakshmi, Anuja Patil, Sudhindra Vooturi

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: New-onset refractory status epilepticus (NORSE) is a relatively new entity. There is a paucity of large studies in NORSE. We analyzed the clinical features, determinants, and predictors of outcome in patients with NORSE.

Methodology: In this open cohort study, data of patients with NORSE admitted in the neurointensive care unit of a tertiary care referral hospital from 2010 to 2023 were retrospectively analyzed. The primary outcome for analysis was good functional outcome defined as score of 4 or 5 on Glasgow Outcome Scale.

Results: Out of 444 patients with status epilepticus 50 patients (11.26%) had NORSE. The mean age was 36.43 ± 25.5 years. The most common etiology was infectious encephalitis (54%) followed by autoimmune encephalitis (24%). The mean stay in the intensive care unit was 16.5 ± 5.3 days. Abnormal magnetic resonance imaging was present in 48% patients. At the time of discharge, only 14% ( n = 7) patients had good outcome. Autoimmune encephalitis predicted good outcome in the entire cohort ( P < 0.01).

Conclusions: Outcome at discharge is poor in most of the patients. Autoimmune encephalitis as the etiology predicted good outcome. Therefore, early identification of patients with autoimmune encephalitis and treatment with immunotherapy should be considered in these patients.

Abstract ID: 45: Electroclinical Spectrum of Epileptic Encephalopathy with Recognition of Genotypic Variants and Assessment of Treatment Outcome: A Single-Center Experience

Bonny Sen, Jasodhara Chaudhuri, Kartik Chandra Ghosh, Joydeep Mukherjee

NRS Medical College Hospital, 138 953 Bose Road, Kolkata, West Bengal, India

Background and Aim: Epileptic encephalopathy (EE) is an electroclinical condition where epileptiform activity itself contributes cognitive and behavioral impairment above and beyond what might be expected from the underlying pathology alone (International League Against Epilepsy (ILAE) 2010). The clinical spectrum of EE is complex and depends on the age of onset, epileptic behavior, environmental and genetic variables. Here, we aimed to expand the electroclinical spectrum of EE.

Methodology: We conducted a single-center, prospective observational study recruiting children aged between 1 and 18 years, fulfilling the ILAE 2010 criteria for EE from May 2022 to April 2023 in XXXX hospital, and analyzed the data based on their clinical presentation, electroencephalographic (EEG) findings, etiological workup with genetic diagnosis, and treatment outcome.

Results: A total of 43 patients of EE were divided into West syndrome ( n = 16) and non-West syndrome ( n = 27) based on their electroclinical profile. All West syndrome patients received oral steroid and 13 received add-on vigabatrin. Non-West syndrome cohort (27) comprised Dravet syndrome (3), Lennox–Gastaut syndrome (6), progressive myoclonic epilepsy (8), and epilepsy aphasia spectrum (3), and the remaining 13 having specific genetic etiology were diagnosed as developmental epileptic encephalopathy. Former showed >50% reduction in seizure frequency in 68% and favorable EEG changes in 56% with a combination of oral steroid and vigabatrin. Add-on 3-month pulse intravenous methylprednisolone therapy showed better seizure reduction (75%) with favorable EEG (50%) and significant cognitive improvement in Vineland social maturity scale scores (75%) ( P = 0.011) compared to ketogenic diet group and anti-seizure medication optimization group in the non-West syndrome cohort.

Conclusions: There is a beneficial role of steroid in both West syndrome and non-West syndrome.

Abstract ID: 46: Stem Cell Therapy in Multiple Sclerosis: A Meta-analysis of Randomized Controlled Trials

Amit Kumar, Surendra Kumar, Manoj Kumar Prasa, Anupa Prasad, Manoj Kumar Prasad, Anupa Prasad, Surendra Kumar

Rajendra Institute of Medical Sciences, Ranchi (RIMS, Ranchi), Jharkhand, India

Background and Aim: This meta-analysis aims to systematically assess the safety and efficacy of stem cell therapy in patients with multiple sclerosis (MS).

Methodology: We conducted a comprehensive literature search across electronic databases, including PubMed, Embase, Web of Science, and the Cochrane Library. Inclusion criteria comprised randomized controlled trials reporting on the effect of stem cell therapy in MS patients. The title/abstract and full-text article screening for identifying the eligible studies was carried out by two independent researchers using the Covidence platform ( http://www.covidence.org ) to obtain potentially eligible studies. Two authors independently conducted the methodological quality assessment using the Cochrane Risk of Bias tool-2. The Grading of Recommendation, Assessment, Development, and Evaluation methodology was used for assessing the certainty of evidence on the GRADEpro platform available online.

Results: Of the 1144 records identified through the four above-mentioned databases, a total of eight studies involving 374 participants were included in the meta-analysis. The mean age at the time of randomization ranged from 35.2 years (1) to 48 years. Pooled analysis based on the types of stem cells indicated no statistically significantly difference in Expanded Disability Status Scale (EDSS) score at 6 months post-randomization between the mesenchymal stem cells groups compared to control (Mean deviation [MD] -0.04, 95% confidence interval [CI]: -0.36–0.28, n = 6, I2 =17%). The progression of disease measured by EDSS did not differ between both the groups at 1 year (RR 0.45, 95% CI: 0.02–11.62, I2 = 85%) in patients received hematopoietic stem cell transplantation (HSCT).

Conclusions: Further research is warranted to elucidate the efficacy of stem cell therapy in patients with MS.

Abstract ID: 47: Genetic Profile Of Muscle Diseases in a Tertiary Care Center

Azma Mohammed, Sita Kanthima Akkireddy, Nagarjunakonda Venkata Sundarachary, Aruna Kumari Upputuri, Bindu Narmada Gottipati

Guntur Medical College, Andhra Pradesh, India

Background and Aim: We are presenting 25 cases of different inherited muscle diseases which are genetically proven.

Methodology: A prospective study was conducted from 2022 to 2024. This case series includes variety of inherited muscle diseases: three cases of myotonia congenita, one case of paramyotonia congenita, two cases of distal myopathies: Nonaka and myofibrillar, 3 cases of metabolic myopathies (Pompe, CPT II deficiency, Myoadenylate deaminase deficiency. 2 cases of congenital muscular dystrophies: Ulrich and Merosin deficient CMD, 1 case of Beckers muscular and Duchene muscular dystrophy, 2 cases of Myotonic dystrophy, 1 case of congenital myopathy with Rigid spine syndrome, 9 cases of LGMDs with different genes LAMA2, CAPN3 (4), SGCG (3); TTN (1)

Results: All 25 cases underwent genetic analysis to confirm the diagnosis of muscle diseases.

Conclusions: This case series highlights the importance of genetic analysis in accurately diagnosing the myopathies. In some cases, like metabolic myopathies, with early diagnosis, we can initiate the patient on enzyme replacement.

Abstract ID: 48: Neurologic Intensive Care Unit in Rural Setup: An Experience

Abhishek Dileep Wankar, Ajoy Sodani

Sri Aurobindo Institute of Medical Sciences (SAIMS, Indore), Madhya Pradesh, India

Background and Aim: The present study is an epidemiological profile of a dedicated neurologic intensive care unit from central India over a period of 4 years. This study was performed in an 1165-bedded tertiary care hospital in central India. All consecutive patients above 15 years of age admitted in neurology intensive care unit (INCU) during a period of 48 months were enrolled

Methodology: Data analysis of the 1575 patients was done.

Results: Results of the study population showed that 968 (62.65%) were males and 577 were females. Majority of the patients (63%) had stroke. Majority of the patients (35.47%) fell in the age group of 41–60 years. Of the patients, 46.86% ( n = 724) came from rural areas. Results revealed that 35.47% patients left the hospital against medical advice, with 71.71% of these patients admitted with strokes. The in-hospital mortality rate was 6.86%.

Conclusions: This study shows the utilization and need of neurologic services in rural India.

Abstract ID: 49: Pimavanserin in Parkinson’s Disease Psychosis in Indian Patients: A Post Hoc Analysis Based on CGI-I Score and Duration of Psychosis

Mridula K. Rukmani, Bhavesh Kathiriya, Yogesh Patidar, Vishalkumar Patel, Ashok Singhal, Bhavinkumar Patel, Surjyaprakash Choudhury, Reginald Varadarajulu, Pattojoshi Amrit, Bhavesh Solanki, Akanksha Sharma, Amitkumar Pande, Keyur Parmar, Selvakumar Jayalekshmi, Nagarjunakonda Sundarachary, Priyanka Walzade, Sanjay Ramteke, Dinesh Kumar, Praveen Kumar, Dipesh Sonawane, Prashant Devkare, Mandodari Rajurkar, Dipak Patil, Pravin Ghadge, Suyog Mehta

City Neuro Center, Hyderabad, Telangana, India

Background and Aim: Psychosis affects up to 60% of Parkinson’s disease patients. Pimavanserin is a nondopaminergic, selective 5-hydroxytryptamine receptor 2A (5-HT2A) inverse agonist used for the treatment of Parkinson’s disease psychosis (PDP). However, treatment response to pimavanserin in PDP patients with varied duration of psychosis is yet to be established. This post hoc analysis aimed to assess the improvement in Clinical Global Impression-Improvement (CGI-I) score based on the duration of psychosis.

Methodology: This is a post hoc analysis of a Phase 3, multicenter, randomized, open-label, assessor-blind, parallel-group, active-controlled, comparative study including 247 Indian patients with PDP (120 in the pimavanserin arm [34 mg once daily] and 127 in the quetiapine arm [25-200 mg/d] for 56 days). Patient data was segregated in the following psychosis duration categories: Group A (<6 months, n = 96), Group B (6–12 months, n = 71), Group C (>12 months, n = 71). Treatment response was assessed on percentage change from baseline (CFB) in CGI-I score.

Results: The mean duration of psychosis was 1.2 years (range: 5 weeks–10.8 years). The percentage reduction in CGI-I at Day 56 was as follows: Group A (-39.7 ± 27.84 and -34.9 ± 23.43), Group B (-54.9 ± 25.39 and -54.7 ± 23.01), Group C (-46.7 ± 19.68 and -28.7 ± 33.18) in the pimavanserin and quetiapine arms, respectively ( P < 0.0001, CFB for both groups). Pimavanserin showed superiority over quetiapine at Day 56 ( P = 0.0059, 95% confidence interval: -31.28 to -4.7) in Group C. Both study treatments were well tolerated.

Conclusions: Pimavanserin was well tolerated and efficacious in improving the psychosis in PDP patients of all disease duration categories and was superior to quetiapine in >12 months category.

Abstract ID: 121: Clinical and Etiological Profile of Pachymeningitis – A Study from a Tertiary Care Center

Kamani Babu, Suryaprabha Turaga, Reshma Sultana

Nizam’s Institute of Medical Sciences, Hyderabad (NIMS, Hyderabad), Telangana, India

Background and Aim: Hypertrophic pachymeningitis is a form of fibrosing chronic inflammatory process that causes thickening of the intracranial dura mater. Though the common symptoms are headache and multiple cranial nerve palsies, the presentation is nonspecific and varied. We aimed to study the clinico-etiological profile of hypertrophic pachymeningitis and highlight unusual presentations.

Methodology: This was a single-centered study where adults aged between 12 and 80 years who had neurologic symptoms and pachymeningeal enhancement on magnetic resonance imaging of the brain/spinal cord were retrospectively identified, over the past 2–3 years, from the electronic database of the institute from the department of neurology and followed over a period of 5 years from when they are diagnosed. The characteristics of the patients, including demographics, clinical features, laboratory data, radiologic features, risk factors, and response to treatment, were classified and analyzed.

Results: A total of 75 patients were included in the study. This was an ambispective study. The most common symptom was headache, and the most common etiology was tuberculosis. Other etiologies included Immunoglobulin G4-related disease (IgG4RD), idiopathic. A case of Churg–Strauss syndrome was also included.

Conclusions: Our study is an attempt to alert the treating physicians and surgeons about atypical presentations of hypertrophic pachymeningitis in clinical practice to avoid undue complications. All patients with chronic headache not responding to conventional treatments should undergo neuroimaging to rule out pachymeningitis.

Abstract ID: 122: Prevalence and Reasons for Delay in Treatment of Tuberculous Meningitis and its Impact On Outcome: A Prospective Study from a Tertiary Care Center in Northern India

Riya Sharma, Siddharth Chand, Aditya Nayan, C. V. Sai Teja, Nareddy Sandeep Reddy, Ritu Shree, Neeraj Singhla, Chirag K. Ahuja, Manoj Kumar Goyal, Manish Modi, Vikas Suri, Ashish Bhalla, Kusum Sharma

Post Graduate Institute of Medical Education and Research, Chandigarh (PGIMER Chandigarh), India

Background and Aim: Though previous studies have shown a high prevalence of delay in initiation of treatment for tuberculous meningitis (TBM), a comprehensive understanding of its prevalence, risk factors, and impact on TBM outcomes remains lacking. So, we planned to systemically study delayed treatment initiation in TBM. We aimed 1. to determine the prevalence and factors contributing to treatment delay in TBM and 2. evaluate the influence of treatment delay on the outcome of TBM.

Methodology: This prospective observational study included 42 patients with definite TBM. All the patients were evaluated and followed up in detail and data was recorded meticulously.

Results: Mean age of patients was 30.9 ± 13.4 years and 63.4% of them were women. Common clinical features included fever (97.6%), headache (97.6%), vomiting (59.5%), seizures (25%), meningeal signs (73.8%), altered sensorium (42.9%), sixth nerve play (26.2%), and focal motor deficit (33.3%). Hyponatremia was seen in 23.8%. The most common radiologic abnormalities included exudates (85.4%), hydrocephalus (64.3%), infarcts (34.1%), and spinal arachnoiditis (39%). Good outcome (modified Rankin Scale 0–2) was seen in 66.7%, while 22% died. Predictors of poor outcome and death included low Glasgow Coma Scale score and high stage of TBM. Mean duration of illness was 65.9 ± 74.6 days. Mean delay in start of antituberculosis treatment after admission was 2.1 ± 2.3 days. Of the patients, 67.5% received wrong diagnosis and 85% received wrong treatment before admission. Delayed treatment initiation (>21 days after the onset of illness) was seen in 64.5%. Delayed treatment initiation did not affect the outcome of TBM.

Conclusions: There is an urgent need to develop cost-effective tests and spread awareness about TBM among medical practitioners.

Abstract ID: 123: Long-Term Outcomes in Cohort of Super-Refractory Status Epilepticus in a Tertiary Care Center

goli Ashwini, Sita Jayalakshmi

KIMS Hospital, Hyderabad (KIMS, Hyderabad), Telangana, India

Background and Aim: Super-refractory status epilepticus (SRSE) is a life-threatening neurologic emergency with high mortality and morbidity, and there is no clear protocol for its management. The aim of this study is to give an overview of the demographics, etiology, diagnostics, treatment, and long-term outcomes of SRSE.

Methodology: This was a retrospective cohort study conducted in KIMS Hospital, Secunderabad from 2010 to 2024. Patients of any age, satisfying the definition for SRSE, irrespective of etiology were included. Immunotherapy was initiated wherever necessary. Functional outcome (Glasgow Outcome Scale, control of seizures, return to work/school) was compared with clinical data at the latest follow-up (0.5–14 years) using logistic regression analysis. In addition, outcomes were compared between new-onset refractory status epilepticus (NORSE) and known epileptics.

Results: Of total 90 patients, 54 were males. Mean age at presentation was 37 (2–85) years. Fifty-nine (66%) were patients with NORSE and 22 (24%) were known epileptics. In total, 56 received immunotherapy and two underwent surgery. Ten patients were lost to follow-up. Of 80, 43 survived. Mortality rate was 46%. Among those who survived, 51% returned to work/school, seizures were well controlled in 39 (91%), while five (12%) had persistent seizures. Moreover, 65% were completely seizure free. Younger age group (mean 12.80 ± 7.79) had persistent seizures than older age group (mean 32.69 ± 19.04) ( P = 0.027). Known epileptics tend to have persistent seizures than NORSE patients in the long term ( P = 0.055). Among 39 patients who showed good control of their condition, 48.7% received immunotherapy during the active phase ( P = 0.032). NORSE constituted 64% among those with favorable generalized onset seizure. No difference was observed in the duration of intensive care unit or hospital stay with long-term outcome.

Conclusions: Early initiation of intravenous anesthetics and immunotherapy is associated with good control of seizures and improved functional outcome in the long term.

Abstract ID: 124: Clinical Subtypes and Radiologic patterns in Progressive Supranuclear Palsy: A Retrospective Analysis at a Tertiary Care Center

Kartika Gulati

Amrita Institute of Medical Sciences, Faridabad, Haryana, India

Background and Aim: Progressive supranuclear palsy (PSP) is an important differential of Parkinson’s disease. The 2017 PSP diagnostic criteria classify it into cortical and subcortical variants. Subcortical variants have longer disease duration and slower progression rate. However, progressive supranuclear palsy- Richardson syndrome (PSP-RS) is overrepresented compared to other variants. Midbrain to pons ratio in magnetic resonance imaging (MRI) aids in PSP diagnosis and subclassification. We aimed to subclassify PSP into cortical and subcortical types and identify the clinical and radiologic correlation in PSP patients.

Methodology: This was a retrospective observational study performed in Amrita Institute of Medical Sciences, Faridabad. Patients who attended movement disorder clinic at Amrita Institute of Medical Sciences, Faridabad, between August 2022 and April 2024, and were provisionally diagnosed as PSP were included as the study population. Clinical and radiologic data were collected using MS Excel. Statistical analysis was then performed.

Results: Out of the total 105 patients, four patients could not be traced. Thirty-four out of 101 patients were females (67 males). The mean age at symptom onset was 66.9 years, and the mean disease duration at presentation was 3.8 years. Most common clinical subtype was progressive supranuclear palsy-corticobasal syndrome (PSP-CBS) (31), followed by progressive supranuclear palsy-parkinsonism (29), progressive supranuclear palsy- Richardson syndrome (21), progressive supranuclear palsy - frontotemporal dementia (PSP-FTD) (18), and progressive supranuclear palsy-progressive gait freezing (3). Most common presenting feature was akinetic rigid syndrome, followed by cognitive dysfunction, dystonia, and gait freezing. The midbrain to pons ratio was measured in 47 cases. Mean midbrain:pons ratio was 0.15 in PSP-RS subgroup, 0.245 in PSP-CBS, 0.22 in PSP-P, and 0.21 in PSP-FTD subgroup. PSP-CBS patients had asymmetric cortical atrophy.

Conclusions: PSP subtypes other than PSP-RS are underrepresented. Classifying PSP into cortical or subcortical subtypes based on clinical and radiologic assessment may aid treatment and prognostication.

Abstract ID: 125: Autoimmune Movement Disorders: An Illustrative Case Series of Eight Patients

Purushottam Singh, Aditya Gupta, Anirban Gupta

Army Hospital Research & Referral, New Delhi, India

Background and Aim: Movement disorders are a prominent and common feature in many autoantibody-mediated neurologic diseases and present as varied clinical presentations. This is especially crucial as they are potentially curable and sometimes a harbinger of an underlying tumour. This study aims to describe the variability of presentations, etiology, management, follow-up, and outcome of autoimmune encephalitis (AIE), a potentially reversible cause of movement disorder.

Methodology: We evaluated eight patients who presented with movement disorders associated with AIE at our tertiary care center between 2021 and 2023. All patients were treated and followed up as per standard protocol.

Results: We evaluated eight patients who presented with movement disorders in association with AIE. Various abnormal movements observed were myokymia, tremors, dyskinesias, stereotypy, dystonia, ataxia, parkinsonism, and stiff limb and spasm Antibodies were detected against GAD-65 ( n = 3), CASPR-2 ( n = 3), and LGI-1 ( n = 2). Diagnoses made were Isaac syndrome, Morvan syndrome, and AIE, and one patient was diagnosed with opsoclonus myoclonus syndrome associated with neuroblastoma. On neuroimaging, five (62.5%) patients revealed normal magnetic resonance imaging of the brain. On treatment, three (37.5%) patients responded well to first-line immunotherapy (intravenous immunoglobulins, steroids, or both). Two patients received second-line therapy consisting of rituximab (Table 2). One patient with paraneoplastic opsoclonus myoclonus ataxia syndrome received adrenocorticotropic hormone (ACTH) and required surgical removal of the tumor. All patients are in remission; six patients are on maintenance immunosuppressants with azathioprine ( n = 3), mycophenolate mofetil ( n = 3), and two are on rituximab.

Conclusions: Our observations suggest that a high index of clinical suspicion with early and aggressive management of these disorders will increase the likelihood of better outcomes.

Abstract ID: 126: Long-Term Functional Outcomes and Caregiver Burden in Survivors After Decompressive Hemicraniectomy for Malignant Hemispheric Infarction: Real-World Experience from South India

Hari Krishna Sai Pramodh Veliveli, Sanjith Aaron, Ajith Sivadasan, Vivek Mathew, Prabhakar Thirumal Appaswamy, Mathew Joseph, Angel Miraclin

Christian Medical College, Vellore (CMC, Vellore), Tamil Nadu, India

Background and Aim: Decompressive hemicraniectomy (DHC) is an important life-saving measure in the management of patients with malignant hemispheric infarctions (MHIs). However, there is paucity of data on the long-term functional outcomes and caregiver burden among Indian patients.

Methodology: The study was a prospective observational study on a historical cohort of patients who underwent DHC over 5 years. A total of 66 patients who underwent DHC for MHIs were followed up for 2 years.

Results: Among the survivors, excellent outcomes were seen in 13 (19.6%) patients and good outcome was seen in 26 (39.3%) patients.

Conclusions: There was no difference in outcomes between right and left hemisphere involvement. Caregiver burden was moderate in this cohort, with higher social insecurity and dysfunctional social life observed among the caregivers with higher disability.

Abstract ID: 127: Non-ataxia Manifestations of Spinocerebellar Ataxia – 12

Pooja Anand, Divyani Garg, Ayush Agarwal, Animesh Das, Divya MR, Awadh Kishor Pandit, Achal Kumar Srivastava

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Spinocerebellar ataxia type 12 (SCA12) is the most common autosomal dominant ataxia in northern India. In clinical practice, non-ataxia manifestations are frequently observed in SCA12 patients, which require early diagnosis and management to improve quality of life. Our aim was to study non-ataxia manifestations of SCA12, which are currently lacking in literature.

Methodology: We conducted a single-center, cross-sectional study on 60 genetically confirmed cases of SCA12. We analyzed non-ataxia manifestations by using the following validated scales: Montreal Cognitive Assessment (MOCA), Hamilton Depression Scale (HAM-D), Hamilton Anxiety Scale (HAM-A), Validated Sleep Questionnaire, and short form health survey questionnaire (SF-36), which were used to assess cognition, depression, anxiety, sleep, and quality of life, respectively.

Results: Hand tremor (75%) was the most common presenting feature, followed by ataxia (15%). Mean MOCA score was 22.87 ± 4.86, with 32 patients (53.34%) having mild cognitive dysfunction. Mean HAM-A score was 8.58 ± 4.09, with 40 patients (66.67%) having mild anxiety. Mean HAM-D score was 9.45 ± 4.48, with 39 patients (65%) having mild depression. Sleep abnormalities were seen in 21 patients (35%).

Conclusions: The burden of non-ataxia manifestations was observed to be high in our study. Hand tremor was the most common and initial non-ataxia manifestation of SCA12, which may be misdiagnosed as essential tremor or parkinsonian tremor. Cognitive and sleep abnormalities and quality of life parameters correlated with severity of ataxia. Recognition and assessment of non-ataxia manifestations may help in providing holistic care to patients with SCA12.

Abstract ID: 128: Cognitive Function and Psychiatric Manifestations in Idiopathic Intracranial Hypertension: A Cross-Sectional Study

Deepshikha Singla, Bhupender Kumar Bajaj

Vardhman Mahavir Medical College, New Delhi (VMMC, New Delhi), India

Background and Aim: Idiopathic intracranial hypertension (IIH) is a neurologic disorder characterized by raised intracranial pressure of unknown etiology. Cognitive dysfunction has been reported in patients with IIH, and this can have negative impact on the quality of life of patients with IIH. The relationship between IIH and cognitive impairment is not well studied. We undertook this study with the aim to assess neurocognitive and neuropsychiatric dysfunction in patients with IIH and to determine their relationship with the clinical features of IIH and lumbar cerebrospinal fluid (CSF) opening pressure.

Methodology: This was a case–control cross-sectional observational study in which 34 patients and 34 age- and gender-matched healthy controls were recruited. Detailed clinical evaluation including comprehensive neurocognitive assessment was done using Indian Council of Medical Research-Neurocognitive Toolbox (ICMR-NCTB) for patients and controls. Neuropsychiatric Inventory Questionnaire was used to screen for neuropsychiatric comorbidities. Headache severity was measured using Headache Impact Test-6 (HIT-6), and lumbar puncture was done for all the patients.

Results: The mean age (± standard deviation [SD]) of patients and controls were 34.38 (±8.623) and 33.29 (±7.783) years, respectively. Mean (±SD) of HIT-6 score and lumbar CSF pressure were 63.26 (±11.944) and 291.91 (±31.09) mm CSF, respectively. Patients performed significantly worse compared to controls on assessment of cognitive function by ICMR-NCTB with Montreal Cognitive Assessment, Trail Making Black and White A and B tests, phonemic fluency, verbal delayed recall, picture naming test, and Modified Taylor Complex Figure test ( P < 0.05). Anxiety, depression, and appetite dysfunction were significantly higher in patients compared to controls ( P < 0.05).

Conclusions: Patients with IIH have multidomain cognitive impairment, which correlates with lumbar CSF pressure.

Abstract ID: 130: ReDem Study: Reversible Dementia Spectrum in Indian Context

Mahammad Mondal, Faheem Arshad, Keerthana BS, Pallavi Nagaraj Sastry, Suvarna Alladi

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Dementia is a major public health challenge due to limited therapies for degenerative forms; yet a notable portion of cases are potentially reversible, emphasizing the importance of actively identifying and promptly treating reversible causes. Our study aims to investigate the prevalence, etiologic spectrum, and clinical indicators predicting reversible dementia.

Methodology: This retrospective study was conducted at a South Asian neuroscience institute from 2017 to 2023 and included patients with cognitive decline and potentially reversible etiologies. Thorough investigations and red flag assessments were conducted.

Results: Out of 1810 dementia cases, 370 (20.4%) had reversible dementia (ReDem). ReDem patients were younger (56.2 vs. 61.9 years) with shorter illness duration (20.9 vs. 24.0 months) than degenerative dementia (DD) patients ( P < 0.001). Reversible etiologies included immune-mediated conditions (20.0%), neuroinfectious diseases (6.6%), psychiatric causes (7.6%), nutritional and metabolic etiologies (10.5%), neurosurgical conditions (14.6%), and miscellaneous etiologies (12.2%). All studied red flags, except seizures, were more common in ReDem patients compared to DD patients ( P < 0.05). Binary logistic regression showed red flags increasing odds of reversible causes, such as early onset (odds ratio [OR]: 2.35), fluctuations (OR: 3.73), rapid decline (OR: 3.02), high-risk exposure (OR: 11.76), high-risk behavior (OR: 35.88), incongruent neuropsychologic assessment (OR: 35.15), abnormal general physical examination (OR: 8.79), and unexplained neurologic findings (OR: 13.82). The presence of >1 red flag increased odd by 5.94 times, >2 by 20.69 times, and >3 by 25.14 times ( P < 0.05 for all). Treatment was provided to all ReDem patients, but 64.1% were lost to follow-up, with the mortality reported being 5.1%. Of the 35.9% followed, 63.9% reported subjective improvement and 31.6% showed Clinical Dementia Rating improvement (mean CDR: 1.3).

Conclusions: This study, the largest on this topic in India and one of the largest globally, emphasizes the importance of recognizing and treating reversible causes of dementia, including comorbidities in DD cases.

Abstract ID: 131: Predictors and Outcome of Symptomatic Intracranial Hemorrhage in Patients with Acute Ischemic Stroke Undergoing Intravenous Thrombolysis

Athira P

Government medical college, Thiruvananthapuram, Kerala, India

Background and Aim: Symptomatic intracranial hemorrhage (sICH) is a serious complication of intravenous thrombolysis (IVT) for acute ischemic stroke (AIS). The study was undertaken to determine the predictors and outcome of sICH.

Methodology: In this prospective cohort study conducted at ****, between May 2023 and May 2024, adult patients with AIS undergoing IVT were recruited. sICH was defined as per the European Cooperative Acute Stroke Study (ECASSIII) criteria, and the cohort was followed up and various clinicoradiologic parameters and outcome were compared between groups with and without sICH to determine the risk factors using a logistic regression analysis and prognosis, respectively. Predictive efficacy of risk prediction models – HAT, SEDAN, THRIVE, SPAN100 – were compared.

Results: Out of the 120 patients thrombolysed, sICH occurred among 9.17%, and all of them had a parenchymal bleed of PH2 category (with extra-axial bleed in two cases). The performance of predictive scores HAT ≥ 3, SEDAN > 3, THRIVE > 5, and SPAN100 = 1 was only modest. Seven of the 11 patients with sICH succumbed to death within a 3-month span.

Conclusions: The study identified five independent risk factors for sICH after multivariate logistic regression – the highest odds was for stroke severity followed by presence of large vessel occlusion, hyperdense middle cerebral artery sign on initial computed tomography, exposure to dual antiplatelet and total anterior circulation stroke. Among the risk prediction scores studied, HAT had the best discriminatory capacity.

Abstract ID: 132: Nerve Enlargement Pattern on Ultrasound can Differentiate Ulnar Neuropathy at the Elbow and Hansen’s Ulnar Neuropathy: An Observational Study from a Tertiary Care Center

Yerasu Muralidhar Reddy, Lalitha Pidaparthi, Jagarlapudi MK Murthy, Kiran ESS, Sudeer Kumar

Renova Institute of Neurological Sciences (RINS), Hyderabad, Telangana, India

Background and Aim: Hansen’s ulnar neuropathy (HUN) and entrapment ulnar neuropathy, commonly referred to as ulnar neuropathy at the elbow (UNE), are the most common causes of focal ulnar neuropathies. Differentiating the two is sometimes challenging. We aimed to examine the utility of the ultrasound (US) in differentiating these conditions.

Methodology: We performed a retrospective analysis of prospectively collected data from 26 nerves of 22 HUN patients and 36 nerves of 28 UNE patients who underwent US. The ulnar nerve cross-sectional area (CSA) was measured at the elbow behind the medial epicondyle, 5 cm proximal and distal to the elbow. The length of the enlarged nerve and the humerocubital nerve ratio (HCR) were calculated. All these parameters were compared between UNE and HUN.

Results: CSA of the ulnar nerve was significantly higher in HUN at all three measurement sites than in UNE ( P < 0.001). The maximum CSA was noted above the elbow in HUN and at the elbow in UNE ( P < 0.001). Lengthy enlarged segment was characteristic of HUN ( P < 0.001). HCR was also significantly different between the two groups ( P < 0.001). The optimum cut-off of HCR (for HUN) was 0.87 (sensitivity 88%, specificity 100%, area under the receiver operating characteristic curve 0.95).

Conclusions: HCR is a useful sonographic indicator for distinguishing HUN and UNE. Other variable favoring HUN is extensive and maximal nerve enlargement above the elbow. UNE is characterized by focal nerve enlargement at the elbow.

Abstract ID: 133: A Study on Natural History and End-of-Life Events in Amyotrophic Lateral Sclerosis – Indian Experience

Anoop Cheriyan, Saraswati Nashi, Nalini A

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Amyotrophic lateral sclerosis (ALS), often referred to as Lou Gehrig’s, is the most common and the best recognized form of the motor neuron diseases characterized by relentless degeneration of both upper and lower motor neurons leading to progressive muscular paralysis. Aims of the study were to (1) describe the clinical characteristics, survival pattern, and end-of-life events in ALS and (2) to compare young-onset ALS with late-onset ALS.

Methodology: This was an ambispective descriptive study conducted at National Institute of Mental Health and Neuro-Sciences, Bangalore. The study period was January 2010–June 2024. Patients diagnosed with ALS (definite, probable, and possible) formed the study population. The sample size was approximately 1000. Last follow-up status will be obtained by telephonic/email/letter questionnaire follow-up for cases coded before June 2022. For patients who succumbed to illness, relatives will be contacted via letter questionnaire/telephonic conversation and the details of end-of-life events and death will be recorded.

Results: We included 1070 patients in the dataset with a sex ratio of 3:1. The most common clinical type was upper limb onset (37.9%), which was followed by lower limb onset (31.1%) and bulbar onset (30.7%). Respiratory onset was noted in only 0.3% of cases. Moreover, 47.19% were classified as definite ALS. Memory disturbances were present in 10% and behavioral disturbances in 5.1% of the study population.

Conclusions: Our study’s demographic and clinical characteristics are largely consistent with existing literature, though some variations highlight the potential influence of regional factors.

Abstract ID: 134: Predictors of Good Functional Recovery in Patients with Acute Ischemic Stroke at 3 Months of the Acute Ischemic Event – A Prospective Cohort Study

Sohini Chakraborty, Awadh Kishor Pandit, M. V. Padma Padma Srivastava, Rohit Bhatia, Manjari Tripathi, Deepti Vibha, Vishnu V. Y., Rajesh Singh, Achal Kumar Srivastava, Ajay Garg, B. D. Charan, Ashish Upadhyaya

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Stroke is the fourth leading cause of death and fifth leading cause of disability in India. Our aim was to assess predictors of good functional outcome (modified Rankin Scale [mRS] 0–2) at 3 months and early neurologic improvement [ENI] = National Institutes of Health Stroke Scale (NIHSS) reduction by 8 or NIHSS of 0/1 at 24 h) in acute ischemic stroke (AIS) patients.

Methodology: A single-center prospective cohort study recruiting 311 adult AIS patients with pre-stroke mRS?1, within 72 h of ictus, fulfilling the inclusion and exclusion criteria, was conducted from February 2022 till November 2023. Demographic data, onset to hospital arrival (Time1), Glasgow Coma Scale (GCS) motor score at presentation, NIHSS at presentation (NIHSS0), at 24 h (NIHSS24), and at 72 h (NIHSS72), systolic blood pressure (SBP), diastolic blood pressure (DBP), Alberta Stroke Program Early Computed Tomography Score (CT-ASPECTS), multiphase-computed tomography angiogram collateral score, perfusion study (Computed Tomography Perfusion), time to intravenous thrombolysis (IVT)/thrombectomy (EVT), single/dual antiplatelet, electrocardiogram, echocardiography, Holter, hemogram, electrolytes, coagulogram, lipid, thyroid, ENI and mRS at 3 months (mRS3) were assessed.

Results: Of the study subjects, 66% were males, having a mean age of 56 years (±16.18). 53% were hypertensive, 35% were diabetic, 33% had dyslipidemia, 38% consumed tobacco while 23% consumed alcohol. In addition, 22%, 31%, 27%, 5%, and 14% had small vessel disease (SVD), large artery atherosclerosis, cardioembolic stroke, stroke of other determined etiology, and stroke of other undetermined etiology, respectively. Mean SBP, DBP, GCS, NIHSS at presentation, CT-ASPECTS, collateral score, time to hospital arrival, time to thrombolysis, and time to thrombectomy were 147 mmHg (±28.58), 86 mmHg (±16.4), 6 (±1), 11 (6–17), 6 (±3), 3 (±1), 6.5 h (3.5–12.5), 5.5 h (3.5–9), and 3.5 h (2.5–4.5), respectively. Moreover, 52% had LVO and 38% achieved ENI. On univariate analysis, younger age of ictus, lower NIHSS at 0, 24, and 72 h, higher GCS at presentation, higher CT-ASPECTS, absence of LVO, higher mTCA collateral score, TOAST of SVD, ENI, absence of cardiac chamber enlargement on echocardiography, absence of arrhythmia diagnosed by either echocardiogram/electrocardiogram/Holter, intake of dual antiplatelet, those who did not undergo decompression surgery, higher serum albumin, lower C-reactive protein, lower D-dimer, lesser time from ictus to hospital, and reduced duration of hospital stay, all were prognostic factors for good outcome at 3 months. On performing multivariate regression, NIHSS at presentation, age, achieving ENI, intake of DAPT, and absence of arrythmia were independent predictors of good outcome at 3 months after adjusting for confounders. With increase in age and NIHSS at 0 h, chances of good outcome significantly decreased with adjusted odds ratio of 0.943 (0.889–0.999, P = 0.049) and 0.791 (0.656–0.954, P = 0.014), respectively, whereas ENI, usage of DAPT, and absence of arrythmia had higher odds of achieving good outcome with odds ratio (OR) of 68.688 (28.127–167.742), 0.268 (0.096–0.749), and 0.296 (0.091–0.962), respectively. On performing multivariate regression, lower admission NIHSS, IVT, use of DAPT, absence of arrythmia, and right hemispheric stroke were significant predictors of ENI after adjusting for confounding factors. With the increase in NIHSS at 0 h, chances of ENI significantly decreased with adjusted odds ratio of 0.795 (0.717–0.881, P < 0.0001). With IVT, use of DAPT, absence of arrythmia, and right hemispheric stroke, odds of ENI increased with OR of 4.312 (2.258–8.233), 0.077 (0.015–0.395), 0.226 (0.112–0.456), and 0.153 (0.058–0.406), respectively.

Conclusions: Prediction of outcome after AIS is important for setting realistic and attainable treatment goals. It informs patients and their relatives properly, facilitates discharge planning, and helps in anticipating possible consequences. Improving our knowledge about predictive factors might enhance rehabilitative procedures in AIS patients.

Abstract ID: 135: Study of Cognitive Impairments in IPD Patients in a Tertiary Care Hospital in Eastern India

Subrata Halder, Ayon O Ghorai

Calcutta National Medical College & Hospital, Kolkata, West Bengal, India

Background and Aim: Cognitive impairments in idiopathic Parkinson’s disease (IPD) patients is an important nonmotor symptom that is ignored most of the time. In the last few decades, several studies revealed cognitive changes range from mild cognitive impairment to dementia in IPD. This study was conducted to see the cognitive derangement in IPD as well as its correlation with disease duration.

Methodology: It is an observational cross-sectional study of IPD patients who presented to our neurology department. One hundred and thirty patients of IPD who fulfilled the inclusion criteria formed the study population. Proper history examination, especially cognitive assessment, was done thoroughly and the data collected was analyzed subsequently.

Results: The M:F ratio in our study population was 1.5:1. In addition, 57% patients were below 60 years of age and the disease duration in 48% and 38% patients was 1–5 and 5–10 years, respectively. Majority of the patients belonged to low socioeconomic status and had below high school education. Montreal Cognitive Assessment is more informative than MMSE for quick assessment of dementia. Impairment of sustained attention was prominent in early as well as progressed disease. Language was preserved even in late stages, except reduced word fluency. Planning, mental flexibility, set sifting, abstract reasoning, and motor sequencing were significantly impaired, especially in late cases. Impairment of working memory, episodic memory, and free recall but recognition was better with cues. Procedural memory was also impaired. Semantics and autobiographical memory were preserved. Constructional apraxia was a prominent feature, irrespective of duration of disease. Bradyphrenia was more prominent as the disease progressed. Visuo-perceptual skill and color vision were relatively preserved even in late stages. No significant apraxia, acalculia, alexia, agraphia, right left disorientation, finger agnosia, dressing apraxia, hemineglect, or topographic disorientation was found.

Conclusions: As there was derangement of multiple domains of cognition, routine assessment of cognitive function is very essential to improve patients’ care.

Abstract ID: 136: The Study of SCN1A and SCN2A Gene Polymorphisms in Epilepsy and Their Effects on Antiepileptic Responsiveness

Kollipara Sumanth, Uma Sinharoy, Anindita Joardar, Alak Pandit

Institute of Post Graduate Medical Education and Research, Kolkata (IPGMER Kolkata), West Bengal, India

Background and Aim: We aimed to study the SCN1A and SCN2A gene polymorphisms among epilepsy patients and to ascertain the function of such genetic polymorphisms in determining the patients’ susceptibility to antiepileptic medications.

Methodology: The samples were collected from a cross section of men and women from our epilepsy clinic after obtaining informed consent from the patients/parents to perform genetic study and also after getting Institutional ethics committee approval. Each patient was investigated in detail, and the genotypes of single-nucleotide polymorphisms were identified by direct DNA sequencing.

Results: A total of 49 epilepsy patients were enrolled in this study, which consisted of 27 (55%) male and 22 (45%) female patients with a male to female ratio of 1.22:1. Forty-two (85%) patients were diagnosed with drug-resistant epilepsy and seven (14.28%) had drug-responsive epilepsy. The average age of patients was 16.57 years and there was no significant difference in age between drug-resistant patients (15.84 years) and drug-responsive patients (20.85 years). The average age at onset of first seizure was 9.42 years for responders and 6.25 years for nonresponders. Forty-two (85.7%) patients had generalized tonic clonic seizures, six (12.24%) patients had focal seizures with impaired awareness, and one (2.04%) patient had focal to bilateral tonic clonic seizures.

Conclusions: There was a significant association between rs2298771 of SCN1A gene, rs17183814 of SCN2A gene, and refractory epilepsy, and thus, these emerged as risk factors for drug resistance. They play a major role in influencing response to antiepileptic drugs.

Abstract ID: 137 : Tirofiban Infusion Therapy in Acute Ischemic Stroke – A Single-Center Experience

Neelamraju Mounika, M. K. Singh, Mukheem Mudabbir, Chakradhar Reddy, Chakradhar Reddy

Plot No 3, Road No. 2, Financial District, Gachibowli, Nanakramguda, Telangana 532

Background and Aim: Perforating artery infarction (PAI) with branch atheromatous disease has a stroke recurrence risk of 18%–33%, and there is also high risk of early neurologic deterioration. Tirofiban, a rapid-acting nonpeptide glycoprotein-IIb/IIIa platelet receptor antagonist, along with aspirin has significantly reduced neurologic fluctuations and shown excellent functional outcomes in acute PAI. Our aim is to report short-term outcomes in acute ischemic stroke (AIS) patients having PAI and who were treated with tirofiban adjunctive therapy.

Methodology: A total of 50 patients presented to the emergency department between January 1 and March 31, 2024 with AIS; among them, 22 patients had PAI. After scrutinizing the inclusion and exclusion criteria, 10 patients were selected for this study. Informed consent was obtained from all patients. A loading dose of tirofiban (0.4 µg/kg/min over 30 min) followed by a maintenance dose of 0.1 µg/kg/min up to 48 h plus aspirin (150 mg) was given for all patients. Outcome measures were modified Rankin Scale (mRS) and National Institutes of Health Stroke Scale (NIHSS) at admission and at the time of discharge.

Results: Among 10 patients recruited, 70% were males and 30% were females. Their mean age was 49.9 years, symptom duration was 14.4 h, mRS was 3, and NIHSS was 4.2. Ninety percent patients had symptom fluctuations. Also, 70% had anterior circulation and 30% had posterior-circulation. At discharge, all patients showed neurologic improvement, with 80% achieving mRS 0–1 and 20% achieving mRS of 2. Mean-hospital stay was 4.3 days, and none had bleeding diathesis.

Conclusions: In our experience, when stringent selection criteria were followed, tirofiban showed significant benefit in acute PAI with minimal-bleeding risk. However, large number of cases from different centers need to be studied for generalizing these results.

Abstract ID: 138: A Multicenter, Cross-Sectional Analysis to Assess the Safety and Usage Pattern of Brivaracetam in the Management of Focal-Onset Seizure – BREEZE Study

Krishnaprasad Korukonda, Arvind Sharma, Yakshdeep Dave, Girish Kulkarni, Rasika Deshpande

Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai (TNMC, Mumbai), Maharashtra, India

Background and Aim: Brivaracetam (BRV), a third-generation antiseizure medication (ASM), offers strong conformational receptor domain binding, faster blood-brain barrier permeability, and better tolerability, making it a potential therapeutic option as an initial line or initial line add-on strategy for focal-onset seizure (FoS). The following study was planned to further understand the role and relevance of BRV in the real-world settings of India.

Methodology: This was a multicentric, cross-sectional, noninterventional study conducted in patients with FoS across India. The study was approved by the central independent ethics committee. Descriptive and analytical statistics were employed using Statistical Package for the Social Sciences version 29.0.1.0.

Results: Per protocol analysis included 8479 eligible patients from 1069 sites. Gender distribution showed 5771 (68.06%) were males and 2708 (31.93%) were females, with a mean age of 41.21 years (12.74 ± standard deviation). Totally, 8019 (94.57%) patients had FoS and 460 (5.43%) patients had focal to bilateral tonic–clonic seizures (FBTCs), with psychiatric comorbidities noted in 3884 (45.80%) cases. In FoS, BRV 50–100 mg twice a day (BID) was administered as monotherapy and as an adjuvant in 51.6% and 48.3% patients, respectively. In FBTCs, BRV 100 mg BID was administered as monotherapy and as an adjuvant in 50.20% and 49.7% patients, respectively. In FoS, 4105 (51.18%) patients switched from Levetiracetam (LEV) to BRV, whereas 3915 (48.81%) patients switched from other ASMs to BRV. Behavioral adverse events (BAEs) accounted for 2059 (50.51%) patients in LEV to BRV switch versus 133 (3.39%) in other ASM to BRV switch. Sodium channel blocker (SCB), Na Valproate, and lacosamide were the most commonly prescribed ASM along with BRV.

Conclusions: BRV remains a pragmatic option for the management of FoS for the treatment-naïve and/or BAE cases.

Abstract ID: 139: Clinical Demographics and Relapse Rates in NMOSD Patients

Niveditha C, Archana N B, Shreyas Kumar Lamani, Vibhav M S, Janardhan D C, Praveen Kumar S, Pramod K, Thejus B

Bangalore Medical College and Research Institute, Bengaluru (BMCRI Bengaluru), Karnataka, India

Background and Aim: Neuromyelitis optica spectrum disorder (NMOSD) is a rare, debilitating autoimmune condition characterized by severe inflammation of the optic nerves and spinal cord, leading to blindness and paralysis. The global incidence of NMOSD varies, with higher prevalence observed in certain ethnic groups such as Asians and Africans. In the USA, the prevalence is estimated at 1–10 per 100,000 individuals. This study aims to address these gaps by investigating the clinical demographics, prognostic factors, and relapse rates in patients with NMOSD.

Methodology: In our tertiary care center, we conducted a retrospective study and analyzed the clinical and demographic characteristics of a cohort of 47 NMOSD patients.

Results: In our study, 66% were female and 34% were male, with a mean age of 35.4. The seropositivity rate was 61%. The most common presentation was longitudinally extensive transverse myelitis (51%), which was followed by optic neuritis (31%). Females were more affected in seropositive NMOSD compared to seronegative NMOSD, which is a statistically significant finding. The median Expanded Disability Status Scale (EDSS) at follow-up was 2 for seropositive NMOSD and 1.5 for seronegative NMOSD. The maximum number of relapses was 2 for seronegative NMOSD and 4 for seropositive NMOSD. Irregular follow-up was the most common cause of relapse. Although maximum relapses were seen in seropositive patients, statistical confirmation was not achieved. No statistically significant association was found between serostatus and disease severity.

Conclusions: This study provides a detailed overview of the clinical and demographic characteristics of NMOSD based on a retrospective analysis of 47 NMOSD patients, extending the information provided by similar recently published studies.

Abstract ID: 140: A Comparative Study of Clinicoradiologic Features and Outcome of Longitudinally Extensive Transverse Myelitis With or Without AQP4/Mog Antibody – A Retrospective Observational Study

Anuradha Mahto, Kamlesh Jagiasi, Pawan Ojha, Bilia Kizhekekudy Aipu, Gaurav Chaudhary, Rakesh Singh

Grant Medical College and JJ Hospital, Mumbai, Maharashtra, India

Background and Aim: Longitudinally extensive transverse myelitis (LETM) is often associated with neuromyelitis optica spectrum disorders (NMOSDs) or myelin oligodendrocyte glycoprotein (MOG)-Ab–associated disease. We aimed to study the difference in the clinical and radiologic features, treatment response, and recurrence of seronegative LETM, when compared to antibody-positive patients.

Methodology: A retrospective observational study was carried out in the inpatient department of neurology in a tertiary care center from April 2022 to April 2024. Written informed consent of the patients and approval from the Institutional Ethics committee were taken.

Results: Forty-three patients with LETM (20 seronegative, 14 anti-AQP4 Ab, and nine anti-MOG positive) were included. Majority (91.3%) of the females had antibody-positive LETM, whereas 60% males had seronegative LETM. Cervical and cervicodorsal cord with central grey matter involvement was present in antibody-positive LETM (52.3% vs. 45% and 95.6% vs. 70%, respectively). At the onset, both groups displayed similar average in modified Rankin Scale (mRS) scores. However, on follow-up, both the groups exhibited significant reduction in mRS score. Eleven patients experienced at least one relapse (6 [26.1%] seropositive and 5 [25%] seronegative).

Conclusions: Patients with seronegative LETM have clinical and radiologic differences when compared to those with seropositive LETM. However, the response to early immunotherapy is beneficial and prevents relapses and disability.

Abstract ID: 141: Peripheral Nerves and Beyond in Pure Neuritic Leprosy: Neuroimaging Study

Sanjeev Kumar Bhoi, Menka Jha, Menka Jha, Priyanka Samal, Yuvraj Lahre, Suvendu Purkait, Pritimayee Behera, Gautom Kumar Saharia

All India Institute of Medical Sciences, Bhubaneswar (AIIMS, Bhubaneswar), Odisha, India

Background and Aim: Pure neuritic leprosy (PNL) is an uncommon form of leprosy involving peripheral nerves alone. Limited information is available on the imaging abnormalities of the central nervous system (CNS) in leprosy patients. This prospective observational study uses magnetic resonance imaging (MRI) to evaluate the involvement of nervous system beyond the peripheral nerve among PNL patients.

Methodology: Patients with symptoms of neuropathy and/or enlarged nerves were screened. Patients underwent a detailed clinical examination, biochemical evaluation, nerve conduction study (NCS), and biopsy of the affected peripheral nerve, preferably the sural nerve. MRIs of the brachial and lumbar plexus, dorsal root ganglia, spinal cord, and brain were evaluated in patients with Hansen’s neuritis on nerve histopathology.

Results: Out of 86 patients screened for PNL, 52 were positive on nerve biopsy. Most of the patients were male (86.53%), and the mean age of patients was 45.72 ± 15.25 years. Asymmetrical polyneuropathy was the most common NCS pattern in 55.76% (29/52) patients. We found abnormal imaging findings in 21 (40.38%) patients. Ganglionitis was the most common finding seen in 14 (26.92%) patients, followed by plexitis (15.38%) and myelitis (11.53%). Patients with MRI lesions were younger and were found to have more functional impairment and raised cerebrospinal fluid protein.

Conclusions: In PNL, many patients have subclinical involvement of dorsal root ganglion, brachial plexus, lumbar plexus, and spinal cord. Exact pathophysiology of CNS involvement is not clear; however, imaging of the above-mentioned regions may help in early diagnosis and prevent complications. Furthermore, to prevent incorrect diagnoses, it is crucial to consider these findings in PNL patients when assessing individuals who exhibit symptoms of peripheral neuropathy.

Abstract ID: 142: Repetitive Transcranial Magnetic Stimulation in New Daily Persistent Headache Patients: A Single-Arm, Open-Label Study

Bharath MM, Vimal Kumar Paliwal

Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow (SGPGI Lucknow), Uttar Pradesh, India

Background and Aim: New daily persistent headache (NDPH) is a continuous, unremitting headache from onset that yields suboptimal results with traditional medications. Repetitive transcranial magnetic stimulation (rTMS) has emerged as a promising noninvasive treatment for migraine. We aimed to evaluate the efficacy of rTMS in reducing the frequency and severity of headaches and associated anxiety and depressive symptoms in NDPH patients.

Methodology: This was an open-label, prospective, single-arm, interventional pilot study conducted between October 2022 and September 2023. All eligible participants received 10 Hz rTMS (600 pulses, 10 trains), delivered to the left prefrontal cortex. The post-rTMS headache severity was recorded weekly for 4 weeks and headache-free days/functional disability, Patient Health Questionnaire-9 (PHQ-9), and Generalized Anxiety Disorder 7 item (GAD-7) scores were recorded at the end of 4 weeks and compared with pre-rTMS parameters. The primary outcome was defined by 50% reduction in headache severity/headache-free days and 6 points reduction in Headache Impact Test-6 score at 4 weeks.

Results: Fifty NDPH patients (mean [standard deviation {SD}] age, 35.06 [13.91] years; 31 females [62%]) participated in this study. Thirty-five patients (70%) reported 50% improvement in pain severity, with a mean reduction of 10.84 (4.88) headache days. Thirty-eight patients (76%) reported a 6 points reduction in HIT score at 4 weeks. Maximum improvement in the above parameters was observed in NDPH patients with chronic migraine. Only two patients reported sound intolerance. The median (interquartile range) PHQ-9 and GAD-7 scores reduced from 11.5 (3.75–20) to 7 (2–15) ( P < 0.001) and from 10 (3–14) to 5.5 (0–9) ( P < 0.001), respectively.

Conclusions: rTMS was well tolerated and effective in reducing pain severity, headache days and headache-related disability, and depressive and anxiety symptoms.

Abstract ID: 143: Neuropalliative Care Interventions in ALS: Development of a Care model

Keerthipriya M S, Seena Vengalil, Priya Treesa Thomas, Saraswati Nashi, Nalini A

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Amyotrophic lateral sclerosis (ALS)/motor neuron disorder is a life-limiting, relentlessly progressive neurodegenerative condition. The care is focused on improving the quality of life of patients and caregivers following palliative care principles. We undertook this study to explore ways of developing a neuropalliative care (NPC) model for ALS patients.

Methodology: This was a retrospective analysis of data collected between January 2022 and December 2023 from ongoing NPC research that started in October 2021 in a tertiary care center. Patients diagnosed with ALS as per Revised El Escorial criteria (REEC) by neurologists were referred for palliative care through the inpatient department or outpatient department. Clinical data collection tools validated for the study were used.

Results: Six hundred and eighty-one ALS patients were recruited during the study period. The M:F ratio was 2.18:1, mean age at presentation was 52.85 ± 12.36 years (16–86), and mean duration of illness was 18.54 ± 15.77 months (2–132). The mean Amyotrophic Lateral Sclerosis Functional Rating Scale- Revised (ALSFRSr) score available in 451 patients was 34.41 ± 8.02 (7–47). In addition, 537 patients received interventions by the medical officer, 250 patients by a nurse, 300 by a social worker, 176 by an occupational therapist, and 291 by a speech-language pathologist. These interventions were provided at the NPC clinic. All 681 patients then received long-term support through telephonic follow-ups made at regular intervals, and a total of 1511 telephonic contacts were made during the study period. Thirty-five patients residing locally received homecare visits. The trigger for palliative care referral was a mere diagnosis of ALS.

Conclusions: There is a need to develop outcome measures to capture the effectiveness of the NPC interventions that can make changes at the policymaking level to improve the availability of NPC for all ALS patients in the country.

Abstract ID: 144: Cerebral Venous Thrombosis (CVT) in Older Individuals: Understanding Risk Factors, Clinical Features, Treatment, and Outcomes

Brunda S, Raghunandan Nadig, G. R. K. Sarma, Thomas Mathew, Sagar Badachi, Sonia Shivde, Akshata Huddar, Sindhu V, Swathi S. Sanjee, Sreerag Kapparath

St. Johns Medical College, Bengaluru, Karnataka, India

Background and Aim: Cerebral venous thrombosis (CVT) occurs more commonly among young adults. It is infrequent in older individuals and may differ in clinical presentations, risk factors, and outcome from CVT in young. Anticoagulation is pivotal in treatment, and preventing risk factors remains essential. Our aim was to study the clinical characteristics, risk factors, and outcomes of CVT in patients aged over 45 years.

Methodology: Case records of patients with CVT over the age of 45 years admitted in our hospital between 2009 and 2023 were studied retrospectively. Demographic details, clinical presentation, neuroimaging, laboratory profile, and treatment given were analyzed with appropriate descriptive statistics.

Results: During this period, 318 patients with CVT were treated, of which 60 were above 45 years of age. Among them, 43 were males (71%). Most common presentation was headache (73%), followed by seizures (41%) and motor deficits (33%). Risk factors included hyperhomocysteinemia (55%), anemia (hemoglobin [Hb] <12) (30%), and polycythemia (Hb >16) (25%). antiphospholipid antibody (APLA)/antinuclear antibody (ANA) was positive in 11 patients. Two patients had malignancy. In addition, 71% of patients had extensive thrombosis involving more than one sinus, with 61% having parenchymal hemorrhagic infarct. Nearly 70% had more than 7 days of hospital stay. Vitamin K antagonist (VKAs) were most commonly prescribed at discharge, while 15 patients were discharged on Non-Vitamin K Antagonist Oral Anticoagulant (NOACs).

Conclusions: CVT is a significant health concern among individuals over 45 years old, particularly males, due to extensive thrombosis and prolonged hospitalization. Identification of risk factors and implementation of better treatment options in older adults are crucial for improving outcomes.

Abstract ID: 145: Unveiling the Hidden Neurology Care Providence: Inpatient Consultation Liaison Neurology in a Tertiary Care Hospital in India: Is the Time Ripe for a Neurohospitalist Subspecialty?

Tejaswini Shanubhogue, Soaham Desai, Sachin Khanchand Udwani, Arth Jayeshkumar Shah, Manthan Manesh Dave

Shree Krishna Hospital & Parmukhswami Medical college, Anand, Gujarat, India

Background and Aim: Neurologic disorders constitute a significant global burden, yet the role of neurologists in inpatient care remains underrecognized. We aimed to assess inpatient neurology liaison consultations in a tertiary care hospital in XXXXXX, India, to quantify this “hidden work” and inform service development.

Methodology: In this prospective cross-sectional study, we analyzed all neurology referrals ( n = 236) over 3 months at XXXXXX tertiary care teaching hospital. We evaluated patient demographics, referral characteristics, diagnoses, and management plans. Interobserver agreement on referral urgency was assessed using Cohen’s kappa.

Results: Most referrals were from Critical Care (48.7%, 95% confidence interval [CI]: 42.3%–55.1%), Medicine (18.6%, 95% CI: 13.7%–23.5%), and Emergency (11.9%, 95% CI: 7.8%–16.0%). Common diagnoses were stroke (39.0%, 95% CI: 32.7%–45.3%), seizures (30.5%, 95% CI: 24.7%–36.3%), and encephalopathy (28.8%, 95% CI: 23.1%–34.5%). Interobserver agreement on urgency was very good (kappa = 0.887, P < 0.001). Neurologists perceived 44.9% (95% CI: 38.5%–51.3%) of patients needing repeat review, but only 31.1% (95% CI: 22.3%–39.9%) of them received it.

Conclusions: Neurologists play a crucial role in managing inpatient emergencies, yet their contributions are often underrecognized. Despite good awareness among referrers on urgency assessment, the gap in re-review consultations highlights a need for service improvement. These findings call for investment in efficient, patient-centered neurology liaison services, potentially through models like neurohospitalists or tele-neurology.

Abstract ID: 146: Spectrum of Seronegative Optic Neuritis in a Tertiary Care Hospital

Reshma Shaik

Nizam’s Institute of Medical Sciences, Hyderabad (NIMS, Hyderabad), Telangana, India

Background and Aim: Optic neuritis (ON) refers to a group of conditions that involve inflammation of the optic nerve. The patients present with partial to complete vision loss within a few days of onset. A gamut of etiologies can be responsible for ON and are broadly classified as typical and atypical. ON seen in multiple sclerosis (MS) is usually referred to as “typical ON.” ON with various etiologies, other than MS, is classified as “atypical ON.” ON with Aquaporin 4 +ve, myelin oligodendrocyte glycoprotein (MOG) +ve, glial fibrillary acidic protein (GFAP), vasculitis is considered as seropositive, other than this, it is considered seronegative ON. Seronegative ON vary from traditional seropositive ON in terms of etiologies, management strategies, and ultimate neurologic outcomes. It is, therefore, very important to distinguish between seropositive and seronegative ON. We aimed to know the spectrum of seronegative ON cases and its outcomes in a tertiary care hospital.

Methodology: We included all the patients with seronegative ON (ON other than MS, neuromyelitis optica MOG, GFAP, vasculitis) from all age groups. The period of study was for 1 year (2023–2024). Total number of patients was 50 (15 male, 35 female). Patients were examined for visual acuity and the fundus was also examined before admission and at the time of discharge and 2 weeks and 1 month after follow-up.

Results: Out of total 50 patients, 10 patients had toxic ON, one patient had high-altitude ON, and others had ON due to unknown causes. Mean age of the patients was 28 years. There was female predominance, and the visual acuity ranged at admission from perception of light to 6/9.

Conclusions: Seronegative ON spectrum is increasing day by day, so early recognition and treatment will lead to significant improvement.

Abstract ID: 147: Demographic, Clinical Profile, and Treatment Outcome of New-Onset Refractory Status Epilepticus (NORSE) and Febrile Infection-Related Epilepsy Syndrome (FIRES) in a Tertiary Care Center

L. Anand, Suryapprabha Turaga, Bhavani M

Nizam’s Institute of Medical Sciences, Hyderabad (NIMS, Hyderabad), Telangana, India

Background and Aim: To study the Incidence, prevalence, clinical profile, and management outcomes in patients presenting in status epilepticus and diagnosed as new-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.

Methodology: This is a single-centered, ambispective, observational study. Demographic data, clinical features, and cerebrospinal fluid (CSF) analysis, electroencephalogram (EEG), and the reports of magnetic resonance imaging of the brain and positron emission tomography scan were collected. Serum and CSF autoimmune encephalitis and paraneoplastic panel were sent. Continuous EEG monitoring was done to see the response to antiseizure medication (ASM) and other treatment. Patients received ketogenic diet. Urine ketosis was monitored daily. Final outcome with different combined treatment modalities was compared along with their efficacy.

Results: Thirty patients who met the inclusion criteria were included in the study. The age of patients ranged from 11 to 58 years. Treatment modalities included multiple ASMs (median five drugs). Midazolam infusion was administered in all patients. Fourteen patients required anesthetics (ketamine) to suppress the seizure activity. Steroids were used in 24 patients and intravenous immunoglobulin (IVIG -2 g/kg) was used in 14 patients. Twelve patients died in the acute phase.

Conclusions: The duration and severity of seizures in the acute period correlated directly with the short-term and long-term clinical outcomes. There is an urgent need for developing new effective therapeutic strategies to treat this acute catastrophic epileptic syndrome.

Abstract ID: 148: Brown–Vialetto–Van-Laere Syndrome: A Close Mimic of Madras Motor Neuron Disease, but Potentially Treatable Syndrome – Experience from a South Indian Center

Dipti Baskar, Sai Bhargava Sanka, Seena - Vengalil, Kiran Polavarapu, Veeramani Preethish Kumar, Saraswati Nashi, Ananthapadmanabha K, Yamini Harikishen, Priya Treesa Thomas, Gautham Arunachal, Atchayaram Nalini

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Mutations in the genes SLC52A2 and SLC52A3 , which encode the riboflavin transporters RFVT2 and RFVT3, respectively, cause Brown–Vialetto–Van-Laere (BVVL) syndrome. Typically, patients present with sensorineural deafness and progressive pontobulbar palsy. Other features include sensorimotor axonal neuropathy, optic atrophy, and respiratory compromise. Here, we describe the clinical and genetic spectrum of eight genetically confirmed BVVL syndrome patients, which closely mimics the elusive Madras motor neuron disease (MMND).

Methodology: This was a retrospective observational study on eight patients with BVVL syndrome and corresponding genetic variants. Baseline demographic, clinical, and neurophysiology details were recorded, and genetic analysis was done by next generation sequencing.

Results: The mean age at disease onset was 11.25 (range: 7–16) years. All had varying illness durations, with a mean duration of 6.75 years (range: 3–12). All had severe sensorineural hearing loss, dysarthria and severe hypophonia, tongue atrophy and fasciculations, impaired tandem gait, and sensory ataxia. Weakness and atrophy of limb muscles were noted in five, facial weakness in four, sensory symptoms in three, and 9–11 cranial nerve palsy in five patients. In the eight patients, five novel variations (two homozygous and two heterozygous variants in the SLC52A3 gene and one heterozygous variant in the SLC52A2 gene) and previously reported one homozygous and one heterozygous variant in SLC52A3 were noted. Six of eight patients reported significant clinical improvement after 3–6 months of high-dose riboflavin therapy (600 mg/day).

Conclusions: This report highlights the importance of genetic confirmation of all patients suspected to have MMND/BVVL syndrome and not mislabel cases as MMND, for which still further advanced genetic testing is warranted.

Abstract ID: 149: Evolution of Sleep Apnea and Arousals in Patients with Ischemic Stroke: A Longitudinal Follow-Up Study in Asian Indian Population

P. R. Srijithesh, Mythirayee S, Doniparthi Venkata Seshagiri, Nitish L. Kamble, Sanjib Sinha, Ravi Yadav

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Sleep-disordered breathing (SDB) is a common condition characterized by pauses in breathing or shallow breathing during sleep. SDB can have a number of detrimental consequences, including daytime sleepiness, fatigue, and increased risk of accidents. In patients with stroke, it is associated with poorer clinical outcome and increased risk of recurrence of stroke. The study objective was to evaluate the frequency, severity, type, and pattern of evolution of SDB and arousals in ischemic stroke patients.

Methodology: Ischemic stroke patients underwent overnight polysomnography within 1 month of stroke and after 3 months, with evaluations at 6, 18, and 24 months.

Results: Of the 141 acute ischemic stroke patients screened, 105 underwent adequate polysomnography. The mean age of patients was 50.5 years (standard deviation [SD]: 12.30); 78.4% of them were male, and the mean body mass index was 26 (SD: 5.4). Also, 81.9% had an apnea-hypopnea index (AHI) 5, with 12.5% having AHI >30. Median arousal index (ArI) was 5.5 (interquartile range [IQR]: 0.5–49.5), which increased with apnea. Follow-up showed 56.8% with AHI ?5 and 12.5% with severe apnea. Median ArI decreased to 4.8 (IQR: 0.5–21.1). Significant changes in severe (19 vs. 7), moderate (17 vs. 11), and mild (8 vs. 15) apnea categories were noted. Sleep efficiency and rapid eye movement (REM) sleep duration improved significantly.

Conclusions: The study revealed a high prevalence of SDB in acute ischemic stroke patients, with improvements in AHI, ArI, sleep efficiency, and REM sleep duration in the chronic phase.

Abstract ID: 150: Long-Term Outcome of Revascularization Procedures for Moyamoya Angiopathy in South Indian Children: Experience from a Prospective Cohort

Nikitha Rafeek, Vaishakh Anand, Suhas Udayakumaran, Vinayan KP

Amrita School of Medicine, Kochi, Kerala, India

Background and Aim: Moyamoya vasculopathy is one among the most common causes of pediatric stroke. Here, we describe the clinical profile and effect of revascularization procedure in 14 children with moyamoya vasculopathy, who had been admitted to a tertiary care hospital in South India.

Methodology: A prospective study was conducted in children identified with moyamoya vasculopathy during the study period (2006–2022). Demographic and clinical details were collected from the hospital database up to the last follow-up visit and by telephonic interviews. Outcome was analyzed based on functional assessment using modified Rankin Scale (mRS), recurrence of stroke, and seizure burden post-revascularization procedure.

Results: Fourteen children were included. There were eight boys and six girls from India ( n = 12) and Maldives ( n = 2). Median age at presentation was 12 months (0.5–96 months), with a median latent period of 1 year (<1 week–10 years) from symptom onset and diagnosis. Six subjects had familial moyamoya. Stroke was the most common initial presentation ( n = 9, 64%). Others included recurrent seizures ( n = 1), transient ischemic attacks ( n = 2), and headaches ( n = 2). Two children were categorized as moyamoya syndrome having Down syndrome and Neurofibromatosis type 1 (NF-1). Radiologically, only ischemic stroke was observed. Unilateral disease was seen in two children, and PCA involvement was noted in seven children. Indirect revascularization was performed in 12 children. Mean follow-up period was 8.3 years (2–16 years). Stroke recurred in one subject. Three out of nine children continued to have seizures during follow-up. Headache resolved in all and one child expired. Mean mRS score improved from 2.9 (1–5) at the onset to 2.5 (0–6) at the last follow-up.

Conclusions: The subtlety of clinical presentation of Moyamoya vasculopathy in children might cause a delay in diagnosis. Revascularization procedures will stabilize the clinical course by improving the cerebral perfusion and may have a positive impact on outcome.

Abstract ID: 151: Reversible Cerebral Vasoconstriction Syndrome – A Case Series from South India

Sapna Erat Sreedharan, Manju Surendran, A. R. Swathy Krishnan, P. N. Sylaja

SCTIMST, Thiruvananthapuram, Kerala, India

Background and Aim: Reversible cerebral vasoconstriction syndrome (RCVS) is a rare cause of stroke, presenting with headache, seizures, focal deficits, or encephalopathy. Very little is known about this rare condition from India. Here, we studied the clinical, imaging characteristics and short-term outcome of RCVS from South India.

Methodology: This was a single-center retrospective study of all subjects with a clinicoradiologic diagnosis of RCVS from January 2014 to December 2023 with a 3-month completed follow-up. Comparisons were made between ischemic and hemorrhagic presentations of RCVS with regard to clinical features, vascular imaging patterns, and outcome.

Results: We had 22 patients fulfilling the criteria for RCVS by Calabrese et al . There were 18 women with a mean age 47.59 years (range 25–69 years). Seven of our patients presented 2 weeks after symptom onset. Almost all our patients had some precipitating factor for RCVS. Headache was the most common presenting symptom in our cohort. Majority (14/22, 63.6%) developed focal neurologic deficits during the course of illness. Four/22 (18%) patients did not report headache during the course of illness. Most common imaging finding at presentation was cortical subarachnoid hemorrhage (SAH) (9/22, 40.9%), followed by infarcts (6/22, 27.2%), while 12/22 (54.5%) developed new ischemic lesions on repeat imaging. Ischemic and hemorrhagic presentations of RCVS did not differ in the clinical presentation or outcome. All patients with ischemic lesions showed diffuse vasospasm on imaging, while those with SAH had both diffuse and focal vascular abnormalities.

Conclusions: Here, we present the largest single series of RCVS from India. Patients can infrequently present without headache and focal vascular abnormalities.

Abstract ID: 153: Clinico-demographic and Laboratory Profile of Myasthenia Gravis Patients in a Tertiary Care Center from South India

Tejeswara Rao Salihundam, Gopi Seepana

Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Myasthenia gravis is the most common autoimmune neurologic disease, with a highly variable presentation. It is a potentially serious, but treatable neuromuscular junction autoimmune disorder, if diagnosed early and confirmed by clinical and laboratory testing. Aim of this study is to evaluate clinical, demographic, and radiologic profile.

Methodology: This study was conducted for 1 year in department of neurology, Andhra Medical College, with a sample size of 44 patients. Inclusion criterion was patients above 18 years of age with a final diagnosis of myasthenia gravis. Exclusion criterion was age less than 18 years. Data were analyzed using Stata MP and Microsoft Excel.

Results: Of the patients, 64.7% were females, with the average age of presentation being 48.2 years. Most common presentation was asymmetric ptosis. Nine percent of patients presented with myasthenic crisis. Fatiguability, fluctuations, and improvement with rest were present in almost all patients. Repetitive nerve stimulation (RNS test) was positive in 72.7% of patients. In addition, AChR Ab (acetylcholine receptor) was positive in 52.6% and Musk Ab was positive in 13.63% of patients. On contrast-enhanced computed tomography chest, thymoma was present in 31.8% of patients. Presence of thymoma was associated with more severe Quantitative Myasthenia Gravis (QMG scores) and was more likely to present with generalized myasthenia.

Conclusions: Myasthenia gravis is a polymorphic disease. It needs appropriate history and evaluation by expertise and availability of serological and electrophysiologic testing for prompt early diagnosis and treatment for good outcome.

Abstract ID: 154: Evaluation of Factors Responsible for Progression in a Cohort of Hirayama Disease

Yamini Priyanka PentaKota, Vishnu VY, Saranya B Gomathy, Ayush Agarwal, Ajay Garg, Padma Srivastava, Rohit Bhatia, Alisha Reyaz

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Hirayama disease is a rare, nonfamiliar, monomelic amyotrophy originally described in 1959. The classical findings are pure motor lower motor neuronweakness involving unilateral or asymmetric bilateral forearms and hands. This plateaus after few years. It causes significant disability in some patients, who can benefit with timely surgical intervention. In our study, we aim to assess the frequency of Hirayama in patients presenting with focal upper limb amyotrophy and to assess factors contributing to progression.

Methodology: It is a single-center, prospective, observational study planned between November 2022 and November 2024. All patients presenting to xxx outpatient department fulfilling the inclusion criteria will be enrolled and assessed every 6 months. Motor system examination with modified Medical Research Council scale and dynamometry and functional scales like - Overall Neuropathy Limitations Scale (ONLS), Multifocal motor neuropathy - Rasch-built Overall Disability Scale (MMN RODS), Amyotrophic Lateral Sclerosis Functional Rating Scale (ALS FRS) are applied to assess patients periodically every 6 months. Twenty-eight patients were followed up till 1 year.

Results: Eighty-eight patients who presented with focal upper limb amyotrophy were screened. Out of these patients, 75 patients (85%) met the diagnostic criteria. Of them, 96% were males. Mean age of patients was 22.5 years (standard deviation (SD)-7.634). The average age at symptom onset was 17.8 years (SD 7.23). The symptoms started in the left upper limb in 44% and in the right upper limb in 42%, with eventual asymmetric bilateral involvement in 68.5%. Moreover, 79% of the patients were found to be historical progressors at the time of enrolling. Baseline ONLS, MMN RODS, and ALS FRS were 1.772 (SD 0.784), 33.4 (SD 7.231), and 44.8 (SD 1.22), respectively. The average power in the worst limb by manual and digital dynamometry was 29.58 (SD 23.5) and 32.153 (SD 26.42) pounds, respectively. Also, 77% had MRC grade 4 in the worst limb. The disability was mild in 78.5% and severe in 3.2% of patients.

Conclusions: Cervical collar and reassurance are the mainstay of treatment in majority of patients.

Abstract ID: 155: Palliative Care Needs, Symptom Concerns, and Caregiver Burden in Chronic Neurologic Disorders: An Analysis of 1000 Cases

Priya Treesa Thomas, Gargi S. Kumar, Seena Vengalil, Suvarna Alladi, Nalini Atchayaram, Ravi Yadav, Suvarna Alladi, Nalini Atchayaram

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Patients with chronic neurodegenerative disorders have multifaceted needs that go beyond the diagnosis and are often unmet. Research on care has predominantly concentrated on the medical symptomatic management, with some attention being given to the holistic care components. Present study aims to understand the symptom burden and palliative care needs in a cohort of patients with neurodegenerative disorders referred to a neuropalliative care clinic in a tertiary hospital.

Methodology: A prospective, cross-sectional study was conducted using mixed methods. We interviewed consecutive patients–caregiver dyads ( N = 1000; adults and children) referred to an interdisciplinary, neuropalliative, and supportive care clinic established as part of the routine neurologic services in a tertiary referral care hospital in Bengaluru, India. The participants were assessed for their clinical and sociodemographic profile, symptom burden (palliative care outcome scale [Integrated Palliative Care Outcome Scale S24]), caregiver burden (Zarit Burden Interview), and palliative care needs (semi-structured needs assessment schedule). The quantitative and qualitative data were analyzed separately and integrated.

Results: Neuromuscular disorders (mainly amyotrophic lateral sclerosis) were most commonly referred, followed by advanced dementia and movement disorders. There was a male preponderance (64%) among the patients receiving the care. Majority of the caregivers (55%) reported severe caregiver burden. There was a positive correlation between symptom burden and caregiver burden ( r = 0.144; P < 0.001), and caregiver burden was more when the care receipt patient was of female gender. The perceived needs included the need for accurate and sensitive communication of information, symptom management, especially of distress symptoms, and support as the disease progresses.

Conclusions: Improving primary palliative care knowledge and skills and building effective networks with various stakeholders are ways forward to address the identified needs.

Abstract ID: 156: Evaluation of Atrial Cardiopathy in Elderly Patients with Non-cardioembolic Acute Ischemic Stroke Including Embolic Stroke of Undetermined Source

Akash Narayan, Sadanand Dey, Debabrata Chakraborty, Amitabha Ghosh

Apollo Multispeciality Hospital, 58, Canal Circular Road, Kadapara, Phool Bagan, Kolkata, West Bengal 754

Background and Aim: Embolic stroke of undetermined source (ESUS) is responsible for about 30% of ischemic stroke. Atrial fibrillation (AF) is an important cause of embolic stroke. Atrial cardiopathy predisposes to thrombus formation and thereby may be responsible for embolic stroke in the absence of AF. A link between atrial cardiopathy and ESUS, if proven, may support anticoagulant therapy in ESUS for secondary prevention. Our aims were to (1) classify various ischemic stroke types according to TOAST classification and to evaluate ESUS in the same subjects and (2) find the association of atrial cardiopathy in ESUS and compare it with non-ESUS group.

Methodology: This observational prospective study was conducted in Apollo Multispecialty Hospital, Kolkata from September 2022 to November 2023. Patients >60 years of age with ischemic stroke were selected for the study and were further classified according to Trial of ORG 10172 in Acute Stroke Treatment (TOAST)classification. Brain and neck vessel imaging, electrocardiogram, echocardiography, 24-h Holter, and N-terminal pro-B-type natriuretic peptide (NT-proBNP) test were done. Data was analyzed for atrial cardiopathy after excluding AF and other cardioembolic causes of stroke with appropriate statistical test of significance using Statistical Package for the Social Sciences 23.0 version software.

Results: Of the 225 eligible patients, 48 (21.3%) were classified as ESUS and 97 (43.1%) as non-ESUS. Atrial cardiopathy was significantly more prevalent in ESUS patients than non-ESUS patients, with NT-proBNP >250 pg/ml and P-wave terminal force in lead V1(PTFV1) >5000 mV ms as independent variables ( P < 0.05).

Conclusions: Markers like NT-proBNP and PTFV1 may, therefore, be potential independent predictors of embolic stroke even in the absence of atrial fibrillation. Future research on the potential use of anticoagulants in ESUS should focus more on subsets of patients with the above markers of atrial cardiopathy.

Abstract ID: 157: Assessment of Cognitive Dysfunction During Migraine Attack and Comparison with Healthy Non-headache Controls: A Prospective Clinic-Based Study from North India

Rahul Nagane, Debashish Chowdhury, A. K. Duggal, Sanjay Rao Kordcal

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi), India

Background and Aim: Cognitive dysfunctions in migraine patients during a headache phase have not been reported in Indian migraine patients. We aimed to compare the cognitive functions in episodic migraine without aura (E-MwA) patients during ictal (headache) states and compare them with non-headache controls (NHCs).

Methodology: E-MwA patients underwent evaluations during an untreated spontaneous attack of E-MwA. All subjects undertook cognitive status assessment by a battery of 16 tests. The means between the two groups were compared by independent Student’s t -test. Post hoc Bonferroni correction was applied. The level of significance was set at P < 0.05.

Results: Eighty-eight patients each in E-MwA and NHC groups were studied (mean age 30.3 ± 8.8 and 31.4 ± 6.6 years, respectively; female:male ratio 3.4:1 and 1:1, respectively). E-MwA patients fared worse in various cognitive tests compared to NHCs, such as Montreal Cognitive Assessment (MoCA) (26.1 ± 2.2 vs. 28.1 ± 0.3), digit span forward (5.2 ± 0.9 vs. 6.3 ± 0.2), digit span backward (3.7 ± 0.8 vs. 4.6 ± 0.1), trail making test A (46.9 ± 2.3 vs. 34.5 ± 2.3) and test B (89.8 ± 3.7 vs. 66.3 ± 3.9), Stroop test for words (60.8 ± 2.1 vs. 46.3 ± 2.2), colors (89.1 ± 2.9 vs. 70.9 ± 3.1), and interference (137.9 ± 4.2 vs. 116.5 ± 4.3) (all P < 0.001). E-MwA patients performed significantly worse in both phonemic and semantic fluency tests (9.1 ± 0.5 vs. 13.3 ± 0.5; mean difference: -4.2 ± 0.7; 95% confidence interval [CI]: -5.5 to -2.8; P = 0.001 and 15.6 ± 0.5 vs. 18.5 ± 0.5; mean difference: -2.9 ± 0.7; 95% CI: -4.2 to -1.5; P = 0.001, respectively), finger tapping test- right (35.6 ± 0.9 vs. 44.7 ± 0.9; mean difference: -9.2 ± 1.3; 95% CI: -11.7 to -6.6; P = 0.001), finger-tapping test- left (32.2 ± 0.9 vs. 42.6 ± 0.9; mean difference: -10.4 ± 1.2; 95% CI: -12.8 to -8.0; P = 0.001), and the famous faces (13.3 ± 0.8 vs. 14.4 ± 0.2; mean difference: -1.1 ± 0.3; 95% CI: -1.6 to -0.6; P = 0.001).

Conclusions: Compared to non-headache controls, migraine patients had significant subjective and objective cognitive dysfunctions during a migraine attack.

Abstract ID: 158: Stimulation-Induced Discharges in Patients with Genetic Generalized Epilepsy: A TMS–EEG Study

Deepak Chadha, Shubham Gupta, Sreeraj V. S., Urvaksh M. Mehta, L. G Vishwanathan, Raghavendra K, Ravindranadh C. M., Sanjib Sinha, Ajay Asranna

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: The combination of transcranial magnetic stimulation (TMS) with electroencephalogram (EEG) has opened new opportunities in epilepsy research, allowing direct investigation and modulation of cortical excitability and induction of epileptiform discharges (EDs) to enhance diagnostic and prognostic capabilities

Methodology: This prospective study analyzed 31 patients of genetic generalized epilepsy (GGE) and 31 matched healthy controls. EEG signals were recorded using a 32-channel TMS-compatible EEG amplifier. Magnetic stimuli were delivered using a figure-of-eight coil connected to a Magstim 200 stimulator. The motor cortex and the dorsolateral prefrontal cortex were localized through neuronavigational techniques. Optimal stimulus intensity was calculated based on resting motor threshold and paired-pulse TMS protocol. Each target received 300 stimuli of long-interval cortical inhibition (LICI), short-interval cortical inhibition (SICI), and motor threshold with a 5-s intertrial interval. Data was analyzed in MATLAB.

Results: In our study, discharges were evident in 51% (16 patients) on single-pulse stimulation, 41% (13 patients) by paired-pulse stimulation (SICI), and 39% (12 patients) with paired-pulse stimulation (LICI) over the motor cortex. In the dorsolateral prefrontal cortex, 3% (one patient) showed discharges through single-pulse stimulation, 23% (seven patients) by paired-pulse stimulation (SICI), and 16% (five patients) by paired-pulse stimulation (LICI).

Conclusions: TMS-induced discharges have important implications in GGE, and further evaluation is needed to establish the role of TMS–EEG in understanding epileptogenesis and improving diagnosis and prognosis in GGE and epilepsy.

Abstract ID: 159: Clinical, Radiologic, and Genetic Revelations: A Case Series on Presenilin Mutations

Deepak Chadha, Keerthana B. S., Gautham A, Faheem Arshad, Suvarna Alladi, Subasree R

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Genetic factors, including mutations and environmental influences, are crucial in dementia. Early-onset Alzheimer’s involves presenilins, while frontotemporal dementia (FTD) is associated with genes linked to non-amyloid proteinopathies. Key genes can cause diverse phenotypes, suggesting pathophysiologic heterogeneity. This case series investigates how presenilins contribute to various non-AD (non- Alzheimer’s disease) phenotypes, a rare phenomenon in the Indian context.

Methodology: The clinical, radiologic, and genotypic data of six patients presenting at the Department of Neurology at National Institute of Mental Health and Neuro-Sciences were analyzed, which revealed presenilin mutations.

Results: The mean age of patients was 53 years, and there were three males and three females. Two patients had the behavioral variant of FTD with a positive family history and two had the non-fluent variant with parkinsonism features. One case exhibited overlapping features of FTD with myoclonic jerks and the posterior cortical variant of AD, while another presented solely with the posterior cortical variant of AD. Imaging revealed frontotemporal atrophy in two cases, frontoparietal and frontoinsular atrophy in each case, parietotemporal atrophy in one case, and diffuse atrophy in another case. Genetic analysis by whole exome showed mutations in presenilin-1 in three patients and in presenilin-2 in three patients, which was further confirmed by Sanger sequencing in three of the patients.

Conclusions: PSEN has been associated with both AD and non-AD phenotypes, highlighting the need for future research to understand the heterogeneous mechanisms by which PSEN contributes to these diseases.

Abstract ID: 160: Greater Occipital Nerve block of Methylprednisolone and Lignocaine Compared with Placebo Improves Disability, Headache Impact, and Quality of Life When Used as a Transitional Preventive Treatment for Episodic Cluster Headache: Results from the Randomized, Double-Blind, Placebo-Controlled ANODYNE Study

Sanjay Rao Kordcal, Debashish Chowdhury, Debashish Chowdhury, Rahul Nagane, Ashish Kumar Duggal

Kasturba Medical College, Manipal (KMC, Manipal), Karnataka, India

Background and Aim: ANODYNE 1 and II studies showed the effectiveness of greater occipital nerve block (GONB) with methylprednisolone and lignocaine as a transitional preventive treatment for episodic cluster headache compared to placebo. Here, we report the results of the patient-reported outcome (PROM) measures of the ANODYNE study. Our aim was to study the efficacy of GONB in improving PROM.

Methodology: This randomized, double-blind, placebo-controlled, parallel-group trial included episodic cluster headache (ECH) patients, aged 18-65, diagnosed by International Classification of Headache Disorders third version criteria, with one or more attacks per 24 h for 7 days before randomization. ECH patients were randomized to receive active GONB (2 ml methylprednisolone [80 mg] and 2 ml lignocaine [2%]) and placebo (4 ml saline injections). PROM included headache impact, assessed by headache-impact-test-6, headache disability, assessed by headache disability inventory (HDI), and cluster headache quality of life (CHQOL). Efficacy analyses, that is, the changes in PROM scores from baseline to Week 4, were performed in a modified intention-to-treat population using a generalized linear model. The trial was registered with the Clinical Trials Registry of India (CTRI/2021/21/038397).

Results: Forty ECH patients were randomized. Thirty-nine patients (19 in the active group and 20 in the placebo group) were available for efficacy analysis. A significantly greater improvement in PROM in the active group compared to placebo was observed. The mean differences in these parameters between the two groups were -10.2 (95% confidence interval [CI]: -12.7 to -7.7; P < 0.001), -13.3 (95% CI: -9.6 to -7.0; P < 0.001), and -11.1 (95% CI: -16.6 to -5.6; P < 0.001) for HIT-6, HDI, and CHQOL, respectively, favoring active GONB. No serious adverse effects were reported.

Conclusions: ANODYNE study found that GONB injection with methylprednisolone and lignocaine, compared to a placebo, resulted in significant improvements in PROM.

Abstract ID: 161: Clinical Profile and Etiologic Spectrum in Children with Paroxysmal Nonepileptic Events – A Prospective Study

Peesapati Narasimhacharyulu, V. Viswanathan

Apollo Children Hospital, Chennai, Tamil Nadu, India

Background and Aim: Paroxysmal nonepileptic events (PNEEs) are group of events with sudden paroxysmal changes in behaviors or consciousness, which resemble epilepsy but are not accompanied by electrophysiologic changes. We aimed to study the possible etiology and analyze clinical phenomenology of PNEE in children and identify differentiating pointers between PNEE and epilepsy.

Methodology: A prospective observational study was conducted over 10 months. Children with history of paroxysmal events characterized by abrupt changes in awareness, behavior, and movement were included. Children with strong family history of seizures, neurologic deficits, and static encephalopathy were excluded. Data of children was collected in a prefixed proforma, including demographic data, semiology of events, differentiating factors from epilepsy, history of stressors, and response to treatment. Children underwent video electroencephalogram (EEG) as per the protocol and details were noted. All the data were coded in Excel and analyzed statistically.

Results: Total number of subjects was 46, consisting of 23 boys and 23 girls. Their mean age was 9.95 years. Mean time lag from onset to diagnosis was 1.53 years. Most common semiology was mixed type (45%), which was followed by unexplained bizarre activity (19%). Most common differentiating features from epilepsy were abrupt onset and offset (97%), followed by occurrence in presence of caregivers (60%). Suggestibility was present in 41%. School absenteeism was very significant (32.6%, mean- 4.7 months). Significant stressors were present in many children. School stressors like bullying/performance issues were seen in 34% of children, with familial conflicts reported in 23%. Video EEG picked up paroxysmal events in 23.9%. Also, 45.6% used antiseizure medications with the average duration being 1.03 years. Follow-up at 6 months was done, which showed improvement in 81% children.

Conclusions: Clear identification of PNEE helps in avoiding unnecessary treatment with antiseizure medications, thus helping child and family both socioeconomically and psychologically.

Abstract ID: 162: Face Mask-Associated Headache: A New Entity or Exacerbation of Underlying Primary Headache Syndromes?

Manish Bhartiya

Command Hospital, Southern Command, Pune, Maharashtra, India

Background and Aim: N95 masks have become an essential tool in the daily working of health-care workers ever since the coronavirus disease 2019 pandemic. Our aim was to study the prevalence of and characteristics of headache in a group of health-care workers using N95 masks in our hospital.

Methodology: This was a questionnaire-based cross-sectional study conducted at a tertiary care center. The individuals who used complete personal protective equipment were excluded from the study. Other hospital staff workers not exposed to high-risk areas and who did not wear the N95 face mask were taken as the control group. Submission of response was taken as consent. The headache syndromes were classified as per the International Classification of Headache Disorders third version criteria. Chi-square test was used to compare the two groups.

Results: Two hundred and fifty-six health-care workers participated in the study, out of which 141 individuals used N95 masks. Thirty-nine (27.7%) respondents developed a new-onset headache with N95 face mask use. Seventeen (43.5%) had headaches that required the use of abortive analgesics. The patients with use of mask for more than 4 h were more likely to experience a new-onset headache. The headache was described as throbbing and frontotemporal by most of the individuals.

Conclusions: The use of N95 masks may be associated with a new phenotype of headache. It may be associated with prolonged use of N95 masks. Further studies are required to study the risk factors and appropriate treatment options for this type of headache.

Abstract ID: 163: Study of Dietary Habits in Patients with Cognitive Decline : A Comparative Study

Shubham Agrawal

Sawai Man Singh Medical College, JLN Marg Jaipur, Rajasthan, India

Background and Aim: (1) We aimed to study the dietary habits of patients with dementia. (2) In addition, we attempted to compare the dietary habits of patients with cognitive decline to those with normal cognitive function.

Methodology: This was a case–control retrospective study. Demographic information and detailed medical history, including age at onset and duration of illness and medications, were noted. Food and drink consumption before the onset of neurocognitive decline for 1 year was assessed from the history given by patient’s spouse using a validated Food Frequency Questionnaire for North Indians. Daily energy consumption of ultra-processed food was calculated by summing the energy consumption in calories of all the foods in that group. Consumption of UPF was expressed as a percentage of total daily energy consumption. Cognitive function assessment was done using Mini-Mental State Examination(MMSE Hindi), Frontal Assessment Battery (FAB), Montreal Cognitive Assessment (MoCA), and Trail Making Test (TMT).

Results: Cereal intake showed a U-shaped relation with early-onset dementia, with the risk increasing for an intake above 350 g/day. A high intake (>400 g/day) of dairy products was also associated with excess risk. A substantial proportion of consumption of ultra-processed food was found higher in the dementia group.

Conclusions: This case–control study provides insights on the role of dietary factors in EOD risk. In particular, our findings suggest a detrimental effect on EOD risk due to a high intake of cereals, dairy products, and some types of sweets. Conversely, the intake of some types of fish, vegetables, dry fruits, and chocolate alongside moderate coffee consumption appears to be beneficial.

Abstract ID: 164: Spectrum of Leukoencephalopathies

Divya Bayatapalle, Balaji Gopalan, Hariharan S

Chengalpattu Medical College, Tamil Nadu, India

Background and Aim: Leukoencephalopathies are disorders that exclusively or predominantly affect the white matter of the brain. In adults, cerebral white matter lesions are a common magnetic resonance imaging (MRI) finding in the clinical routine. They can be either nonspecific and age related or indicative of severe neurologic disease. The etiology of white matter lesions is very heterogeneous and includes congenital, vascular, inflammatory, neoplastic, neurodegenerative, metabolic, toxic, and traumatic origins. Here, we are going to present various white matter diseases we encountered at our hospital from April 2023 to April 2024. We aimed to study the clinical profile and establish diagnosis of white matter diseases of the brain.

Methodology: This was an observational study. Patients who presented to the outpatient department and those admitted at the emergency, medical, and neurology wards, whose MRI brain showed predominantly white matter changes were included in the study and those with age-related vascular causes and traumatic causes were excluded. After completing history, clinical examination, and neuroimaging, patients were evaluated accordingly for etiology.

Results: Thirty-six cases who were above 18 years of age were included in the study. The study population consisted of 19 females and 17 males. Among the 36 cases studied, there were four leukodystrophy cases (cerebrotendinous xanthomatosis, adrenoleukodystrophy, Sjogren Larsson syndrome, L-2-hydroxyglutaric aciduria),four are infectious (2Dengue, Herpes Simplex encephalitis, Scrub typhus), six are Inflammatory (3 Multiple Sclerosis, 1 Neuro Myelitis Optica, 1 Acute Disseminated Encephalomyelitis, 1 Steroid Responsive Encephalopathy with associated Thyroiditis), four are Metabol (4 Central Pontine Myelinosis), six are Vascular (2CADASIL, 2 CARASIL, 2VASCULITIS) and Unknown cause are of 12.

Conclusions: Though the list of white matter diseases is unending, it is necessary to have an exhaustive list of investigations, so that treatable causes can be recognized and permanent disabilities can be avoided.

Abstract ID: 165: Spectrum of Atypical Presentations of CNS Tuberculosis, Clinical Profile, Diagnosis, and Outcomes – A Case Study of 21 Patients

Naren Polavarapu, Jawahar Marimuthu, Nanda Kumar Vasudevan, Balasubramanian Samivel, Velusamy Subramanian, Rajagambeeran V

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Central nervous system (CNS) tuberculosis (Tb) is severe form of extrapulmonary Tb that can lead to significant morbidity and mortality if not promptly diagnosed and treated. This study highlights the spectrum of atypical CNS-Tb manifestations encountered in a single tertiary care center.

Methodology: A prospective study was conducted between May 2023 and May 2024 including 21 patients. We collected data on patient demographics, clinical presentation, diagnostic methods, radiologic findings, treatment regimens, and clinical outcomes.

Results: Our study included seven males and 14 females, all immunocompetent, with a mean age of 32.4 years (range: 15–75 years). The most common presenting symptom was focal neurologic deficits (85.7%); majority had paraparesis (52.4%), followed by vision loss (19%) and cranial nerve deficits (14.3%). Other symptoms included headache (57.1%), fever (42.9%), new-onset seizures (28.6%), and altered sensorium (9.5%). Nearly half of the patients had isolated neurologic Tb involvement (47.6%); the remaining patients had disseminated Tb (52.4%), among which majority had pulmonary Tb. Magnetic resonance imaging (MRI) of the brain and spinal cord revealed spinal arachnoiditis in eight (38%), optochiasmatic arachnoiditis in three (14.3%), Tb meningitis in six (28.6%), multiple CNS tuberculomas in nine (42.9%), myelitis (longitudinal extensive transverse myelitis) in five (23.8%), vascular complications in four (19%), secondary vasculitis with multiple infarcts in three, cerebral venous thrombosis (CVT) in one, and solitary tuberculomas in two (intramedullary at D12 vertebral level and dorsal midbrain, respectively). MRI was abnormal in all cases (100%). Cerebrospinal fluid (CSF) analysis showed significantly elevated protein and lactate dehydrogenase and decreased glucose in 15 cases (71.4%). CSF cartridge based nucleic acid amplification test (CB-NAAT) was positive in three cases (14.3%), and sputum/ bronchoalveolar lavage (BAL) CB-NAAT was positive in three cases (14.3%). All patients received first- or second-line antitubercular therapy, with adjunctive corticosteroids, of which 18 (85.7%) showed significant improvement and three patients continued to deteriorate, of which two died (9.5%).

Conclusions: This case study highlights various atypical clinical and radiologic manifestations of CNS-Tb and underscores the importance of having high index of suspicion for CNS-Tb in differential diagnosis of patients presenting with unexplained neurologic syndromes in endemic areas like India.

Abstract ID: 166: Nonmotor Features in Cervical Dystonia – A Northern Indian Perspective

Gurdeep Rajan

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi), India

Background and Aim: Nonmotor features in cervical dystonia (CD) are essentially linked to its pathophysiology; it is an area of immense unexplored possibilities. Previous studies have reported a mixed account. The current study aims to assess the sleep quality, daytime sleepiness, anxiety, and depression and ascertain their relationship with dystonia severity in CD.

Methodology: A cross-sectional study was conducted at the Neurology Department, Govind Ballabh Pant Institute of Postgraduate Medical Education (G.I.P.M.E.R), Delhi. CD patients were assessed for the severity of the dystonia using Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS),, for depression, anxiety, sleep quality, using modified Tsui scale, and daytime sleepiness using Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Pittsburgh Sleep Quality Index (PSQI) and Epworth Sleepiness Scale (ESS), respectively.

Results: Thirty patients were recruited with a mean age of 50.4 ± 11.2 years, and the M:F ratio was 4:1. The mean TWSTRS and Tsui score were (33.0 ± 13.2) and (10.6 ± 4.8), respectively. Thirty percent had severe dystonia (Tsui score >12.5). The mean scores for PSQI, ESS, BDI, and BAI were (6.1 ± 3.6), (2.4 ± 2.9), (8.4 ± 4.8), and (1.1 ± 1.8), respectively. Only five patients had depression, whereas all had mild anxiety. No correlation was found between the sleep and mood disturbances with dystonia severity.

Conclusions: Nonmotor features are an integral part of symptomatology, management of which forms a holistic plan for the dystonia therapy. Presence of mild anxiety in all patients is a silent bystander and roots intricately with the pathophysiology remaining as an enigma, warranting further research.

Abstract ID: 167: Renal Manifestations in CASPR2 Antibody-Associated Diseases: A Retrospective Case Series

Rajender Kandikonda

Amrita School of Medicine, Kochi, Kerala, India

Background and Aim: Contactin-associated protein 2 (CASPR2) antibody-associated diseases are characterized by central and peripheral nervous system manifestations. Membranous nephropathy with proteinuria is reported with voltage-gated potassium channel complex antibodies, but no data is available on CASPR2 antibodies. We aimed to study the renal manifestations in CASPR2 antibody-associated neurologic diseases.

Methodology: A retrospective study was conducted in a tertiary care teaching hospital in South India from 2013 to 2023. Patients aged 18 years or above were included in the study if serum or cerebrospinal fluid tested positive for CASPR2 antibodies in cell-based assay. Those with additional antibody positivity were excluded.

Results: There were 22 patients (15 males, mean age 57.59 + 14.68 years) with CASPR2 antibody positivity. Eight presented with central nervous system involvement (encephalopathy, behavioral abnormalities, seizures, sleep disturbances, and dysautonomia) and 14 with peripheral nervous system symptoms (paresthesias, muscle twitching, and cramps). Eight (36.36%) had renal manifestations as new-onset proteinuria – heavy proteinuria- 3, moderate proteinuria- 3, mild proteinuria- 2, after excluding all other causes. Three patients underwent renal biopsy, which showed membranous nephropathy. Proteinuria improved with immunotherapy in seven patients. Six patients received rituximab therapy preceded by intravenous methylprednisolone (IVMP), and two of them received additional doses of intravenous immunoglobulin also. One received IVMP, oral steroids with injection cyclophosphamide, and later azathioprine; the other patient did not opt for treatment.

Conclusions: Proteinuria is the common renal manifestation CASPR2 antibody-associated neurologic disease. Improvement with immunotherapy indicates a possible autoimmune pathogenesis involving glomeruli. Further studies are required to delineate the exact mechanism underlying this association.

Abstract ID: 168: Clinical study on Sleep and Epilepsy in a Tertiary Care Center: Study on the Bidirectional Relationship Between Sleep and Epilepsy: How Nighttime Sleep Can Trigger Epileptic Seizures and How Antiepileptic Medications Affect Sleep Quality and Seizure Control

Jasmine P

Tirunelveli Medical College, North High ground Road, Tirunelveli, Tamil Nadu, India

Background and Aim: The connection between sleep and epilepsy was first identified by ancient Greeks. Aristotle observed that both sleep and seizures involve altered states of consciousness and a separation of the soul from the body. This study investigates how nighttime sleep can trigger epileptic seizures, how nocturnal seizures disrupt sleep patterns, and how antiepileptic medications impact the sleep quality and seizure control. Recent advances in neurophysiology, neuropsychology, sleep medicine, and genetics offer promising opportunities to enhance our understanding of the relationship between sleep and epilepsy, leading to direct clinical benefits. This study aims to (1) examine the effect of sleep on epilepsy, (2) investigate how epilepsy affects sleep, (3) explore the impact of comorbid sleep disorders as a differential diagnosis of epilepsy, (4) assess the effect of antiepileptic medications on sleep and seizure control, and (5) evaluate whether the dose or timing of antiepileptic drugs can improve therapeutic outcomes.

Methodology: Two hundred epileptic patients attending the neurology outpatient department participated in this study. Comprehensive histories, clinical examinations, routine electroencephalograms, and magnetic resonance imaging of the brain epilepsy protocols were conducted. Detailed records of sleep disturbances during non-rapid eye movement (NREM) and rapid eye movement (REM) phases, as well as seizure occurrences during these periods, were maintained. The National Institute of Mental Health and Neuro-sciences sleep disorder questionnaires, Children’s Sleep Habits Questionnaire (CSHQ), Epworth Sleepiness Scale, and Pittsburgh Sleep Quality Index were used to evaluate sleep disturbances.

Results: Postictal somnolence was observed in 8% of patients. Seizures disrupted sleep continuity in 43% of patients by increasing wakefulness after sleep onset and causing sleep fragmentation. Sleep was disturbed in 33% of patients during the immediate postictal and interictal periods. Increased sleep latency and frequent nocturnal awakenings (insomnia) were present in 52% of patients with epilepsy. Excessive daytime sleepiness was found in 44% of patients. Comorbid sleep disorders, such as obstructive sleep apnea (OSAS), were found in 13% of patients, exacerbating epilepsy symptoms and creating a vicious cycle.

Conclusions: In conclusion, this study highlights the critical interplay between sleep and epilepsy. Epilepsy significantly disrupts sleep, and addressing these sleep disturbances is vital for achieving optimal seizure control. By integrating sleep management into the treatment of epilepsy, clinicians can improve both sleep quality and seizure outcomes, ultimately enhancing the quality of life for patients with epilepsy. Future research should continue to explore the mechanisms underlying the sleep–epilepsy relationship and develop targeted interventions to address these challenges.

Abstract ID: 169: Interictal Calcitonin Gene-Related Peptide Levels in Migraine, Tension-Type Headache, and Non-headache Controls: A Comparative Study of a Large Cohort of 160 Subjects

Hari Yadav, Debashish Chowdhury, Bhawana Mahajan, Sanjay Rao Korcdal, Rahul Nagane, Ashish Duggal

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi), India

Background and Aim: Whether the serum calcitonin gene-related peptide (CGRP) levels from peripheral blood during the interictal headache-free states can be used as a biomarker for migraine is still uncertain. We aimed to estimate the CGRP levels from peripheral blood during the interictal headache-free states in patients with episodic migraine without aura (MwA), episodic migraine with aura (MA), chronic migraine (CM), chronic tension-type headache (CTTH), and non-headache controls.

Methodology: Serum CGRP levels from peripheral blood from the antecubital vein were measured in all subjects using enzyme-linked immunosorbent assay. For MwA and MA patients, CGRP was measured during a headache-free state for at least 72 h. For CM and CTTH patients, CGRP was measured during either a headache-free state or with mild headache (visual analog score of <2) for at least 72 h. Use of any acute medications was not allowed for 24 h before sampling.

Results: A total of 160 subjects across five groups (32 patients each for MwA, MA, CM CTTH, and healthy controls) were studied. Compared to the mean CGRP level of 18.5 ± 8.9 pg/ml in non-headache controls, CGRP was significantly elevated in patients with MwA 31.9 ± 27.3 ( P = 0.012), MA 30.6 ± 24.0 ( P = 0.011), and CM 35.3 ± 15.5 ( P ≤ 0.001). However, there was no difference in CGRP levels between the non-headache controls and CTTH patients (18.5 ± 8.9 vs. 22.5 ± 11.4; P = 0.124). There was no significant difference in CGRP levels between MwA and MA patients ( P = 0.838) and MwA and CM patients ( P = 0.551).

Conclusions: Migraine patients had elevated peripheral venous CGRP levels interictally compared to non-headache controls. The subtypes of migraine could not be differentiated from each other based on CGRP levels. CTTH patients did not show elevated CGRP.

Abstract ID: 170: Case Series of Immune Reconstitution Therapy in RRMS Patients

Nemalikonda Spoorthi

KIMS Hospitals, Minister Rd, Krishna Nagar Colony, Begumpet, Secunderabad, Telangana, India

Background and Aim: In view of less studies on the efficacy of alemtuzumab in relapsing-remitting multiple sclerosis (RRMS) patients, we wanted to analyze the long-term remission rates and disease disability reduction in RRMS patients. We aimed to assess the remission with two doses of alemtuzumab in RRMS patients. This is a retrospective observational study conducted in RRMS patients from a tertiary care hospital with at least 5 years of follow-up.

Methodology: A total of four female patients of RRMS were studied with a mean age of 18 years. Initial diagnosis was made after age 20 for three patients and for one patient at 14 years of age. All four were advised to be on oral acyclovir as prophylaxis for 12 months from the infusion date.

Results: Before alemtuzumab treatment: Among the four, one had four relapses in 2 years, one had seven relapses in 12 years, another had seven relapses in 10 years, and the last one had multiple relapses in 10 years. Two patients were on three disease-modifying therapies (DMTs) before initiation of the treatment, one was on two DMTs, and only one was on a single DMT. After two cycles of alemtuzumab, two out of four patients had neither clinical nor radiologic relapse. One had radiologic relapse and the last one with the most aggressive form had one clinical relapse with a corresponding enhancing lesion after two cycles.

Conclusions: This study has shown alemtuzumab offering promising results when used as immune reconstitution therapy in RRMS patients, but needs long-term follow-up to know the efficacy and its tolerability.

Abstract ID: 171: A Cross-Sectional Study to Assess the Correlation of Waist–Calf Ratio with Carotid Atherosclerosis in Patients Presenting with Ischemic Stroke

Homesh Bhole, Sujata Jayant Khatal, Shankar Prasad Gorthi

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim: Carotid atherosclerosis is a well-known risk factor for ischemic stroke. However, screening of all patients by using computed tomography (CT)/magnetic resonance (MR) angiography is cumbersome and costly; hence, search for cost-effective methods is mandatory. Calf circumference is a marker of peripheral lean mass. We aimed to estimate the association between waist–to–calf ratio and carotid atherosclerosis in patients presenting with ischemic stroke.

Methodology: This was a prospective, cross-sectional, observational study from a single tertiary care center in western Maharashtra. Fifty-eight patients of ischemic stroke were included. A detailed history was taken, a clinical examination was conducted, and biochemical indices were measured. Anthropometric measurements were calculated as per standard and defined methods in the form of waist circumference, calf circumference, and waist–calf ratio. Carotid atherosclerosis was diagnosed on the basis of CT angiography or MR angiography of the brain and neck.

Results: The study includes 58 patients. Mean age of presentation was 57.19 ± 14.95 years and male to female ratio was 2.22. Vascular risk factors noted were: 21 (36.20%) patients were diabetic, 19 (32.75%) were hypertensive, eight (13.79%) had history of ischemic heart disease, six (10.34%) were smokers, 25 (43.10%) patients were tobacco chewers, and 12 (20.68%) patients had a history of alcohol consumption. There were 38 patients who had carotid atherosclerosis (waist–calf ratio was >2.5 in eight patients). We found an association between waist–calf ratio and carotid atherosclerosis with chi-square 3.62, degree of freedom 1, and P -value of 0.05, which was statistically significant.

Conclusions: Waist–calf ratio is an important anthropometric marker of carotid atherosclerosis and can be used in screening of high-risk patients. Studies on larger groups are suggested.

Abstract ID: 172: Study of Clinical and Genetic Profile of CMT

Ankur Vivek, Vijaya Nath Mishra, Abhishek Pathak

Institute of Medical Sciences, Varanasi (IMS, Varanasi), Uttar Pradesh, India

Background and Aim: Inherited neurologic diseases represent a complex spectrum of disorders that significantly impact individuals’ quality of life, posing challenges both to patients and health-care providers. Among these conditions, charcot-marie-tooth (CMT) stands out as a focal point due to its intricate interplay between genetic predispositions and neurologic manifestations.

Methodology: This study was a cross-sectional, observational study. It was conducted on all consecutive patients with suspected genetically determined CMT presenting to the neurology outpatient/inpatient department services of SS Hospital (Sir Sunderlal Hospital Benaras Hindu University.

Results: A total of 33 patients of CMT were studied. Mean age of patients was 27.42 years and the M:F ratio was 20:13. Mean age of onset of disease was 17.96 years. Majority of them were wheelchair bound (19). Most of the patients belonged to lower middle socioeconomic status (15), with a majority of them living outside the Gangatic zone. CMT 2Z was the most common type found in our study.

Conclusions: Our findings confirm that a large number of patients with a representative variety of CMT subtypes have been enrolled, and that the frequency of achieving a molecular diagnosis and distribution of the CMT subtypes reflect those previously reported. This study confirms that it is possible to assess patients in a uniform way between international centers, which is critical for the planned natural history study and future clinical trials. These data will provide a representative baseline for longitudinal studies in CMT.

Abstract ID: 173: Predictors of Hyponatremia in Guillain–Barré Syndrome and its Influence on Outcome

Vishal Jha, Jayantee Kalita, Roopali Mahajan, Gutti Nagendra Babu

Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow (SGPGI Lucknow), Uttar Pradesh, India

Background and Aim: Hyponatremia has been reported in Guillain–Barré syndrome (GBS), but its predictors and role in functional outcome have not been reported comprehensively. We report the occurrence of hyponatremia in GBS and its association with severity and neurophysiologic subtypes. We also evaluated its influence on disability and death.

Methodology: Five hundred and twenty-nine patients with GBS were evaluated retrospectively, and the data since 1998 was retrieved from our prospective GBS registry. The clinical and neurophysiologic subtypes were noted; clinical severity was graded on a 0–6 scale (Hughes). Serum sodium at admission was noted, and presence of hyponatremia was categorized as mild (130–134.9 mg/dl), moderate (125–129.9 mg/dl), and severe (<125 mg/dl). The predictors of hyponatremia were evaluated by univariate followed by multivariate analysis. The clinical outcome of patients with and without hyponatremia was also analyzed.

Results: We found that 193/529 (36.5%) patients had hyponatremia at admission: 132 (25%) had mild, 49 (9.3%) had moderate, and 12 (2.3%) had severe hyponatremia. The predictors of hyponatremia on univariate analysis included disease severity on admission ( P < 0.001), facial palsy ( P < 0.001), bulbar palsy ( P < 0.001), autonomic involvement ( P < 0.001), and respiratory involvement and assisted ventilation ( P < 0.001). Occurrence of hyponatremia was not associated with age ( P = 0.43), gender ( P = 0.02), clinical subtype ( P = 0.22), electrophysiologic subtype of GBS ( P = 0.26) or intravenous immunoglobulin therapy ( P = 0.58). Hyponatremia was associated with days of hospitalization ( P < 0.001), 1-month outcome ( P < 0.001), and 3-month outcome ( P < 0.001). On multivariate analysis, the independent predictors of hyponatremia were facial palsy (adjusted odds ratio [AOR] = 1.82, 95% confidence interval [CI] 1.16–2.83; P = 0.009) and autonomic dysfunction (AOR = 1.08, 95% CI 1.08-2.68; P = 0.02). Three (0.56%) patients died and two of them had hyponatremia. Hyponatremia was associated with poor outcome at 3 months ( P = 0.03).

Conclusions: 36.5% patients with GBS may have hyponatremia, especially those with facial palsy and autonomic dysfunction. Occurrence of hyponatremia may suggest poor outcome at 3 months.

Abstract ID: 174: A Prospective Study on Correlation of Radiologic and Electrophysiologic Characteristics with Progression to Proximal Limb Involvement in Hirayama Disease

Somya Singhal, Sweety Medical Trivedi, Sanjeev Jha

Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow (SGPGI Lucknow), Uttar Pradesh, India

Background and Aim: This study was conducted with the aim of correlating electrophysiologic and neuroimaging findings with clinical progression to proximal limb involvement in patients with distal-predominant Hirayama disease (HD).

Methodology: This is a prospective, hospital-based, observational, follow-up study conducted in 23 consecutive patients of Neurology Outpatient Section of Sanjay Gandhi Post Graduate Institute of Medical Sciences from September 2022 to April 2024. The data was collected with the help of a preformed questionnaire. Nerve conduction study, electromyography (EMG), and dynamic cervical spine magnetic resonance imaging study were performed in all patients to confirm the diagnosis. The follow-up study at 3, 6, 9, and 12 months was done with a repeat clinical examination and electrophysiology.

Results: Clinical: In the study cohort, only eight (35%) patients presented with bilateral symptoms; however, on detailed examination, 15 (65%) patients had bilateral involvement. Radiologic characteristics: Cord atrophy was present in 20 (86%) patients. All patients in the cohort had epidural thickness. Epidural thickness involving both above and below C5 vertebrae level was found in 18 (78%) patients, whereas five (21%) patients had epidural thickness below C5 vertebrae only. Electrophysiology: On EMG, in five patients with epidural thickness below C5 level, neurogenic changes were restricted to C7–T1 myotomes. Of 18 patients with epidural thickness above C5 level as well, 11 (61%) had neurogenic changes restricted to C7–T1 myotomes, whereas in seven (39%) patients, neurogenic changes were present in C5 and C6 myotomes as well. However, at 12 months follow-up, none of them showed progression in EMG or proximal muscle weakness.

Conclusions: Patients of HD with epidural thickness above C5 vertebrae level may have neurogenic changes on EMG in C5 and C6 myotomes, but do not have clinical prognostic implication about progression of disease to proximal myotomes.

Abstract ID: 175: Study of Incidence of Intracranial Hemorrhage Post-Thrombolysis in Acute Ischemic Stroke

Kanika Mathur, Kanika Mathur, Deepak Bhosle, Shankar Prasad Gorthi, Dulari Gupta

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim: Thrombolysis with intravenous recombinant tissue plasminogen activator is the most widely used and approved form of reperfusion therapy in acute ischemic stroke (AIS), with post-thrombolysis hemorrhagic transformation being the most feared complication. We aimed to study the incidence of intracranial hemorrhage (ICH) following thrombolysis and the predictability of SEDAN (consisting of five criteria – random blood sugar levels, early ischemic sign on computed tomography [CT] brain, hyperdense artery sign on CT brain, age, and NHISS score) score of ICH.

Methodology: A prospective, observational, cross-sectional study was conducted at a single medical college in western Maharashtra. National Institutes of Health Stroke Scale (NIHSS) was assessed, and patients fitting the criteria for undergoing thrombolytic therapy with intravenous recombinant tissue plasminogen activator were selected. SEDAN score was assessed to anticipate the risk of ICH post-thrombolysis. A CT of the brain plain scan was repeated 24 h post-thrombolysis or earlier (if there was any clinical deterioration causing an increase in NIHSS score >4 points) to look for ICH.

Results: A total of 62 patients were included in study. Out of these, 42 (67.7%) were males and 20 (32.3%) were females. The patients’ mean age was 55.9 ± 13.64 years. Mean NIHSS at presentation was 11.56 ± 6.06, and mean SEDAN score was 2.13 ± 1.41. Out of 62 patients, seven (11.29%) patients developed intracerebral haemorrhage (ICH). The SEDAN score was found to correlate with post-thrombolysis ICH (chi-square- 13.82, degree of freedom- 5, P value- 0.02)

Conclusions: The previous study conducted to validate the predictability of SEDAN score for ICH post-thrombolysis in AIS gave significant results (area under the receiver operating characteristic curve 0.76, 95% confidence interval, P < 0.001). Our study proves the same. Therefore, SEDAN score is a valid predictive tool for ICH post-thrombolysis in AIS.

Abstract ID: 176: Validating the Prognostic CVTGS score in Cerebral Venous Sinus Thrombosis Patients at a University Medical College in Western Maharashtra

Chaitra Kannadka, Sukanya Dasgupta, Shankar Prasad Gorthi, Dulari Gupta

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim: Cerebral venous sinus thrombosis (CVST) is a rare but serious neurologic condition characterized by thrombosis in the cerebral venous sinuses. This study aims to validate the prognostic cerebral venous thrombosis grading scale (CVT-GS) score in CVST patients, assessing its predictive value against the modified Rankin Scale (mRS) at 30 days post-admission.

Methodology: This prospective single-center study was conducted at a university in western Maharashtra. All patients of CVST were studied, and detailed clinical history, risk factors, neurologic examination, laboratory and radiologic investigations, treatment, and outcomes were recorded. CVTGS score was assigned at presentation, which was categorized into mild (0–2), moderate (3–7), and severe (8–13), and patients were assessed using mRS on day 30.

Results: The study included 74 patients. Mean age of patients at presentation was 35.54 ± 12.47, and the male to female ratio was 1.55. Risk factors included smoking (31%), alcohol use (34%), and use of oral contraceptive pill (55%). Most common symptom on presentation was headache (59%), with 32% patients presenting with seizures. Transverse sinus (59%) was the most commonly involved sinus, followed by sigmoid sinus (49%) and superior sagittal sinus (37%). Thirteen percent of patients had poor functional outcome as measured by mRS >2. The Spearman correlation coefficient between CVT-GS scores and 30-day mRS scores was calculated, which revealed a significant positive correlation ( r = 0.69, P < 0.01). Higher CVT-GS scores correlated with worse outcomes on the mRS scale.

Conclusions: The CVT-GS scale is a valuable prognostic tool for predicting 30-day outcomes in CVST patients. This study validates its use, providing a reliable method for early prognosis. Further studies with larger sample sizes are recommended to confirm these findings.

Abstract ID: 177: To Study the Clinical Profile and Long-Term Follow up of Myasthenia Gravis Patients on Combination Therapy with Oral Azathioprine and Prednisolone at a Tertiary Care Hospital

Sachin Adhav, Rahul Chakor, Swaleha Nadaf

Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai (TNMC, Mumbai), Maharashtra, India

Background and Aim: Myasthenia gravis (MG) is the most common autoimmune disorder that affects the neuromuscular junction. MG is a largely treatable disease, but can result in significant morbidity and mortality. This can usually be prevented with timely diagnosis and appropriate treatment. Herein, we present the clinical profile and long-term follow-up of MG patients who presented to our hospital and were on combination therapy with oral azathioprine and prednisolone.

Methodology: **** hospital is a tertiary care hospital in Mumbai. In the last 5 years, a total of 61 MG patients were followed up in immunology outpatient department. These 61 patients with acquired MG confirming the inclusion criteria were included in study. Variables studied were age, sex, MG subgroup, Myasthenia Gravis Foundation of America (MGFA) class at onset, peak, and last follow-up, and treatment details with follow-up of patients on combination therapy with oral azathioprine and prednisolone.

Results: Analysis included 61 (31 males and 30 females) patients. Mean age at onset was 39.6 years. Fifty-four patients were on combine regimen with azathioprine and prednisolone and were followed up for 5 years. Fifteen (27%) patients were in successful pharmacologic remission with prednisolone 0.05 mg/kg/day and azathioprine 1 mg/kg/day. Five (9.25%) patients were in complete stable remission after stopping azathioprine and prednisolone. Azathioprine was successfully stopped in 11 (20%) patients after a mean follow-up interval of 3.8 years and was tapered to 1 mg/kg in 18 (33.33%) patients after a mean follow-up period of 3.5 years.

Conclusions: MG affects all age groups. Azathioprine and prednisolone combination therapy is effective in controlling the disease without significant side effects with complete stable remission.

Abstract ID: 178: Association of Human Leukocyte Antigen (HLA) Class II Alleles in Patients with Ethambutol-Induced Optic Neuropathy (EtON)

Prabhjit Kaur, Aastha Takkar, Valliappan Muthu, Kathirvel S, Paramjit Singh, Ramandeep Singh, Biman Saikia, Vivek Lal

Post Graduate Institute of Medical Education and Research, Chandigarh (PGIMER Chandigarh), India

Background and Aim: Ethambutol (EMB)-induced optic neuropathy (EtON) is a well-known and potentially devasting adverse effect, but its exact etiopathogenesis is still unknown. Recent National Tuberculosis Elimination Programme (NTEP) guidelines allow the prolonged (>8 months) use of EMB, increasing the cumulative drug exposure and hence the risk of EtON. It is prudent to identify population at risk for developing EtON for convenient and safe duration and dose adjustments of this important bacteriostatic anti-tuberculosis treatment (ATT). We assessed the role of genetics (mitochondrial and human leukocyte antigen [HLA] Class II profiling) in patients of EtON. Our aim was to evaluate the association of mitochondrial genes and HLA class II alleles in patients with EtON.

Methodology: This was prospective, observational, case–control study carried on 100 patients with EtON and 190 matched controls. Clinical, detailed neuro-ophthalmologic examination, serum and cerebrospinal fluid investigations, and detailed imaging were carried out in all patients. All possible risk factors of EtON were noted for subsequent multivariate analysis. Five milliliters of blood sample was stored in an ethylenediaminetetraacetic acid vial at -20°C for mitochondrial genetic assessment and HLA typing. DNA was isolated using Qiagen Mini kit. Isolated DNA was further processed for HLA typing using polymerase chain reaction - single specific prime (PCR-SSP) method. For mitochondrial mutations, REPLI-g Mitochondrial DNA Kit was used to amplify mitochondrial DNA and polymerase chain reaction - restriction fragment length polymorphism (PCR-RFLP) method was used to assess the mutations.

Results: Median age (range) of the patients was 47 (18–73) years, and median duration of intake of EMB (range) was 6 (2–23) and 6 (6–9) months in cases and controls, respectively. Majority of the patients had pulmonary tuberculosis. All patients were receiving weight-based EMB as per the existent guidelines (15–20 mg/kg/day), and the median dose (range) was 18.83 (10.5–36.53) mg/kg/day. Most patients with EtON had severe vision loss (80%) and complete visual field loss (59%). Thickness of optic nerves and optic chiasma was reduced significantly in the EtON group ( P ≤ 0.0001) compared to the normative data on imaging. All the patients were found to have wild mitochondrial mutations. The allele frequency of HLA-DQB1*05:01 allele was found to be significantly high compared to controls ( P ≤ 0.0001). Similarly, the allele frequency of HLA-DQB1*03:01 ( P = 0.03), HLA-DRB1*03:01 ( P = 0.04), and HLA-DRB1*12:01 ( P = 0.03) was found to be higher in cases compared to controls. The later three were significantly associated with good visual outcome at 6 months follow-up on multivariate analysis (R-1.00).

Conclusions: This is the first study highlighting the association of EtON with HLA Class II alleles. Our findings provide an insight into the role of HLA alleles in the etiopathogenesis of EtON.

Abstract ID: 179: Deciphering Disease Mechanisms – Computational Validation of Structural and Functional Consequences of Novel Genetic Variants in Developmental Epileptic Encephalopathy (DEE)

Ramshekhar N. Menon, Alfiya Fasaludeen, Moinak Banerjee

SCTIMST, Thiruvananthapuram, Kerala, India

Background and Aim: The advent of next generation sequencing (NGS) techniques allowed to identify a large number of novel variants and its use in clinical contexts are still challenging. A comprehensive understanding of the molecular mechanisms of disease, as well as the structure, function, and biological network of the proteins involved in its pathogenesis, is required to device precision therapy. This study aims to assess the functional and structural impact of novel variants using in silico prediction tools.

Methodology: To assess the impact of novel variants on protein function and structure, various computational prediction tools were used. We performed mutant protein stability prediction using iMutant and MuPro, protein interaction prediction using the STRING database, functional pathway analysis using the DAVID database, functional prediction of voltage-gated sodium channel variants using FunNCion, variant pathogenicity prediction using AlphaMissense, and the prediction of variants’ impact on protein structure and function using Molecular Dynamics Simulation (MDS).

Results: The in silico functional analysis of 16 novel pathogenic disease-causing variants in voltage-gated sodium channel revealed that 75% of the variants were loss of function variants and 25% were gain of function variants. MDS of three novel variants (STXBP1:c.A434C:p.Y145S, UFSP2:c.344T>A;p.V115E, and SHQ1:c.850T>C;p.Y284H) associated with developmental epileptic encephalopathy (DEE) revealed the potential impact of deleterious variants on the protein structure and stability. DEE phenotypes included Dravet syndrome in 62.5% (10/16), early infantile epileptic encephalopathy (EIEE) ( STXBP1 ), West syndrome ( UFSP2 ), and neurodevelopmental disorder with dystonia and reflex seizures ( SHQ1 ).

Conclusions: This is the first study which validates computational approaches for assessing the functional impact of novel variants.

Abstract ID: 180: Impact of Subthalamic Deep Brain Stimulation on Survival of Indian Patients with Parkinson’s Disease

Athul Sanjeev, Sayoojya Sachidanandan, Syam Krishnan, Krishnakumar Kesavapisharady, Asish Vijayaraghavan, Divya KP, Gangadhara Sarma

SCTIMST, Thiruvananthapuram, Kerala, India

Background and Aim: Deep brain stimulation (DBS) is the standard of care for patients with Parkinson’s disease (PD) experiencing motor complications in the mid-stages of the disease. DBS improves patients’ quality of life remarkably; its impact on long-term survival in patients from the developing world is unclear. We aimed to examine the long-term survival of patients with PD, who had undergone DBS of the subthalamic nucleus (STN) bilaterally, and the factors associated with mortality from our surgical cohort over a span of 25 years.

Methodology: The survival status of patients with PD who had undergone STN DBS in our center and with a minimum of 5 years of follow-up after surgery was examined. Families of patients who were not on follow-up were contacted over the telephone.

Results: One hundred and ninety-five patients satisfied the inclusion criteria; among them, nine (4.6) patients were not contactable. Eighty-five patients (43.6%) had died; 53 (62.4%) of the deaths were due to complications of immobility. The mean survival after surgery was 18.8 (±) 2.8 years, and the mean disease duration at death was 10.2 (±) 2.6 years. Postural instability–gait dominant phenotype, more severe motor disability, poorer activities of daily living, and poorer levodopa response at baseline were among the factors associated with mortality.

Conclusions: DBS may contribute to the prolonged survival of PD patients. Survival is influenced by baseline PD phenotype, severity, and levodopa response. Complications of immobility related to the natural progression of PD contribute to mortality.

Abstract ID: 181: Long-Term Efficacy and Safety of Greater Occipital Nerve Block of Methylprednisolone and Lignocaine for Episodic Cluster Headache: Results from a 1-Year Open-Label Treatment Phase of ANODYNE Trial

Akhil Sahib, Debashish Chowdhury, Sanjay Rao Kordcal, Rahul Nagane, Ashish Duggal

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi)

Background and Aim: The long-term efficacy and safety of greater occipital nerve block (GONB) for the preventive treatment of episodic cluster headaches (ECHs) remains unknown. This study aims to find the long-term efficacy and safety of GONB in patients with ECH, following a double-blind treatment phase (DBTP).

Methodology: Open-label treatment phase (OLTP) of ANODYNE trial included patients who opted out of DBTP and those with persistent attacks at the end of DBTP (Week 4). They received second GONB with 2 ml of methylprednisolone (80 mg) and 2 ml of 2% lignocaine. The efficacy and safety during OLTP were assessed at Week 8 and Week 12. The patients were followed up to 52 weeks. Recurrence of cluster period and its treatment were recorded. Descriptive summaries were provided. Data was reported as observed, without imputation for missing data. The trial was registered with the Clinical Trials Registry of India (CTRI/2021/21/038397).

Results: Fifty-two weeks follow-up was available for 39 patients. Five patients in the placebo group and none in the active group required a repeat GONB (27.8% vs. 0%; P = 0.022). Remission time following the second GONB was significantly lesser for the active group (9.0 ± 2.6 days vs. 16.2 ± 7.7 days; P = 0.002). A complete remission was achieved in 77.7% patients at Week 8 and 100% patients at Week 12. Twelve patients had attack recurrence after Week 12. These occurred at a median of 37 weeks from the time of randomization (range: 21–51 weeks). All were treated with a third GONB. Eleven patients became attack free by median 9 days that lasted till Week 52. Repeat GONBs were well tolerated.

Conclusions: The result of this study showed long-term beneficial effects of single/repeated GONB with good tolerability.

Abstract ID: 182: Comparison Study of VKA and NOACS in the treatment of Cerebral Sinus Venous Thrombosis and Their Functional Outcomes

Srinath Vasagiri, Anuja Patil

KIMS Hospital, Hyderabad, Telangana, India

Background and Aim: Comparison of vitamin K antagonists (VKA) and novel oral anti-coagulants (NOACS) in the treatment of cerebral sinus venous thrombosis (CSVT) and their functional outcomes

Methodology: This is an ambispective study including patients admitted with CSVT in a tertiary care hospital from January 2019 to December 2023. Patients aged >14 years, with a confirmed diagnosis of CSVT, based on imaging with or without intracranial hemorrhage were included. Patients with CSVT associated with central nervous system infection or due to head trauma, pregnancy, bleeding diathesis, severe renal impairment, or liver disease were excluded.

Results: The median age of onset was 38.5 years. Males were more commonly involved (61.6%), but female patients tended to be younger ( P -value= 0.031). VKA was used in 13 patients (25%) and NOACS in 39 patients (75%). Good outcomes (modified Rankin Scale [mRS] 0–3) were seen in 92.31% and bad outcomes (mRS 4–6) in 7.69%. Of those aged >45, 17.6% had worse outcome and of those aged <45, only 2.85% had bad outcome, but it was not statistically significant ( P -value= 0.055). Patients with glasgow coma scale (GCS) 10 or less had worse outcomes compared to patients with GCS >10 at admission ( P =0.045). Social outcomes were good in 61.5% in the VKA group and 84.6% in the NOACS group ( P = 0.07).

Conclusions: Functional outcomes were comparable in both the groups – VKA and NOACs. Age <45 years was associated with good outcomes. GCS less than 10 was associated with worse functional outcomes.

Abstract ID: 183: Clinical Profile, Diagnostic Delay, Patient-Reported Outcomes, and Atypical Features in Episodic Cluster Headache Patients: A Prospective Study from a Tertiary Care Headache Clinic

Aastha Bansal, Akhil Sahib Sahib, Sanjay Rao Kordcal, Ashish Duggal Duggal, Debashish Chowdhury Chowdhury

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi), India

Background and Aim: There is limited data on the clinical profile of episodic cluster headache (ECH) in the Indian population. Herein, we report on the clinical profile of ECH patients.

Methodology: Demography and clinical characteristics of headaches were documented prospectively for consecutive ECH patients diagnosed by International Classification of Headache Disorders third version criteria and attending the Headache Clinic from December 2021 to January 2023. Assessment of cranial autonomic symptoms (CASs) and signs and restlessness during the attacks was made based on a questionnaire. Patient-reported outcome measures (PROMs) were also assessed. PROMs included headache impact, assessed by headache impact test (HIT-6), headache disability, assessed by headache disability inventory (HDI), and cluster headache quality of life (CHQOL).

Results: Forty ECH patients (35 males, five females; mean age: 36.9 ± 11.4 years) were studied. The average duration of cluster period (CP) was 35.4 ± 18.3 days. Lacrimation and conjunctival injection were the most common CASs. All patients experienced restlessness during attacks. The mean delay in diagnosis was 5.3 years. ECH was misdiagnosed as side-locked migraine (75%), trigeminal neuralgia (17.5%), and paroxysmal hemicrania (7.5%). Circannual periodicity was present in 29/40 (72.5%) patients. Most CPs occurred in the last quarter of the year (October–December) (11/29 patients). Circadian periodicity was present in 25/40 patients (62.5%); 16 had daytime attacks and the remaining nine had attacks predominantly at night. Fourteen patients (35%) had atypical features. Mild interictal pain (median 4 h [range 1–6 h]) was seen in 20% of patients. Side-switching and facial-onset cluster attacks were seen in two patients each. The mean HIT-6, HDI, and CHQOL scores were 69.1 ± 3.2, 79.4 ± 7.4, and 84.1 ± 8.7, respectively, suggesting significant headache impact, disability, and poor quality of life.

Conclusions: ECH patients received delayed diagnosis and had significant headache-related disability and poor quality of life.

Abstract ID: 184: A Cross-Sectional Study of Epstein–Barr Virus Status in Primary Demyelinating Disorders and its Association with Clinical Features, Fatigue, and Disability

Tarang Panchal, Debashish Chowdhury

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi), India

Background and Aim: Epstein–Barr virus (EBV) status in relapsing remitting multiple sclerosis (RRMS), neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte-associated disease (MOGAD) may differ. We aimed to study the EBV status in primary demyelinating disorders and its association with clinical features, fatigue, and disability.

Methodology: During a relapse-free period (>1 month), qualitative estimation of EBV IgM, IgG, and Epstein–Barr nuclear antigen (EBNA) in serum was done with Vitek immunodiagnostic assay kits using enzyme-linked fluorescent assay and classified into seven groups as per manufacturer’s guidelines. Multiple sclerosis symptom severity was estimated by “SYMPTOMSCREEN,” fatigue by “modified fatigue impact scale,” and disability by Expanded Disability Status Scale (EDSS).

Results: Eighty-one patients (27 RRMS [mean age 30.6 years, median 29, M:F 20:7], 24 NMOSD [mean age 30.2 years, median 28, M:F 20:4], and 30 MOGAD [mean age 32.9 years, median 32, M:F 23:7]) were studied. Overall, 77.7% patients were females. The mean disease duration was 94, 75, and 32 months for RRMS, NMOSD, and MOGAD, respectively. EBV seropositivity (positive IgG and EBNA) was seen in 21 (72.4%) RRMS, 15 (62.5%) NMOSD, and 23 (76.7%) MOGAD ( P = 0.512) patients. One patient each with NMOSD and MOGAD had primary EBV seropositivity (positive IgG and IgM, negative EBNA) and one patient of NMOSD had primary infection (only IgM positive); isolated IgG positivity was seen in one patient (MOGAD) and isolated EBNA positivity in five patients. Two patients of NMOSD were seronegative. EVB seropositivity had no significant association with clinical symptoms, fatigue, and disability.

Conclusions: EBV seropositivity was similar in patients with three common primary demyelinating disorders. Seropositivity had no significant association with clinical symptoms, fatigue, and disability.

Abstract ID: 185: Clinical and Radiologic Profile of Dementia in a North Indian Tertiary Care Hospital and its Associated Family Caregiver Burden: An Observational Study

Sai Teja, Karthik Vinay Mahesh, Sameer Vyas, Harmandeep Singh, Sandeep Grover, Vivek Lal

Post Graduate Institute of Medical Education and Research, Chandigarh (PGIMER Chandigarh), India

Background and Aim: To study the epidemiological, clinical, and investigational profile of dementia in a tertiary care hospital in North India and assess for family caregiver burden.

Methodology: This study was a prospective, observational study that included 91 newly diagnosed patients with major neurocognitive disorders who presented to our center (Post Graduate Institute of Medical Education and Research) from Feb 2022 to June 2023. Detailed history as well as clinical neurologic examination was done in all patients. All hematologic, biochemical, optional investigations if required (whole body/brain positron emission tomography (PET), neuroimaging included magnetic resonance imaging (MRI) brain with T1 + T2 weighted images with SWI/ DWI/ADC & FLAIR sequences, other investigations including, EEG wherever deemed necessary was done to rule out other mimickers of dementia. The severity of dementia was assessed with the dementia rating scale by the primary investigators. Caregiver burden was assessed by family burden interview schedule (Pau and Kapurwas done after obtaining informed consent.

Results: The current study included 91 patients with the mean (standard deviation [SD]) age of presentation being 63.36 (8.73). Males constituted 54 patients (59.3%) and females constituted 37 (40.7%). Among the subtypes of major neurocognitive disorder, the most common was FTD (40 [44%]) followed by AD (22 [24.2%]), VD (10 [11%]), CBS (6 [6.6%]), and Creutzfeldt-Jakob disease (CJD) (4 [4.4%]). The mean (SD) Mini-Mental State Examination (MMSE) was 11.78 (9.47) in our group, and the mean (SD) Montreal Cognitive Assessment (MoCA) score was 9.59 (8.38). Mean MMSE and MoCA were least in CJD with 8.25 (10.90) and 5.25 (8.06), respectively. The family burden score assessed by the Pai and Kapur scale was 6.93 (5.03) with a maximum score of 23. The highest burden was noted in the financial domain and disruption of family routine activities. Caregiver burden was the maximum in CJD with a score of 11 (4.16), followed by frontotemporal dementia (FTD) (7.88 [5.65]) and vascular dementia (VD) (7.20 [3.71]) and was the least in Alzheimer’s disease (AD) (4.68 [4.24]) group. Positive MRI findings including cortical atrophy were seen in 89% of cases, whereas others had normal or only white matter changes. PET was done in 42 patients with a dominant area of atrophy was correlated with the dominant area of hypometabolism on Z scores in 40 patients, Z scores being normal in 2

Conclusions: Caregiver burden was the maximum in major neurocognitive disorder with higher neuropsychiatric symptoms like CJD followed by FTD, vascular dementia, and the least burden was found in AD. Financials and disruption of routine family activities cause a major burden for caregivers compared to the physical and emotional components.

Abstract ID: 186: Case Series of Spontaneous Intracranial Hypertension

Parameswaran K

Indo American Hospital, Brain and Spine Centre, Vaikom, Kerala, India

Background and Aim: We aimed to report 30 consecutive case series of spontaneous intracranial hypotension (SIH) who presented to our hospital between January 2016 and March 2024.

Methodology: We retrospectively analyzed 30 consecutive cases of SIH who presented to our hospital.

Results: We studied 30 cases of SIH. Their age varied between 20 and 69, with a mean age of 35. Male /female is 18/12 with slight female predominance. All had classical postural headache. Eight patients were presented with status epilepticus secondary to cortical venous infarct, one presented with psychosis/cognitive decline as frontotemporal dementia syndrome, and 12 patients presented with bilateral subdural hematoma – 3 needed burr hole evacuation. MRI of the brain showed classical features of SIH in all (bilateral subdural collection 100%, venous distension 100%, brainstem descent 100%, bilateral subdural hematoma in 40%, venous infarct in 40%). MRI whole spine screening with fat-saturated T2 space showed spinal longitudinally extensive cerebrospinal fluid (SLEC) collection in most of the patients (28/30). All were SLEC type 1 A and B. All patients underwent nontargeted medium-volume autologous epidural blood patch. All of them made complete recovery without any recurrence. Follow-up period ranged from 8 years to 3 months.

Conclusions: SIH is a completely treatable entity. Very late presentation with atypical features can mislead diagnosis, so a proper history of postural headache at onset and careful imaging interpretation are essential.

Abstract ID: 187: A Prospective Comparative Study of Headache Impact, Disability, and Quality of Life in Patients with Episodic and Chronic Migraine in a Large Cohort of 300 Patients

Arijit Pal, Manju Yadav, Debashish Chowdhury

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi), India

Background and Aim: Episodic migraine (EM) and chronic migraine (CM) patients have differences in disease profile. The aim of this study is to compare the headache impact, disability, and quality of life in patients with EM and CM.

Methodology: EM and CM patients diagnosed by the International Classification of Headache Disorders third version criteria were recruited. Headache impact, migraine disability, and quality of life were measured by headache impact test (HIT-6), Migraine Disability Assessment (MIDAS), and Migraine-Specific Quality of life (MSQ) questionnaire, respectively. MSQ includes measures for Role Function-Restrictive (MSQ-RR), Role Function-Preventive (MSQ-RP), and Emotional Function (MSQ-EF).

Results: Three hundred (150 EM and 150 CM) patients were studied (EM: 117 females and 23 males; CM: 135 females and 15 males). CM patients were significantly older than EM patients (mean age at presentation 34.1 ± 10.1 years vs. 31.7 ± 9.9 years; P = 0.04). There was a diagnostic delay of 3.4 ± 1.3 years and 5.3 ± 1.2 years for EM and CM patients respectively. Monthly migraine days were 9.3 ± 3 and 19.6 ± 6 for the EM and CM groups, respectively ( P < 0.05). CM patients had significantly greater ( P < 0.05) headache impact (HIT-6 score 67.5 ± 6.2 vs. 62.3 ± 3.7) and disability (MIDAS score 62.6 ± 18.1 vs. 43.1 ± 16.2) and poorer MSQ-RR (32.2 ± 14.6 vs. 42.8 ± 12.8), MSQ-RP (45.1 ± 14.6 vs. 56.6 ± 12.4), and MSQ-EF (48.4 ± 14.6 vs. 67.5 ± 11.5) compared to EM patients. Attack severity did not differ between the two groups.

Conclusions: CM patients received delayed diagnosis and had significantly greater headache impact, higher disability, and poorer quality of life compared to EM patients.

Abstract ID: 188: Efficacy and Tolerability of Combination Treatment of Botulinum Toxin and Greater Occipital Nerve Block in Patients with Resistant Migraine: An Open-Label, Prospective Study

Manju Yadav, Arijit Pal, Sanjay Rao Kordcal, Debashish Chowdhury

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi), India

Background and Aim: The role of combination treatment of botulinum toxin A (BoT-A) and greater occipital nerve block (GONB) for resistant migraine (RM) remains unknown. Our objective was to find the efficacy and tolerability of combination treatment of BoT-A and GONB in patients with RM.

Methodology: RM patients, aged 18–65 years, diagnosed by the European Headache Federation’s consensus criteria, received monthly GONB and three-monthly BoT-A. The primary end point was mean change in monthly headache days (MHD) at 6 months. Key secondary outcomes included mean change in monthly migraine days (MMD) and 50% responder rate. Efficacy analysis was done using one-way analysis of variance for repetitive measures. The level of significance was set at P < 0.05.

Results: Sixteen RM patients (13 F:3 M, mean age 29.6 ± 10.8 years) were studied. The mean duration of RM was 3.3 ± 1.9 years. At baseline, number of failed preventive medications was 3.7 ± 1.1. The mean MHD and MMD at baseline were 23.6 ± 3.9 and 18.5 ± 4.2, respectively. Following the combination treatment, at 6 months, MHD and MMD were significantly reduced compared to baseline (-13.3 ± 1.1 [confidence interval {CI}: -10.4 to -16.3; P < 0.001] and -11.5 ± 0.9 [CI: -9.0 to -13.9; P < 0.001], respectively). Fifty percent responder rate was 56.3%. Adverse effects were mild and transient that included pain and mild bleeding at the injection site. No serious adverse effects were reported.

Conclusions: This open-label, prospective study undertaken for the first time in the world showed good efficacy and tolerability of the combination treatment of BoT-A and GONB in patients with RM over 6 months.

Abstract ID: 189: Clinical Spectrum of MOGAD in a Tertiary Care Hospital: A Case Series Study

Praveen Singh, Shobana N, Sacratis S, Selvakumar CJ, Sadeeshkumar V, Manoj Prakash J

Coimbatore Medical College Coimbatore, Tamil Nadu, India

Background and Aim: Anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibody-associated disease (MOGAD) is an immune-mediated neurologic disorder with a broad spectrum of clinical presentation that is often difficult to distinguish from other demyelinating diseases, such as multiple sclerosis and neuromyelitis optica spectrum disorder. The clinical spectrum of MOGAD is expanding over time. However, the long-term management and prognosis of this disorder are still controversial. This study aimed to report the clinical profiles and treatment outcomes of MOGAD in our center.

Methodology: This is a single-center, case series study. Clinical and paraclinical data, along with treatment outcomes of patients with MOGAD were analyzed.

Results: A total of 20 patients were identified, of which 14 (70%) patients were women, and the median age at disease onset was 30 years (range 20–67). A total of 27 episodes were observed, with optic neuritis (ON; 75%) being the most frequent presentation, and 70% of them were unilateral. Other presentations included three longitudinal extensive transverse myelitis (LETM; 15%), one acute disseminated encephalomyelitis (ADEM)-like presentation (5%), and one patient presenting with the diagnostic criteria of area postrema syndrome (APS).

Conclusions: The clinical characteristics of MOGAD are heterogeneous, in which, ON, LETM, ADEM, and APS may occur. The long-term prognosis is relatively favorable in most patients. However, it is notable that some patients may have highly relapsing courses and accumulating disabilities. Large-size, multicenter studies are warranted to determine the risks of relapse and prognosis.

Abstract ID: 190: Utility of EEG and MRI in Late-Onset Seizures – A Prospective Observational Study

Donti Ramakrishna Anusha

Manipal Academy of Higher Education, Manipal (MAHE, Manipal), Karnataka, India

Background and Aim: Geriatric epilepsy includes pre-elderly (60 years old) and new-onset epilepsy in elderly. Recognizing seizures in this population is challenging because of clinically subtle presentation of seizures in most cases. This study aims to demonstrate the role of electroencephalogram (EEG) and magnetic resonance imaging (MRI) in late-onset seizures and epilepsy.

Methodology: Consecutive patients with late-onset seizures and epilepsy attending the neurology services were enrolled in the study after obtaining their informed consent and applying the inclusion and exclusion criteria. All the patients were stratified into late-onset seizures and late-onset epilepsy groups. EEG and MRI were done in all patients.

Results: One hundred and nine elderly patients presented with seizures, with males constituting 62 of them. Of the 109 patients, 67 were in seizures group and 42 were in epilepsy group. In the seizures group, MRI findings were normal in 61.1% and abnormal in 39.9%. In the epilepsy group, all patients had abnormal MRI. In the seizures group, 68.6% and 31.4% of patients had normal and abnormal EEG, respectively. Of them, generalized slowing (25.3%) was the most common finding, followed by focal slowing (5.9%), focal spikes (2.9%), and generalized spikes (1.4%). In the late-onset epilepsy group, 47.6% and 52.4% of patients had normal and abnormal EEG, respectively. Focal slowing (30.9%) was the most common finding in them, followed by generalized slowing (21.4%), focal spikes (7.1%), focal electrographic seizures (7.1%), generalized spikes (2.3%), and generalized electrographic seizures (2.3%). EEG changes were statistically significant favoring normal EEG in the seizures group ( P = 0.0287) and focal slowing and focal electrographic seizures favoring the epilepsy group ( P = 0.0008).

Conclusions: The yield of abnormal EEG was low in elderly. If abnormal, generalized slowing was the most common abnormality. Patients with abnormal MRI tended to have epilepsy rather than unprovoked seizures.

Abstract ID: 191: An Observational Study of Quality of Life in Patients with Advanced Parkinson’s Disease Pre- and Post-Deep Brain Stimulation

Jayasree Manikinda

KIMS Hospital, Hyderabad (KIMS, Hyderabad), Telangana, India

Background and Aim: The aims of the study were to assess the quality of life of patients with advanced Parkinson’s disease (PD) before and after deep brain stimulation (DBS) and assess the correlation between unified Parkinson’s disease rating scale (UPDRS) motor scores and quality of life in patients with PD pre- and post-DBS.

Methodology: A total of 41 patients with advanced PD (>5 years disease duration or motor fluctuations and dyskinesias) were included in the study. All patients were evaluated with UPDRS part 3 motor scores in OFF and ON state. Quality of life was assessed using Parkinson’s Disease Questionnaire 39 (PDQ-39). The association between PDQ-39 and UPDRS motor scores was evaluated using Pearson correlation coefficients.

Results: The mean total PDQ-39 score was 45.92, whereas the average UPDRS 3 motor score was 70.67. The total PDQ-39 score and almost all the domains of PDQ-39 except for stigma showed a significant ( P <0.05) correlation with UPDRS 3 motor scores. Post-DBS patients constituted 26.8% of the study population. Average UPDRS 3 motor scores and PDQ-39 scores were 79.45 and 72.55 in pre-DBS and 66.8 and 34.2 in post-DBS patients, respectively.

Conclusions: UPDRS motor scores strongly correlated with PDQ-39 total and individual domain scores, suggesting a strong association between poor quality of life and greater impairment in advanced PD patients.

Abstract ID: 192: Effect of Anodal Transcranial Direct Current Stimulation on Naming in Aphasic Patients with Acute Ischemic Stroke – A Randomized, Double-Blind, Sham-Controlled Study

Devavrata Sahu, B. K. Bajaj, Jyoti Garg, Ashish Kumar Duggal

ABVIMS and Dr RML Hospital, New Delhi, India

Background and Aim: Studies on non-invasive brain stimulation techniques such as transcranial magnetic stimulation (TMS) and transcranial direct current stimulation (tDCS) have shown positive results in rehabilitating chronic aphasia after a stroke. However, their effects on acute ischemic stroke patients with aphasia have not been well studied.

Methodology: This was a prospective, double-blinded, randomized trial. Using predefined inclusion and exclusion criteria, 28 patients were enrolled and randomized into two 14-patient groups. One group received 5 days of anodal tDCS to the Broca’s area, whereas the other received sham stimulation. The outcomes were a change in the Accuracy, Reaction Time, and Picture Naming Test (PNT) scores on the Indian Council of Medical Research PNT at 4 weeks after the last session of anodal tDCS.

Results: Both groups had similar baseline characteristics. Naming Accuracy results at 4 weeks after tDCS compared to baseline were statistically significant in the anodal tDCS group (Friedman test, P = 0.002). Reaction time results at 4 weeks after tDCS compared to baseline were statistically significant in the anodal tDCS group (Friedman test, P = 0.001). PNT score results at 4 weeks after tDCS compared to baseline were statistically significant in the anodal tDCS group (Friedman test, P = 0.002). No such results were obtained in the sham stimulation group ( P = 0.992). A mixed analysis of variance comparing the anodal tDCS and sham stimulation groups showed a significant effect of both time and stimulation on both groups, eliminating time as a confounding factor (F (4, 104) = 4.27, P = 0.003 for Accuracy, F (4, 104) = 4.47869, P = 0.004 for Reaction Time, and F (4, 104) = 4.42, P = 0.002 for PNT scores).

Conclusions: Transcranial direct current stimulation is helpful in patients with aphasia after acute stroke, but larger studies are needed to confirm these findings.

Abstract ID: 193: Study of Clinical Profile of Patients Presenting with Subacute and Chronic Ataxia: A Study from a Tertiary Care Hospital in Eastern India

Soumyadarshan Nayak, Rajlaxmi Satpathy, Ashok Kumar Mallick

SCB Medical College, Cuttack, Odisha, India

Background and Aim: Ataxia is a common presentation of various neurologic disorders. It poses a challenge in the diagnosis of the cause, and many patients remain undiagnosed. It is essential to understand the demographic pattern and clinical features for early diagnosis and effective management of the underlying disorder.

Methodology: This prospective, observational study was carried out at SCB Medical College, Cuttack, from October 2021 to September 2023. Detailed demographic, clinical, laboratory, and electrophysiologic data were collected.

Results: The hospital-based incidence of ataxia was 116 (4.9%). Out of 116 patients, 64.6% were males and 35.4% were females. Mean age of the study population was 33.21 years. The mean age of disease onset was 33.1 years. The mean duration of disease was 2.08 years. The most common presenting symptom was motor weakness observed in 84 (72.4%) patients with ataxia. Other presenting symptoms were sensory (31%), vertigo (18.9%), convulsions (13.8%), visual abnormality (11.2%), ocular signs (13.8%), cranial neuropathies (14.6%), autonomic dysfunction (4.3%), and pseudo-dystonia (2.6%).

Conclusions: Ataxia is a common manifestation encountered in various neurologic conditions, which can have acute, subacute, episodic, or chronic presentation. It can be challenging to diagnose and may require investigations such as brain imaging, genetic testing, immunological studies, and blood investigations. The cause of ataxias should be identified early, and the treatable conditions should be treated promptly with active monitoring.

Abstract ID: 194: The Role of PET–CT scan in the Diagnosis and Outcome of Autoimmune Encephalitis

Laxmi Khanna, Ritu Ranjan Verma, Mandaville Gourie Devi

Sir Gangaram Hospital, New Delhi, India

Background and Aim: Autoimmune encephalitis has a subacute presentation with an estimated prevalence of 0.7–12.6 per 100,000 people. However, the diagnosis is hindered by a lack of appropriate investigations. Positron emission tomography–computed tomography (PET–CT) scans can predict the effect of treatment, recurrence, and progression of the disease, with detection of associated neoplasms. Hence, this test is a biomarker of autoimmune encephalitis. The aims of this study were to compare two groups of hospitalized patients: group 1 with PET–CT scans and group 2 with magnetic resonance imaging (MRI) brain scans. The clinical spectrum, hospital course, abnormal antibody assay, abnormal imaging, rehabilitation, outcome, and the quality of life after treatment were studied.

Methodology: Two cohorts of 30 hospitalized patients with and without PET–CT scans were included in a retrospective study that spanned 5 years from 2018 to 2023. PET–CT scans were not done in the second group due to delayed presentation, lack of awareness, or lack of resources for PET–CT.

Results: The clinical features, hospital course, and outcome were severe in those whose diagnosis was delayed due to lack of fluorodeoxyglucose positron emission tomography (FDG PET)–CT scans ( P < 0.001). The sequels were higher in the patient group without PET–CT scans ( P < 0.009). Recovery was better in the PET–CT group ( P < 0.010). In patients who were not investigated by PET–CT, the clinical features were severe with refractory seizures, behavioral and personality changes, impaired memory, and automatisms.

Conclusions: FDG PET–CT scans are cost-effective imaging modalities which can identify different forms of encephalitis based on specific imaging patterns. This test enables early diagnosis, treatment, and prevention of complications of this dreaded condition.

Abstract ID: 195: Update on Muscle Imaging in Myositis

Siphora Bijjiga, Boby Varkey Maramattom

Aster Medcity, Kochi, Kerala, India

Background and Aim: Dermatomyositis [DM] is a common idiopathic inflammatory myopathy with muscle and skin changes. Prevalence of DM in India is 14–22/100,000. Magnetic resonance imaging (MRI) is now increasingly used in the diagnosis and follow-up of clinical disease. Our aim was to study the MRI abnormalities in muscle and their clinical significance.

Methodology: A retrospective analysis of DM patients who presented to our institute over a 4-year period was performed. These patients had undergone whole-body Short Tau Inversion Recovery (STIR) muscle imaging as well as imaging of limited thigh sequences.

Results: Seventeen patients with DM were included in the analysis. Twelve patients had undergone a whole-body MRI and five had undergone only thigh muscle MRI.

Conclusions: Extensive muscle and subcutaneous high signal intensities were demonstrated in most patients. As our study utilized a whole-body muscle imaging MRI sequence, it adds novel findings to the existing literature on MRI in DM.

Abstract ID: 196: An Observational Cross-Sectional Study of Clinical Spectrum in Patients of Myasthenia Gravis at a Tertiary Care Centre In Northeastern India

Hamsa K M, Marami Das

Guwahati Medical College and Hospital, Bhangagarh, Guwahati, Assam, India

Background and Aim: Myasthenia gravis is a relatively uncommon autoimmune disorder characterized by antibodies targeting the neuromuscular junction. This condition can manifest with drooping eyelids to paralysis of respiratory muscles. In the context of this disease, we only have access to very few studies of myasthenia patients from India. This study aimed to identify the detailed clinical presentation, age of symptom onset, gender distribution, antibody status, thymus abnormalities, and myasthenic crisis in myasthenia patients.

Methodology: An observational, cross-sectional study of myasthenia gravis patients was conducted from May 2022 to April 2024.

Results: Sixty-nine patients with acquired myasthenia were included in the study (37 females and 32 males). The youngest patient was 4 years old and the eldest was 72 years old. Sixty-six (95%) patients had positive neostigmine test. Thirty-six (52%) had generalized myasthenia, 29 (42%) had bulbar symptoms, and 18 patients (26%) had only ocular symptoms. Laboratory tests revealed that 49 (71%) had anti-acetylcholine receptor antibodies, nine had anti-muscle-specific kinase antibodies (anti-MUSK) antibodies, and 11 (15%) patients were double negative. RNS (repetitive nerve stimulation) tests were positive in 46 (66%) patients. Sixty-five (94%) had good response to anti-acetylcholinesterase medications. Thymus abnormalities were observed in 24 patients (34%): 13 with thymic hyperplasia and 11 with thymoma. Eleven (15%) of these patients presented with myasthenic crises. Eleven (15%) required intravenous immunoglobulin and four (5%) required rituximab. Twenty-nine (42%) of our patients had infections as precipitating factors for myasthenia.

Conclusions: Despite its serious nature, myasthenia gravis responds well to treatment when promptly addressed. The increasing incidence of anti-MUSK antibodies is changing the treatment landscape, with a shift toward the primary use of rituximab. Given that one-fifth of patients present as myasthenic crisis, it is of utmost importance for the emerging generation of neurologists to be well informed and receive appropriate training for the timely intervention and management of these patients.

Abstract ID: 198: Clinical Profile of Anti-GAD Antibody Syndromes

Jaydip Ray Chaudhuri, Gnanageetha B B, Rajashekar Reddy Reddy, Mohan Krishna J J, Anirudh Randhi, Swathi Alluri, Pavan K, Sandeep Kumar, Kamala Priya

Yashoda Hospital, Hyderabad, Telangana, India

Background and Aim: Anti-glutamic acid decarboxylase (anti-GAD) antibody is a rare type of antibody formed against glutamic acid decarboxylase enzyme, which is essential for the production of gamma-aminobutyric acid, an inhibitory neurotransmitter present in the brain. Low level of GABA is associated with a spectrum of neurologic disorders ranging from motor to cognitive disorders, known as anti-GAD antibody neurologic syndromes. Our aim was to study the varied clinical manifestations of anti-GAD antibody syndrome.

Methodology: In this paper, we present a series of seven cases that had presented to our hospital with different neurologic presentations, which, after detailed evaluation, were found to be due to anti-GAD antibody autoimmunity. Three patients presented with sensorimotor polyneuropathy, which, on detailed evaluation, was found to be due to anti-GAD autoimmunity. Three other patients presented with stiff person syndrome phenotype, two of whom had the stiff limb variant. One patient presented with sensory seizures, which was diagnosed as GAD65 antibody-associated epilepsy. These patients were treated with corticosteroids, intravenous immunoglobulin, plasma exchange (PLEX), immunosuppressants, and rituximab.

Results: All the above patients showed good response to corticosteroid and Immunotherapy.

Conclusions: From this study, we can conclude that anti-GAD antibody-associated syndrome can have a myriad of clinical manifestations involving both the peripheral and central nervous systems, and hence, it is important for the treating physician to investigate for these antibodies, especially in the elderly with subacute to chronic presentations, as it is a potentially treatable cause.

Abstract ID: 199: Efficacy of a Virtual Car Navigation-Based Online Neurofeedback Game Using Consumer-Grade EEG on Enhancing Attention Skills in Stroke and Mild Cognitive Impairment Patients

Subasree Ramakrishnan, Suhail A T, Vinod A Prasad

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: The burden of cognitive impairment is increasing worldwide. Neurofeedback training (NFT) is emerging as a promising nonpharmacologic intervention for enhancing cognition in healthy and cognitively impaired patients. In this study, we aimed to examine the efficacy of a real-time neurofeedback game using a four-channel consumer-grade electroencephalography (EEG) system on enhancing overt and covert attention skills in stroke and mild cognitive impairment (MCI) patients.

Methodology: The proposed NFT was conducted across 15 patients, which included seven stroke and eight mild cognitive impairment subjects. The game was based on the navigation of a car in a Graphical Use Interface on a computer screen, with the car’s speed proportional to the player’s attention. Sample entropy of EEG signals was the metric for computing attention score. The attention level of the player was computed from EEG signals streamed in real time and was displayed in the form of a progress bar, which acted as feedback to the player. The efficacy of the proposed neurofeedback game was evaluated using different performance measures. Data acquisition, signal processing, attention score computation, and car navigation took place in a synchronized mode as a closed-loop system.

Results: The attention score significantly improved from the first session to the final session by 1.25%–12.55% in the stroke group and 1.84%–44.14% in the MCI group. The cognitive performance and attentional resource index (BATR) also increased in nine subjects from session 1 to session 3.

Conclusions: To our knowledge, this is the first EEG–brain computer interface study for enhancing overt and covert attention in the cross-sectional stroke–MCI patients.

Abstract ID: 200: Peripheral Nerve Hyperexcitability Syndromes – A Clinical and Etiologic Study in a Tertiary Care Hospital

Anusha Induri, shobana N, SACRATIS M, SADEESH KUMAR V, SELVA KUMAR CJ

Coimbatore medical college and hospital, Coimbatore, Tamil Nadu, India

Background and Aim: Peripheral nerve hyperexcitability (PNH) syndromes are an uncommon group of conditions characterized by spontaneous discharges from motor nerve fibers. These are largely immunological, and some forms are genetic. Here, we present seven cases of PNH syndromes secondary to (1) mercury toxicity due to indigenous medicine intake and (2) likely viral infection triggering autoimmunity, with an aim to describe clinical presentation, etiology, voltage-gated potassium channel antibodies (VGKC) antibodies, and electromyography (EMG) findings.

Methodology: Patients who presented with symptoms like muscle twitching, cramps, and neuropathic pain involving mostly the lower limbs between March 2023 and 2024 were examined clinically. Investigations like nerve conduction studies, EMG, VGKC antibody test, heavy metal screening, antinuclear antibody profile, and computed tomography (CT) of the chest and abdomen were conducted in them, apart from routine investigations.

Results: A total of seven patients were included in the study. The patients had muscle fasciculations and severe intractable neuropathic pain, predominantly in the lower limbs, without other axonal neuropathic diseases. One patient presented as chronic fatigue syndrome (CFS), three patients as Issacs, and three patients as Morvan’s. There was a history of indigenous medicine intake in five patients, in whom serum mercury levels tested were found to be elevated. History of prior fever with skin lesions was found in two patients. All the patients had VGKC antibody test positive, with EMG showing myokymic and neuromyotonic discharges. CTs of the chest and abdomen were normal and negative for autoimmune profile. Six patients responded to steroids and one patient required immunosuppression.

Conclusions: This series of PNH cases presents with secondary causes like indigenous medicine intake and viral infection. Neurophysicians should be aware of these as one of the secondary causes of PNH, along with other causes like neoplasms and autoimmune diseases.

Abstract ID: 201: A Comparative Study Between Transcranial Doppler and Magnetic Resonance Angiography to Diagnose Intracranial Artery Stenosis in Acute Ischemic Stroke Involving Anterior Circulation

Antony Sherly Rose Martin Charles, Kishore R, Arun Raj E, Rajasekaran M, Kannan N, Prabhu A, Kishore R, Arun Raj E, Prabhu A

Government K.A.P Medical College, Tiruchirappalli, Tamil Nadu, India

Background and Aim: Our aim is to compare the transcranial Doppler (TCD) results with magnetic resonance angiography (MRA) findings in diagnosing intracranial artery stenosis in patients with ischemic stroke involving the anterior circulation.

Methodology: As a prospective study, TCD and MRA were done in 52 patients with ischemic stroke involving the anterior circulation and admitted in neurology department, KAPV Government Medical College for past 6 months. MRA was done by 1.5-T TOF (time-of-flight) method. TCD was done with EMS 9U Shenzen Delica medical equipment, M Mode, 2 MHz probe. Both magnetic resonance imaging and TCD were done on the same day. Kappa coefficient was used to compare the results of TCD and MRA. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated, and MRA was considered as a comparator.

Results: Of the 102 cerebrovascular accident (CVA) patients, 52 patients were included in the study. Thirty-six were males and 16 were females. Patients aged >40 years were included. The agreement between TCD and MRA was 0.64, which was calculated by kappa coefficient for the anterior circulation. The stenotic vessel identification ability of TCD was sensitivity 80%, specificity 90%, PPV 92.3%, and NPV 57%.

Conclusions: TCD is one of the noninvasive methods to assess intracranial artery stenosis. TCD and MRA have substantial agreement in diagnosing acute ischemic stroke involving the anterior circulation. TCD can be used as a noninvasive diagnostic test to assess intracranial artery stenosis in ischemic stroke involving the anterior circulation.

Abstract ID: 202: Combining Smartphone-Delivered and Clinician-Delivered Intervention: A Plausible Model for Service Delivery for People with Aphasia?

Apporva Pauranik, Rajath Shenoy, Gopee Krishnan

Vishesh Jupiter Hospital, Indore, Madhya Pradesh, India

Background and Aim: A wide range of tasks have investigated the treatment of word-finding difficulties in aphasia. Many researchers have incorporated technology to facilitate self-practice of traditional therapy tasks. However, some therapy tasks are less easily adapted for self-practice by people with aphasia, even with technology. For example, while repetition in the presence of picture (RIPP) can be relatively easily implemented as self-practice, semantic feature analysis is more challenging due to the need for feedback and adaptation depending on the participant’s response. We examined a plausible model for clinical service delivery using two modes of service delivery with two different therapy approaches: a smartphone-based home program using RIPP and clinician-delivered therapy using Semantic Feature Analysis (SFA).

Methodology: We used a single case methodology with six people with chronic aphasia who had a predominant difficulty in picture naming. Participants were given a set of 300 pictures for naming. One hundred and twenty items that were difficult to name were selected for treatment and divided into four matched lists (30 each). These lists were randomly allotted to four conditions: smartphone-delivered treatment, clinician-delivered treatment, untreated but probed, and untreated pre-post only. We had three treatment orders with two participants per order: clinician-delivered treatment (using SFA) followed by smartphone-delivered treatment (using RIPP), smartphone-delivered treatment followed by clinician-delivered treatment, and simultaneous provision of both treatment modes. We analyzed the picture naming results using weighted statistics.

Results: Of the six participants, four showed significant item-specific improvements in naming following smartphone-delivered treatment with three of these participants also showing improvement with the clinician-delivered treatment. There was no significant difference between the tasks for any participant.

Conclusions: This study reinforces the potential for technology-based home practice to improve word retrieval through the use of simple tasks. Such technology-based interventions have advantages over pen and paper “homework” as they allow for (remote) monitoring by clinicians.

Abstract ID: 203: Application of Updated Paraneoplastic Score Care Diagnostic Criteria in Suspected Cases

Sankavi K, Shobana N, Socratis M, Selvakumar C.J., Sadeeshkumar V

Coimbatore Medical College, Tamil Nadu, India

Background and Aim: Paraneoplastic neurologic syndromes (PNSs) are an immune-mediated heterogenous group of neurologic disorders that occur in cancer patients. PNS diagnostic criteria have been updated recently after 16 years. These updated criteria provide a standardized framework for assessing PNS and ensure consistency in evaluation. Our aim was to implement an updated scoring system for diagnosing paraneoplastic syndromes in suspected cases.

Methodology: Fifteen eligible patients with suspected PNS from our department were enrolled in this study. Here, the data including patients’ clinical paraneoplastic phenotypes, presence of neuronal antibodies (neuronal surface, intraneural antibodies), and associated cancer were evaluated. PNS care scoring system was utilized, and the level of diagnostic evidence was assessed for guidance of clinical decision-making, including monitoring of disease progression and further diagnostic workup.

Results: Out of 15 patients, nine were males and six were females. The most common clinical phenotype was Morvan’s syndrome (six patients), followed by sensory neuropathy (five patients), limbic encephalitis (three patients), and stiff person syndrome (one patient). According to PNS care scoring system, a total of two patients met the definite diagnostic level, six patients met the probable criteria, four patients met the possible criteria, and three patients met the criteria for non-PNS. This updated PNS care scoring system is more specific as it requires cancers consistent with antibody and clinical phenotype.

Conclusions: Updated PNS care scoring system plays a critical role in enhancing clinical practice and can serve as a research tool in the field of PNS.

Abstract ID: 204: Impact of Genetic Variants on Sodium Valproate-Induced Weight Gain in Persons with Epilepsy of South Indian Origin: A Candidate Gene Association Study

Anamika Sekhar, Kesavan Ramasamy, Pradeep P. Nair, Harivenkatesh Natarajan

Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry (JIPMER Puducherry), India

Background and Aim: Sodium valproate, a commonly used anticonvulsant for treating epilepsy, is associated with a variety of side effects, the most frequent being weight gain. Association between genetic factors and valproate-induced weight gain is not studied in South Indian (SI) population. Hence, the study aims to (1) evaluate the association between nine single-nucleotide polymorphisms (SNPs) in drug metabolizing enzyme, appetite stimulation, energy homeostasis genes, and valproate-induced weight-gain in persons with epilepsy (PWE) from SI and (2) compare fasting plasma insulin, testosterone, adiponectin, resistin, and leptin levels in cases and controls between relevant genotypes.

Methodology: Two hundred and sixteen PWE aged 18–65 years of both genders were included within 1 month of initiating valproate monotherapy. Body weight was measured at baseline and after 6 months of follow-up. Genotyping of extracted DNA was done by quantitative reverse transcription polymerase chain reaction (qRT-PCR), and biochemical parameters were estimated by enzyme-linked immunosorbent assay.

Results: Of the 216 PWE recruited and followed up, 97 people were classified as cases (weight ? 5% of the baseline weight), while 97 were controls. The allelic distributions of all of SNPs in this study were checked for Hardy–Weinberg equilibrium. Two SNPs in genes such as ?2 catalytic subunit of AMPK ( PRKAA2 ) –rs10789038 and resistin ( RETN )-rs1862513 showed significant associations with valproate-induced weight gain ( P = 0.0019 and P = 0.0012, respectively). There was no statistically significant difference between wild-type and variant genotypes of LEPR, CYP2C19*2, and RETN in cases and controls and fasting plasma leptin, testosterone, and resistin levels.

Conclusions: It has been shown that genetic polymorphisms in PRKAA2 rs10789038 RETN rs1862513 may have value in predicting valproate-induced weight gain.

Abstract ID: 205: Computer-Based Assessment Tool (COMBAT) for Android and iOS-Based Smartphones for the Diagnosis of Primary Headache Disorders

Vaibhav Deorari, Debashish Chowdhury, Kolin Paul, Pritesh Srivastava, Ashish Kumar Duggal

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi), India

Background and Aim: There is a paucity of digital diagnostic tools for headache disorders. We aimed to develop a branching logic questionnaire and a computer-based assessment tool (COMBAT) for Android and iOS-based smartphones for the diagnosis of primary headache disorders using International Classification of Headache Disorders third version.

Methodology: The development of COMBAT was done in four phases. In phase 1, an assessment of the core diagnostic features of primary headaches and a review of case records from the headache clinic were done. Then a questionnaire and the branching logic algorithm in phase 2 were developed. In phases 3 and 4, validation of the tool was done using different sets of users against the diagnosis provided by two headache experts (gold standard). Both English and Hindi versions were created.

Results: We created 15 versions of COMBAT and improved upon its features. COMBAT finally included 143 questions. Three hundred and eighty-five patients each were recruited for analysis in phase 3 and 4. By phase 4, sensitivity, specificity, and accuracy for diagnosis of migraine headache were 95.54%, 89.8%, and 94.8%, respectively; for tension-type headaches were 84%, 98.06%, and 97.14%, respectively; for trigeminal autonomic cephalalgias headaches were 83.3%, 99.46%, and 98.7%, respectively; and for “other primary headaches” were 50%, 98.69%, and 98.44%, respectively. Only four patients each remained unclassified during phases 3 and 4. The mean test duration for the application of COMBAT was 10.6 (standard deviation- 2.85) min. In addition to the diagnosis of primary headaches, COMBAT could diagnose episodic from chronic headaches, medication overuse headaches, and possible secondary headaches.

Conclusions: “COMBAT,” a computer-based mobile application tool, demonstrated a high degree of accuracy, sensitivity, and specificity for diagnosing primary headache disorders.

Abstract ID: 541: Phenotypic, Electrophysiologic, and Imaging Spectrum of Hirayama Disease from Northern India

Saranya Gomathy, Yamini Priyanka, Ajay Garg, William L. Macken, Ayush Agarwal, Tanveer Ahmed, Rohit Bhatia, Vinay Goel, Kanwaljeet Garg, Robert D. S. Pitceathly, Michael G. Hanna, M. V. Padma Srivastava, Venugopalan Y. Vishnu

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Hirayama disease (HD) is a lower motor neuron (LMN) disorder the manifests as asymmetric weakness, atrophy, and fasciculations of one or both distal upper limbs. There is a lack of knowledge of this entity among clinicians due to its rarity. This study aimed to review the clinical, electrophysiologic, and radiologic characteristics of HD.

Methodology: All patients with clinically suspected HD between January 2018 and December 2022 were reviewed. Search criteria included insidious-onset progressive pure motor LMN weakness of distal upper limb (s), electromyography (EMG) showing chronic denervation in C7–T1 myotomes, and dynamic cervical magnetic resonance imaging (MRI) suggesting HD-like anterior dural displacement, asymmetric cord flattening, cord atrophy/hyperintensity, epidural flow voids, and enhancement of epidural crescent. Demographic details, clinical examination findings, and electrophysiology database were analyzed using Statistical Package for the Social Sciences software version 26.0.

Results: One hundred forty patients met the HD diagnostic criteria. Of them, 98.6% were males. Mean age of patients was 21.2 ± 4.8 years. Average age of symptom onset was 17.8 years. Median duration of symptoms was 3 (1.5–4) years. All had distal amyotrophy. Sixty-nine (49%) had unilateral and the rest (51%) had asymmetric bilateral involvement. Family history was confirmed in two patients. Cold paralysis, oblique atrophy, and polyminimyoclonus were seen in 105 (75%), 139 (99%), and 89 (63.5%) patients, respectively. EMG was abnormal in all ( n = 106), and sensory nerve action potential (SNAPs) were normal in all. EMG changes in the uninvolved limb were seen in 33% (18/54) patients. Dynamic MRI revealed anterior dural displacement in 134 (96%) patients, cord atrophy in 111 (79%), and asymmetric cord flattening in 124 (88.5%). Contrast MRI in 72 (51%) patients revealed epidural enhancement in 64 (89%) patients. Four underwent surgery.

Conclusions: Good quality, head-to-head randomized trials on conservative versus surgical management need to be performed in future.

Abstract ID: 556: Long-Term Epilepsy Control, Neurodevelopmental Outcome, and Quality of life in Children and Adolescents with Lennox–Gastaut Syndrome

Indrasish Ray Chaudhuri, Vinayan Kollencheri Puthenveettil, Vaishakh Anand, Bindu R

Amrita School of Medicine, Kochi, Kerala, India

Background and Aim: Lennox–Gastaut syndrome (LGS) is a chronic epilepsy of childhood that persists into adulthood with its intractable seizures and neurocognitive impairment. Our present study aimed to assess the epilepsy, neurodevelopmental outcome, and quality of life (QoL) outcome and their predictors in a follow-up cohort with LGS.

Methodology: A cross-sectional evaluation in a follow-up cohort of LGS (aged 2–25 years with age <18 years at diagnosis), between June 2023 and March 2024, was performed at a tertiary care center in South India. The subjects were assessed for epilepsy severity, functional status (gross motor and hand function), social quotient, behavioral comorbidities, QoL, and caregiver burden using the Early Childhood Epilepsy Severity Scale, Gross Motor Function Classification System, Manual Ability Classification System, Vineland Social Maturity Scale, Diagnostic and Statistical Manual of Mental disorders-5 criteria, Pediatric Quality of Life Inventory (PedsQL)-Epilepsy module, and Zarit caregiver burden scale, respectively.

Results: Ninety-six patients with LGS (63.5% boys) with at least 1-year regular follow-up were studied. The majority (56.3%) had underlying structural etiology. The mean (+ standard deviation [SD]) age of the cohort was 10.3 (5.8) years. The long-term outcomes observed were ongoing epilepsy (73/96), unfavorable motor status (77/96), moderate to severe socio-adaptive deficits (68/96), autism spectrum disorder (47/96), attention-deficit hyperactivity disorder (39/96), impaired QoL (78/96), and moderate to severe caregiver burden (79/96). Interictal sleep electroencephalogram showing generalized paroxysmal fast activity was associated with ongoing epilepsy in the last follow-up ( P = 0.01). Epilepsy control correlated with motor and cognitive outcomes.

Conclusions: With the increasing survival of children and adolescents with this severe chronic epilepsy, improvement in epilepsy control can translate into better long-term neurocognitive outcomes and improvement in QoL with less caregiver burden.

Abstract ID: 501: Deciphering Posterior Reversible Encephalopathy Syndrome with Spinal Cord Involvement (PRES-SCI): Etiology, Presentation, Radiology, and Outcomes

Pritam Majumdar, Thomas Matthew, Shagun Bhardwaj, Sharath Kumar GG, Sai Kantha, Raghunandan Nadig, Gosala Raja KukkutaSarma

St. Johns Medical College, Bengaluru, Karnataka, India

Background and Aim: Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic syndrome usually accompanied by subcortical white matter hyperintensities in the parieto-occipital region. Here, we report two cases of ours along with an analysis of all 39 cases reported in literature of rare spinal cord variant of this disease.

Methodology: We have described the demographics, presentation, radiology, and outcomes of two cases from our cohort, along with 39 cases from the literature.

Results: The median age of our cohort was 24 years (range 1.5–67), with 61% being male. All patients presented with hypertension, with a mean systolic blood pressure of 206 ± 24.2 mmHg and a mean diastolic blood pressure of 122.5 ± 20.5 mmHg. Renal dysfunction was observed in 68.3% of patients. The majority of patients presented with headaches (81%) and vision difficulties (63.4%). Hypertensive retinopathy was found in 56% of patients upon examination. Neurologic symptoms were reported by only 20% of patients. Cortical involvement was seen in 46% of patients, while brainstem involvement was observed in 83% of patients. Cervical cord involvement was present in 98% of patients, with an average of over five segments affected. Cord swelling was noted in 66% of patients. The median clinical follow-up was at 9 days, and radiologic follow-up was at 35 days, showing complete resolution.

Conclusions: Being a close mimicker of neuromyelitis optica spectrum disorder, it is of utmost importance to identify this disease to avoid wrong investigations and treatment.

Abstract ID: 502: KCDT7 Gene-Related Progressive Myoclonic Epilepsy with Ataxia

Sonalika Behera, Ashok kumar Mallick, ASHOK KUMAR MALLICK, KALI PRASANNA SWAIN

Shree Ram Chandra Bhanja Medical College, Cuttack, Odisha, India

Background and Aim: Progressive myoclonus epilepsy (PME) with ataxia a very rare disorder consisting of myoclonus, tonic–clonic seizures with ataxia. Ataxia is usually the first presentation followed by multiple episodes of seizure and myoclonus. It usually does not affect the intellect of patients. These are rarely inherited neurodegenerative disorders caused due to various etiologies.

Methodology: A 3-year-old female child presented to the neurology outpatient department with ataxia, myoclonus, and seizures with normal antenatal, natal, postnatal history and negative family history. Extensive workup was done to find out the cause.

Results: General examination revealed head size of 48 cm with no other abnormalities. Nervous system exam revealed presence of cerebellar signs. No pyramidal signs and sensory abnormalities were present. Cognition was normal. Electroencephalogram was normal, and magnetic resonance imaging of the brain was also normal. Other blood tests were normal. Whole exome sequencing revealed a novel homozygous mutation of variant c.533C>T in exon 4 of the KCDT7 (potassium channel tetramerization domain containing protein 7) gene that was compatible with the diagnosis of PME3 with or without intracellular inclusions.

Conclusions: This study reported a rare case of KCDT7 -related PME with ataxia in the Indian population and identified a novel KCDT7 mutation in a child with PME. This study contributes to the genetic diagnosis and counseling of families with PME.

Abstract ID: 503: Burnside–Butler Syndrome – Pathogenic Variant or VUS?

Suranjana Basak, Sudesh Prabhakar, Amit Shankar Singh, Harpreet Singh Mann, Jeenendra P. Singhvi

Fortis Hospital Sector 62, Mohali, India

Background and Aim: Burnside–Butler syndrome is an inheritable genetic condition characterized by the partial deletion of specific genetic material located on chromosome 15q11. Individuals with this medical condition display a variety of neuropsychiatric disorders – psychosis, aggression, mood disorders, developmental disorders involving learning difficulties, language delays, autism spectrum disorders, attention-deficit/hyperactivity disorder.

Methodology: This is a case report including the clinical spectrum, cytogenetics, and neuroimaging.

Results: A 7-year-old boy r/o Canada, born as full term normal vaginal delivery (FTNVD), cried 3 min after birth, following which he was admitted to the intensive care unit for 4 days and operated for bifid scrotum and hypospadias and was discharged after complete recovery. He presented to our neurology outpatient department with complaints of two episodes of generalized tonic clonic febrile seizures at the age of 2 and 5 months. Patient also had developmental delays of approx. 4 month in physical attributes and intellectual disability. He had good grasping capacity, normal hearing-vision, poor writing, and diminished attention. The mother also reported attention-seeking behaviors. She reported per vaginal (PV) bleeding during the fourth month of pregnancy, and antenatal care (ANC) scan during the eighth month reported fetal growth and developmental delay. The patient also had a family history of paternal grandfather having epilepsy. Neuro exam revealed-impaired cognition, MMSE 18, and short attention span. Language and motor delays were noted; in addition, the patient had stuttering speech, autistic behavior-irritability, tantrum throwing, and attention-deficit hyperactivity disorder (ADHD). There were no sensorimotor abnormalities, and all reflexes were bilateral ++. Incoordination and gait ataxia were present. Magnetic resonance imaging of the brain + spectroscopy s/o microcephaly. Rhombencephalosynapsis. Fusion of dentate nuclei and abnormal configuration of fourth ventricle were found posteriorly. Asymmetric prominence of extra-axial cerebrospinal fluid layering along the anterolateral aspect of the left cerebellum was noted. Cisternal prominence was noted. Vermis was absent. Brain parenchymal volume to intracranial volume was low. Cytogenetics reported chromosome 15 deletion, uncertain significance. Deletion of 15q11.2 (312kbp).

Conclusions: The case highlights the complex nature of symptoms despite being reported “uncertain significance,” wherein there are significant clinical and radiologic features.

Abstract ID: 504: Beyond Organs: IgG4-Related Disease Extending to Peripheral Nerves

Siphora Bijjiga

Aster Medicity, Kochi, Kerala, India

Background and Aim: Peripheral neuropathy, characterized by numbness and tingling, can have diverse etiologies. This case report discusses a 64-year-old male patient presenting with simultaneous onset of numbness and tingling in both hands and feet, which progressively worsened despite initial treatment. The aim is to explore the diagnostic process and management of this patient’s neuropathy, which is ultimately attributed to chronic inflammatory demyelinating polyneuropathy secondary to IgG4-related disease.

Methodology: Patient’s medical history was reviewed, including a history of treated tuberculosis (TB) 30 years ago and no significant family medical history. Initial clinical examinations and vital signs were normal, but neurologic examinations revealed sensory impairments. Comprehensive diagnostic tests were conducted, including serum vitamin B12 levels, intrinsic factor antibody, complete blood count, liver function tests, renal function tests, protein electrophoresis, nerve conduction studies (NCV), magnetic resonance imaging, ultrasound, positron emission tomography scan, and various biopsies.

Results: Initial tests indicated a vitamin B12 deficiency, leading to a diagnosis of pernicious anemia with peripheral neuropathy. However, the patient’s symptoms worsened, with additional weakness in both feet and hands. NCV results confirmed subacute-onset progressive distal sensory motor neuropathy. Further investigations revealed negative results for human immunodeficiency virus, HV, hepatitis B virus surface antigen (HBSAg), TB diagnostic panel, and various autoimmune markers. The final diagnosis was CID secondary to IgG4-related disease, confirmed by elevated serum IqG4 levels and characteristic histologic findings.

Conclusions: The complexity of neuropathic conditions is highlighted. Effective management, involving high-dose intravenous methylprednisolone followed by oral prednisolone, led to improvement in both sensory and motor symptoms, illustrating the potential for positive outcomes with appropriate diagnosis and treatment.

Abstract ID: 505: Double Trouble: A Rare Co-occurrence of Double-Seropositive Myasthenia Gravis and Overlap Myositis

Cankatika Choudhury, Cankatika Choudhury, Akhil Sahib, Neera Chaudhry

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi), India

Background and Aim: Idiopathic inflammatory myositis (IIM) occurring in the context of connective tissue disorders is not uncommon. It is better characterized as overlap myositis. Adding to this is the rare association of myasthenia gravis (MG) with IIM. Very few cases of myositis and myasthenia overlap have been reported in literature so far. Here, we report the first case of a double-seropositive (anti-acetylcholine receptor antibody [anti-AChR]) and (anti-MuSK) MG and overlap myositis in a young female.

Methodology: A 22-year-old Asian woman presented with drooping of both eyelids without any double vision for 11 months, difficulty in swallowing liquids more than solids, with nasal intonation of speech for 10 months, progressive difficulty in climbing stairs followed by difficulty in raising arms overhead for 5 months. This was associated with pain in small and large joints of the body with discoloration of fingers on cold exposure. There was bilateral frontalis over action and asymmetric ptosis. MG composite score was 11, and Myasthenia Gravis Foundation of America (MGFA) class was III A. Ice pack test was positive with >2 mm improvement of ptosis in each eye. Muscles around hips, thighs, and arms were tender. Power was Medical Research Council (MRC) grade 4-/5 around bilateral shoulder and elbow and 4/5 around both hips and knees, respectively.

Results: C-reactive protein was Positive (creatine phosphokinase(CPK)-1333 IU/L). Anti-acetylcholine receptor antibody (anti-AChR) binding was positive. Anti-muscle-specific kinase (Anti-MuSK)antibody was positive. Myositis panel was strongly positive (3+) for Ro-52 and PM-Scl 100.

Conclusions: Patients of IIM should also be evaluated for myasthenia if ptosis and jaw weakness are present despite high CPK.

Abstract ID: 506: Decompensated Liver Failure with Longitudinally Extensive Myelitis – An Unusual Clinical Combination in Sjogren’s Syndrome

Yamini Priyanka PentaKota, Divyani Garg, Ayush Agarwal, Tanuja Kabir, Ajay Garg, Kameshwar Prasad, Achal Kumar Srivastava

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Sjögren’s syndrome is a chronic autoimmune disorder of exocrine glands which affects individuals in the fourth to fifth decade. Hallmark features are keratoconjunctivitis sicca and dry mouth due to lymphocytic infiltration of the glands. Multisystemic involvement may occur. We report an unusual combination of liver cirrhosis and myelitis as a presenting feature of Sjogren’s syndrome.

Methodology: A 60-year-old female presented with acute-onset weakness of the right lower limb 2 months back followed by left lower limb weakness 1 month back with bowel and bladder involvement and sensory loss below D4 level. She also had abdominal distension for 6 months before the onset of these symptoms. On evaluation, she was found to have decompensated liver failure with esophageal varices. Magnetic resonance imaging showed longitudinally extensive transverse myelitis (LETM) from D1 to D9 level.

Results: Autoimmune etiology was suspected, considering the conglomeration of chronic liver disease and myelitis. Serum neuromyelitis opticaand myelin oligodendrocyte glycoprotein were negative. Anti-SS-A and SS-B antibodies were strongly positive. Schirmer’s test was positive. Whole body and brain positron emission tomography scan was normal. A diagnosis of Sjogren’s syndrome was made. She was started on plasmapheresis as pulse steroids were not beneficial, with which she showed improvement. Long-term management with rituximab therapy was planned.

Conclusions: Sjögren’s syndrome should be kept as a differential for LETM, especially in the background of multisystem involvement.

Abstract ID: 507: An interesting Case of Brainstem Glioma Presenting as Demyelination

Suresh Palanisamy, Sivakumar S, Thiruvarutchelvan

Government Mohan Kumaramangalam Medical College Salem, Tamil Nadu, India

Background and Aim: Brainstem glioma is a highly devastating disease, and any mass-like lesion in the brainstem can raise suspicion of this diagnosis. However, other inflammatory, demyelinating, or degenerative diseases can mimic brainstem glioma in clinical presentation and imaging features. Therefore, diagnosis based solely on imaging is often insufficient for brainstem lesions and may lead to incorrect diagnosis and treatment.

Methodology: Our patient, a 22-year-old, right-handed male, developed weakness of the right lower limb for 1½ months and upper limb for 1 month with slurring of speech and had fever for a day before the onset of illness, with no history of sensory loss or bladder/bowel involvement or lower limb weakness and no history of trauma or neck pain or breathing difficulty. On examination, the patient had horizontal nystagmus bilaterally. Patient had right gag reflex absent and left gag reflex diminished. Tongue wasted the on left side with deviation to the left on protrusion. Spinomotor examination showed weakness as the power grading reveals right shoulder 3/5, Elbow 3/5, Wrist 3/5, Right hip 3/5, Knee 3/5 and ankle 3/5. Deep tendon reflexes were brisk and B/L plantar- Extensor with normal sensory examination

Results: Magnetic resonance imaging (MRI) of the brain showed diffuse hyperintense lesion in the left half pons and entire medulla and cervical cord up to C2 level showing demyelination. So, the patient was started with intravenous methylprednisolone. But there was no clinical improvement and aquaporin antibody was negative. Repeat MRI of the brain with magnetic resonance (MR) spectroscopy showed peak choline/creatinine ratio 3.5, suggestive of diffuse brainstem glioma, and the patient was sent for stereotactic surgery.

Conclusions: This case is an example of brainstem glioma masquerading as demyelination stresses the importance of detailed MRI and tissue diagnosis when in doubt.

Abstract ID: 508: A Clinicolaboratorical Study of Guillain–Barré Syndrome with Special Reference to Phrenic Nerve Study

Rajarshi Chakraborty, Rajesh Verma

King George’s Medical University, Lucknow (KGMU Lucknow), Uttar Pradesh, India

Background and Aim: Guillain–Barré Syndrome (GBS) is an acute acquired autoimmune inflammatory disorder of peripheral nerves and roots. The clinical subtype of GBS include acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), and variants. Respiratory insufficiency is an important predictor of poor prognosis in GBS. We aimed to perform phrenic nerve conduction study (NCS) to predict respiratory involvement and outcome in GBS.

Methodology: A total of 75 patients (including nine coronavirus disease 2019 [COVID-19] +ve patients) of GBS admitted to our hospital over 2 years were thoroughly evaluated with clinical examination, blood tests done in the laboratory, and NCS including phrenic nerve study.

Results: The median age of patients was 31 years (range 1–75 years) with a male:female ratio of 2:1. Subtype analysis showed AIDP (10.67%), AMAN (38.66%), and AMSAN (50.67%). The mean Erasmus Guillain- Barre Syndrome Respiratory Insufficiency Score (EGRIS) score at admission was 3.8 ± 0.17 days and mean Medical Research Council (MRC) score at admission was 30.35 ± 1.80, with the need for ventilatory assistance observed in 24 (32%) patients with a mean duration of ventilatory stay of 23.83 ± 3.50 days. It reduced to 16 ± 3.89 days in COVID-19 patients with GBS due to increased early deaths. Phrenic nerve study corroborated with future requirement of ventilatory support and this was statistically significant. Mortality was observed in eight (10.67%) patients, which increased to two (22.22%) patients in COVID-19 illness, with ventilatory deaths occurring in eight (33.33%) patients that increased to two (50%) in COVID-19 illness.

Conclusions: Respiratory insufficiency and subsequent ventilatory requirement is an important predictor of poor outcome in GBS. Phrenic NCS can be incorporated in routine NCS protocol to provide information about impending requirement of ventilatory support.

Abstract ID: 509: KCNC1 Mutation: An extremely Rare Progressive Myoclonic Epilepsy Presenting as Myoclonic Epilepsy and Ataxia due to Potassium Channel Mutation (MEAK)

Sidharth S, Manjari Tripathi, Deepti Vibha, Jasmine Parihar, Animesh Das, Rajesh Kumar Singh

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Progressive myoclonic epilepsy (PME) include phenotype leading to myoclonic jerks, seizures, and progressive neurologic impairment. KCNC1 is an extremely rare potassium channel mutation known to cause PME with ataxia.

Methodology: We present a 24-year-old male born of a nonconsanguineous marriage with low birth weight and near-normal developmental milestones. At the age of 4 years, he developed multiple episodes of generalized myoclonic jerks responsive to valproate. At the age of 7 years, he developed tremulousness of both hands-on activities, scanning dysarthria, gait instability, and mild cognitive impairment. He had generalized tonic clonic seizures along with myoclonic jerks. His seizures were controlled on medication since 14 years of age. On examination, he had gaze-evoked horizontal nystagmus and broken saccades with normal fundus. Motor examination was normal. Cerebellar examination revealed abnormal signs bilaterally. Blood investigations and lactate levels were normal. Magnetic resonance imaging of the brain revealed diffuse cerebellar atrophy. Skin biopsy did not reveal any Lafora bodies. Electroencephalogram showed generalized epileptiform discharges. Whole exome sequencing revealed KCNC1 c.959G>A variant at exon 2 with autosomal dominant pathogenic variant.

Results: Myoclonic epilepsy and ataxia due to potassium channel mutation (MEAK) identification in a PME phenotype has unravelled many of the unclassified PME spectrum diseases, and whole exome sequencing is useful for making the diagnosis.

Conclusions: Whole exome sequencing in PME with MEAK phenotype can help in diagnosing rare mutation of KCNC1 .

Abstract ID: 510: Papillon-Léage–Psaume Syndrome and Neuronal Migration Disorders

Vikas Lakhanpal, Bhawna Lakhanpal

All India Institute of Medical Sciences (AIIMS), Bathinda, India

Background and Aim: We present a case of orofacial digital syndrome type 1 with seizures, cognitive defects, and neuronal migration disorders, highlighting the need for evaluation and management of neuronal migration defects in orofacial digital syndrome (OFD) syndromes.

Methodology: An early adolescent girl presented with focal seizures with dyscognitive features and intellectual disability. General physical examination revealed a lobulated tongue with tongue nodules, widely spaced eyes, hypoplasia of alar nasi, and alopecia areata along with brachydactyly. Neurologic examination showed Montreal cognitive assessment score of 17 out of 30. There were no signs pertaining to the motor, sensory, or cerebellar system. Her brain magnetic resonance imaging was suggestive of complete corpus callosum agenesis, interhemispheric cyst, and polymicrogyria along with heterotopia, suggestive of neuronal migration disorders [Figure 2]. Ultrasound abdomen showed normal bilateral kidneys.

Results: Molecular genetic analysis using next-generation whole exome sequencing revealed a heterozygous missense variant in exon 3 of the OFD1 gene (chrX:g.13736596T>C; Depth: 113x) that results in the amino acid substitution of serine for leucine at codon 77 (p.Leu77Ser; ENST00000340096.11). ENST00000340096.11 matches with NM_0036113.3; both are MANE-selected transcripts for OFD1 gene. This OFD1 variation is classified as a variant of uncertain significance. The variant has not been reported in the 1000 genomes, gnomAD (v3.1), gnomdAD (v2.1), TOPMed, and our internal databases. The in silico predictions of the variant are probably damaging by PolyPhen-2 (HumDiv) and damaging by SIFT, LRT, and MutationTaster2.

Conclusions: The present case highlights the importance of full neurologic, neuroradiologic, and neuropsychologic evaluation, besides renal and orofacial abnormalities in OFD1 patients, as seizures and cognitive defects can be the only presenting complaints in OFD1 .

Abstract ID: 511: Multiaxial Neurologic Syndrome Secondary to Hypoparathyroidism: A Rare Case

Vikas Lakhanpal, Bhawna Sharma, Sameer Peer

All India Institute of Medical Sciences (AIIMS), Bathinda, India

Background and Aim: Disorders of calcium metabolism can lead to enhanced excitability of the nervous system and varied neurologic symptomatology.

Methodology: We describe a multiaxial neurologic syndrome in a hypocalcemic state secondary to hypoparathyroidism, along with distinct radiologic changes in the brain.

Results: A female in her late 40s presented with complaints of behavioral issues, parkinsonism, myoclonus, and seizures. On examination, she had positive trousseau and Chvostek signs suggestive of facial hyperexcitability. Montreal’s cognitive assessment score was 18/30. However, there was a cogwheeling type of rigidity along with bradykinesia. Her calcium level was 4.3 mg/dl with normal vitamin D3 levels and significantly reduced parathyroid hormone levels. Magnetic resonance imaging revealed multiple blooming foci in the basal ganglia region and dentate nuclei. She was managed with calcium, vitamin D supplementation, along with antiseizure medications and showed remarkable improvement in her symptoms; by 2 months, she improved fully to near-normal status.

Conclusions: Chronic hypocalcemia may manifest in the form of psychiatric and neurologic symptoms. Underdiagnosis and misdiagnosis are common due to multiaxial neurologic involvement in metabolismespecially hypocalcemia. Idiopathic hypoparathyroidism is a crucial differential diagnosis in patients with manifestations of chronic hypocalcemia.

Abstract ID: 512: The Conundrum of Idiopathic Intracranial Hypertension (IIH): Challenges in Diagnosis and Management

Neelamraju Mounika, M.K Singh, Chakradhar Reddy, Rahul Konduri

Plot No 3, Road No. 2, Financial District, Gachibowli, Nanakramguda, Telangana

Background and Aim: Many atypical presentations of idiopathic intracranial hypertension (IIH) have been reported in the literature with multiple cranial nerve palsies in varied frequencies. The mechanism by which cranial neuropathies develop in IIH is unknown; however, it is thought to be due to intracranial pressure imposing traction forces on cranial nerves. There have been a very few case reports of cranial nerve palsies and neck pain in IIH patients with high opening pressure that resolved similar to our case report. We aimed to raise clinical awareness about the management of atypical presentations of IIH.

Methodology: A 28-year-old female, with a history of migraines, experienced neck pain and right hemicranial radiating pain post-cricket ball injury on the neck, which was trivial. Symptoms included severe neck pain radiating to the right arm and headache. Examination revealed bilateral Grade 3 papilledema and right facial lower motor neuron (LMN) weakness. Initial high opening cerebrospinal fluid (CSF) pressure (35 cm) following CSF drainage showed clinical improvement. Neuroimaging showed features suggestive of IIH. Negative serological tests ruled out other causes. Despite Diamox initiation, she had recurrence of severe arm pain and facial weakness. Repeat lumbar puncture showed elevated pressure (38 cm). Due to fluctuating symptoms, a shunt was offered, but the patient refused and continued conservative management, resulting in symptomatic improvement subsequently.

Results: Regular follow-ups revealed a gradual resolution of the presenting symptoms and improvement, supporting the efficacy of the treatment regimen.

Conclusions: This case expands the understanding of IIH by acknowledging its diverse clinical spectrum. It emphasizes the necessity for personalized diagnostic strategies and increased awareness among health-care providers to effectively identify and manage atypical IIH presentations.

Abstract ID: 513: l-2-Hydroxyglutaric Aciduria: A rare Cause of Global Developmental Delay – A Case Report

Praveen Singh, Shobana N, Sacrates M, Selvakumar CJ, Sadeeshkumar V

Coimbatore Medical College Coimbatore, Tamil Nadu, India

Background and Aim: l-2-hydroxyglutaric aciduria (L2HGA) is an autosomal recessive inherited metabolic disorder caused by pathogenic variants in the L2HGDH gene (14q22.1) encoding mitochondrial 2-hydroxyglutarate dehydrogenase. (1) First described the disease in 1980 and since then, cases from different regions have been reported worldwide with an estimated prevalence of less than 1/1,000,000 births. (2) The biochemical hallmark of L2HGA is increased concentration of l-2-hydroxyglutaric acid in body fluids, which was relatively higher in urine, cerebrospinal fluid, and to a lesser extent in plasma. Final and accurate diagnosis is made by molecular analysis (whole exome sequencing) of L2HGDH gene. (3) No specific treatment exists for L2HGA; management is mainly supportive with specific therapies including flavin adenine dinucleotide sodium and levocarnitine chloride. Methodology: Clinical history followed by detailed neurologic examination was taken and further workup was done accordingly.

Results: Whole exome sequencing confirmed L2HGDH gene mutation suggestive of L2HGA.

Conclusions: L2HGA may be an etiology of global developmental delay.

Abstract ID: 514: Finger Drop as the Initial Manifestation of Hirayama disease: An Unusual Presentation of a Rare Disease

Varun Singh, Sanchit Shailendra Chouksey, Akansha Jain, Rameshwar Nath Chaurasia, Abhishek Pathak, Anand Kumar, Deepika Joshi, Vijaya Nath Mishra

Institute of Medical Sciences, Varanasi (IMS, Varanasi), Uttar Pradesh, India

Background and Aim: Focal amyotrophy, unilateral or asymmetric bilateral weakness, and wasting of muscles innervated by C7, C8, and T1 are the hallmarks of Hirayama disease. We are reporting a case of Hirayama disease, whose presenting feature was finger drop, a very unusual feature.

Methodology: A 19-year-old male presented with progressive inability to extend his left hand’s middle and ring fingers for 4 months. He did not have pain over fingers, loss of sensation, atrophy, fasciculations, headache, or neck pain. His higher mental function test, cranial nerves, and sensory examination were normal. Motor examination revealed severe extensor weakness in his left third and fourth fingers and mild weakness in the left index finger and triceps (not part of presenting complaints). Deep tendon reflexes were normal.

Results: Routine blood investigations were normal. Electromyogram revealed fasciculations and neurogenic motor unit action potential (MUAP) in C7, C8, and T1-innervated left upper limb muscles, suggestive of chronic reinnervation. Magnetic resonance imaging of the cervical spine in forward flexion showed prominent epidural space in the lower cervical–upper thoracic spine with multiple prominent flow voids within and forward migration of the posterior dural sac causing dynamic cord compression.

Conclusions: Finger drop is a very unusual presenting manifestation due to focal and selective atrophy of the anterior horn cell.

Abstract ID: 515: Clinical Profile and Precipitating Factors in Patients With Breakthrough Seizures – A Cross-Sectional Observational Study

Subhangi Thakur Hameer, Parampreet Kharbanda, Jitupam Baishya, Kamalesh Chakravarty

Post Graduate Institute of Medical Education and Research, Chandigarh (PGIMER Chandigarh), India

Background and Aim: We aimed to assess the clinical profile and precipitating factors in patients presenting with breakthrough seizures in a tertiary care center in northern India.

Methodology: We conducted a cross-sectional observational study in a tertiary referral hospital and research center in northern India involving patients >14 years of age, diagnosed as a case of epilepsy and in remission for at least 3 months and presenting with breakthrough seizures. Data was collected using a predefined proforma and various validated assessment scales.

Results: Out of the 45 patients enrolled over a 3-month period, breakthrough seizures were more common in males (66.7%) compared to females (33.3%). The mean age was 29.4 ± 12.8 years. Generalized tonic–clonic seizures were the most common semiology present in 71.1% of the study population. In the study, the most common precipitating factor was missed doses, accounting for 20%, followed by self-tapering of antiepileptic medications, which was around 17.8%. The most common drug used was levetiracetam (around 60%).

Conclusions: Finding from this study indicates that missed doses of anti-epileptic drugs (AEDs) are a major factor in the onset of breakthrough seizures, followed by self-tapering of AEDs. These factors have to be recognized and corrected to prevent mortality and morbidity as well as financial burden in people with epilepsy.

Abstract ID: 516: A Case of Chronic Progressive Ataxia with Hearing Loss

Mude Naik

Thanjavur Medical College, Thanjavur, Tamil Nadu, India

Background and Aim: A 52-year-old male, with no comorbidities, presented with ataxia, which was insidious in onset and progressive over a period of 1 year, associated with dysarthria and hard of hearing for a period of 6 months. On examination, the patient had staccato speech, sensorineural hearing loss, and spasticity of all four limbs with pancerebellar signs. He was clinically diagnosed as case of spinocerebellar ataxia. On neuroimaging, the patient was found to have superficial siderosis with lesions in brainstem, temporal lobe, cerebellum, and spinal cord.

Methodology: After informed consent was obtained, detailed history was taken and the patient was thoroughly examined. Relevant investigations were done. Neuroimaging was conducted. Genetic studies were sent and reports awaited.

Results: A 52-year-old male presented with chronic progressive ataxia with sensorineural hearing loss and spasticity of all four limbs, the characteristic triad of superficial siderosis, and was confirmed with neuroimaging also.

Conclusions: Superficial siderosis is an extremely rare condition, which usually presents in those with structural pathology or recurrent subarachnoid hemorrhage. Detailed investigations are needed to be labeled as idiopathic and if any treatable causes are diagnosed, they should be addressed.

Abstract ID: 517: Central Diabetes Insipidus, Optic Neuritis, and Cerebral Venous Thrombosis in a Young Male – A Rare CNS Mishmash

Sanchit Chouksey, Varun Kumar Singh, Rameshwar Nath Kumar Chaurasia, Akansha Kumar Jain

Institute of Medical Sciences, Varanasi (IMS, Varanasi), Uttar Pradesh, India

Background and Aim: Immunoglobulin G4-related disease (IgG4-RD) is a multisystem disease characterized by sclerosing lesions and elevated serum IgG4 levels. Neurologic manifestation is rare and varies in severity. Combined manifestation of cerebral venous thrombosis (CVT), optic nerve involvement, and central diabetes insipidus (CDI) in a patient with IgG4-RD has not been reported so far.

Methodology: A 26-year-old male presented with polydipsia and polyuria for 3 months and acute-onset, painful, bilateral, sequential (right followed by left) blurring of vision with right hemicranial headache for 2 months. His Glasgow Coma Scale score was 15. Visual acuity (VA) in the right eye was perception of hand movement with relative afferent pupillary defect and in the left eye was 6/12. Fundus examination revealed bilateral grade 1 papilledema.

Results: Laboratory workup showed raised erythrocyte sedimentation rate and C-reactive protein. Magnetic resonance imaging of the brain revealed hypertrophic cranial pachymeningitis, hypophysitis, and left transverse and sigmoid sinus thrombosis [. Autoimmune and thrombophilia profile was negative. Urine osmolality was low and serum osmolality was high, suggestive of CDI. Serum IgG and IgG4 levels were elevated (1694 mg/dl and 2.86 g/dl, respectively), confirming the diagnosis of IgG4-RD. He received pulse steroid therapy followed by oral steroid, anticoagulant, and desmopressin, which led to improvement in headache, polydipsia, polyuria, and vision at day 45

Conclusions: IgG4-RD may present with a constellation of neurologic symptoms and signs. A rare combination of CVT, CDI, and optic neuropathy as seen in the present case will need anticoagulant with desmopressin apart from immunosuppressant for a favorable outcome.

Abstract ID: 518: Limb Gridle Pattern of Myasthenic Syndrome – A Rare Case Report

Ramachandran MohanRaj

Thanjavur Medical College, Thanjavur, India

Background and Aim: Congenital myasthenic syndromes are a heterogenous group of genetic neuromuscular diseases. They pose a diagnostic dilemma, as features characteristically associated with neuromuscular disorders may be subtle. Here, we are reporting an 8-year-old male child who presented with limb gridle pattern of weakness and positive family history. His condition was evaluated as congenital myopathy, but finally turned out as congenital myasthenic syndrome with novel DOK7 mutation.

Methodology: An 8-year-old male child, second born out of third-degree consanguineous marriage, with no significant peripartum events presented with mild motor developmental delay associated with symmetrical proximal muscle weakness of all four limbs with spinal deformity and normal intelligence and no cranial nerve involvement. After getting informed and written consent from the guardian of the child, relevant investigations including serum creatine phosphokinase (CPK) were done. All investigations were found to be within normal limits. After counselling the parents, whole genome sequencing was done.

Results: Early age of presentation, limb gridle pattern weakness with significant family history and normal CPK, and electrophysiologic studies made us to suspect congenital myasthenic syndrome. Whole genome sequencing showed DOK7 mutation, and the child responded satisfactorily to oral salbutamol.

Conclusions: Early diagnosis and treatment of DOK7 congenital myasthenic syndromes (CMS) with salbutamol, which is inexpensive and safe, results in good prognosis.

Abstract ID: 519: Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay – Genetically Proven

Apsara P S, Murugan P K, Justin C

Madurai Medical College Madurai, Tamil Nadu, India

Background and Aim: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a neurodegenerative disorder, with the characteristic triad of slowly progressive cerebellar ataxia, spasticity, and peripheral neuropathy. Recent studies indicate that ARSACS is not limited to Quebec, but is variably present worldwide, including the novel spastic ataxia of Charlevoix-Saguenay (SACS) variant. However, only a few genetically proven cases of ARSACS have been reported from India.

Methodology: A 35-year-old male presented with gradually progressive unsteadiness and difficulty in walking since the age of 23 years; his gait was markedly unstable, and also, his speech was slurred. Third-degree consanguinity was present at the level of his parents, but no family member was affected with neurologic deficits. On examination, the patient had bilateral cerebellar signs with scanning speech, slow saccades, horizontal gaze-evoked nystagmus, truncal ataxia, and wide-based ataxic gait. Other neurologic examination revealed normal cognition and motor system with bilateral leg spasticity.

Results: T2-weighted brain magnetic resonance imaging showed multiple linear hypointensities in the pontine parenchyma. Superoanterior vermian and superior cerebellar peduncle atrophy, as well as mild atrophy of the cervical spinal cord were noted. Nerve conduction studies showed sensorimotor demyelinating polyneuropathy. Genetic testing showed a homozygous, pathogenic variant, c.7504C>T p.Arg2502Ter (73X/74X), on chromosome 13 in the SACS gene.

Conclusions: ARSACS must be suspected in early-onset cerebellar ataxia combined with spasticity and peripheral neuropathy. Typical radiologic features such as superior cerebellar vermian atrophy and linear pontine hypointensities greatly enhance the diagnosis. Ultimately, the diagnosis is confirmed by genetic studies.

Abstract ID: 520: Dirt Spasm: A Tale of Fighting Spirit in a Resource-Poor Setting

Rameshwar Chaurasia, Sanchit Shailendra Chouksey, Varun Kumar Singh, Akansha Jain

Institute of Medical Sciences, Varanasi (IMS, Varanasi), Uttar Pradesh, India

Background and Aim: Tetanus is a neuromuscular disorder caused by an anaerobic spore-forming bacterium, Clostridium tetani , found in soil and feces. Primary immunization programs have significantly reduced the incidence of tetanus in developed countries.

Methodology: A 50-year-old male belonging to poor socioeconomic status presented to us with episodic tonic spasm with a lock jaw following trauma to the right great toe. He was diagnosed with generalized tetanus and managed in a resource-poor setting with proper intensive care unit protocol despite having economic restraint.

Results: On admission, he was intubated and mechanically ventilated. He was kept in the intensive care unit in isolation. He was managed with antibiotics, intramuscular tetanus toxoid, diazepam, and other supportive treatment. Due to intermittent muscular spasm, injection diazepam was started and gradual escalation was done. After stabilization, diazepam was tapered gradually, and he was weaned from the ventilator. He was discharged on day 61 with no complications.

Conclusions: A high degree of suspicion and strict vigilance are needed, particularly in unimmunized patients, as the history of exposure is sometimes missing. Benzodiazepines, neuromuscular blockade, and mechanical ventilation are the mainstays of treatment. Careful monitoring and adherence to protocolized care help reduce the cost burden and mortality. Maintaining up-to-date tetanus immunization status ensures optimal protection against infection.

Abstract ID: 521: Bilateral SDH in a Patient with SIH Treated Successfully with Bilateral MMA Embolization and Targeted Epidural Blood Patch

Raja Kakollu, Jagadish B. Agadi, Sharath Kumar G G

Apollo Hospitals, Sheshadripuram, Bengaluru, Karnataka, India

Background and Aim: A 46-year-old male presented in April 2023 with a history of headache of 4 weeks duration, which was of occipital, moderate (5/10), throbbing type. It started following a helicopter ride in crouched position for a long time. There was positive Postural variation, which aggravates on sitting, standing, sneezing, coughing, with a severity of 8/10 and completely relieved in supine position. It was not associated with nausea, vomiting, giddiness, tinnitus, or visual obscuration. The patient did not have any comorbidity, nor he reported of any past history.

Methodology: Examination revealed bilateral papilledema, no cranial nerve deficits, and all DTRs were brisk. Imaging (will be included in the poster presentation)Ultrafast dynamic computed tomography myelogram was done followed by targeted epidural blood patch at D1–D2 level which was Uneventful, no complications and recovered. Repeat magnetic resonance imaging after a month showed resolution of spinal epidural collection, SDH. Two months later, he presented with recurrence following flight travel. He had similar orthostatic headache associated with nausea and vomiting. No deficits were found on examination. Imaging (will be included in the poster presentation) showed increase in bilateral subdural collection with multiple internal septations.

Results: Bilateral MMA embolization was done i/v/o SDH, followed by repeat epidural blood patch at the C7–D1 level. The patient recovered. There was no recurrence. Imaging on subsequent follow-ups showed gradual decrease in size of subdural collection. The patient has been asymptomatic since then.

Conclusions: Incidence of cSDH is on the rise, and it causes high morbidity and mortality. Middle meningeal artery embolization (MMA) is a promising alternative for chronic subdural hematoma (cSDH).

Abstract ID: 522: Unraveling the Mystery of Cancer’s Hidden Threat: PNET Presenting with Rapidly Progressive Dementia and Myoclonus, A Unique Paraneoplastic Syndrome

Nayana Bhuyan, Nayana Bhuyan, Arpan Mitra, Abhishek Pathak, Ankur Vivek, Akansha Jain

Banaras Hindu University, Varanasi (BHU, Varanasi), Uttar Pradesh, India

Background and Aim: Paraneoplastic neurologic syndrome (PNS) is an immune-mediated disorder affecting any part of the nervous system, in the presence of cancer. This syndrome is rarely reported in pancreatic neuroendocrine tumor (PNET). Here we present a case of a 60-year-old male presenting as seronegative PNS with clinical features of rapidly progressive dementia. Myoclonus due to PNET is rare.

Methodology: A 55-year-old male presented with forgetfulness of recent events, with apathy followed by mutism and sudden shock-like jerky movements of all four extremities since 7 months. On examination, Glasgow Coma Scale (GCS)- E4V1M5, grade II clubbing Mini-Mental State Examination (MMSE) 4/30, cortical myoclonus was present.

Results: Routine investigations showed RBS 54, glycated hemoglobin- 4.8, and C-reactive protein- 53. Cerebrospinal fluid random blood sugar 24. Magnetic resonance imaging of the brain showed bilateral T2/fluid attenuated inversion recovery hyperintensities of bilateral parasagittal parietal lobe as well as left temporal lobe, suggestive of encephalitis. Autoimmune, paraneoplastic, and vasculitic panel was negative. Patient responded to steroids with complete resolution in 2 weeks. CAP study was suggestive of neoplastic etiology (PNET).

Conclusions: PNS can be associated with PNET, which is very rare, and patient should be aggressively investigated for optimal treatment of these cases with rare presentation.

Abstract ID: 523: Development of a Duchenne Muscular Dystrophy Registry to Optimize Care

Ashish Sharma, Deepti Malik, Naveen Kumar Bhardwaj

AIIMS Bilaspur, Himachal Pradesh, India

Background and Aim: Duchenne muscular dystrophy (DMD) is the most prevalent and lethal of the inherited dystrophies. Globally, the incidence is reported at 1 in 3500 live male births. There is currently no cure for the disease. With the possibility of gene therapy becoming available, patients who would qualify for such treatment need to be identified. Furthermore, understanding the disease expression in a population is essential to focus targeted interventions, such as corticosteroids, to ensure they are safe and effective in the local setting. By maintaining a DMD-specific disease registry, this information can be obtained. This proposal will describe the concept and design of the DMD disease registry using Research Electronic Data Capture (REDCap).

Methodology: The registry will be developed using REDCap’s web-based online design, accessed through the Clinical Research Center in AIIMS Bilaspur. Electronic case report forms will be created from these clinical data using REDCap and for specific variables, serial entries will be possible relating to disease progression. International data standards will be adopted as proposed by TREAT-NMD (neuromuscular registry in Europe), a global network of registries on DMD, to ensure our data is compatible with this and other international registries.

Results: Retrospective data entry combined with dynamic prospective recording of data will be utilized in this project. Building on an existing database, previously confirmed DMD boys will be eligible for inclusion into the registry. As our registry will be on-going study, sequential analysis of accumulated data will be done going forward to review trends on our patients with DMD.

Conclusions: This proposal describes the concept and design of a DMD registry and the steps which will be followed for its establishment with REDCap. The focus is to consolidate clinical and genetic information of DMD, commencing with the local center’s patient cohort but rolling out access to other tertiary care centers to create a national resource, which is internationally relevant.

Abstract ID: 524: SLE and Myasthenia Gravis – An Unusual Coexistence

Veerappan V S, Saravanan S

Tirunelveli Medical College, Palayamkottai, Tirunelveli, India

Background and Aim: Systemic lupus erythematosus (SLE) and myasthenia gravis (MG) are two autoimmune diseases that have a relapsing–remitting course and positive antinuclear antibodies. SLE can precede or follow the development of MG. Coexistence of SLE and MG is a rare phenomenon.

Case Report: A 33-year-old female patient developed mononeuritis multiplex and was diagnosed with SLE 10 years back. Serum antinuclear antibodies and anti-ds DNA were positive. Anti-cardiolipin and antiphospholipid antibodies were negative. She was treated with six cycles of cyclophosphamide and was put on maintenance therapy with azathioprine, chloroquine, and steroids. She was admitted with slurring of speech, drooping of eyelids, difficulty in swallowing, and difficulty in walking. She had diurnal variations in her symptoms. On examination, she had proximal muscle weakness and signs of fatigability. Oral hydroxychloroquine (HCQS) was stopped, and there was no improvement even after 1 week of discontinuing this drug. Magnetic resonance imaging of the brain and chest X-ray were normal. Serum creatinine kinase was normal. Nerve conduction studies showed a decremental response of more than 20%.

Discussion: The findings were consistent with neuromuscular junction disorder. Anti-acetylcholine receptor antibody was positive. She was treated with oral pyridostigmine 60 mg twice daily. Clinical improvement was seen within 48 h.

Conclusions: In conclusion, the association between SLE and MG is complex. In MG patients who had undergone thymectomy, any clinical and serological suspicion of SLE should be carefully evaluated. In lupus patients complaining of decreased muscle strength and fatiguability, the possibility of MG should be ruled out.

Abstract ID: 525: A Case of Acute Brainstem Syndrome with Neurogenic Pulmonary Edema

Gopikrishnan R

Thanjavur Medical College, Tamil Nadu, India

Background and Aim: A case of acute brainstem syndrome with neurogenic pulmonary edema is described here.

Case Report: Patient was admitted with interactable hiccups/vomiting.

Discussion and Conclusions: Patient with NMOSD needs aggressive management. If not responding to pulse steroids, next line of management (intravenous immunoglobulin/plasmapheresis) should be immediately started. Neurogenic pulmonary edema will also improve with other neurologic symptoms.

Abstract ID: 526: A Case of Cerebral vasculitis Secondary to Rheumatoid Arthritis at the Burnt out End of the Disease

Naresh Chinthala, Arnesh Bhattacharya, Jyoti Garg, Ashish Kumar Duggal, Arnesh Bhattacharya

Thanjavur Medical College, Thanjavur, India

Background and Aim: CNS vasculitis is a rare complication of rheumatoid arthritis that is usually seen in advanced burnt-out stages of the disease. Patients may present with strokes, infarcts and hemorrhages with characteristically low CRP and ESR without active joint inflammation.

Case Report : A 60-year-old lady with a long-standing history of rheumatoid arthritis presented with a history of recurrent strokes, seizures and encephalopathy. She had multiple joint deformities without tenderness. ESR and CRP were low, but anti-CCP and RF were strongly positive. MRI brain with MRA brain and neck vessels showed multiple areas of cortical infarcts and microhemorrhages with eccentric narrowing of both supraclinoid internal carotid arteries without any vessel wall enhancement. CSF showed elevated protein-positive anti-CCP.

Discussion: The patient was given IV pulse methylprednisolone and later maintenance steroids with a plan for adding Rituximab. The patient showed significant cognitive as well as radiological improvement with corticosteroids.

Conclusions: In patients with a history of rheumatoid arthritis, particularly burnt-out disease, rheumatoid arthritis-associated vasculitis should be considered in the differential diagnosis of recurrent strokes. Treatment with steroids and other immunomodulators can provide substantial benefits.

Abstract ID: 527: Isolated Intramedullary Spinal Neurocysticercosis Managed Medically with Excellent Outcome

Saurav Raja, Ashish Kumar Duggal, Jyoti Garg, Naresh Chinthala, Ashish Kumar Duggal, Jyoti Garg Tamil Nadu

Background and Aim: Neurocysticercosis is one of the most common parasitic infections of the central nervous system and is caused by the larvae of the tapeworm Taenia solium. Spinal cysticercosis is a rare manifestation of neurocysticercosis, accounting for 0.7 to 5.85% of all cases. Isolated intramedullary spinal neurocysticercosis (NCC) can create challenges in terms of diagnosis and management.

Case Report: A 26-year-old male presented with complaints of backache for 2 months and insidious onset and progressive weakness of bilateral lower limbs for 2 weeks with early involvement of bowel and bladder. Upon neurological examination, the patient exhibited paraplegia with the lower limb muscle power of MRC grade 1 and a sensory level located at T6. Spinal imaging revealed a well-defined cystic lesion, hyperintense on T2 images and complete suppression on FLAIR. The patient was managed medically with oral steroids and anthelminthic drugs, and intensive physiotherapy was advised. The patient showed significant improvement and was able to walk independently within 2-3 weeks. The oral steroid was tapered slowly, and antihelminthics were continued for a period of 6 weeks. The patient improved completely over a period of 3 months.

Discussion and Conclusions: Intramedullary spinal neurocysticercosis is a rare but treatable clinical entity. The treatment includes both medical and/or surgical management. Steroids should be given along with medical therapy as they reduce therapy-associated inflammatory reactions. Surgical therapy is required when patients present with severe and progressive neurological deficits, which do not improve on medical therapy.

Abstract ID: 528: Stroke a rare Presentation of Pediatric Systemic Lupus Erythematosus

Anand Kumar, Deepika Joshi, Varun Kumar Singh, Abhishek Pathak, Rameshwer Nath Chaurasia, Vijaya Nath Mishra

Banaras Hindu University, Varanasi (BHU, Varanasi), Uttar Pradesh, India

Background and Aim: Central nervous system (CNS) involvement is relatively uncommon in pediatric systemic lupus erythematosus (SLE). Most common manifestation of CNS-SLE in pediatric patients is neuropsychiatric in the form of depression, memory impairment and psychosis.

Case Report: A 6-year-old girl presented with complaints of acute onset deviation of angle of mouth to right side, weakness of left upper and lower limbs and difficulty in speech progress over 3-4 hours. There are no history of headache, loss of consciousness, seizure, bladder and bowel incontinence or sensory loss. There was a significant history of low-grade fever with bilateral lower limb weakness 9 months back which improved significantly with residual mild spasticity in bilateral lower limbs. On examination, patient was conscious, irritable, aphasic but comprehended simple commands. There was left upper motor neuron-type 7th cranial nerve palsy and 3/5 power in left upper and lower limbs while normal power in right limbs. Reflexes were brisk in all 4 limbs with clonus at bilateral ankle and bilateral extensor planter reflex. Blood complete blood counts suggest mild anemia, and liver and renal function tests were normal. Magnetic resolution imaging (MRI) brain shows diffusion restriction left putamen, globus pallidus and right external capsule suggestive of small vessels infarcts. On further evaluation, the ESR and CRP were found to be very high, ANA 3+v (Homogenous), ANTI dsDNA +ve, PM-SCL ++, NUCLEOSOME++, HISTONE+ with low complement C2 and C3 levels. On the basis of ACR/EULAR classification criteria for SLE (2019), diagnosis of pediatric CNS-SLE was made and patient was started on intravenous (IV) pulse methylprednisolone for 5 days. Patient was also started on aspirin 37.5 mg/day and started on cyclophosphamide 500 mg/m2 IV once monthly.

Discussion and Conclusions: Childhood onset SLE has a poor prognosis, compared to adult onset SLE. Early diagnosis and prompt aggressive treatment may reduce the morbidity in such patient.

Abstract ID: 529: Ipsilateral Ictal Ptosis: A Rare and Localizing Manifestation of Epilepsy

Shalini S, Pradeep R

MS Ramaiah Medical College, Bengaluru, Karnataka, India

Background and Aim: Ictal ptosis, characterized by drooping of the eyelid during a seizure, is an exceedingly rare phenomenon. It is often overlooked as a diagnostic clue in epilepsy, especially in cases of focal seizures. This case report aims to highlight ictal ptosis as a valuable localizing sign in epilepsy, emphasizing its clinical significance and underlying pathophysiology.

Case Report: A 35-year-old female presented with focal seizures with impaired awareness since 10 years, presenting with a rare manifestation of ipsilateral ictal ptosis. The patient experienced recurrent episodes of sudden, transient drooping of the left eyelid, lasting for 30-60 seconds, accompanied by mild confusion and unresponsiveness with oral and limb automatisms. Her neurological examination between episodes was unremarkable. Electroencephalography (EEG) revealed focal epileptiform discharges originating from the left temporal region, correlating with the ictal events. Magnetic resonance imaging (MRI) of the brain showed left MTS. The patient was on multiple AEDs with minimal response.

Discussion: The patient responded well after the cortico-amygdalohippocampectomy (CAH).

Conclusions: Ictal ptosis, though rare, serves as a significant localizing sign in epilepsy. Clinicians should maintain a high index of suspicion for this presentation to facilitate accurate diagnosis and management. This case highlights the need for increased awareness of ictal ptosis among healthcare providers to improve outcomes in patients with focal epilepsy.

Abstract ID: 530: Oxygen Embolism due to Acute Hydrogen Peroxide Toxicity: Uncommon Presentation of Stroke With Quadriparesis - A Case Report

Devashish Ruikar, Vishrant Bharati, Gajanan Chavan, Shirish N. Patil

MIMSR Medical College Latur, Maharashtra, India

Background and Aim: Hydrogen peroxide is a commonly used surface disinfectant nowadays used in households, and in hospitals there are not much data of H2O2 toxicity in literature, specially of CNS toxicity.

Case Report: We present a case report of a 56-Yr-gentleman—dhobi by profession who accidentally consumed H2O2 which they use for cleaning stains over the clothes, after the 30-50 ml of oral consumption, immediately he had frothing of mouth, nausea and vomiting, and after 20-30 min he had breathing difficulty and then confusion. After hospital admission, he had gradual onset progressive weakness of right and then left-sided body leading to quadriplegia within 2 to 3 hours of the poisoning. He managed conservatory. After 1 week, his clinical condition and CNS weakness recovered and he was discharged power gr 4 in all 4 limbs. His MRI brain was s/o- Dw and flair changes in frontoparietal and occipital region with infarcts probably due to oxygen embolization.

Discussion and Conclusions: Hydrogen peroxide and its CNS manifestations are not much reported in medical literature, and more data are needed on rare and potentially fatal toxin.

Abstract ID: 531: Delayed Organophosphorus Compound Poisoning Induced Myeloneuropathy- A Rare Case Report

Sowmy Saravanan

Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry (JIPMER Puducherry), India

Background and Aim: Accidental or intentional ingestion of organophosphate is common in South India, mainly in agricultural community. It can present as acute cholinergic crisis, intermediate syndrome and organophosphate-induced delayed neuropathy (OPIDN). Neuropathy target esterase is thought to be the target of OPIDN initiation. It is characterized by distal degeneration of some axons of both the peripheral and central nervous systems occurring 1-4 weeks after exposures. Here, we describe the case of a 19-yr-old female presenting with OPIDN.

Case Report: A 19-yr-old girl, without significant family history, present with chronic progressive, spastic paraparesis, pure motor with wasting hypertonia hyperreflexia, without sensory, bowel and bladder or other cranial nerves, cerebellar or higher mental function involvement. The total duration of illness is 6 months, preceded by organophosphorus poisoning, with requirement of ventilator support and prolonged intensive care support 6 months prior to the symptom onset.

Discussion: Common etiologies for myeloneuropathy like B12, vitamin E and copper deficiency were ruled out, infectious etiologies like HIV HTLV 1 and Lyme CMV were ruled out, and whole exosome for inherited causes was also reported normal.

Conclusions: It has been proposed that OPIDN is caused by the covalent inhibition of neuropathy target esterase (NTE). For instance, the administration of potent NTE inhibitors in animals causes neuropathy, and mutations of the NTE gene are associated with slowly developing motor neuron disorders similar to OPIDN. The inhibition of the NTE activity by organophosphate may perturb the metabolism of important membrane phospholipids, potentially leading to neuropathy.

Abstract ID: 532: Rare Occurrence of Miller Fisher Variant of Guillain-Barré Syndrome Following Modified Radical Mastectomy in a Patient with Breast Cancer

Soumya Bhowmik

AIIMS Kalyani, West Bengal, India

Background and Aim: Guillain–Barré syndrome (GBS) is an acute immune-mediated polyneuropathy characterized by progressive symmetric weakness and areflexia. Miller Fisher variant of GBS is a rare subtype, presenting with a triad of ophthalmoplegia, ataxia and areflexia.

Case Report: A 64-year-old female with a history of invasive ductal carcinoma of the left breast underwent MRM surgery with axillary lymph node dissection. On the fifth postoperative day, she developed mild weakness and difficulty in eye movements. Her condition deteriorated over the following 48 hours, with the development of bilateral ophthalmoplegia. Neurological examination showed bilateral complete ophthalmoplegia with mild ptosis, gait ataxia without limb ataxia and absent deep tendon reflexes.

Discussion: Laboratory results, including complete blood count and metabolic panel, were within normal limits. Cerebrospinal fluid (CSF) analysis showed albumino-cytologic dissociation with a protein level of 134 mg/dl and 2 cells. Nerve conduction studies indicated the impersistence of late responses, while brain MRI results were normal. On the basis of clinical context and supportive investigations, the primary diagnosis of Miller Fisher variant of Guillain–Barré syndrome was kept.

Conclusions: The case underscores the importance of recognizing neurological complications in postoperative patients, especially those with underlying cancer. This report adds to the limited body of the literature on post-surgical MFS, suggesting a greater need for awareness and monitoring of similar cases in the future.

Abstract ID: 533: Trident sign in spinal cord sarcoidosis

Haritha S, Sowmini P.R., Mugundhan K

Stanley Medical College, Chennai, Tamil Nadu, India

Background and Aim: Spinal cord sarcoidosis is very rare, affecting only 1% of the patients with sarcoidosis. We present a case of spinal cord sarcoidosis magnetic resonance imaging (MRI) findings demonstrating the characteristic “trident sign,” which is pathognomonic for the same.

Case Report: A 45-year-old female presented with subacute onset of pain in the neck radiating along the spine till low back, of four months duration, numbness started in the foot ascended up to neck and stiffness of all four limbs since three months, tight band like sensation in the chest region and weakness of both hands in the past two weeks. On examination, wasting and weakness of bilateral hand muscles were noted along with spasticity in all four limbs, absent superficial abdominal reflex bilaterally, inverted biceps and triceps reflex, other DTR brisk bilaterally, plantar extensor; diminished fine touch and vibration sense below C5 level; and impaired joint position sense in all four limbs, with rest of the neurological examination being normal.

Discussion: MRI cervical spine was done which revealed LETM from CV junction to C7 vertebrae with trident sign—due to central canal subpial and crescent-shaped posterior subpial contrast enhancement, which is pathognomonic of spinal cord sarcoidosis. Other causes of LETM were ruled out. In addition, ACE levels were elevated and she had mild hypercalcemia, which were also in favor of the diagnosis of sarcoidosis.

Conclusions: Trident sign is a characteristic sign that should prompt the clinician to have high index of suspicion of spinal cord sarcoidosis. This is helpful especially in a resource-poor setting.

Abstract ID: 534: It is a CIDP but not Responding to First Line Medications. What is Next?

Prabhudev Hiremath, Mahendra V Javali, P T Acharya, Pradeep R, Anish Mehta, Lakshmipriya

MS Ramaiah Medical College, Bengaluru, Karnataka, India

Background and Aim: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a common entity but with myriad clinical presentations and complex pathophysiology. Lately, nodopathy and paranodopathy subgroup of disorders, which were initially considered as a part of CIDP spectrum gaining a traction in view of complicated pathogenesis, intricate clinical presentation and resistance to first-line anti-CIDP medications. European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) considers nodo-paranodopathy spectrum of disorders as a separate entity in view of varied pathophysiology, clinical presentation, treatment and prognosis.

Case Report: Patient was presented in neurology outpatient department in view of progressive weakness and ataxia since 3 months. She was admitted for evaluation. Her history, examination and nerve conduction study suggested the possibility of distal acquired demyelinating symmetric polyneuropathy (DADS). She was treated with IVIG, 2 gm/KG/Over 5 days. She showed transient improvement over few days to worsen again. After giving IVIG twice, she was given IV methylprednisolone (IVMP) in view of poor response to IVIg. However, she did not respond to IVMP. In view of typical CIDP picture with poor response to IVIG and IVMP, nodo-paranodopathy was suspected and evaluated for the same.

Discussion: Her serum showed positivity for antibodies against neurofascin 140 and 155 confirming our suspicion. She was treated with rituximab.

Conclusions: Acute or chronic demyelinating neuropathy with resistance to IVIg should prompt the work up for nodo-paranodopathy mediated neuropathy.

Abstract ID: 535: A Novel Radiological Diagnostic Marker in Ataxia Telangiectasia

Sai Apoorva Reddy, Thejus B, Archana Beckett Netto, Praveen S. Kumar, Nagaraja B.S, Sharath Kumar GG

Bangalore Medical College and Research Institute, Bengaluru (BMCRI Bengaluru), Karnataka, India

Background and Aim: Ataxia-telangiectasia (AT) is an autosomal recessive disorder characterized by progressive cerebellar degeneration, oculocutaneous telangiectasia, immunodeficiency and susceptibility to cancer as well as radiation toxicity. It is caused by pathogenic variants in the ataxia-telangiectasia-mutated (ATM) gene. MRI brain in patients typically demonstrates cerebellar volume loss, compensatory enlargement of the 4th ventricle, scattered small white matter T2 hypointensities are often identified in patients with AT indicating tiny hemosiderin deposits related to thrombosis or vascular leaks from telangiectatic vessels. In patients with AT on SWI imaging, the dentate nuclei can have deficient iron signals, which otherwise should be visible in SWI.

Case Report: We report a rare case of a 15-year-old female with ataxia telangiectasia with iron deficient signals in the bilateral dentate nuclei in the MRI brain sequence—SWI. A 15-year-old female with a normal birth and developmental history till the age of 2 years after which the patient presented with a progressive disease in the form of cerebellar ataxia, ataxic dysarthria, peripheral neuropathy, recurrent falls, oculomotor apraxia, recurrent respiratory tract infections, myoclonus, chorea, conjunctival telangiectasias. Laboratory tests showed mild iron deficiency anemia and serum alfa fetoprotein levels- 195 ng/ml, NCS of all 4 limbs showed bilateral axonal sensorimotor polyneuropathy, MRI brain with SWI was done, and it showed marked cerebellar atrophy with cerebral microbleeds and loss of iron signals in the bilateral dentate nucleus.

Discussion and Conclusions: Recognition of iron-depleted signals in the bilateral dentate nuclei may help in further radiological confirmation of this disease.

Abstract ID: 538: Quantification and Clinical Correlation of Posterior Cranial Fossa Cerebrospinal Fluid Volume in Primary Hemifacial Spasm using Magnetic Resonance Imaging

Rohan R Mahale, Anudeep Davaluri

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Primary hemifacial spasm (HFS) is due to neurovascular conflict (NVC) at root entry zone of the facial nerve. Whether reduction of posterior cranial fossa (PCF) cerebrospinal fluid (CSF) volume is a risk factor for HFS is not clear. The study aims at the radiological assessment of PCF CSF volume and its clinical correlation.

Methodology: 50 cases of primary HFS and 50 age- and sex-matched controls were recruited. PCF CSF volume was quantified in 3-T brain MRI (3D-DRIVE seq) using 3D Slicer software version 5.0.3 r30893/7ea0f43. Space between outline of brainstem/cerebellum (inner boundary) and posterior fossa endocranium (outer boundary) was PCF CSF volume.

Results: The mean age at presentation of cases was 50.7 ± 10.7 years (42-69 years), and controls were 52.4 ± 8.7 years (45-68 years). The mean duration of symptoms was 3.5 ± 1.3 years (1.5 to 8 years). About 52% of patients had grade 2 (mild) severity of HFS. The mean PCF CSF volume of patients was 13725.1 ± 909.5 mm3, and controls were 14458.5 ± 973.5 mm3 (p < 0.001). The mean PCF CSF volume of females with HFS was 13714.8 ± 852.5 mm3, and female controls was 14521.8 ± 973.5 mm3 (p = 0.006). PCF CSF volume was significantly associated with the presence of HFS (p = 0.007), the severity of HFS (p < 0.001) and the presence of NVC (p = 0.02).

Conclusions: PCF CSF volume was lesser in HFS patients and was associated with the presence of HFS, the severity of HFS and the presence of NVC. Females with HFS had smaller PCF CSF volume. Small PCF CSF volume is a risk factor for HFS particularly in females with HFS.

Abstract ID: 539: Gabapentin and Pregabalin on RLS patients on Hemodialysis in Relation to Anxiety and Depression

Ruchika Tandon Tandon, Ajay Chavan, Varsha Ambwani, Anupama Kaul, Vimal Paliwal

Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow (SGPGI Lucknow), Uttar Pradesh, India

Background and Aim: Restless legs syndrome (RLS) in renal failure patients is bothersome, and there is a limited data on the utility of pregabalin and gabapentin in these patients with comorbid anxiety and depression. Hence, we compared the effect of gabapentin and pregabalin in chronic kidney disease (CKD) patients on maintenance hemodialysis (MHD) with restless legs syndrome with regard to anxiety and depression.

Methodology: We classified 100 RLS patients on MHD into anxiety- and depression-based categories and gave them either pregabalin or gabapentin and compared treatment response in them at 3 months using t tests.

Results: Patients on both pregabalin and gabapentin improved significantly at 3 months, and there was no significant difference in between gabapentin and pregabalin. However, the anxiety group improved more at 3 months and gabapentin was better than pregabalin at three months in those with anxiety. Also, there was no significant difference between different age and sex groups with regard to their response to these two drugs at 3 months.

Conclusions: Hence, we concluded that both pregabalin and gabapentin may be equally effective in treating RLS in MHD patients. Gabapentin may be a better choice in the anxious patients.

Abstract ID: 540: Cerebellar Cognitive Affective Syndrome in Cerebellar Disorders due to Various Etiologies: A Study of 30 Patients

Himanshu Kaushal, Rajendra Kumar Sureka, Jatinkumar Jain, Madhugandha Wahane, Amit Kumar Agarwal, Jatinkumar Jain

Mahatma Gandhi Medical College and Hospital, Jaipur, Rajasthan, India

Background and Aim: Cerebellar cognitive affective syndrome is the syndrome of neurocognitive symptoms and signs including executive function, linguistic processing, spatial cognition and affect regulation with or without motor and/or balance symptoms.

Methodology: Cross-sectional study was conducted in the Department of Neurology in tertiary care hospital in North-east Rajasthan in India. Thirty patients were recruited. Inclusion criteria are patients with cerebellar symptoms/signs with age more than 18 years. Exclusion criteria: other causes including hepatic and renal failure, dyselectrolytemia, thyroid disorder, etc., were excluded. The cognitive functions were studied in the patients using the two assessment tools, namely cerebellar cognitive affective syndrome scale (CCAS-S) and mini-mental state examination (MMSE) scale.

Results: 70% were males, 66.66% were illiterate, out of 30 patients, 11 patients had vascular events, 8 had degenerative ataxia, and 6 had drug-induced cerebellar signs, 3 MSA-C, 2 alcohol-induced cerebellar degeneration. A total of 228 patients had definitive CCAS. 93.33% failed in semantic, 90% in phonemic fluency task, 93.33% in category switching, 86.66% and 93.33% in digit forward and backward, 70% and 80% in registration and recall, and 73.33% in cube drawing/copying. 66.6% had inattention. 60% had blunted affect.

Conclusions: CCAS is highly underdiagnosed and underreported condition. While assessing cognitive impairment in patients with cerebellar lesions, it can be inferred that the routinely administered screening MMSE scale can underrate the patients of CCAS because it is generally normal or shows mild cognitive impairment CCAS scale on the other hand has yield for diagnosis of cerebellar cognitive affective syndrome.

Abstract ID: 542: Cognitive, Neuropsychiatric and Electrophysiological Assessment of Post Covid-19 Patients- A 6 Months Trajectory

Rahul Naik, Samhita Panda, Anka Arora, Gopal Krishna Bohra

All India Institute of Medical Sciences, Jodhpur (AIIMS, Jodhpur), Rajasthan, India

Background and Aim: Post-COVID-19 condition, a constellation of cognitive decline, neuropsychiatric manifestations and fatigue has been observed in individuals who have recovered from COVID-19. The extent of affected population and progression of the disorder have been areas of interest.

Methodology: We conducted a prospective observational study at a tertiary care hospital on patients who had COVID-19 infection at least 6 months prior. Cognitive functions, anxiety, depression and fatigue were assessed. P300 was performed, and the same set of tests were repeated after 6 months.

Results: Among 102 patients with history of COVID-19 infection >6 months prior, 38.2%at baseline and 43.84% at 6 months were cognitively impaired. A high incidence of anxiety (80.4%) and depression (69.6%) was found. 46.1% experienced significant fatigue at baseline. 48%at baseline and 61.64% at 6 months had prolonged P300 latency. All scales including frontal assessment battery (FAB), Hamilton anxiety scale (HAM-A), Hamilton depression rating scale (HDRS) and fatigue severity scale (FSS) were found to be more significantly affected in cognitively impaired group (<0.05).

Conclusions: The study underscores those long-term neuropsychiatric implications of COVID-19, including cognitive decline, anxiety, depression and fatigue can present even years after infection and may also worsen progressively. Anxiety was more prevalent in our patients than in previous studies, while depression and fatigue were observed to increase with time in the post-COVID-19 condition. Interestingly, abnormality in P300 latency and amplitude was found even in patients in whom cognitive decline was not found clinically with additional worsening with time.

Abstract ID: 543: Diagnostic Accuracy of Imaging Findings in Tubercular Meningitis and Spinal Tubercular Arachnoiditis and Correlation with Outcomes -An Ambispective Cohort Study

Chitrangada Yadav, Elavarasi Arunmozhimaran, Ajay Garg, Deepti Vibha, Manjari Tripathi, Rajesh Singh, Animesh Das, Rohit Bhatia, Mamta Bhushan, Vishnu VY, Achal Srivastava, Awadh Pandit, Anu Gupta, Ayush Agarwal, Devyani Garg, Jasmine Parihar, Deepti Vibha, Animesh Das, Vishnu VY

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Approximately 10 million people are infected with tuberculosis each year, especially immunocompromised patients, such as HIV-infected individuals or people with risk factors like under-nutrition, diabetes, smoking and alcohol consumption 1. Tubercular meningitis is a paucibacillary, infection of the central nervous system caused by mycobacterium tuberculosis, and is the commonest cause of chronic meningitis in tropical countries and a major public health concern.

Methodology: The inclusion criteria included all patients who were admitted in neurology, general medicine or geriatric medicine or medical oncology wards with chronic meningitis diagnosed on the basis of composite clinical criteria, imaging criteria as well as natural history of the illness, and were at least 14 years of age of all sexes. Patients who are positive for antibodies to HIV and pregnant females were also included.

Results: The mean age in TBM group was 32 years and in non-TBM group was 30.64 years. Headache followed by fever was the most common symptom in both the groups. The specificity of focal nodular enhancing lesions was 93%, and spinal arachnoiditis was 81%.

Conclusions: Imaging findings suggestive of tubercular meningitis did not significantly correlate with a modified Rankin Scale at 3.

Abstract ID: 544: Polyneuropathy due to N Hexane Intoxication – A Case Series

Pradeep Lalwani, Shubhakaran Khichar, Amita Bhargava, Nidhi Yadav

DR. S N Medical college, Jodhpur, Rajasthan, India

Background and Aim: N-hexane, a volatile organic chemical, is notorious for its neurotoxicity in occupational settings. The early recognition of n-hexane toxicity manifestations is essential to prevent its harmful effects in persons dealing with the substance. We present four patients of polyneuropathy after exposure to hexane by inhalation.

Methodology: Three males and one female, aged between 32 and 45 years, are working in a needle manufacturing factory for the past 10 years. In September 2023, due to malfunctioning of exhaust of furnace burners due to which gas particles of mixture (N-hexane, silicone and isopropylene) could not be burnt out, patient was exposed to gas through inhalation. Among the 40 workers working in the plant, four were directly involved in mixing process and they developed neuropathy symptoms and hence they were evaluated for cause.

Results: We found that all patients presented with painful slowly progressive weakness and numbness for 1 month. Cerebrospinal fluid showed albumino-cytological dissociation. NCV was significant of primary demyelinating with secondary axonal sensory motor polyneuropathy which in contrast differs from toxin neuropathy which is primary axonal. Excluding the other possible treatable underlying pathologies, the diagnosis of n-hexane neuropathy was kept based on the occupational history and toxic substance exposure. Patients were managed with symptomatic treatment and had marked clinical and electrophysiological improvement on 6-month follow-up.

Conclusions: N-hexane neuropathy should be considered in a patient with subacute or chronic neuropathy if there is history of prolonged to this chemical in a poorly ventilated workplace.

Abstract ID: 545: Spectrum of Clinical Profile & Follow-Up of Adenovirus Associated Neurological Disease in Children – in a Tertiary Care Institution in South India

Muhammed Shadique, Mohinish Sundar, Sonia Rosalind Martina, Shree Shruthi Badrinath, Neeraj Elango, Jered Livingston, Leema Pauline

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: To characterize the spectrum and salient clinical features of adenovirus-associated neurologic disease in children and to analyze outcome based on mRS scale

Methodology: A prospective observational study was conducted between January 2023 and August 2023 among children admitted with CNS disease associated with adenovirus infection at the Institute of Child Health, Madras Medical College. Demographic details, age at presentation, presenting complaints, neurological manifestations, investigations done, imaging findings, treatment given and outcome were analyzed. Inclusion criteria were (1) children 0–12 years of age, (2) confirmed adenovirus infection by throat swab PCR and (3) the presence of CNS manifestations like seizures and encephalopathy. Exclusion criteria were 1) children without CNS symptoms and 2) children with preexisting neurologic disease.

Results: A total of 150 adenovirus-positive cases were admitted during the study period, among which 20 (13.3%) had neurological manifestations.

Conclusions: Early suspicion and focused management of adeno-associated illness during the epidemic is the key to prevent long-term sequelae. Standardized protocols regarding the use of antivirals and immunotherapy in such cases are required.

Abstract ID: 546: Assessment of Non-Motor Symptoms in Motor Neuron Disease- A Case-Control Study

Kheta Sharma

All India Institute of Medical Sciences, Jodhpur (AIIMS, Jodhpur), Rajasthan, India

Background and Aim: Motor neuron disease (MND) is a progressive neurodegenerative disorder involving motor neurons. The emergence of non-motor symptoms (NMSs) is attributed to the spreading of pathology to other areas of the brain. The “disease spreading hypothesis” provides a more comprehensive explanation for the diverse NMSs observed in neurodegenerative diseases. Our study aimed to investigate the spectrum of NMSs in patients with MND and to draw comparisons between different subgroups, shedding light on the broader implications of the disease-spreading hypothesis.

Methodology: This study enrolled 22 patients diagnosed with MND along with 22 healthy controls. Evaluation using the non-motor symptoms score (NMSS) was conducted, and relevant statistical analyses were performed to derive various results.

Results: Our comprehensive study focused on spectrum of NMSs, shedding light on the overall burden experienced by patients with MND. The MND group exhibited significantly higher total NMSS scores compared to healthy individuals (20.77 ± 17.14 vs. 3.45 ± 4.90; P value = 0.000). We observed a significantly higher frequency of NMSs in MND patients (90.9%) compared to controls. Of these, the most significant non-motor symptoms in MND patients were in the domain of fatigue (86.4%), dysphagia/choking (36.4%) and unexplained pain (22.72%).

Conclusions: The higher prevalence of NMSs in the disease group compared to the control group supports the hypothesis that MND may be characterized as a “multisystem disease.” This explains the importance of integrating the evaluation and management of NMSs into a comprehensive and contemporary healthcare approach for individuals with MND.

Abstract ID: 547: Study of Etiology, Clinical Spectrum and Associated Features of Delirium in Patients Admitted in Neurology Ward of a Tertiary Care Centre: A Case Control Study

Arpan Mitra, Abhishek Pathak

Institute of Medical Sciences, Varanasi (IMS, Varanasi), Uttar Pradesh, India

Background and Aim: Delirium, one of the oldest disorders known to medicine, was well described 2,500 years ago in the medical writings of Hippocrates. The word “delirium” was first used as a medical term to describe mental disorders occurring during fever or head trauma. Delirium is an acute mental status change characterized by abnormal and fluctuating attention. There is a disturbance in level of awareness and a reduced ability to direct, focus, sustain and shift attention. The syndrome of delirium can be a physiological consequence of a medical condition or stem from a primary neurological cause.

Methodology: Study design: This will be a case-control study, admitted to a neurology ward over a 1.5-year period. Participants Cases: All consecutive cases with a diagnosis of delirium admitted in neurology ward would be enrolled. Inclusion criteria for cases were patients aged 18 years and over, admitted to a neurology ward with evidence of delirium or developed incident delirium during hospitalization Controls: Control patients would be selected randomly from medical patients who had no diagnosis of delirium during admission. Cases and controls would be stratified according to admission location and equal ratio of one case, and two controls would be selected.

Results: A total of 120 cases were included in the study. Accordingly in 1:2 ratio, 240 controls are taken. Mean age of patients is 59.37 ± 18.68 years. Male/female ratio is 67:53. Most common cause of delirium found is hyponatremia followed by stroke and meningoencephalitis. Most common cause of hyponatremia is SIADH.

Conclusions: Delirium is a common presentation of neurological diseases. Prospective randomized trials are needed to further investigate spectrum of delirium.

Abstract ID: 548: Electrophysiological Profile of Guillain Barre Syndrome Patients and their Functional Outcome in a Tertiary Care Centre – An Observational Study

Reshma Venugopal

Government Medical College, Trivandrum, Kerala, India

Background and Aim: To determine the association between the electrophysiological profile and functional outcome of Guillain–Barre syndrome patients as assessed by Hughes score.

Methodology: Prospective observational study over 18 months among 96 Guillain–Barre syndrome patients admitted in the Neurology department and the electrophysiology study done within 2 weeks of symptom onset or at the time of admission and at the time of discharge. The baseline data and clinical characteristics at the time of admission were collected with a structured proforma, and the quality of life and functional outcome were assessed by the MRC sum score and Huges score both at the time of admission and discharge. Institutional ethics committee clearance is obtained.

Results: The frequency distribution of the GBS variants in our study population is AIDP constituting 36.5%, and axonal variants constituted 32.2% AMAN, 25% AMSAN and 15.6% AMAN with reversible conduction block and the rest 6.3% by Miller Fisher variant.

Conclusions: Electrophysiology has a major role in diagnosis and predicting the prognosis of GBS patients. Knowledge of these factors can be helpful in the betterment of management and prognostication of patients with GBS.

Abstract ID: 549: Evoked Potential Changes in Patients with Parkinsonism - An Observational Study

Hemant Luniwal, Samhita Panda, Samhita Panda

All India Institute of Medical Sciences, Jodhpur (AIIMS, Jodhpur), Rajasthan, India

Background and Aim: Background: Parkinsonism is defined as a clinical syndrome characterized by tremor, rigidity, bradykinesia and postural instability. Nonmotor symptoms (NMSs) such as autonomic, sleep, cognitive and sensory symptoms are increasingly being recognized as a major cause of disability in parkinsonism and contribute to the decline in quality of life. Most sensory symptoms are clinically subtle in parkinsonism. This study aims to demonstrate subclinical sensory involvement using electrophysiological evaluation of evoked potentials in parkinsonism. Aim: To assess visual, auditory and somatosensory evoked potential in patients with Parkinsonism.

Methodology: This study enrolled 30 patients diagnosed with parkinsonism based on history and clinical examination and 28 healthy controls. Evoked potentials [(visual evoked potentials (VEPs), brainstem auditory evoked potentials (BAEPs), somatosensory evoked potentials (SSEPs)] were done in all cases and controls. Pertinent statistical analyses were performed to analyze results.

Results: This study revealed that compared to healthy controls latencies of N75, P100, N145 latencies of VEP, individual latencies of wave I to V of BAEP and N20, P22, P37 and N45 latencies of SSEP were prolonged in parkinsonism and were also statistically significant (p <0.05). It was also noted that in majority, the latencies of evoked potentials were more prolonged in idiopathic Parkinson’s disease (PD) compared to atypical PD though not statistically significant.

Conclusions: Nonmotor symptoms including sensory, visual and olfactory symptoms are common in PD and difficult to recognize and assess based on usual scoring systems. The evoked potentials can be used to objectively quantify and confirm the presence of central conduction abnormalities and therefore can be used to assess disease progression by comparing evoked potentials during follow-up period.

Abstract ID: 550: Uncommon Sequelae of a Common Poisoning

Karnati Kumar, Sanjeev Kumar Bhoi, Sanjeev Kumar Bhoi

All India Institute of Medical Sciences, Bhubaneswar (AIIMS, Bhubaneswar), Odisha, India

Background and Aim: Background: Organophosphorus (OP) compound poisoning clinically presents with acute cholinergic symptoms, intermediate syndrome, and rarely as OP-induced delayed neuropathy and myeloneuropathy. The thoracic cord is predominantly affected. The pathogenesis of this myeloneuropathy in humans is still not well perceived. Aim of Study: To determine the clinical features and predictors of the development of delayed myeloneuropathy in patients with OP poisoning.

Methodology: Patients of OP ingestion with delayed myeloneuropathy, presented to the neurology OPD, were evaluated with detail poisoning history, clinical examination, MR imaging, nerve conduction, electromyography studies, visual, auditory, somatosensory and motor evoked potentials to elicit the pattern of disease manifestation and progression.

Results: Among the 19 patients who were included, 10 (52.6%) patients had consumed chlorpyrifos and permethrin composition. Eleven (57.8%) patients had undergone gastric lavage. Six (31.5%) had experienced acute worsening, and 10 (52.9%) patients had developed intermediate syndrome. OPIDN had appeared after a median of 4 (1–8) weeks after the poisoning. All patients had lower limb hypertonia with wasting and distal more than proximal weakness with pure motor or sensorimotor involvement. MRI showed thoracic cord atrophy in 3 (15.7%) patients. MEP study revealed reduced amplitude in lower limbs on lumbar stimulation but absent on cortical stimulation in 5 (26.3%) patients.

Conclusions: Thoracic cord atrophy was associated with myeloneuropathy after organophosphorus poisoning as delayed complications. Early gastric lavage may delay the progression of OPIDMN. Further research is needed to evaluate pathogenesis of organophosphorus-induced thoracic involvement.

Abstract ID: 551: Impact of Inpatient Neurology Liaison Services: An Audit in a Rural Indian Teaching Hospital

Tejaswini Shanubhogue, Soaham Desai, Arth Jayeshkumar Shah, Sachin Khanchand Udwani, Manthan Manesh Dave

Shree Krishna Hospital & Parmukhswami Medical College, Anand, Gujarat, India

Background and Aim: Liaison neurology, a service where neurologists assess inpatients admitted under other specialties, plays a crucial role in managing complex neurological cases, which often remains under-recognized. We sought to quantify this “hidden work” and evaluate its impact. Our objectives were to audit inpatient neurology liaison consultations, evaluate the demographics, reasons for consultations, diagnoses and management plans and assess its impact on patient care.

Methodology: In this prospective cross-sectional study, we analyzed all neurology referrals (n = 236) sequentially generated over three months from September to November 2022 at XXXXXX. We collected data on patient demographics, referral characteristics, diagnoses, management plans and impact of service on patient care and outcomes using a standardized proforma. Statistical analysis was performed using Microsoft Excel and SPSS 20.

Results: Most referrals were from critical care (48.7%, 95% CI: 42.3-55.1%), medicine (18.6%, 95% CI: 13.7-23.5%) and emergency (11.9%, 95% CI: 7.8-16.0%). Neurology referrals were seen in mean time of 196.13 minutes (95% CI: 171.11-221.15, SE = 12.70) for routine and 4.55 minutes (95% CI: 4.02-5.06, SE-0.254) for urgent referrals. Mean consultation time per patient was 22.9 minutes (CI-21.88-23.91, SE = 0.52) excluding the time for documentation and intradepartmental discussion. An impact in management by formulating definitive diagnosis (50.8%), new investigations (83.5%) and treatment plan suggestion (95.3%) was seen.

Conclusions: There is a substantial impact of inpatient neurology liaison services in a rural Indian tertiary care hospital, and this “hidden work” needs to be acknowledged. Investment in efficient neurology liaison services is necessary to improve patient care and outcomes.

Abstract ID: 552: Prospective Evaluation of Visual Pathway for Early Detection of Ethambutol induced Optic Neuropathy

Rizwan Mohammad, Netravathi M, Vikram V. Holla, Nagarathna S, Jitender. Saini

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Systematic analysis of patients who were taking ethambutol is to identify the early signs of optic nerve damage and prevent irreversible vision loss.

Methodology: Patients who were diagnosed with TB meningitis were taken as cases. Patients who presented to OPD, had completed one year of ATT and were in the maintenance phase without ethambutol were taken as controls. Both cases and controls underwent detailed neurological, VEPs and ophthalmological (visual acuity and OCT) examination.

Results: In the present study, 12.7% had an acute presentation, and the majority (40%) presented with a headache, followed by fever (33%). In controls, 15% had an acute presentation, and the majority (29%) presented with a headache, followed by altered sensorium (17%). The P100 latencies of the visual evoked potentials of the cases at baseline and follow-up at 6 months in both eyes were found to prolong significantly (p <0.0001). The P100 latencies of cases at baseline and controls at 12 months follow-up in right eyes were found to be prolonged significantly (p < 0.031). The P100 latencies in cases at 6 months and controls at follow-up at 12 months in left eyes were found to be prolonged significantly (p < 0.029). The RNFL showed thinning of almost all layers in visual quadrants when compared to baseline in cases and 6 months at follow-up.

Conclusions: The present study suggests that prolonged exposure to ethambutol increases the risk of visual dysfunction in patients with TBM. Further research to validate and expand on our findings, larger sample sizes and longer follow-up times is required.

Abstract ID: 553: Intriguing Cases of Atypical Central Nervous System Tuberculosis: Unveiling the Uncommon

Shashanka Chillapuram, Sandhya Manorenj

Deccan Institute of Medical Sciences, DMRL X road, Santosh nagar main Rd, Kanchan Bagh, Hyderabad Telangana, India

Background and Aim: Central nervous system (CNS) tuberculosis presents significant diagnostic and therapeutic challenges, particularly in its atypical forms, which can mimic other neurological conditions. This presentation aims to highlight the diversity of atypical CNS tuberculosis cases, focusing on unique clinical, radiological and pathological features to enhance diagnostic awareness and improve patient outcomes.

Methodology: A cross-sectional study of atypical CNS tuberculosis cases was conducted at a tertiary care center in South India from January 2023 to May 2024. Detailed patient histories, clinical presentations, imaging studies, laboratory results and treatment regimens were reviewed. Cases were selected based on their deviation from typical presentations, including unusual symptoms and rare imaging findings.

Results: A total of 10 patients with atypical manifestations of central nervous system tuberculosis were identified. The mean age of the study population was 35.5 years, with a predominance of females (60%). Among the included patients, atypical presentations are seen as follows: 1) brain tuberculoma with paradoxical Longitudinally extensive transverse myelitis (n = 1), 2) chronic suppurative otitis media with TB meningoencephalitis (n = 2), 3)subacute onset of hiccoughs and vomitings secondary to tuberculoma involving medulla oblongata (n = 1), 4) rare involvement of trigeminal nerve with CNS TB (n = 1) 5) provoked cerebral sinus venous thrombosis secondary to CNS TB (n = 1), 6) normal pressure hydrocephalus secondary to CNS TB (n = 1), 7) pregnancy with TB meningoencephalitis with CSF Gene XPERT positivity (n = 2), 8) extensive basal exudates and meningeal enhancement resembling spider leg appearance and 9) TB meningoencephalitis presenting as HSV-1 encephalitis involving temporal cortex and subcortex.

Conclusions: Atypical CNS tuberculosis represents a diagnostic challenge due to its varied presentations and non-specific findings. Increased awareness and understanding of these uncommon cases can lead to earlier diagnosis and improved patient outcomes.

Abstract ID: 554: Efficacy of Metformin in Idiopathic Intracranial Hypertension: A Randomized Double Blind Placebo Controlled Trial

Yatharth Dixit, Vivek Lal, Ashish Kumar Kakkar, Aastha Takkar Kapila, Karthik V M, Samir Malhotra, Rimesh Pal, Basav Raj Tigari

Post Graduate Institute of Medical Education and Research, Chandigarh (PGIMER Chandigarh), India

Background and Aim: Idiopathic intracranial hypertension (IIH) is a disorder commonly seen in obese women of child-bearing age and has been considered akin to metabolic syndrome. Management includes weight loss, oral pharmacological agents and surgical procedures. Given the lipid-lowering and pleiotropic effects of metformin, we aimed to assess efficacy of oral metformin as compared to standard management in patients of IIH.

Methodology: This was a prospective, double-blind, randomized controlled trial carried out on 34 patients of IIH in a tertiary care center. The study population constituted patients meeting inclusion criteria as per revised Friedman (2013) and ICHD-3 criteria. Primary outcome was to assess efficacy of oral metformin in improvement of perimetric mean deviation (PMD), and secondary outcomes consisted of weight reduction, improvement in Composite Visual Outcome (CVO) and Headache Impact Test-6 (HIT-6) and frequency of adverse effects.

Results: Baseline characteristics of two groups were comparable. Significant weight loss was noted in the metformin group as compared to standard therapy. Improvement in PMD was also noted on addition of metformin though the difference was not statistically significant. CVO and HIT-6 also did not show any significant difference. Two patients discontinued intervention due to severe adverse effects within first week of initiation of therapy itself.

Conclusions: We conclude that oral metformin is an efficacious, safe and readily available option for weight loss in patients with IIH. A larger sample size and longer duration are required to ascertain benefits of visual outcome.

Abstract ID: 555: Evolution of Central Nervous System Tuberculoma with Different Anti-tubercular Therapies on Serial Magnetic Resonance Imaging: A Retrospective study

Potluri Vivek, Atchayaram Nalini, Jitender Saini, Netravathi M, Seena Vengalil

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: The optimal treatment guidelines and duration of ATT in CNS tuberculoma are still unclear. This study aimed to describe the evolution of CNS tuberculomas on serial MRI with three different ATT regimens.

Methodology: There is a retrospective, observational, monocenter study of serial MRI brain imaging findings of tuberculomas in patients receiving different ATT regimens. Sixty-eight patients who were clinically and radiological presumed to have CNS tuberculomas were included in the study. HIV-positive patients and pregnant women were excluded. Three different ATT regimens used were: a) Group A: HRZE in the intensive phase followed by HR in the maintenance phase, b) Group B: HRZEM in the intensive phase followed by HRM in the maintenance phase and c) Group C: HRZE in the intensive phase followed by HRZ in the maintenance phase. Serial MRI scans were done at 0, 6, 12 and 18 months after starting anti-tubercular treatment.

Results: Twenty-eight patients (41.2%) showed complete resolution of tuberculoma, among which the highest number of patients were present in group A (60%), followed by group B (33.3%) and group C (27.3%). By 18 months, 25 patients (36.8%) had complete resolution of tuberculomas, 2 patients (2.9%) had near complete resolution, 29 patients (42.6%) had more than 50% resolution of tuberculoma size and 12 patients (17.6%) had less than 50% decrease in size of tuberculoma.

Conclusions: Rate of resolution of tuberculomas did not improve with the addition of an extra drug to the conventional HRZE + HR regimen nor did it lead to earlier resolution of CNS tuberculomas.

Abstract ID: 557: To Study the Prevalence of Headache and its Characterization after Intermittent Photic Stimulation in Patients Undergoing EEG - A Hospital Based Study

Iqbal Singh, Monika Singla Singla, Gagandeep Singh Singh

Dayanand Medical College, Ludhiana (DMC, Ludhiana), Punjab, India

Background and Aim: Migraine and epilepsy are both common neurological disorders, with important similarity of both being episodic disorders. Also, the post-ictal headache is mainly migrainous in nature. Intermittent photic stimulation during EEG recordings may contribute to a correct diagnosis of ictal headache. Headache, epilepsy and photosensitivity, these all three conditions are known to facilitate each other. Recognition of headache as an epileptic manifestation per se represents a challenge yet. The aim of this study was to study the prevalence of headache and its characterization in patients undergoing EEG with intermittent photic stimulation.

Methodology: An observational study was conducted at EEG Laboratory, DMCH from December 1, 2022, to December 1, 2023. Patients with age > 8 years presenting to OPD, emergency, ward, undergoing an EEG were included. A structured interview was conducted using a standardized questionnaire via Google Forms shared on WhatsApp. Patients were categorized into migraine-like headache group and other headaches group.

Results: Out of 249 patients, 30.5% patients had history of migraine, and 18.1% had family history of migraine. Headache after EEG/IPS was noted in 12.9% of patients. Out of the 84 patients who had headache after the EEG/IPS test, and 59.2% had past history of migraine as compared to 22.5% patients without past history of migraine (p value <0.001).

Conclusions: Post-ictal headache is often neglected, regardless of its severity. The physician should bear this association in mind when dealing with epileptic and migraine patients to be able to offer such patients an accurate diagnosis and appropriate treatment.

Abstract ID: 558: Continuous Video EEG: Role in Diagnosis and Management of Children with Medically Intractable Epilepsy in a Developing Health Care System

Ira Chaudhary, Ajay Goenka Goenka

Mahatma Gandhi Medical College & Hospital, Jaipur, Rajasthan, India

Background and Aim: CVEM is one of the most important investigations for the correct diagnosis and management of unclassified paroxysmal events and medically intractable epilepsy. The objective of study is to emphasize the utility of continuous video-EEG monitoring (CVEM) at a community hospital in the developing healthcare system of India.

Methodology: The study was a prospective analysis of children, at a community hospital in India from Nov 2023 to Jan 2024; with an age range from 5 to 18 years. The patients with a diagnosis of medically intractable epilepsy who underwent CVEM with their target episodes captured were included in the study. Out of 56 patients, 40 were enrolled in the study based on the inclusion criterion. Variables including age, seizure semiology, routine EEG, MRI Brain, number of anti-seizure medications and duration of seizures were assessed.

Results: We captured 92 target episodes among these 40 patients. A total of 16 (40%) children have epileptic seizures (12 focal onset and 4 generalized onset). Among the 24 (60%) of patients with non-epileptic episodes, 12 (30%) were diagnosed with conversion disorder, 6 (15%) with Tourette’s Syndrome, 4 (10%) with dyskinesia and 2 (5%) with parasomnias. Abnormal EEG 11/16, 68.7% vs 5/24, 20.8% and MRIs 4/16, 25% vs 4/24, 16.6% were seen in children with final diagnosis of epilepsy and non-epileptic episodes, respectively.

Conclusions: Although found to be expensive and time-consuming, in many cases an accurate diagnosis can only be achieved after CVEM. Abnormal MRI brain and routine EEGs are suggestive but not confirmatory for diagnosis of epilepsy.

Abstract ID: 559: Congruence and Discrepancy Between Video Electroencephalogram (VEEG) & Magnetic Resonance Imaging (MRI) of Brain in Focal Drug Refractory Epilepsy: Insights from a Large Indian Cohort

Sidharth S, Manjari Tripathi, Deepti Vibha, Rajesh Kumar Singh, Jasmine Parihar, Animesh Das, Elavarasi A, P. Sarat Chandra, Shailesh B Gaikwad, Madhavi Tripathi

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Co-relation of epileptiform discharges with the focal structural lesions on imaging in individuals with DRE is crucial in identifying surgical candidates. This study aims to determine the concordance of scalp VEEG with structural lesion on MRI brain in individuals with focal drug-resistant epilepsy (DRE), potentially predicting the success of epilepsy surgery.

Methodology: This retrospective observational study was conducted from January 2017 to March 2024 at the Epilepsy Monitoring Unit (EMU), Department of Neurology, All India Institute of Medical Sciences (AIIMS) Delhi. Inclusion criteria included individuals with DRE having a single focal structural lesion on MRI brain and having undergone VEEG monitoring for at least 48 hours. Exclusion criteria included MRI-negative DRE, multiple or expansive lesions and pregnancy. Statistical analysis involved calculating concordance rates between video EEG and MRI lesions using Fisher exact test.

Results: Of 2009 screened individuals, 407 met the inclusion criteria, with imaging and VEEG data available for 378 individuals (187 adults and 191 children; 250 males and 128 females). The focal lobar distribution of lesions was predominantly temporal (65.87%), followed by frontal (22.75%), occipital (5.82%) and parietal (6.08%). Concordance of epileptiform discharges was highest in temporal lesions (70.28%), followed by frontal (54.65%), parietal (26.08%) and occipital lesions (9.09%). The chi-square test indicated a significant difference in concordance rates among different regions (p < 0.05).

Conclusions: Temporal lobe lesions showed the maximum congruency of video EEG epileptiform discharges with focal lesion on MRI brain whereas frontal lobe lesions showed moderate level agreement.

Abstract ID: 560: Assessing Quality of Life of Drug Resistant and Drug Sensitive Epilepsy Patients and it’s Predictors: A Comparative Cross Sectional Study

Aparna Amarnath, Shaji C V, Prasanth S R

TD Medical College, Alappuzha, Kerala, India

Background and Aim: Background epilepsy is a chronic neurological disease and is associated with a stigma and lower quality of life. Seizure control is the part where we currently focus, but the importance of their quality of life (QOL) has not been addressed. AIMS: 1. To compare the QOL between drug-sensitive and drug-resistant epilepsy patients in a tertiary care center and 2. To study the predictors of poor QOL of epilepsy patients.

Methodology: A comparative cross-sectional study was done in our tertiary care center from October 2022 to January 2024. After getting ethical clearance, a total of 225 patients were studied of which 111 were drug-sensitive and 114 were drug-resistant by consecutive sampling technique. Using semi-structured pretested questionnaire and Malayalam version of QOLIE 10, patients were interviewed and data entry was done. Data was analyzed in SPSS software (version 27). Baseline characteristics between drug-sensitive and drug-resistant groups were assessed using Chi-square and Mann–Whitney U test. Factors associated with poor QOL in epilepsy were determined using chi-square test. Multiple logistic regression was done to determine predictors of poor QOL, p value of <0.05 was considered to be statistically significant.

Results: There is a significant difference in distribution of QOLIE 10 score between drug-resistant 2.5 (1.9,3.5) and drug-sensitive 1.4 (1.2,1.7) (p value < 0.001). Total patients were divided into two groups based on median QOLIE 10 score (2.5). Chi-square test was done to determine factors associated with poor QOL (QOLIE 10 score <2.5). Predictors of poor quality of life found were history of drug resistance (OR = 33.26;95%CI =7.645-11.7), use of levetiracetam (OR = 2.95;95% CI = 1.221-7.103), associated psychiatric illness (OR = 5.61;95% CI = 2.385-13.19) statistically significant with a p value <0.001 and R2 53%.

Conclusions: QOL is significantly decreased in DRE epilepsy. Patients with DRE, psychiatric illness and those on levitiracetam had poor QOL.

Abstract ID: 561: Risk of Atherosclerosis among Patients with Epilepsy on Long Term Anti-Seizure Medicines

Abhinav Ankur

Vardhman Mahavir Medical College, New Delhi (VMMC, New Delhi)

Background and Aim: Epilepsy is one of the most common neurological disorders affecting approximately 70 million people worldwide. Long-term intake of antiepileptic medicines has been reported to be associated with increased risk of atherosclerosis. Dyslipidemia and carotid artery intima media thickness (CA-IMT) are considered biomarkers for risk of atherosclerosis. We investigated patients on antiepileptic medicine monotherapy for atherosclerosis risk and compared with that of age- and sex-matched healthy controls.

Methodology: Fifty patients with epilepsy aged 12-40 years on anti-seizure medicine monotherapy were recruited. The patients and 50 healthy age- and gender-matched volunteers were clinically examined in detail, and their body mass index, serum lipid profile and CA-IMT were determined.

Results: All the patients were on monotherapy of phenytoin or carbamazepine or valproate or levetiracetam. Patients on ASM were frequently overweight and had statistically significantly higher serum total cholesterol, non-HDL cholesterol, LDL: total cholesterol compared to controls (P < 0.05). CA-IMT was significantly higher among patients compared to controls (P < 0.05).

Conclusions: Patients on antiepileptics including phenytoin, carbamazepine, sodium valproate and levetiracetam have increased risk of atherosclerosis.

Abstract ID: 562: Nuptiality Behavior in Epilepsy, A Psycho-Behavioral Research at a Tertiary Care Hospital in South India

Saikiran K, Surya Prabha Turaga

Nizam’s Institute of Medical Sciences, Hyderabad (NIMS, Hyderabad), Telangana, India

Background and Aim: This study attempted a cross-sectional analysis of a group of patients with epilepsy, drug resistance and non-responders and incorporated the QOLIE 31 Questionnaire and a marital outcome questionnaire to assess the quality of marital life. Emotional well-being was the domain observed to be worst affected in the group of patients.

Methodology: This cross-sectional study at our tertiary care center comprised 139 consenting adults, screened at the OPD prospectively over two years, who were on two or more drugs, for a period of one year or more. Excluded were those with below average intelligence and seizures of psychogenic nature. QOLIE 31 (Quality of life in Epilepsy 31) forms were used to assess quality of life, and a questionnaire for assessing the marital behavior. Appropriate statistical analyses were carried out to compare QOLIE 31 T scores and marital life characteristics among the subgroups of the study patients.

Results: The QOLIE 31 T scores were least among the age group of 18-32, with emotional well-being being the domain to be worst affected. Responders to ASMs were found to have statistically significant higher scores. Divorce rate (8.5%) was above the national average among PWE, particularly in those with secondary epilepsy.

Conclusions: Significant vortices exist in the current healthcare system to address the social needs of PWE, particularly to target adherence to therapy, and provide health education regarding the disease.

Abstract ID: 563: Seizure Outcomes and Predictive Factors in Epilepsy with Myoclonic Atonic Seizures (EMAS): A Retrospective Long Term Follow Up Study

Nishanth Chinthoju, Vaishakh Anand, Vinayan Kollencheri Puthenveettil

Amrita School of Medicine, Kochi, Kerala, India

Background and Aim: EMAS is an epileptic syndrome that begins in early childhood with an incidence of approximately 1 in 10,000 children and 2% of all childhood epilepsies. There is a paucity of data regarding the clinical course and treatment response in children with EMAS in India. AIM: To evaluate the seizure outcome and factors predictive of poor outcomes in EMAS.

Methodology: A retrospective study was done in tertiary care. Subjects diagnosed with EMAS with prior normal development and under regular follow-up were included. Baseline demographic parameters, EEG and neuroimaging characteristics were collected. Seizure outcome and EEG evolution in the follow-up were collected.

Results: 25 subjects (M = 76%). Age of onset of seizures is from 1.3 to 4.7 years (mean: 2.6 years). Apart from myoclonus (100%) and atonic (64%), other seizures are head drops (64%), absence (20%), tonic-clonic (72%), tonic seizures (12%), non-convulsive status epilepticus (1%) and epileptic encephalopathy (28%). EEG at onset captured interictal epileptiform discharges and background slowing in 88% and 68%. After treatment with anti-seizure medications (ASM), 44% responded with single ASM, and 28% developed drug-refractory epilepsy required keto diet. EEG was normal in 68% of patients in 1 year of treatment. Outcome was poor in tonic, recurrent non-convulsive status epilepticus. Frequent generalized spike and waves and the presence of generalized paroxysmal fast activity (GPFA) were the EEG features suggestive of a poor outcome.

Conclusions: EMAS-childhood-onset epileptic encephalopathy with polymorphic seizures is often underdiagnosed. Failure to start the early treatment adversely affects developmental outcome. The presence of poor prognostic factors warrants the need for aggressive treatment with appropriate antiseizure drugs and early initiation of keto diet.

Abstract ID: 564: Thyroid Function Abnormalities in Patients on Anti-Epileptic Therapy

Rohith M G, Ashalatha Radhakrishnan, Manisha K Yallapalli, Nandana J

SCTIMST, Thiruvananthapuram, Kerala, India

Background and Aim: Epilepsy is a chronic disease with around 8.2/1000 prevalence in the general population and in developing countries, and it is more than 10/1000. AEDs used to treat epilepsy are known to have many associated effects including endocrine, metabolic and reproductive disturbances. We undertook this study to determine thyroid dysfunction in epileptic patients on AEDs. Aims: To determine the incidence of subclinical hypothyroidism in those on AEDs and to determine effect of older versus newer AEDs on thyroid function.

Methodology: This is an observational, retrospective, cross-sectional study carried out in 203 epileptic patients who were admitted in epilepsy ward between July 2013 and March 2014 at R. Madhavan Nayar Center for Comprehensive Epilepsy Care, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, southern India. Detailed data including demographics, seizure type, duration of anti-epileptic therapy and thyroid function including TSH (thyroid stimulating hormone), fT4 (free thyroxine) and fT3 (free tri-iodothyronine) levels are collected from electronic medical records (EMR) and compared between the older and newer AEDs.

Results: Among 203 patients on AEDs, 27 patients (13.3%) developed subclinical hypothyroidism with maximum abnormality seen with valproate and clobazam. Twenty-four patients were associated with low fT4 and low fT3 levels with maximum reduction caused by those on clobazam, oxcarbamazepine and valproate. There was no significant difference noted between monotherapy versus polytherapy AEDs and thyroid dysfunction.

Conclusions: Thyroid dysfunction in epilepsy on therapy is an important disturbance which has to be diagnosed and followed up while patients on AEDs. Our data showed older generation drugs had more thyroid dysfunction than the newer AEDs.

Abstract ID: 565: Impact of Valproate Discontinuation on Seizure Control in Women of Reproductive Age

Sridhar S.K, Ashalatha Radhakrishnan

SCTIMST, Thiruvananthapuram, Kerala, India

Background and Aim: We analyzed seizure control in reproductive age group women on valproic acid (VPA) and studied the difference in seizure control between VPA maintenance and after changing to other anti-seizure medications (ASMs), because of reproductive age group or impending pregnancy.

Methodology: All women with epilepsy (WWE) from prospectively maintained electronic medical records (EMR) from 2013 to 2022 were analyzed. We included WWE in age group 18-45 years, both primary generalized and focal epilepsy. We studied their clinical profile, ASMs use, seizure control while on VPA and after changing to other ASMs and adverse effect and pregnancy related complications.

Results: There were 69 WWEs who were well controlled on VPA, enrolled in this study after exclusions. Among them, 28 (40.58%) had diagnosis of JME, and 30 (43.47%) were having other subtypes of GGE, whereas 11 (15.9%) patients had focal onset epilepsy. Among these patients, 26 (37.68%) only needed VPA dose more than 800 mg/d for their control of seizures, remaining patients required less than 800 mg/d of VPA. Also, VPA mono-therapy needed for about 21 (30.43%) with the mean dose of 660 mg/d. Out of the 69 patients who were well controlled on VPA, 18 patients were switched to other ASMs. Among these switch group (n= 18), 10 (55.55%) had lost their seizure control after switching from VPA. Among the patients who continued on VPA, teratogenicity occurred in 3 patients with the mean dose of 966 mg/d.

Conclusions: Switching to alternative ASMs in WWE who are already well controlled on low-dose VPA (<800 mg/d) leads to poor seizure control and increased likelihood of resulting in polypharmacy.

Abstract ID: 566: Personality Disorders in Patients with Idiopathic Generalized Epilepsy

Ankith Bhasi, Ramshekhar N. Menon, Ashalatha Radhakrishnan

SCTIMST, Thiruvananthapuram, Kerala, India

Background and Aim: To estimate the prevalence of personality disorders in persons with idiopathic generalized epilepsy (IGE) and to compare it with the general population.

Methodology: The study was conducted in a tertiary epilepsy care center in South India. Study population included 57 normal controls and 57 cases (18 years of age, confirmed cases of IGE). Exclusion criteria included history of any psychiatric disorder or family history of any psychiatric illness. Self-administered questionnaire was used for study data collection. IPDE-ICD10 screening questionnaire was applied to investigate personality traits, and Bear-Fedio inventory (BFI) was used to study different behavior traits in patients with IGE and controls. To compare the prevalence of personality disorders in cases and controls, we applied chi-square test. For ascertaining the relation between different demographic variables with the presence of personality disorders, Pearson’s chi-square test and Fischer exact test were applied. Mann–Whitney U test was used to analyze the significant difference of Bear-Fedio inventory (BFI) scores between cases and controls.

Results: Compared to controls, a higher prevalence of personality disorders was noted among IGE patients (87.72% in cases versus 36.84% in controls). Personality traits like borderline (p = 0.000), anankastic (p = 0.000), anxious (p = 0.000), histrionic (p = 0.000), paranoid (p = 0.098) and impulsive (p = 0.002) were considerably higher in cases with IGE. This was comparatively higher among females (p = 0.034) than males. In BFI also, significantly higher scores were found among cases in all behavioral traits except hypermoralism, altered sexual and aggression.

Conclusions: Personality disorders were significantly higher in cases with IGE. An early intervention in the form of counselling may be considered to improve their quality of life, in addition to drugs, since a majority of them need life-long treatment.

Abstract ID: 567: Factors Influencing Clinical Outcomes of Acute Ischemic Stroke Managed with Mechanical Thrombectomy

Keerthi Gopu, Subhash Kaul DR, Pradeep Mucharla DR

KIMS Hospital, Hyderabad (KIMS, Hyderabad), Telangana, India

Background and Aim: Mechanical thrombectomy (MT) is known to improve the outcomes of patients experiencing an acute ischemic stroke (AIS) due to large vessel occlusion (LVO). However, despite an endovascular reperfusion rate close to 90%, only half of the patients are functionally independent at 90-day follow-up. Identification of factors influencing clinical outcomes of LVO strokes who undergo MT is essential. This study was aimed to investigate the clinical, radiological and procedural factors that may influence clinical outcomes of LVO strokes treated with MT.

Methodology: In this bispective observational study, 52 AISs with LVO patients who underwent MT were included. IV thrombolysis was done in eligible patients. Patients were divided into good or poor outcome groups according to modified Rankin Score (mRS), good outcome: mRS 0-3, poor outcome: mRS 4-6. Statistical comparison of the clinical (National Institutes of Health Stroke Scale [NIHSS score]), radiological (occlusion site, Alberta stroke program early CT score [ASPECTS score]) and procedural factors (IV thrombolysis, time to intervention, thrombolysis in cerebral infarction [TICI score], post-procedure symptomatic intracerebral hemorrhage [sICH]) between two groups was done.

Results: Of the 52 patients, 35 (67.3%) had good outcome. Patients with lower NIHSS score (p < 0.04), higher ASPECTS score (p < 0.001) and no post-procedure sICH (P < 0.05) had good outcome. Factors like age, gender, IV thrombolysis, time to intervention and TICI score did not have statistically significant correlation with outcome at 90-day follow-up.

Conclusions: Mechanical thrombectomy had absolute benefit in LVO stroke. Lower NIHSS score, higher ASPECTS score and the absence of post-procedure sICH were reliable predictors of good outcome at 90-day follow-up.

Abstract ID: 568: Utility Of MR Vessel Wall Imaging in Ischemic Strokes due to Intracranial Stenosis

Payal Patil, Rajesh Benny, Rakeshsingh Singh, Vicky Thakkar, Akash Walinjkar, Jayashree Dahiphale

Fortis Hospital, Mulund, Mumbai, India

Background and Aim: AIM: To study the utility of vessel wall imaging in ischemic strokes. BACKGROUND: Conventional imaging techniques are limited to evaluating changes in the vessel wall lumen. High-resolution magnetic resonance vessel wall imaging (MRVWI) can allow visualization of the vessel wall and help differentiating atherosclerotic stenosis from primary angiitis of the central nervous system (PACNS).

Methodology: This was a prospective study in a tertiary care center over 6 months from October 2023 till now. Ten consecutive patients presenting to the emergency room with acute ischemic stroke due to intracranial stenosis were enrolled. Their symptoms, demographic characteristics, comorbidities and relevant investigations (including workup for systemic vasculitis) were noted. MR VWI was done in all patients. All patients were treated according to the standard of care. A follow-up MRI was done on all patients after 3 months.

Results: Seven elderly with focal neurodeficits with comorbidities had eccentric enhancement of luminal stenosis. These patients were diagnosed with atherosclerotic stenosis and treated with antiplatelets, statins and correction of their risk factors. Three young patients had recurrent symptoms without any vascular risk factors and had concentric, homogenous vessel wall enhancement. These were treated as primary CNS angiitis (with systemic steroids/immunosuppressants) and had complete recovery after treatment.

Conclusions: MRVWI should be considered in all patients presenting with ischemic strokes due to intracranial stenosis. It helps to differentiate between vasculitis (concentric) and atherosclerosis (eccentric) stenosis. MR-vessel wall imaging can also be used to monitor the disease activity in the case of primary CNS angiitis.

Abstract ID: 569: Clinico Demographic and Radiological Profile of Symptomatic Intracranial Atherosclerotic Disease Patients from North India

Ira Chaudhary, Gaurav Goyal Goyal

Mahatma Gandhi Medical College & Hospital, Jaipur, Rajasthan, India

Background and Aim: Intracranial atherosclerotic disease is a major cause of stroke, with a 15% recurrence rate. Therefore, in order to design primary prevention efforts and future management strategies, it is crucial to determine the risk factor profile and artery that are more typically impacted in patients with ICAD. Therefore, the study’s objective was to analyze the demographic, clinical and radiological profiles of patients with symptomatic intracranial atherosclerotic disease.

Methodology: Retrospective analysis of patients with ages ranging from 18 to 100 years old who were admitted to a tertiary care hospital in India between January 2019 and December 2023 was conducted. Patients with angiographically proven intracranial atherosclerotic disease and symptoms suggestive of cerebral ischemia were included in this investigation. Among 200, 131 patients based on inclusion criteria were enrolled in the study and variables including demographics, risk factors, the vessel implicated and the degree of stenosis were evaluated.

Results: A total of 131 of the 200 patients that were examined were part of the ICAD group, and their mean age was 62.60 ± 10.94 years. In both groups, males were more frequently impacted. Smoking, drinking, CAD, diabetes mellitus, hypertension and dyslipidemia were more common in the ICAD group (no statistical significance). The supraclinoid region of the ICA was the most frequently afflicted artery (left, 38.46 > Right, 23.07%) and anterior circulation >posterior circulation (87.79 vs. 39.69). 53.3% of supraclinoid ICA patients had moderate stenosis

Conclusions: In order to enhance preventative measures and identify new treatment targets, our study established the relationship between modifiable and non-modifiable risk factors and ICAD, as well as the artery segment involved.

Abstract ID: 570: Rare Mysterious Speech Disorders in Acute Stroke

Apsara P S, S Ramu, P K Murugan

Madurai Medical College Madurai, Tamil Nadu, India

Background and Aim: A patient with a motor speech disorder should be able to produce normal expressive language in writing and to comprehend both spoken and written language. Motor speech disorders include dysarthrias, disorders of speech articulation, apraxia of speech and four rarer syndromes: aphemia, foreign accent syndrome, acquired stuttering and the opercular syndrome. Aim of this study is to identify these rare speech disorders which are usually clinically missed in patients with acute stroke.

Methodology: This is a prospective observational hospital study which was performed in patients with acute stroke who had difficulty in speech without abnormalities of language and normal cranial function.

Results: In this study, out of 30 patients, stuttering is seen in 1 patient, foreign accent syndrome in 1, aphemia in 4, opercular syndrome in 4, apraxia of speech in 10 and dysarthria in 10 patients. In these patients, MRI brain showed lesions in left hemispheric insula/left perisylvian network/subcortical connections were involved.

Conclusions: A patient with a motor speech disorder should be able to produce normal expressive language in writing and to comprehend both spoken and written language. If a listener transcribes into print or types the speech of a patient with a motor speech disorder, the text should read as normal language. Hence, we should not misinterpret these rare mysterious speech disorders as language disorders.

Abstract ID: 571: A comparative study on Sovateltide and Aspirin versus Aspirin alone in Patients with Acute Ischemic Stroke

Soumya Kanduri, Praveena Gannavarapu

Pragyna neuro mind clinic, Sadguru Heights, Carpenter’s Colony, Srinivasa Nagar, Madhurawada, Kommadi, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Ischemic stroke is a significant health issue worldwide leading to serious long-term disability and is one of the leading causes of mortality. Acute management of stroke is restricted to causative recanalization and restoration of cerebral blood flow. This study was done with an aim to compare the efficacy and outcomes in patients who were given Sovateltide and aspirin with patients given aspirin alone within 24 hours of acute ischemic stroke.

Methodology: A hospital-based prospective study with a sample of 100 patients of 40-90 years was considered, who presented to the hospital within 24 hours of stroke onset. The patients were divided randomly into two groups of 50 patients each. Patients who were given Sovateltide and aspirin were named group S, and patients who were given aspirin alone were named group A. Both groups of patients were assessed using NIHSS, MRS, Barthel’s index at the time of admission, discharge, 1-month and 3-month follow-up, and the scores were then compared.

Results: With regard to all the indices, there was a significant reduction in the scores from admission to discharge, 1-month and 3-month follow-ups in both the groups. This reduction was more in the group S compared to group A. (Reductions in NIHSS in group S from admission to 1-month and 3-month follow-ups were 4.38, 7.38 and 8.40, respectively, whereas in group A, the reductions were 2.78, 3.48 and 3.96, respectively.)

Conclusions: This study concludes that a combination of Sovateltide and aspirin is a better choice than giving aspirin alone with better reductions in stroke indices in patients presenting within 24 hours of acute ischemic stroke.

Abstract ID: 572: Relevance of Noncontrast Computed Tomography (NCCT)-Based Alberta Stroke Program Early CT Score (ASPECTS) in Predicting Severity of Acute Ischemic Stroke at Presentation and its Functional and Cognitive Outcome at 90 Days

Arnesh Bhattacharya, Jyoti Garg, Ashish Kumar Duggal, Jyoti Garg, Ashish Kumar Duggal, Kuljeet Singh Anand

ABVIMS and DR RMLH, Baba Kharag Singh Marg, New Delhi- 111

Background and Aim: Alberta Stroke Program Early Computed Tomography Score (ASPECTS) is a noncontrast computed tomography (NCCT)-based topographic scoring system that provides quantitative measure of early ischemic changes. The score was initially developed for evaluating candidacy for stroke thrombolysis, but currently also predicts functional and cognitive outcomes of stroke. The aim of the study is to assess if ASPECTS score at presentation can predict the clinical severity of stroke at presentation and also its functional and cognitive outcome at 90 days after the event.

Methodology: Thirty-five patients with acute ischemic stroke presenting within 48 h of onset were included in the study. National Institutes of Health Stroke Scale (NIHSS) score was ascertained at presentation, and ASPECTS score was calculated (less than 6 and 6 or greater). On presentation, NIHSS score and length of hospital stay were considered to be markers of early severity, and modified Rankin Scale (mRS) and Montreal Cognitive Assessment (MoCA) scores were assessed at 90 days. Patients with MoCA less than 26 were considered to be having post-stroke cognitive impairment.

Results: Correlation between ASPECTS and NIHSS, stay length, 90-day mRS and MoCA were -0.452, -0.632, -0.778, and 0.618, respectively. ASPECTS of less than 6 by univariate analysis was seen to be a risk factor for more severe strokes in acute setting with greater morbidity and cognitive decline at 90 days. Cardioembolic strokes also tended to have greater post-stroke cognitive decline.

Conclusions: ASPECTS score is a surrogate marker of early and long-term stroke severity and its impacts.

Abstract ID: 573: Analyses of Mode of Transportation of Stroke Patients to a Tertiary Care Center in North Western India- Is it Time to Make Amends

Taniya Upadhyaya, Kaavya Rao, Neetu Ramrakhiani

Fortis Hospital Jaipur, Rajasthan, India

Background and Aim: The burden of stroke and the disability following it is increasing in India. The interval between the onset of symptoms and arrival at the emergency room is critical for the management of stroke patients. We performed the study to evaluate the trends in the modes of transport of suspected stroke patients at a tertiary care corporate hospital in North-western India and study their outcomes at discharge and follow-up.

Methodology: Data from a prospective stroke registry of 1100 patients at a tertiary care hospital between August 2016 and January 2023 were analyzed. Patients were categorized into 5 modes of transport: 1) ambulance, 2) own transport, 3) hired, 4) public and 5) unknown.

Results: Among the 1100 patients arriving at the emergency room, 35% used the ambulance, whereas 48.18% used their own transport. 13.45% used hired vehicles, and 2.8% used public transport. 59.7% and 18.5% belonged to the urban and semi-urban populations, respectively, whereas 21.45% belonged to the rural population. 69% belonged to the lower middle socioeconomic subgroup, whereas 28.18% and 2.09% belonged to the upper middle class and below the poverty line, respectively. Mean MRS of patients reaching by emergency services was higher than those reaching by other modes of transport.

Conclusions: There is a strong gap in the availability of pre-hospital care and ambulance network even in tertiary care centers in metropolitan and corporate setups with around 65% of patients with stroke arriving hospitals using own transport or hired vehicles.

Abstract ID: 574: Clinical Features and Treatment Outcome in Moya Moya

Sirisha Yasa, Sirisha Yasa

KIMS Hospital, Hyderabad (KIMS, Hyderabad), Telangana, India

Background and Aim: Aims and methods: To analyze the clinical and radiological profile of MMV. To assess the benefit of medical management vs early surgical intervention over long-term follow-up among the variants.

Methodology: This is a single-center retrospective data analysis.

Results: About 23 patients diagnosed with moya moya vasculopathy were identified. Of these 15 were females (65%). Mean age was 18 years (range from 3 months to 62 years). Among comorbidities, 6 had hypertension, and 6 had diabetes. Six patients and 2 had hypothyroidism. Progression of symptoms occurred over 1 week to 12 years. Among the cohort, 17 (73%) were idiopathic, while 6 secondary (27%), including varicella vasculitis-2, SLE-APLA-related vasculopathy-1, Takayasu arteritis-1, Neurofibromatosis-1 and Intracranial atherosclerotic disease (ICAD)- 1. Postpartum intracranial hemorrhage was seen in 2 cases. Presenting feature was seizures 4 (22%), TIA 4 (22%), hemiparesis 10 (43%), vision loss 3 (13%), headache 3 (13%). Unilateral ICA involvement was seen in 2 (1-ICAD and 1- MMD), while posterior (PCA) extension was seen in two. Progression was seen in 20 while on aspirin. Surgical ECA-ICA bypass was done about 4 months after clinical onset in 18 who remained asymptomatic with good mRS (0-3) at mean 6-month follow-up despite residual deficits. Rest had medical management, among which 1 died, one with Takayasu arteritis remained asymptomatic on immunotherapy, and one with varicella vasculitis had radiological and clinical resolution after anti-viral treatment, while 2 had lost follow-up.

Conclusions: A thorough etiological workup is crucial as some causes of MMV are reversible with timely medical management. Early surgical intervention is beneficial in MMV to prevent neurological sequelae.

Abstract ID: 576: Role of Sovateltide in Management of Acute Ischemic Stroke – Prospective Case Control Study

Sarath Aleti, Anjan Pyal, CH Ratna Kishore, D. Ravi Varma

Citi Neuro Center, Hyderabad, Telangana, India

Background and Aim: Sovateltide is a novel endothelin B receptor agonist. Aim of this study is to show if Sovateltide is effective in reducing stroke morbidity in addition to standard treatment of care.

Methodology: All patients of age 18 years with acute ischemic stroke who present to the hospital within 24 hours of onset were included. Treatment group patients were given injection Sovateltide 0.3 micg/kg/dose diluted in 5 ml 0.9% w/v normal saline. Patients received 3 doses each day as intravenous bolus at an interval of 3 hours on day 1, 3 and 6. First dose was given within 24 hours of onset of stroke. NIHSS (National Institute of Health Stroke Scale) and mRS (modified Rankin Scale) were noted before giving the first dose, 24 hours, 30 days and 90 days after first dose.

Results: Thirty-two patients were recruited in our study. Mean NIHSS and mRS on day 0, 1, 30 and 90 were 4.5 ± 2.9, 2.5 ± 2.1, 0.7 ± 1.4, 0.3 ± 0.8 & 3.4 ± 1.1, 2.4 ± 1.2, 0.8 ± 1.1, 0.6 ± 1 in Sovateltide group. Mean NIHSS and mRS on day 0, 1, 30 and 90 were 5.7 ± 5.4, 5.1 ± 5.4, 2.5 ± 3.1, 1.3 ± 1.8 and 3.4 ± 1.3, 2.8 ± 1.7, 1.7 ± 1.6, 1.3 ± 1.2 in control group. On comparison, p value was statistically significant for NIHSS on day 30 and day 90 favoring a better outcome in Sovateltide group

Conclusions: Addition of Sovateltide to standard of care would be helpful in reducing morbidity in acute ischemic stroke and lead to better outcomes

Abstract ID: 577: Role of rTMS in Post Stroke Spasticity in Acute Stroke: A Randomized Sham Control Study

Mupparaju Venkata Divya, Sanjeev Kumar Bhoi

All India Institute of Medical Sciences, Bhubaneswar (AIIMS, Bhubaneswar), Odisha, India

Background and Aim: To evaluate the effectiveness of repetitive transcranial magnetic stimulation (rTMS) and to prevent the post-stroke spasticity in acute stroke patients.

Methodology: A total 38 patients with acute stroke (7-30 days) were enrolled and randomly divided into two groups which include 19 patients in each rTMS and SHAM group. rTMS arm received 3 daily sessions, and each consists of 1000 pulses of 1 Hz rTMS at an intensity of 90% of resting motor threshold (15 minutes) applied over the primary motor area of the contra-lesional hemisphere. SHAM group rTMS intensity was 0% but with a similar sound and scalp sensation. Spasticity (modified Ashworth scale) and modified Rankin score were assessed during pre-rTMS, and at 1 month. Change in baseline and post-rTMS motor evoked potential amplitude and latencies were also recorded.

Results: There is significant development of spasticity at shoulder [0.42 ± 0.51 vs 1.0 ± 0.33] (p = 0.001), elbow [0.36 ± 0.49 vs 0.84 ± 0.6 (p = 0.003)] and wrist [0.21 ± 0.42 vs 0.63 ± 0.49 (p = 0.002)] in SHAM group at 1-month compared to rTMS group. There is a significant improvement in MRS in both groups in 1-month follow-up. There is a significant improvement in handgrip strength in both groups at 1-month follow-up compared to baseline.

Conclusions: Low-frequency rTMS applied to contralesional hemisphere can delay in development of spasticity compared to SHAM stimulation. However, further research with longer duration follow-up is needed. Keywords: rTMS; spasticity; MAS; MEP; prevention

Abstract ID: 579: Anticoagulation Status and Outcome in Cerebral Venous Thrombosis: A Single Centre Retrospective Study from South India

Sangeeth T A, Deepak Menon, Manisha Gupta, Karthik K, Pritam Raja, Subasree Ramakrishnan, Sangeetha Seshagiri, Jitender Saini, Srijithesh PR, Girish B. Kulkarni

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Early initiation and maintenance of anticoagulation appears to be the mainstay of treatment of cerebral venous thrombosis (CVT) but the evidence supporting the intensity and duration of anticoagulation is limited.

Methodology: We retrospectively collected data of patients admitted with CVT over a 5-year period, who had a minimum of 6 months of clinical follow-up and three or more prothrombin time international normalized ratio (PT INR) values spread over 6 months. Data collected included demographic, clinical and radiological parameters, anticoagulation status during the follow-up, complications and clinical status at the last follow-up.

Results: We identified 204 patients, and the mean age was 34.4 ± 11.1 years. The majority had a provoked etiology (194, 95.1%) for CVT. After initial anticoagulation with unfractionated heparin, all patients transitioned to acenocoumarol or warfarin and maintained for a mean duration of 16.02 ± 11.2 months. Time in therapeutic range (TTR) of INR 2- 3 was only 5.1 ± 11.8 percent days and time spent in an INR of 1 to 1.5 for 68.7 ± 31.8 percent days. The average INR over 6 months was 1.37 +/- 0.33. Duration of follow-up was 18.9 ± 13.25 months, and a good outcome was noted in 183 (89.7%) of patients. Complications were seen in 29 (14.2%) patients. Multivariate analysis showed only the CVT GS score to be an independent predictor of good outcome.

Conclusions: Maintenance of an intensive level of anticoagulation may not be required in patients with CVT and may be particularly true when a transient and treatable risk factor is the provoking etiology.

Abstract ID: 580: Clinical Characteristics, Radiological Profile, Recovery Pattern and Outcome of Post-Stroke Aphasia in Arterial and Venous strokes-A comparative study

Shalini Boya

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Aphasia, defined as “the loss or deterioration of verbal communication due to an acquired lesion of the nervous system,” persists as a disability in 21%–38% of stroke survivors. In India, 25% of ischemic stroke patients exhibit aphasia, a strong predictor of poor functional recovery. Cerebral venous thrombosis (CVT) accounts for 0.5%–1% of all strokes, with aphasia observed in 19%–24% of CVT patients. However, longitudinal studies on post-stroke aphasia recovery, especially in the context of venous strokes, are scarce in India. Our study aims to compare and correlate the clinical and radiological profiles of patients with post-stroke aphasia. These patients’ natural course, recovery patterns and outcome predictors were also examined.

Methodology: A prospective study on patients with post-stroke aphasia from venous and arterial strokes assessed and compared clinical and radiological profiles. Recovery was monitored using the Western Aphasia Battery in regional languages at admission, discharge, 3 months, 6 months and 1 year, and voxel lesion site mapping was done on available MRI images.

Results: This study included 90 patients: 40 with arterial strokes and 50 with venous strokes. The mean age at presentation was 44.3 years for arterial strokes and 37.4 years for venous strokes, with mean hospital stays of 13.08 and 19.36 days, respectively. Follow-up data at 1 year were available for 67.5% of arterial stroke patients and 66% of venous stroke patients. At admission, global aphasia was predominant in 80% of arterial stroke patients and 64% of venous stroke patients.

Conclusions: It emphasized the importance of early assessment.

Abstract ID: 581: Study of Genetic and Neurological Profile of Inherited myopathy among patients at a Tertiary Care Hospital in Varanasi

Ankur Vivek, Vijaya Nath Mishra, Abhishek Pathak

Institute of Medical Sciences, Varanasi (IMS, Varanasi), Uttar Pradesh, India

Background and Aim: Inherited neurological diseases represent a complex spectrum of disorders that significantly impact individuals’ quality of life, posing challenges both to patients and healthcare providers. Among these conditions, inherited myopathy stands out as a focal point due to its intricate interplay between genetic predispositions and neurological manifestations. The aim of this study was to study the genetic and neurological profile of disease in patients with inherited myopathies.

Methodology: This study was a cross-sectional observational study. It was conducted on all consecutive patients with suspected genetically determined myopathy presenting to neurology outpatient/inpatient department services of SS Hospital IMS BHU (Tertiary Care Centre) from September 2022 to May 2024.

Results: A total 125 patients of inherited myopathy were taken. Out of which 51 had LGMD, 11 had FSHD, 32 had channelopathy, 16 had dystrophinopathy, 14 had congenital myopathy and 1 had mitochondrial myopathy. Mean age was 23.74 years with M/F ratio of 98:27. Majority of patients belong to lower middle socioeconomic status (78) with majority of them living outside the Gangatic zone. Majority of the patients had proximal muscles pattern of involvement. Among the LGMD group, there was positive correlation seen between the duration of disease and levels of CPK and LDH.

Conclusions: In our study, most common myopathy was found out to be LGMD followed by channelopathy. This study for the first time will expand the land scope of our knowledge of inherited myopathy in India.

Abstract ID: 583: Navigating the Anomalous: An Electrophysiological study and understanding the clinical significance of the Accessory Deep Peroneal Nerve

Vishal Sharma, Gosala Raja Kukkuta Sarma, Raghunandan Nadig, Thomas Mathew, Akshata Huddar, Sagar Badachi, Sonia Shivde, Akshata Huddar, Raghunandan Nadig, Thomas Mathew, Loganathan K

St. Johns Medical College, Bengaluru, Karnataka, India

Background and Aim: The accessory deep peroneal nerve (ADPN) is an often-overlooked anomalous nerve of the lower limb. This anomaly can lead to atypical clinical and electrophysiological presentations of peroneal nerve lesions, potentially resulting in misdiagnosis and inappropriate management decisions. Additionally, an attempted biopsy of the sural nerve may injure the ADPN if not recognized. This study aims to highlight the clinical significance of ADPN, assess its prevalence in Indian patients and quantify its contribution to the CMAP of EDB.

Methodology: A prospective observational study was conducted over a six-month period, involving 110 patients referred for nerve conduction studies. Nerve conduction studies were performed using a Nihon Kohden machine. Active recording electrodes were placed over the EDB muscle, and stimulation was performed at three sites: ankle (S1), fibular neck (S2) and posterior to the lateral malleolus (S3). The proportion of contribution of ADPN to CMAP of ADPN was calculated (A3/A1). Descriptive statistics were used for data analysis.

Results: Out of 200 limbs studied, ADPN was present in 28%. The mean amplitude of CMAPs of the ADPN was 95.82 µV. The percentage of CMAPs of ADPN relative to the ankle CMAP was categorized as follows: 0-5% (55.35% of limbs), 5-10% (17.85%), 10-15% (7.14%), 15-20% (16.07%), 20-25% (1.78%) and 25-30% (1.78%).

Conclusions: ADPN is a common and important anomalous nerve in our patients. In a significant number of patients, it contributes substantially to the CMAP of EDB, so we suggest a routine stimulation behind the lateral malleolus to miss the ADPN to avoid injury to motor fibers during nerve biopsies/ankle surgical procedures

Abstract ID: 584: Delayed Organophosphorous Compound Induced Myeloneuropathy- Case Series of Two Patients From South India

Sowmy Saravanan, Rajeswari Agoram, Sunil Narayan, Ramkumar Sugumaran

Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry (JIPMER Puducherry), India

Background and Aim: Accidental or intentional ingestion of organophosphate (OP) is common in South India, mainly in agricultural community. It can present as acute cholinergic crisis, intermediate syndrome and organophosphate-induced delayed neuropathy (OPIDN). Neuropathy target esterase (NTE) is thought to be the target of OPIDN initiation. It is characterized by distal degeneration of some axons of both the peripheral and central nervous systems occurring 1-4 weeks after single or short-term exposures. Here, we describe the case series of two patients presenting with delayed OPC-induced myeloneuropathy.

Case Report: We are presenting a case series with two patients, a 19-yr old female and 28-yr old male, both the patients with a significant history of OPC consumption and hospitalization in intensive care, with delayed presentation of chronic symmetric progressive bilateral lower limb pure motor paraparesis, with weakness, wasting, hypertonia and hyperreflexia with other CNS examination being normal.

Discussion: Common etiologies for myeloneuropathy like B12, vitamin E and copper deficiency and infectious etiology like HIV HTLV 1 were ruled out, and whole exsome sequencing for inherited causes was also reported normal. MRI spine in the patients was also not suggestive of structural etiology for myelopathy.

Conclusions: It has been proposed that OPIDN is caused by the covalent inhibition of neuropathy target esterase. The administration of potent NTE inhibitors in animals causes neuropathy, and mutations of the NTE gene are associated with slowly developing motor neuron disorders similar to OPIDN. The inhibition of the NTE activity by organophosphate may perturb the metabolism of important membrane phospholipids, potentially leading to neuropathy.

Abstract ID: 585: Clinicoradiological and Electrophysiological Profile of Hirayama Disease

Karthika Valaparambil, Sruthi S. Nair, Soumya Sundaram

SCTIMST, Thiruvananthapuram, Kerala, India

Background and Aim: Hirayama disease (HD) is a rare disease characterized by asymmetric weakness and amyotrophy of the upper limbs with no objective sensory involvement. Our study aimed to describe the clinical, radiological and electrophysiological characteristics of patients with HD.

Methodology: This is a retrospective observational study of patients satisfying the Haushan criteria of HD registered between January 2013 to December 2023. Data collection included clinical details along with electrophysiological and radiological parameters.

Results: We identified 71 patients, among whom all but one were males. The mean age of patients was 19.1 (±3.86) years. The median duration of illness at diagnosis was 2 (0.3-14) years. The most common clinical presentation in our study cohort was bilateral asymmetric upper limb involvement. Polyminimyoclonus was the most frequently detected clinical examination finding in our cohort. Atypical findings of proximal upper limb involvement and hypereflexia of the limbs were noted. The most frequent electrophysiological abnormality was the reduced CMAP in ulnar nerve. The electrophysiological correlate of the reversed split hand index was observed in 52 (73.2%) patients. The most recurring radiological abnormality in our cohort was thinning of the cord.

Conclusions: Revisiting the atypicalities of this rare disease assumes importance in this era of newer modalities of treatment.

Abstract ID: 586: Amyotrophic Lateral Sclerosis Beyond Motor System

Sri Sai Venkata Haritha Putrevu, Gopi Seepana

Andhra Medical College Visakhapatnam, Andhra Pradesh, India

Background and Aim: Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disorder involving motor neurons in motor cortex, brainstem and spinal cord. In recent times, ALS is known to involve other systems in addition to motor system resulting in non-motor symptoms Aim: (a) To compare the incidence of these non-motor symptoms between limb onset and bulbar onset patients ALS and (b) To explore the burden of Non-Motor Symptoms on quality of life in patients with ALS

Methodology: Patients admitted to neurology department, fulfilling the criteria of ALS, were included. Every patient was asked to report non-motor symptoms during the disease course. Non-motor symptoms were considered significant based on standard scales like hospital anxiety and depression scale for anxiety and depression, Epworth sleepiness scale for daytime sleepiness, numeric pain scale for pain, ALSAQ 5 scores for quality of life. Individuals were subjected to relevant investigations like nerve conduction studies, electromyography, MRI of spine and brain

Results: In total of 18 patients, 15 were male (84%), and 3 (16%) were female. Most had onset in fifth decade (7 patients) with a mean age of onset 50.77. Mean disease duration was 10.7 months. Five patients had bulbar onset, and 13 patients had spinal onset. Itching was reported in one patient with bulbar onset ALS. Tingling was seen in 4 patients and numbness in 2 patients of spinal onset ALS. Executive dysfunction was seen in 6 patients (23% in spinal and 60% in bulbar). Depression was seen in 3 patients of spinal onset and none in bulbar onset.

Conclusions: Varied and few non-motor symptoms are present in each patient. Larger sample is required to define association of non-motor symptoms with severity of disease and any differences between bulbar and spinal onset ALS.

Abstract ID: 587: To Evaluate Relationship Between Metabolic Profile, Quality of Life and Polysomnography Detected Sleep Disorders in Myasthenia Gravis

Satyajit Parhi, Satyajit Parhi, SK Bhoi, Menka Jha, Priyanka Samal, Suprava Naik

All India Institute of Medical Sciences, Bhubaneswar (AIIMS, Bhubaneswar), Odisha, India

Background and Aim: Myasthenia gravis (MG) is a chronic autoimmune disease affecting neuromuscular junction. 40-60% was associated with sleep disorder breathing (SDB) and restless leg syndrome. Till date, study is sparse regarding relationship between myasthenia gravis quality of life (MGQOL) and polysomnography (PSG) detected sleep disorders. Aim is to evaluate relationship of sleep disorder, sleep parameters with quality of life (QOL) in myasthenia gravis patients. Secondary objective is to know prevalence of metabolic abnormality and its correlation with QOL in MG patients.

Methodology: A total of 21 stable myasthenia patients were included in this prospective study. Basic clinical parameters, demographic profile, thymus status, subjective sleep score (ESS, PSQI), MGQOL, metabolic parameters, PSG detected sleep parameters were evaluated. At 2-month follow-up, subjective sleep score was documented

Results: Obstructive sleep apnea (OSA) was found in 5 (23.80%) patients and apnea hypopnea index (AHI) (>5/hour) in 6 (28.57%) patients. Periodic limb movement (>5/hour) was found in 6 (28.57%) patients. ESS > 10 was seen in 11 (52.38%) patients, with PSQI > 5 in 14 (66.67%) patients. In MGQOL 15 r score, mean was 7.52 ± 2.73 with highest scoring item “frustration by MG.” In PSG parameters, MG patients are found to be early and light sleepers with reduced REM. Metabolic syndrome was found in 5 (23.80%) patients, but no correlation was found between QOL and subjective sleep score.

Conclusions: Sleep abnormality in the form of decreased sleep latency and REM sleep duration was found in this study. The presence of OSA and AHI >5 positively correlated with poor QOL in stable MG patient. Metabolic syndrome was seen in one-fourth of the MG patients. Metabolic profiles did not correlate with MGQOL score.

Abstract ID: 588: A Case Series of Iliopsoas Abscess- Clinical Presentation, Diagnosis and Treatment

Taniya Upadhyaya, Kaavya Rao, Neetu Ramrakhiani

Fortis Hospital Jaipur, India

Background and Aim: To describe the varied clinical presentation, diagnostic modalities and treatment of psoas abscess, a potentially serious condition.

Methodology: We conducted a retrospective analysis of medical records for a series of patients with confirmed psoas abscess who were treated at our institution between October 2020 and May 2024. Demographic data, clinical presentation, diagnostic methods, treatment options and outcomes were analyzed and summarized.

Results: Eight patients were included in the study. The mean age was 55.62 years, and males and females were equally affected. Common symptoms included severe backache not responsive to standard analgesics and diagnostic methods included basic blood workup, ultrasound of local area, MRI of spine/CECT of abdomen. Most patients were treated with IV antibiotics, both empirical and as per culture sensitivity, or ATT as per etiology, drainage of pus collection in most patients and the majority showed improvement after treatment. However, two patients experienced complications during the course of treatment and succumbed.

Conclusions: Psoas abscess is a rare but important clinical entity that requires early recognition and appropriate treatment. Our case series provides valuable information on the clinical presentation, diagnosis and treatment of psoas abscess, which may aid in the management of this condition in clinical practice.

Abstract ID: 589: Study of Serum Tumor Necrosis Factor Alfa Levels in Diabetic Peripheral Neuropathy Patients from Eastern India

Sonalika Behera, Ashok Kumar Mallick, Kali Prasanna Swain

Shree Ram Chandra Bhanja Medical College Cuttack, Odisha, India

Background and Aim: Background and Aims: TNF alfa is an inflammatory marker upregulated in diabetic peripheral neuropathy. This study aims to assess the role of TNF alfa in diabetic neuropathy patients and how the levels of TNF alfa levels vary with severity of neuropathy in Indian type 2 diabetic subjects.

Methodology Consecutive patients of type 2 Diabetes Mellitus (DM) with or without Neuropathy attending SCBMCH, Cuttack neurology OPDs in one year were recruited. Patients having neuropathy due to nutritional deficiency, chemotherapy, hypothyroidism and patients with malignancy, pregnancy and autoimmune disease were excluded from the study. 94 included subjects divided into Group 1 (healthy controls, n = 36), Group2 (diabetes without neuropathy, n = 28), Group 3 (diabetes with neuropathy, n = 30). Diabetic peripheral neuropathy was diagnosed on clinical examinations and electrophysiology study. Grading of neuropathy was done based on Toronto Clinical Neuropathy Scoring System. TNF alfa levels were measured by using ELISA done in standard kits adapted to Lisa scan (Evolis twin plus, Biorad). All collected data were analyzed statistically by keeping p value of <0.05 as significant.

Results: S. TNF alfa levels in group I, group II, group III are 23.94+-3.23, 42.50+-11.32,71.14+_6.52, respectively (p = 0.01). HBA1c is positively correlated with S. TNF alfa levels (r= 0.218, p = 0.015). Serum TNF alfa levels are significantly increasing with the progression of diabetic neuropathy indicating the extent of peripheral nerve damage (p value 0.008).

Conclusions: TNF alfa is positively correlated with severity of diabetes. S. TNF alfa level increased in diabetic neuropathy, and it is significantly high in severe DPN patients

Abstract ID: 590: Clinico-Electrophysiological Involvement of Facial Nerve and its Relevance in Outcome of Guillain Barre Syndrome

Chaudhari Hemabhai, Jyoti Garg, Ashish Duggal

ABVIMS & DR RML HOSPITAL, New Delhi, India

Background and Aim: Guillain–Barré syndrome (GBS) is an acute, frequently severe and fulminant autoimmune polyradiculoneuropathy. Incidence ranges between 10 and 20 cases per million annually, and cranial nerve involvement ranges from 50% to 75% of patients with GBS. Facial paralysis was reported at a frequency of up to 50%, usually bilateral, and is a poor prognostic indicator. Aim: To assess the incidence of facial paralysis caused by GBS with the clinical and electrophysiological features and outcomes of patients in our study.

Methodology: In a prospective observational study done between November 2022 and April 2024 of a total of 33 adult patients who fulfilled the NINCDS criteria for GBS, clinical and electrophysiological data, including facial NCS, EGRIS score at the time of admission, Hugh GBS disability score and MRC sum score, done on admission and at discharge and finally outcome were recorded.

Results: Cranial nerve dysfunction occurred in 63% of patients. The facial nerve was most commonly involved, followed by bulbar dysfunction, and 3 patients had ophthalmoparesis. Autonomic dysfunction occurred in 6 patients (18.18%). Respiratory difficulty was noted in 7 cases (21.21%), and 4 patients required mechanical ventilation. Facial nerve conduction study was abnormal in 11 (33%) cases), and bilateral, symmetric involvement was present in all cases.

Conclusions: The simplicity of the technique and the information yield is adequate justifications for routine determination of facial nerve conduction in GBS, which can predict impending respiratory insufficiency, higher disability and outcome.

Abstract ID: 591: To Study Clinical Profile and Long Term Follow up of Myasthenia Gravis Patients on Combination Therapy with Oral Azathioprine and Prednisolone at Tertiary Care Hospital

Sachin Adhav, Rahul Chakor, Swaleha Nadaf

Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai (TNMC, Mumbai), Maharashtra, India

Background and Aim: Myasthenia gravis (MG) is most common autoimmune disorder that affects the neuromuscular junction. MG is largely treatable disease but can result in significant morbidity and mortality. This can usually be prevented with timely diagnosis and appropriate treatment. Herein presenting the clinical profile and long-term follow-up of MG patients on combination therapy with oral Azathioprine and prednisolone presented to our hospital.

Methodology: Hospital is a tertiary care hospital in Mumbai. In last 5 years, total of 61 MG patients was followed up in immunology OPD. These 61 Patients with acquired MG confirming inclusion criteria were included in study. Variable studied were: age, sex, MG subgroup, MGFA class at onset, peak and last follow-up and treatment details with follow-up of patients on combination therapy with oral azathioprine and prednisolone.

Results: The analysis included 61 (31 males and 30 females) patients. Mean age at onset was 39.6 years. Fifty-four patients were on combined regimen with azathioprine and prednisolone and were followed up for 5 yrs. Fifteen (27%) patients were in successful pharmacological remission with prednisolone 0.05 mg/kg/day and azathioprine 1 mg/kg/day 5 (9.25%) patients were in complete stable remission after stopping azathioprine and prednisolone. Azathioprine was successfully stopped in 11 (20%) patients after mean follow-up interval of 3.8 yrs and tapered to 1 mg/kg in 18 (33.33%) patients after mean follow-up period of 3.5 yrs.

Conclusions: MG affects all age groups. Azathioprine and prednisolone combination therapy is effective in controlling the disease without significant side effects with complete stable remission.

Abstract ID: 592: Hirayama Disease / Monomelic Amyotrophy: MRI Abnormality of Extensive Dural Detachment Beyond Cervical Spine

Vijay B, Chandrajit Prasad, Nalini Atchayaram, Seena Vengalil, Deepak Menon, Saraswati Nashi

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: Hirayama disease (HD) is typically a progressive focal amyotrophy and weakness of the distal upper limb (s) typically involving C7, C8 and T1 segmental myotomes with sparing of muscles innervated by C5 – 6 myotomes. The typical post-contrast flexion MRI demonstrates forward displacement of posterior dura, anterior cord migration, more pronounced lower cervical cord flattening, atrophy, epidural flow voids and an epidural high-intensity crescent-shaped mass. Aim of the study is to describe the extent of dural detachment in HD

Methodology: Retrospective study. The MRI of patients with HD was analyzed by a senior neuroradiologist and the first author. Standard protocol for HD includes T1, T2 and contrast study of the cervical spine and entire spine in neutral and flexion. Patients were scanned in the supine as well as prone position to acquire maximum neck flexion and capture of the entire spine. Patients who demonstrated dural detachment beyond the cervical spine were selected and details are described. Data entry was done in SPSS version 23 and statistical analysis was done.

Results: Level of maximum atrophy was at C5-C6[56/132 (42.4%)], at C6-C7[48/132 (36.3%)], extensive cord atrophy of >5 spinal segments in 4 cases. Dural detachment was observed in all (100%), cranial extent was at C2 and caudal extent as low as D10[2/132, 1.51%)], D8[4 (3.03%)], D7[4 (3.03%)], D6[11 (8.33%)], D5[29 (21.9%)], D4[24 (18.18%)], D3[22 (16.66%)], D2[10 (7.57%)], D1[26 (19.69%)]. Epidural venous congestion was noted in all (100%). Maximum detachment was seen at the level of C5, C6, C7

Conclusions: Till date, MRI abnormalities have been described to exist only in the cervical spine. We have now documented that the dural detachment could documented that the dural detachment could involve the entire length of the cord.

Abstract ID: 593: Demystifying Pain in Parkinson’s Disease: Types, Mechanisms and Knowledge Gaps

Soaham Desai, Devangi Desai

Shree Krishna Hospital, Pramukhswami Medical College, Karamsad, Anand, Gujarat, India

Background and Aim: Pain is a prevalent and disabling symptom in Parkinson’s disease (PD), yet our understanding of its diverse manifestations and underlying pathophysiology remains limited. This scoping review aimed to comprehensively characterize the types of pain experienced in PD and elucidate potential pathological processes driving its manifestation.

Methodology: Adhering to the PRISMA scoping review guidelines, a systematic literature search was conducted across Embase, MEDLINE and Scopus databases. Rigorous screening, data charting and descriptive thematic analysis were performed on the included studies.

Results: The review identified five distinct pain phenotypes in PD: musculoskeletal, dystonic, neuropathic, akathetic and central pain. While the overall mean prevalence of pain was 53.2%, substantial variability (19-83%) existed, influenced by assessment methodologies. Proposed pathological mechanisms centered on central sensitization, peripheral rigidity/dystonia and neuroinflammation. However, a paucity of studies validating these mechanisms through pathology or imaging highlighted significant evidence gaps. Moreover, the delineation between central and peripheral mediators remained elusive, and detailed characterization of non-neurologic comorbidities contributing to pain was lacking.

Conclusions: This review highlights critical knowledge gaps hampering targeted pain management in Parkinson’s disease.

Abstract ID: 594: Two Old Sisters

Vignesh Kumar, Rajagambheeran Venugopal

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Leukoencephalopathy with vanishing white matter (LVWM) is a disease inherited in an autosomal recessive manner. Ovarioleukodystrophy refers to LVWM in females who exhibit signs or symptoms of progressive ovarian failure. We describe LVWM with secondary amenorrhea and infertility in two sisters.

Methodology: Patient 1, a 25/F presented with a 5-year history of difficulty in walking and stiffness of all four limbs. She also had secondary amenorrhea for the last three years. Patient 2, 29/F also had similar complaints from the same age.

Results: On examination, both patients had normal cognitive function. Both of them had spasticity in all four limbs. Power was 4/5 in all four limbs. All deep tendon reflexes were grossly exaggerated. Ankle clonus was present bilaterally. Babinski’s reflex was positive bilaterally. There was mild difficulty in finger nose test and finger finger nose test. Spastic gait was noted. Sensory, autonomic and extrapyramidal examination was normal. Routine blood investigations, TSH was normal. FSH and LH were increased and estrogen and progesterone were decreased. USG’s abdomen showed atrophic uterus and small ovaries. MRI Brain showed diffuse T2/FLAIR hyperintensity involving bilateral subcortical, central and deep white matter along frontal and occipital horn of lateral ventricles. Genetic analysis in patient 1 showed homozygous missense mutation in exon 13 of EIF2B4 gene on chromosome 2:g.27364531A>G, with substitution of proline for serine at codon 481.

Conclusions: The possibility of VWMD needs to be considered in adult patients who present with features of leukodystrophy and associated premature ovarian failure.

Abstract ID: 595: Determination of Precipitating Factors for Breakthrough Seizures among Patients with Epilepsy in a Tribal Area of Jharkhand: A Retrospective Study

Pratibha Prasad

All India Institute of Medical Sciences, Patna (AIIMS, Patna), Bihar, India

Background and Aim: Breakthrough seizures are an essential aspect to consider while treating epilepsy. No study has been done on the precipitating factors for breakthrough seizures in the tribal area of Jharkhand. Hence, awareness regarding the possible precipitating factors of breakthrough seizures is vital to prevent their occurrence. AIM: To identify the frequency and precipitating factors for breakthrough seizures in epilepsy.

Methodology: This cross-sectional study was conducted between July 2022 and October 2023 at AIIMS Deoghar, Jharkhand Centre of Excellence for the tribal area in Jharkhand. The study included epileptic patients who had been receiving anti-epileptic drugs for at least six months before the survey.

Results: 300 patients with epilepsy were included. 46% of epileptic patients had breakthrough seizures. Non-adherence to anti-epileptic drugs (p < 0.0001); the number of anti-epileptic drugs (AED) (p < 0.0006), duration of treatment (p < 0.05), level of education (p < 0.04), awareness of triggering factors (p < 0.05) and sleep deprivation (p < 0.05) carried the significant association with breakthrough seizures. In contrast, no association was found with gender, occupation and stress.

Conclusions: Breakthrough seizures are preventable. Hence, there is a need to create awareness of this issue to prevent its occurrence.

Abstract ID: 596: Clinical and Etiological Profile of Anterior Visual Pathway Dysfunction in Eastern India- A Tertiary Care Hospital Based Study

Jyoti Kiran, Ramesh Bhattacharya, Kartik Chandra Ghosh, Gourango Prasad Mondal, Santosh Trivedi

Calcutta National Medical College and Hospital Kolkata, West Bengal, India

Background and Aim: The clinical presentation and etiology behind vision loss due to anterior visual pathway dysfunction are numerous and varied. The mode of onset, associated clinical symptoms and neuroophthalmic examination play a critical role in diagnosis. There are ancillary investigations that can support the diagnosis. Literature has scanty data on this topic from eastern India. Hence, this study was taken up with the objective to evaluate the various neurological causes of visual impairment affecting anterior visual pathway and their clinical profile presenting to a tertiary institute in eastern India.

Methodology: INCLUSION CRITERIA: Patients with clinical evidence of anterior visual pathway dysfunction. EXCLUSION CRITERIA: Patients with local ophthalmological causes or posterior visual pathway dysfunction contributing to visual impairment will be excluded from the study. STUDY DESIGN: Institution-based observational cross-sectional study

Results: A total of 56 patients of anterior visual pathway dysfunction were included in our study. Demyelinating optic neuritis (ON) was the most common etiology, observed in 57% of patients followed by AION in 14% and toxic amblyopia in 9% of patients. IIH, infectious neuropathy, non-infectious granulomatous disease, LHON, trauma and CVST were the other observed causes. In the ON cohort majority were idiopathic.

Conclusions: The majority of patients having anterior visual pathway dysfunction had demyelinating diseases. The other important causes were AION followed by toxic and infective optic neuropathy.

Abstract ID: 597: Impact of Health Education on Stroke Risk factor (SRF) Modification Among Doctors in a Medical College

Kiran Bala, Sakshi Agarwal, Sakshi Agarwal, Surekha Dabla

World College Of Medical Sciences & Research And Hospital, Haryana, India

Background and Aim: Stroke remains the second-leading cause of death and the third-leading cause of death and disability combined. Primary prevention remains the key instrument in reducing stroke burden.

Methodology: A prospective study with intervention conducted at PGIMS, Rohtak. After taking informed consent 100 doctors (J/R, S/R, faculty) of either gender were enrolled, 93 completed the study. SRF was assessed by clinical evaluation and Stroke Population Risk Tool (SPoRT) at baseline and 6 months after health education. (Information pamphlets and education material highlighting the key points of stroke prevention strategy as per the WSO.)

Results: The mean age was 29.36 ± 5.64 yrs, (M 47.3%, F 52.7%). Reported risk factors were: HBP 3 (3.2%), diabetes 3 (3.2%), hypercholesterolemia (4.3%), smoking 5 (5.4%), depression 5 (5.4%), anxiety 21 (22.6%), stress 90.3% family history of SRF-HBP 54 (58%), diabetes 51 (54.8%), hypercholesterolemia 33 (35.4%), ischemic heart disease 26 (28%) and stroke18 (19.3%). Twelve (12.9%) individuals had poor diet and 31 (33.3%) physical inactivity. SPoRT score was”3” “4,” “5,” “6” and “7” in 30 (32.2%), 10 (10.8%), 14 (15.1%), 8 (8.6%) and 4 (4.3%), respectively. After intervention at 6 months, some nonsignificant reduction in drinking and smoking was seen without any change in other risk factors.

Conclusions: 1. Health education using digital media has had no significant impact in modifying the stroke risk factors even in doctors. 2. There is an urgent need for intervention modules to motivate and encourage people to adopt a healthy lifestyle right from childhood and these should be included in the teaching curriculum in schools and colleges.

Abstract ID: 598: Effect of Edaravone Therapy on Amyotrophy Lateral Sclerosis Functional Rating Score (ALS-FRS) in Patients of Amyotrophy Lateral Sclerosis (ALS) in Central India: A Retrospective Open Label Study

Priyanka Kashyap, Dharmendra Singh

All India Institute of Medical Sciences, Bhopal (AIIMS, Bhopal), Madha Pradesh, India

Background and Aim: Purpose: To study the effect of intravenous edaravone on ALS-FRS score and survival rate in ALS patients

Methodology: Methods: It is a retrospective study of 3.5 years on Definitive/probable ALS on the basis of revised El Escorial criteria where we compared the effect of IV Edaravone on ALS-FRS score among intervention group (IV Edaravone receiving group/case group) and ALS patients who did not receive IV Edaravone (non-intervention group/control group). The data were collected, demographic profiles were noted and scores were calculated at baseline and at the end of each cycle till 6 cycles of edaravone and compared with the ALS-FRS sores of non-receiving group at the same time. We also assessed the survival benefits among the two groups.

Results: Out of 82 patients of ALS, a total of 25 patients of clinically definite ALS admitted in neurology unit at AIIMS Bhopal were matched for baseline characteristics who received IV Edaravone for 6 cycles (case group/intervention group), and 25 patients who did not receive IV Edaravone therapy (control group/non-intervention group) either due to their financial constraints or not consented were selected. The patients who did not fulfil the diagnosis (ALS mimics under evaluation) and those who failed to receive IV Edaravone (22- ALS Mimic under evaluation, 10-did not consent for the study). The mean age of the participants was 49 ± 5.3 years. Majority of the them were male, 20 (80.8%). Lower limb onset was noted in 21 (84.0%) of the patients, followed by bulbar onset in 4 (16%), the ALS-FRS score difference was not statistically significant among the two groups.

Conclusions: IV Edaravone did not improved the ALSFRS score in intervention group when compared to non-intervention group, neither the survival rates improved.

Abstract ID: 599: Study of Cognitive Functions in Patients with Multiple Sclerosis at a Tertiary Care Center

Neeraj Kumar, Surekha Dabla, Kiran Bala

Pt B.D. Sharma PGIMS Rohtak, Haryana, India

Background and Aim: Multiple sclerosis is autoimmune inflammatory central nervous system disease. Cognitive abnormalities affect 43-70% of patients with MS, which can be a source of vocational and social impairment. The aim of our study was to assess cognitive functions in patients with MS using various scales (BICAMS, SDMT, CVLT2 & BVMTR) for different cognitive domains.

Methodology: Cross-sectional study was conducted in inpatient and outpatient in the Department of Neurology at XXXXXX over a period of one year 2021-2022. Ethical clearance was obtained from the Institutional Ethics Committee and Institutional Scientific Research Committee. After written informed consent forty-four patients diagnosed with all types of MS as per Mc Donald criteria 2017, aged between 18 and 55 years, who can speak single English word without any previous premorbid learning disability, non-correctable severe visual/auditory impairment were enrolled in the study.

Results: Statistical analysis was performed by the SPSS 26 program for Windows. The relationship of cognitive functions was assessed using Spearman’s correlation. A statistically significant (p value <0.05) correlation in mean SDMT and EDSS was observed. It was observed that there was no significant difference in mean SDMT, mean CVLT2 and mean BVMTR when compared with different groups based on age, education, duration of illness, number of clinical attacks, EDSS, detection of OCB in CSF.

Conclusions: Considering the burden of cognitive impairment in MS, routine assessment of these parameters is recommended.

Abstract ID: 600: Decade of Resilience: A Case Series of Prolonged Survival in Subacute Sclerosing Panencephalitis with a Review of Literature on ‘Elite Survivors’

Archita Makharia, Divyani Garg, Ayush Agarwal, Ajay Garg, Achal Kumar Srivastava

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: SSPE has an expected survival of 3-4 years post-symptom onset. We introduce the term “elite survivors” for individuals with SSPE surviving beyond ten years.

Methodology: We analyzed symptomatology, EEG, anti-measles antibody index, and neuroimaging of “elite survivors” of SSPE at our center, comparing them with ten previously documented cases, to discern patterns in disease progression and treatment response.

Results: We included four patients (male: female = 3:1). Age at presentation ranged from 9 years and at enrolment ranged from 10 to 26 years. The female patient represents the only female “elite survivor.” None had a history of measles infection. Three were unvaccinated against measles. Treatment strategies included combinational intrathecal Interferon alpha and Isoprinosine (two patients), and Interferon alpha alone (two patients). Survival ranged from 10 to 17 years; the 17-year survival is the second longest reported in the literature. All patients demonstrated clinical stabilization with resolution of myoclonus albeit with neurobehavioral issues. Follow-up EEGs were normal for two. CSF analysis showed low titers of persistent measles antibodies in one patient and negative in another. Repeat neuroimaging indicated ongoing white matter changes and brain atrophy in all patients.

Conclusions: This study enriches SSPE literature by examining factors influencing disease progression through the lens of “elite survivors.”

Abstract ID: 601: A Multi-Modal Tele-Rehabilitation Intervention for Individuals with Dementia: A Randomized Controlled Trial for Manastik Mobile App

Soubhik Das, Pushkraj Marne

KS52, MIT TBI, Kothrud, MIT, Pune, India

Background and Aim: The Manastik mobile application introduces a pioneering multi-modal tele-rehabilitation intervention designed specifically for individuals with dementia. This randomized controlled trial (RCT) aims to evaluate the efficacy and user satisfaction of the Manastik app in providing comprehensive cognitive and physical rehabilitation remotely. Dementia poses significant challenges in terms of cognitive decline and physical deterioration, necessitating innovative solutions that enhance patient care and engagement. The Manastik app leverages the integration of multiple therapeutic modalities, including cognitive exercises, physical activities, social interaction features and caregiver support tools. The intervention is tailored to meet the unique needs of dementia patients, promoting cognitive stimulation, physical health and social connectivity.

Methodology: Study Design Participants Randomization and Blinding Intervention Control Group Outcome Measures Data Collection and Analysis Ethical Considerations Implementation and Monitoring

Results: Participants using the Manastik app will show significant improvements in cognitive function (MMSE scores) and physical mobility (TUG test) compared to the control group. Additionally, quality of life (QoL-AD scale) will improve, and caregiver burden (ZBI scores) will decrease in the intervention group. High user satisfaction will be reported.

Conclusions: The Manastik app exemplifies the potential of tele-rehabilitation to transform dementia care, offering a scalable and accessible solution that can be integrated into routine clinical practice. This study underscores the importance of multi-modal approaches in addressing the multifaceted challenges of dementia and highlights the app’s role in enhancing patient outcomes and caregiver support. Future research will focus on long-term impacts and the integration of advanced technologies such as artificial intelligence to further personalize and optimize the intervention.

Abstract ID: 602: A Review of Heterogenous Clinical Neurological Manifestations of Primary Sjogren’s Syndrome in a Tertiary Care Centre

Soorya K, Balasubramanian S, Uma Maheswari E, Jawahar M, Velusamy S

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Primary Sjögren’s syndrome (pSS) is an autoimmune connective tissue disease affecting the exocrine glands, leading to damage of their structure and impairment of their function. Neurological disorders are one of the extraglandular manifestations of the disease and are sometimes very painful. The aim of our study is to the analyze the varied neurological manifestations of primary Sjogren’s syndrome.

Case Report: In this case series, we present 7 cases with heterogenous clinical neurological manifestations of primary Sjogren’s syndrome patients.

Discussion: Seven cases of primary Sjogren’s syndrome with varied neurological manifestations were analyzed. The clinical heterogenicity exhibited were: 2 cases presented as mononeuritis multiplex (case 1 and case 2), case 3 presented as subacute sensory motor polyradiculoneuropathy and autonomic dysfunction, case 4 was a motor neuron disease-like presentation, case 5 presented as trigeminal neuropathy with sensory motor polyneuropathy, case 6 was a CNS demyelination with dorsal root ganglionopathy and case 7 was a hypokalemic periodic paralysis and distal RTA. There were female preponderance with 6 females and 1 male in our study. All cases were proven by lip biopsy and antibodies positivity. The most common presentation seen was peripheral neuropathy.

Conclusions: Involvement of nervous system is related to more aggressive course of the disease and need more intensive treatment. Hence, patients should be regularly evaluated for new symptoms.

Abstract ID: 603: Clinical Profile and Treatment Response in Patients with CASPR2 Antibody-Associated Disease

Mitul Kasundra

Mit’ jyotinagar street no-1, Rajkot, Gujarat, India

Background and Aim: The clinical spectrum of contact in-associated protein-like 2 (CASPR2) antibody-associated disease is wide and includes Morvan syndrome. Studies describing treatment and long-term outcomes are limited. Aims: We report the clinical profile and emphasize response to treatment and long-term outcome in ten patients with CASPR2-antibody-associated disease

Methodology: Clinical, radiological, electrophysiological, treatment, follow-up, and outcome data were collected by retrospective chart review.

Results: Clinical manifestations included Isaac syndrome (n = 8) and Morvan syndrome (n = 2). nine patients were positive for LGI1 antibody. Associated features included memory impairment (n = 1), autonomic symptoms (n = 7), hallucination (n = 2), hyponatremia (n = 1). Patients were treated with intravenous methylprednisolone initially if not improved then with inj.IvIg (n = 4)and plasma exchange (n = 1)during the acute symptomatic phase followed by oral steroids and immunosuppression agents. to maintain remission. Mean-modified Rankin score at admission (pre-treatment), discharge and last follow-up were 2.75, 1.7 and 0.42, respectively. All patients were followed up for a mean duration of 15 months. All of them improved completely. Relapse occurred in two patients. One patient had relapse after 4 months and other had after 8 months, but responded favorably to inj. rituximab.

Conclusions: CASPR2 antibody-associated disease has favorable response to immunotherapy with complete improvement and good outcome. Marked clinical overlaps between patients with both LGI1 and CASPR2 antibodies that include dysautonomia, neuromyotonia, neuropathic pain, memory impairment, insomnia and hallucination.

Abstract ID: 604: To Study Cognitive Dysfunction and Depression in Patients With Type 2 Diabetes Mellitus in a Tertiary Care Hospital

Harpreet Kaur, Sudesh Prabhakar, Kiran Pal Singh, Hardeep Singh, Chasham Mitra

Fortis Hospital, Mohali, Punjab, India

Background and Aim: Cognitive dysfunction and depression are frequently underdiagnosed in type 2 DM patients. Literature suggests that their relation with diabetes control has been insufficiently explored. This study aimed to compare the prevalence of cognitive dysfunction and the prevalence and severity of depression in different groups of patients with type 2 DM, categorized by their HbA1c levels.

Methodology: This observational cross-sectional comparative study included 90 patients with type 2 DM with at least a high school education and proficiency in English. Participants were evenly distributed into three groups based on HbA1c levels (6.5% to 7.0%, 7.1%-9%, >9%). Cognitive function was evaluated using the Montreal Cognitive Assessment (MoCA), where scores < 26 indicated dysfunction. Depression was assessed using DSM-5 criteria and the Hamilton Depression Rating Scale (HDRS) for severity.

Results: The study found that cognitive dysfunction was most prevalent in poorly controlled diabetics (>9% HbA1c), followed by those moderately controlled (7.1%-9% HbA1c), and least prevalent in well-controlled patients (?7% HbA1c). Depression rates were 10% in well-controlled patients, 30% in moderately controlled patients, and 53.3% in poorly controlled patients, with significant differences observed among the groups. Moderate depression was significantly higher in poorly controlled patients compared to others.

Conclusions: Effective glycemic control is crucial in managing type 2 DM and its associated conditions. Regular screening for cognitive dysfunction and depression in these patients is vital for early intervention. The findings highlight the need for interdisciplinary care with early involvement of neurologists and psychiatrists thus improving overall health outcomes in patients with type 2 DM.

Abstract ID: 605: Atypical Movement Disorders as Clinical Presentation of Subacute Sclerosing Panencephalitis

Mitul Kasundra

S.M.S. Medical College, Jaipur, Rajasthan, India

Background and Aim: Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis occurring due to persistent mutant measles virus affecting children and adolescents and manifestations in form of psychiatric, cognitive decline, seizures and movement disorders are prominent. Subsequently, myoclonic seizures usually lead to a final stage of akinetic mutism. Aim: To highlight atypical presentation of SSPE and to create high index of awareness about phenotypic spectrum of the disease especially in developing countries.

Methodology: In this case series, we enrolled five children with subacute sclerosing panencephalitis whose presenting features were less common movement disorders like chorea, parkinsonism and unilateral myoclonus. All children had fulfilled the diagnosis as per modified Dyken criteria and were examined for movement disorders. We also assessed their clinical profile and disease severity via Jabbour staging

Results: out of five children 1 child had chorea, 2 children had parkinsonism and 2 children had unilateral myoclonus. MRI brain of all 4 patients showed T2 hyperintensities in subcortical white matter. EEG revealed periodic high amplitude (200-500MV) complexes in 4-10 seconds interval, nonspecific generalized slowing, frontal rhythmic delta activities. All the 4 patients were subjected to various investigations including viral markers, urinary copper and autoimmune profile. There were no significant differences in laboratory features among children with different movement disorders.

Conclusions: Movement disorders were frequent in subacute sclerosing panencephalitis. Hyperkinetic disorders were dominant. Movement disorders may manifest even in earlier stages of subacute sclerosing panencephalitis and may be the heralding feature. SSPE is a great mimicker and index of suspicion should be high in appropriate clinical setting.

Abstract ID: 606: Pregnancy-Associated Nodopathy Masquerading as Acute CIDP: A Rare Case Report

Arvind Visweshwar, P. K. Murugan, Venkateswaran, Justin

Madurai Medical College, Tamil Nadu, India

Background and Aim: We present a case of a pregnant woman who presented with symptoms suggestive of acute CIDP (Chronic Inflammatory Demyelinating Polyneuropathy) but was diagnosed with nodopathy, highlighting the diagnostic challenges and management considerations in pregnant patients presenting with neurological symptoms resembling CIDP.

Case Report: A 28-year-old pregnant woman in her third trimester developed progressive weakness, numbness and tingling in all limbs over four weeks, consistent with demyelinating polyneuropathy, potentially AIDP. Due to pregnancy, concerns arose about immunomodulatory therapy safety. She received IVIG and supportive care, improving on GBS disability scale. A month later, she relapsed, managed with IVIG again, showing partial improvement. Post-delivery, she presented with acute quadriparesis, neck weakness and hand/leg wasting. NCS indicated nodopathy, not CIDP. MRI showed no significant pathology. Treated with IV rituximab, she significantly improved, regaining full function and 6 months post-therapy she is currently symptom-free and fully mobile

Discussion: Initially, NCS indicated demyelinating polyradiculopathy with prolonged latency and absent F wave. After IVIG, conduction velocity and latency improved. In the second episode, NCS revealed reduced CMAP amplitude and prolonged latency. Postpartum, NCS showed reduced CMAP amplitude with conduction block, suggesting nodopathy. EMG showed fibrillation potentials. CSF was normal, autoimmune profile negative.

Conclusions: This case highlights nodopathy as a differential diagnosis in pregnant patients with CIDP-like symptoms. Tailored diagnostics and therapies are crucial for maternal and fetal well-being. Further research is needed to understand nodopathy’s pathophysiology and optimal management during pregnancy.

Abstract ID: 607: Craniovertebral Anomalies - Varied Presentation and Pathology - A Case Series

Ramachandran MohanRaj

Thanjavur Medical College, Thanjavur, Tamil Nadu, India

Background and Aim: Craniovertebral junction is the most important interface between the skull and the spine, comprising various bony and soft tissue structures. Anomalies of these regions can lead to diverse neurological presentations. Prompt identification and treatment are needed to avoid unnecessary complications. Here, we are presenting a series of patients presented with various neurological complaints and diagnosed to have CVJ anomalies.

Methodology: In the period of 6 months, patients attended to neurology out-patients department were examined thoroughly, and necessary neuroimaging was done. Of these, 7 patients were diagnosed as CVJ anomalies and all of them had different presentations with varied pathology.

Results: Over the period of 6 months, 7 patients were diagnosed to have CVJ anomalies by clinical and neuroimaging. Of these, 2 patients presented with chronic headache, who were being treated as migraine, one patient presented as transient flaccid quadriparesis which spontaneously improved, another with chronic spastic quadriparesis with chronic neck pain, one patient being treated with AED for drop attacks and one patient with multiple cranial nerve palsy. All these patients were initially evaluated for respective common causes and finally diagnosed as CVJ anomalies. These patients were managed with surgery according to need and improved.

Conclusions: CVJ, a distinct ligamento-osseous craniospinal axis structures, possesses unique embryological origins and specific biomechanical requirements. Diagnosing complexities in this region poses a challenge for imaging specialists and neurophysicians.

Abstract ID: 608: Clinico-Radiological and Genetic Spectrum of Childhood-Onset Hereditary Spastic Paraplegia: Experience from a Single-Center Retrospective Cohort

Vaishakh Anand, Amol Kumar Jadhav

Amrita School of Medicine, Kochi, Kerala, India

Background and Aim: Hereditary spastic paraplegias (HSP) are a group of genetically and clinically diverse inherited neurological disorders characterized by progressive weakness and spasticity in the lower limbs. It can be associated with other neurological or non-neurological symptoms (complicated forms, cHSP) or occur in relative isolation (pure forms, pHSP). HSP is usually reported in adults, and pediatric-onset forms have rarely been investigated in isolation. Our study aimed to explore the clinical, radiological and genetic spectrum of pediatric-onset HSPs in South Indian children.

Methodology: This retrospective chart review was conducted at a tertiary care center between January 2019 and December 2023 in children aged < 18 years with either pHSP or cHSP.

Results: 45 children (62% boys) with HSP were investigated, and genetic causes were identified in 18 children. Mean (SD) age at symptom onset was 3.5 years (1.2 years). Clinical features included: gait difficulty (31/45), delayed motor milestones (20/45), ataxia (6/45), cognitive impairment (5/45), and urge incontinence (6/45). MRI showed white matter changes in 10/45 (22%) and thinning of the corpus callosum in one child. All of them had gradual worsening spasticity, despite treatment. The majority of children had recessive form of HSP with positive family history in 13/45 (29%). SPG11 and KIF were the most frequent autosomal recessive and dominant HSPs respectively. Complicated phenotype was seen in almost half of study cohort.

Conclusions: Our study highlighted the clinico-radiological and genetic heterogeneity of pediatric HSP. High index of clinical suspicion with knowledge of genetic heterogeneity can lead to early diagnosis and timely institution of neurorehabilitative services thereby improving the quality of life in an otherwise degenerative disease.

Abstract ID: 609: Diverse Clinico-etiological Presentations of Osmotic demyelination syndrome - A Case Series

Laksheeba Duraisamy

Thanjavur Medical College, Thanjavur, Tamil Nadu, India

Background and Aim: Osmotic demyelination syndrome is a rare cause of secondary demyelination. It includes both central pontine and extrapontine myelinosis. Case reports of ODS were reported in the settings of rapid correction of serum osmolality or serum sodium. Here, we are reporting a series of patients diagnosed with ODS with varied pathogenesis and clinical presentations.

Methodology: After obtaining informed written consent, a detailed history and thorough examination were done. Relevant investigations were done. Neuroimaging and electrophysiological studies were carried out.

Results: ODS usually occurs in the setting of rapid correction of serum sodium. In our series of patients, all of them had normal serum sodium at the time of presentation and without prior history of correction. Two patients were alcoholics, one presented as subacute ataxia and dysarthria, and another with acute locked-in state mimicking vascular etiology. One was female, who was non-alcoholic with long-standing poor glycemic control, presented with chronic quadriparesis with dysarthria. All of them were evaluated and diagnosed as ODS based on clinical and neuroimaging and managed conservatively and improved

Conclusions: ODS is rare preventable complication of rapid fluctuations of serum sodium and osmolality. This potentially irreversible complication can be prevented by frequent monitoring of electrolytes. Hence ODS should be suspected in the setting of other precipitating factors like malnutrition and hyperglycemic state, with clinical features of intra-axial involvement.

Abstract ID: 610: Surgical Treatment of Epilepsy and its Outcome – From a Tertiary Care Hospital in India

Muthukani Sankarnarayana, A N S Madhuri

Apollo Hospitals Chennai, Tamil Nadu, India

Background and Aim: • Surgical treatment has become a viable and effective option for patients with drug-resistant epilepsy (DRE) who are poorly responsive to antiseizure medications (ASMs). Given that about 20% of individuals with epilepsy are resistant to ASMs, there would be approximately one million potential candidates for epilepsy surgery in India. • Yet, only <1000 epilepsy surgeries are currently being done annually and only 2 out of every 1000 eligible patients undergo epilepsy surgery in India • To share our experience with epilepsy surgery and provide an overview of the surgical treatments of epilepsies.

Methodology: This was a retrospective analysis of the demographics and clinical and investigative features of patients who underwent epilepsy surgery between January 2021 and August 2024.

Results: The study group included 10 patients with an age ranging from 4 years to 36 years. The mean (SD) age was 18.9 (11.2). The majority of our patients underwent an anterior temporal lobectomy and amygdalohippocampectomy (ATL), craniotomy procedures were done in two patients, and vagal nerve stimulation was done in one patient. The outcome of the Engel Outcome Scale was IA: completely seizure-free since surgery for 9 patients, and IV-B: no appreciable change for 1 patient, and in the ILAE Outcome Scale—class 1 in 9 patients and class 5 for 1 patient.

Conclusions: In carefully selected patients with DRE, surgery offers an excellent chance of becoming seizure-free with significant improvement in overall quality of life.

Abstract ID: 611: Navigating a Rare Neurological Conundrum: Quadriparesis in Neurocysticercosis with Hydrocephalus

Vamsi Krishna Mudamanchu

Vydehi Institute of Medical Sciences and Research Centre, Bengaluru (VIMS, Bengaluru)

Background and Aim: This case report highlights a rare and perplexing clinical presentation in a 55-year-old female who presented with a 1.5-year history of progressive weakness in her lower limbs, leading to walking difficulty. Upon examination, the patient displayed spastic quadriparesis, a magnetic gait, exaggerated deep tendon reflexes (DTR) in all four limbs, and extensor plantar responses. MRI brain and spine revealed non-communicating hydrocephalus accompanied by a cystic lesion located at the foramen magnum along with arachnoid webs, adhesions, and arachnoiditis, with associated mild compression of the upper cervical cord. The patient then underwent ventriculo-peritoneal (VP) shunt decompression along with cyst. The cyst excision biopsy report was suggestive of neurocysticercosis. Patient was then started on anthelmintic and steroids, following which patient had significant clinical improvement.

Methodology: Observational study of uncommon presentation of common disease.

Results: Cerebrospinal fluid (CSF) analysis displayed an unremarkable profile.

Conclusions: This case serves as a compelling illustration of the diagnostic challenges that clinicians may encounter when presented with a rare neurological presentation. Early recognition and treatment of neurocysticercosis are crucial to prevent complications such as seizures and neurological deficits. This case underscores the significance of a multidisciplinary approach involving neurologists, radiologists, pathologist, and infectious disease specialists to accurately diagnose and manage patients with complex presentations.

Abstract ID: 612: Clinical and Radiological Spectrum of Acquired Primary Demyelinating CNS Disorders from a Tertiary Care Hospital

Vamsi Krishna Mudamanchu

Vydehi Institute of Medical Sciences and Research Centre, Bengaluru (VIMS, Bengaluru)

Background and Aim: The term demyelination describes a loss of myelin with relative preservation of axons. The etiology of acquired primary demyelinating disorder is multifactorial. These demyelinating disorders are recognized by their clinical presentation, which may be monofocal or multifocal onset, course such as monophasic or polyphasic/relapsing, and specific neuro-imaging features of the brain and spinal cord. Consensus definitions and criteria are available for the diagnosis of most inflammatory demyelinating disorders such as MS, ADEM, and NMO.

Methodology: This is a retrospective cross-sectional descriptive study carried out in the department of Neurology in Vydehi Institute of Medical Sciences and Research Centre, Bengaluru, between January 2021 to April 2024. The study design is retrospective observational study.

Results: During this period, 38 patients with various demyelinating disorders were diagnosed. 2. There were 21 (55%) males and 17 (45%) females, with an M: F ratio of 1.2:1. 3. The mean age at presentation was 34.05 years The etiologies identified were as follows: idiopathic: 19, multiple sclerosis: 11, neuromyelitis optica spectrum disorder: 4, myelin oligodendrocyte antibody diseases: 4.

Conclusions: The acquired primary demyelinating disorders are not uncommon in our local population. Idiopathic demyelination was the most common, followed by multiple sclerosis. NMOSD and MOGAD had the same incidence in our adult population. However, larger studies over a longer period are required to confirm these findings and to observe the further conversion of idiopathic cases into MS or NMO.

Abstract ID: 613: Hyperhomocysteinemia and Cerebral Venous Thrombosis: Clinical Evaluation and Outcomes in Ten Patients

Perumalla Kiran, Veeranna Mohan Gadad, Veeranna Mohan Gadad, Veeranna Mohan Gadad

SS institute of Medical Sciences, Davangere, Karnataka, India

Background and Aim: Cerebral venous thrombosis (CVT) is a rare but serious condition characterized by the formation of blood clots within the cerebral veins and sinuses. Hyperhomocysteinemia is a recognized risk factor for thrombosis. Our aim is to present the clinical findings of these patients diagnosed with CVT associated with hyperhomocysteinemia.

Methodology: Ten patients were diagnosed with CVT and confirmed hyperhomocysteinemia. Inclusion: confirmed CVT via imaging, elevated homocysteine levels, and exclusion of other risk factors for thrombosis.

Results: Patient demographics and clinical presentation: age range: 25 to 45 years, common symptoms: headache, focal neurological deficits, and seizures; imaging findings: all patients had confirmed CVT on MRI/MRV, common sites: superior sagittal sinus, transverse sinus. Laboratory: elevated homocysteine levels in all patients. Clinical improvement was observed in all patients. Follow-up imaging showed recanalization or significant reduction in thrombus size.

Conclusions: CVT associated with hyperhomocysteinemia requires early identification and treatment which can improve outcomes in patients with CVT.

Abstract ID: 614: To Present Two Rare Cases of Cognitive Impairment Associated with Anti-Titin Antibody Anti-Titin Antibody an Innocent Bystander or Causative Agent of Cognitive Impairment?

Srikanth Gadam, Jaydip Ray Chaudhuri, Sandeep Kumar

Yashoda hospital Somajiguda, Hyderabad, Telangana, India

Background and Aim:

Methodology: We present two cases of rapidly progressing cognitive decline. The first patient, aged 60 years, presented with difficulty in findings ways, loss of interest in routine activities, difficulty in recalling names of familiar people, irritability, and forgetfulness in the form of misplacing objects since 6 months. On examination, the ACE III score was 75/100. The second patient, aged 65 years, presented with complaints of difficulty naming objects and difficulty in identifying familiar persons, as well as forgetfulness for three months. on evaluation, his ACE III score was 69/100.

Results: In the first patient, the serum and CSF autoimmune encephalitis panel were negative. The paraneoplastic panel showed anti-titin antibody positivity. CSF analysis, ANA profile, antithyroid antibodies, and complete hemogram were normal. MRI brain showed diffuse cerebral atrophy and small vessel ischemic changes. PET-CT brain showed hypometabolism in frontal and temporal lobes S/O front temporal dementia. Second patient’s autoimmune encephalitis panel was negative. CSF analysis, ANA profile, antithyroid antibodies, and complete hemogram were normal. MRI brain showed diffuse cerebral atrophy, left temporal gliosis, and small vessel ischemic changes. PET-CT brain showed moderate to severe reduced FDG uptake in left frontal, bilateral parietal, and temporal cortex of the brain and anterior cingulate cortex suggestive of front temporal dementia (semantic variant).

Conclusions: We present two cases of rapidly progressive cognitive decline showing anti-titin antibody positivity. After thorough literature search, there are no case reports showing association of anti-titin antibodies with dementia. Furthermore, number of cases are required to prove the association of anti-titin antibodies with dementia.

Abstract ID: 615: Dentatorubral-Pallidoluysian Atrophy (DRPLA) in Three Successive Generations of an Indian Family

Ranjot Kaur, Ayush Agarwal, Divyani Garg, Pooja Sharma, Mohd. Wasiq, Roopa Rajan, Ajay Garg, Mohd. Faruq, Achal K. Srivastava

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Dentatorubral-pallidoluysian atrophy (DRPLA) is a rare, progressive neurodegenerative disorder with an autosomal dominant inheritance pattern. It is characterized by six cardinal features: ataxia, epilepsy, cognitive impairment, myoclonus, chorea, and psychiatric abnormalities. The diagnosis is established in the presence of clinical features and heterozygous pathogenic CAG trinucleotide repeats in the ATN1 gene. The onset varies from childhood to old age and is inversely proportional to the number of CAG repeats. Individuals presenting before the age of 20 years typically present with epilepsy, myoclonus, and cognitive impairment, whereas later presentation is associated with ataxia, chorea, and neuropsychiatric symptoms.

Methodology: We present a series of three cases from successive generations in an Indian family.

Results: A 46-year-old gentleman presented with progressively increasing pancerebellar syndrome and seizures (unknown onset to bilateral tonic-clonic) for the past eight years. The seizures were refractory to anti-seizure medications. He had become increasingly forgetful and withdrawn for the past 2–3 years. His father had developed similar complains at the age of 70 years. The patient’s son was suffering from progressively increasing intellectual impairment, behavioral abnormalities, and seizures since the age of 10 years. An MRI brain revealed severe cerebellar atrophy and genetic testing revealed pathogenic heterozygous CAG trinucleotide repeats (CAG repeats – 58; normal: 6–35) on the ATN1 gene. Testing in his father (CAG repeats – 50) and son (CAG repeats – 69) revealed similar findings with increasing number of repeats in successive generations.

Conclusions: A wider gain in understanding is required for such rare neurodegenerative disease.

Abstract ID: 616: Double Trouble: Syphilis and/or Antiphospholipid Syndrome

Archana minimahesan, Harikrishnan R, Anilkumar Sivan, John K. John

Believers Church Medical College Hospital, Thiruvalla, Kerala, India

Background and Aim: We report the case of a 63-year-old gentleman k/c/O T2DM, hypertension, who presented initially with imbalance while walking, heaviness of head and numbness for two days. On examination, he had mild gait ataxia and minimal right hand dysmetria. An MRI brain stroke protocol with MRA was normal. Considering a diagnosis of MRI-negative stroke, he was started on dual antiplatelets and statins and sent home. During follow-up, he had persisting heaviness and numbness of head.

Methodology: One month later, he again presented with giddiness and multiple episodes of vomiting. On examination, his GCS was full. An MRI brain with MRA showed acute right pontine infarct and focal moderate stenosis of mid basilar artery.

Results: He was incidentally detected to have syphilis (VDRL+, TPHA+) in October 2023. Retaking history, he was found to have MSM contact history. He took oral doxycycline for 28 days, and serial monitoring of VDRL over months showed decreasing titers. During this admission, VDRL showed a four-fold increase in titers. CSF study showed lymphocytic pleocytosis and raised protein. CSF VDRL was nonreactive. So, he was diagnosed with meningovascular syphilis. APLA profile (workup for stroke) was found to be positive. He was treated with IV penicillin, and antiplatelets were continued. Follow-up VDRL at 6 weeks was negative. APLA remained positive.

Conclusions: APLA should be performed in all syphilis positive patient as it warrants lifelong anticoagulation.

Abstract ID: 617: A Clinico-Electrophysiological Study of Guillain–Barre Syndrome Amidst COVID-19 Illness

Rajarshi Chakraborty, Rajesh Verma

King George’s Medical University, Lucknow (KGMU Lucknow), Uttar Pradesh, India

Background and Aim: Guillain–Barré syndrome (GBS) is an acute acquired autoimmune inflammatory disorder of peripheral nerves and roots. The pathogenesis is essentially an aberrant post-infectious immune response in a genetically susceptible host milieu. Respiratory support is required in 14–44% patients of GBS during their course of illness, with a reported mortality of 5–10%, primarily due to autonomic failure and respiratory failure.

Methodology: A total of 75 patients (including 9 COVID-19-positive patients) of GBS admitted to our hospital over two years were thoroughly evaluated with clinical examination, blood laboratory tests, and nerve conduction study, including phrenic nerve study.

Results: The median age was 31 years (range 1–75 years) with male–female ratio 2:1, and subtype analysis showed AIDP (10.67%), AMAN (38.66%), and AMSAN (50.67%). The mean EGRIS score at admission was 3.8 ± 0.17 days, and mean MRC score at admission was 30.35 ± 1.80 with need for ventilatory assistance observed in 24 (32%) patients with a mean duration of ventilatory stay of 23.83 ± 3.50 days. It reduced to 16 ± 3.89 days in COVID-19 patients with GBS due to increased early deaths. Mortality was observed in 8 (10.67%) patients which increased to 2 (22.22%) patients in COVID-19 illness with ventilatory deaths occurring in 8 (33.33%) patients that increased to 2 (50%) in COVID-19 illness.

Conclusions: The development of GBS in COVID-19 illness had a worse outcome with increased mortality and morbidity.

Abstract ID: 618: A Study on the Clinical and Etiological Profile of Drug Resistant Epilepsy in a Tertiary Care Hospital of Eastern India

Ayon Ghorai, Subrata Halder

Background and Aim: There has been a growing body of research in relation to DRE due to its debilitating consequences and their propensity to reoccur. Early identification and prompt institution of therapy result in good outcome. To study the clinical characteristics of patients presenting with DRE and to analyze the etiological spectrum of DRE patients.

Methodology: This retrospective observational cross-sectional study was conducted at neurology ward and OPD of XXXXXXX. Between March 2023 and February 2024, 57 patients of DRE were evaluated. Diagnosis is based on ILAE definition. An MRI brain and EEG were performed for all. Additional investigations were conducted as etiology concerned.

Results: After ruling out pseudo-resistance, 57 patients (32 M, 25 F) were included. The age of presentation varied from 4 months to 61 years, with the maximum number of patients in the age group of 21–30 years. The most common seizure semiology was FIAS in 28 (49.1%) patients, FAS 11 (19.4%) patients, GTCS in 6 (10.5%) patients, and multiple seizure in 12 (21%) patients. MRI findings showed normal results in 10 (17.5%) patients, MTL sclerosis in 15 (26.3%), gliosis in 6 (10.5%), diffuse brain atrophy, and HIE in 5 (8.7%).. Among other causes, focal cortical dysplasia, limbic encephalitis, tuberculoma, etc., were found.

Conclusions: Etiological evaluation of DRE patients is important to assign proper treatment and assess the outcome. MRI brain is one of the most important investigations in identifying focus.

Abstract ID: 619: A Changing Trend in Cerebral Venous Sinus Thrombosis

Priya Robert, Shaji C V

Government TD Medical College Alappuzha, Kerala, India

Background and Aim: INTRODUCTION – This is an uncommon form of stroke, predominantly found in young patients. AIMS AND OBJECTIVES – Primary objective: To study the clinical presentation of cerebral venous sinus thrombosis. Secondary objective: 1. To ascertain the etiology of cerebral venous sinus thrombosis.

Methodology: MATERIALS AND METHODS STUDY DESIGN: Descriptive study; STUDY SETTING: Department of Neurology, tertiary care center; STUDY POPULATION: Patients attending neurology OPD/general medicine OPD and IP with cerebral venous sinus thrombosis. STUDY PERIOD: January 2022 – JULY 2023 INCLUSION CRITERIA: 1. Age above 18 years; 2. Patients attending the neurology and general medicine department having MR venography or contrast enhanced CT scan showing evidence of cerebral venous thrombosis. EXCLUSION CRITERIA: 1. Subjects with isolated cavernous sinus thrombosis. A total of 44 patients included in this study. Study protocol was explained in detail to the patients/relatives and informed written consent in regional language obtained from them before conducting the study. Detailed history and clinical examination with MRI brain with MR venogram or CECT brain was conducted. History regarding DVT, malignancy, OCP use, etc., was interrogated and recorded. Relevant investigations were conducted in appropriate cases. Correlation between clinical and etiological findings was made. Data analysis was performed using SPSS17.

Results: Results – The mean age of the study participants was 39 years (14.6%). A total of 52% were males, and 48% were females. Out of the 23 males 65% and among 21 females, 9.5% had alcohol use. A total of 48% of males had history of smoking. A total of 41% were overweight, and 14% were obese. A total of 16% had anemia, and 5% had polycythemia. Headache (95.4%), vomiting (47.7%), seizure (36.4%), altered sensorium (22.7%), fever (11.4%), focal deficit (38.6%), aphasia (2.3%), diplopia (13.6%), ophthalmoplegia (25.0%), and papilledema (52.3%) were observed.

Conclusions: Alcoholism is strongly associated with male CVT ( P < 0.001). High index of suspicion for CVT is indicated if an alcoholic person presents with headache with raised intracranial pressure.

Abstract ID: 620: Complex Interplay of Autonomic Nervous System During Yoga Nidra, Sleep, and Wake in Patients with Coronary Artery Disease

Anupama Gupta, Alka Arya, Arun EV, Gautam Sharma

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Autonomic imbalance associated with increased sympathetic activity and reduced vagal tone has been strongly implicated in the pathophysiology of myocardial infarction and sudden cardiac death. In post-MI patients, reduced heart rate variability (HRV) is a marker for autonomic dysfunction and a strong predictor of increased mortality. Yoga Nidra (YN) modifies the autonomic balance on favorable side through complex interplay between the sympathetic and parasympathetic nervous system. The aim of current study is to explore how autonomic variation on HRV during YN is different from sleep and wake states in post-MI patients.

Methodology: Male patients with coronary artery disease, who were ongoing 24 h Holter recording, were given with YN sessions in the morning hours between 10–12 AM. These sessions were given by a trained yoga therapist, and duration and time for various sections of YN session was noted manually. Sleep was marked on Holter recording according to patient’s sleep log and baseline shift of heart rate. The day time HRV was measured from end of YN session to the sleep onset. HRV parameters during YN, sleep, and wake state were compared.

Results: A total of nine sessions with six male patients with coronary artery disease were recorded during Holter recordings. The mean age of participants was 56.88 ± 7.81 years. Autonomic profile during YN is similar to sleep state, except for a significant reduced in TINN during YN (306.43 ± 108.51 vs. 184.98 ± 107.67, P = 0.041). The percentage of HF is significantly higher during YN sessions compared to wake state (19.67 ± 11.27 vs. 8.45 ± 5.27, P = 0.024).

Conclusions: “Yoga Nidra” effectively modulates autonomic nervous system, and percentages of HF power were significantly increased during YN sessions.

Abstract ID: 621: Acute Onset Oculopharyngeal Weakness in a Young Female – Atypical Presentation of a Common Disorder

Preeti Gupta, Rajesh Verma, Rajarshi Chakraborty

King George’s Medical University, Lucknow (KGMU Lucknow), Uttar Pradesh, India

Background and Aim: Myasthenia Gravis is commonly associated with antibodies targeting the acetylcholine receptor (AChR). A subset of patients has antibodies against muscle specific kinase (MuSK) and anti-LRP4 antibodies. However, seronegativity varies in various types. In this case report, we tried to extrapolate different diseases presenting with acute onset oculopharyngeal weakness.

Methodology: A 28-year-old non-pregnant female presented with complaints of acute onset, rapidly progressive slurring of speech, difficulty swallowing, and ptosis of bilateral eyelids for one day duration. She had history of double vision three days prior without diminution and blurring of vision, reddening of eyes or pain on eye movements. There is no associated history of headache, seizures, any limb weakness, paresthesias, and bowel bladder involvement. There was no other significant history. On examination, general examination was within normal limits. On CNS examination, patient was well oriented to time, place, and person with normal higher mental functions. Her speech was characterized by flaccid dysarthria. Cranial examination revealed bilateral cranial nerve 3, 4, 6, 9, and 10 palsy. Motor examination showed normal tone and a power of 4/5 at proximal shoulder and hip, DTRs were normal. Rest examination was within normal limit. Patient worked up for oculopharyngeal weakness.

Results: Hemogram, KFT, LFT, and viral markers were within normal limits. Vasculitis profile, including ANA and ANCA, was normal. On suspecting MG, ice pack test and neostigmine test were performed which showed transient improvement. NCV done was normal study. Repetitive nerve stimulation test showed greater than 10% decremental response in bilateral orbicularis oculi muscles. Cranial magnetic resonance imaging was normal. Anti-AChR and MuSK antibodies levels were within normal range. Patient given IVIG 2 g/kg over five days and showed clinical improvement.

Conclusions: Double seronegative MG is seen in 15% of generalized MG and 50% of ocular MG. Early treatment shows good clinical outcomes.

Abstract ID: 622: Status Epilepticus: Clinico-Radiological and Electroencephalographical Spectrum and It is In-Hospital Outcome

Abhay Ranjan, Amitabh Kumar, Neetu Sinha

IGIMS Patna, Bihar, India

Background and Aim: The objective of this study is to evaluate the clinical, radiological, and electroencephalographical spectrum of patient with status epilepticus, and it is in-hospital outcome.

Methodology: Patients with status epilepticus were evaluated including their demographics, history of epilepsy, antiepileptic drug (AED) default, and status epilepticus type. Blood counts, serum chemistry, ECG, cranial MRI, cerebrospinal fluid, and EEG were performed. Death during hospital was also recorded.

Results: The study included 22 patients with status epilepticus (12 female and 10 male). A total of 95.5% of patients had convulsive, and 4.5% had nonconvulsive SE. The etiology of status epilepticus was autoimmune in 22%, infection in 18%, drug default in 13%, metabolic and hypoxic ischemic sequele in 9% each, and miscellaneous in 16% of patients. No cause could be determined in 13% of status epilepticus patients. Following first AED, status epilepticus was controlled in 36% and 36% responded to second antiepileptic medication. Out of 23 patients, 13 (57%) had non-refractory status epilepticus, and 10 (43%) had refractory status epilepticus. Out of refractory status epilepticus (RSE), five were super RSE. Cranial MRI was abnormal in 55%. Generalized background slowing was the most common EEG abnormality. Overall mortality was 21%. Mortality in RSE was 30%, while mortality in super RSE was 40%. Mortality in NRSE was 15%.

Conclusions: Most common etiology for status epilepticus was autoimmune and infection. About half of the patient had RSE. Mortality was more in patient having super RSE.

Abstract ID: 623: Thalamic Experiential Hallucinosis Presenting as Self-Biting Behavior at Stroke Onset

Soaham Desai, Manthan Dave, Sachin Udwani

Shree Krishna Hospital Pramukhswami Medical College, Anand, Gujarat, India

Background and Aim: The thalamus plays a critical role in integrating sensory information. Damage to this region can disrupt normal sensory processing, leading to vivid sensory hallucinations, a phenomenon known as “experiential hallucinosis.” This case report presents an unusual manifestation of this condition, where the patient exhibited self-biting behavior at the onset of a thalamic stroke.

Methodology: A 64-year-old male farmer was brought in after suddenly biting his right hand twice during sleep. According to witnesses, the patient seemed to be in a dream-like state but was alert upon awakening, with no signs of a seizure. The patient reported feeling heaviness in the right half of his body and a sensation of being forcefully held and unable to free himself. He claimed to have bitten his hand in an attempt to free himself from what he perceived as a thief who had entered his room and muzzled him.

Results: Physical examination revealed right-sided sensorimotor deficits. An MRI brain confirmed a left thalamic hemorrhage. Toxicology screening and electroencephalography were unremarkable, and a psychiatric evaluation did not reveal any significant findings.

Conclusions: This case represents an unusual presentation of a thalamic stroke, with self-biting behavior likely triggered by vivid experiential hallucinations. It highlights the potential for thalamic strokes to manifest with complex neuropsychiatric symptoms distinct from typical lacunar syndromes and expands our understanding of the symptomatic presentations associated with disruptions in sensory processing.

Abstract ID: 624: Neurological Disorders in Pregnancy and Postpartum Period: A Prospective Observational Study on Prevalence and Associated Maternal-Fetal Outcomes

Soaham Desai, Devangi Desai, Harsh Marvania

Shree Krishna Hospital Pramukhswami Medical College, Anand, Gujarat, India

Background and Aim: Neurological disorders contribute significantly to maternal morbidity and mortality during pregnancy and the postpartum period, accounting for approximately 20% of maternal deaths. Early recognition and management are crucial for optimizing outcomes. Objectives: To determine the prevalence of neurological disorders among pregnant and postpartum women (up to 6 weeks) and evaluate their association with maternal and fetal outcomes.

Methodology: A prospective cross-sectional observational study was conducted at Shree Krishna Hospital, Karamsad, Anand, Gujarat, India, between September 2022 and April 2024. Adult pregnant or postpartum patients with neurological conditions were included. Data on maternal, fetal outcomes, comorbidities, and investigations were collected and analyzed using appropriate statistical methods.

Results: The study included 108 participants (10% prevalence) with neurological problems, 85% antenatal, and 15% postpartum. The most common comorbidities were hypertension (13%) and hypothyroidism (11%). The prevalent neurological disorders were pre-eclampsia (56%), eclampsia (19%), and HELLP syndrome (10%). Headache was the most common symptom (11%). While 87% of patients recovered fully, fetal outcome was good in 63%. Approximately 20% required ICU admission, and one-third of neonates required NICU admission.

Conclusions: This study highlights the significant burden of neurological disorders, particularly hypertensive disorders, among pregnant and postpartum women. Early recognition and management are crucial for improving maternal and fetal outcomes and reducing intensive care admissions.

Abstract ID: 625: Unraveling the Link: Myasthenia Gravis and Papillary Carcinoma of the Thyroid

Arvind Visweshwar, Murugan, Venkateswaran

Madurai Medical College, Tamil Nadu, India

Background and Aim: We present a rare case of a 65-year-old female who initially presented with ocular symptoms suggestive of myasthenia gravis (MG) but was incidentally diagnosed with papillary carcinoma of the thyroid during diagnostic workup. This case highlights the importance of comprehensive evaluation and interdisciplinary collaboration in managing patients with autoimmune disorders, as well as the significance of considering concurrent pathologies in atypical clinical presentations.

Methodology: A 65-year-old female presented to our neurology clinic with a three-month history of intermittent diplopia and ptosis, particularly worsening with prolonged periods of near vision and alleviating with rest. She denied any difficulty in swallowing, voice changes, or limb weakness. Neurological examination revealed bilateral ptosis, more pronounced on sustained upgaze, and mild limitation of extraocular movements, consistent with ocular onset MG. Acetylcholine receptor antibody testing was positive, confirming the diagnosis.

Results: Given the coexistence of autoimmune MG and the patient’s age, a thorough evaluation for associated autoimmune conditions was initiated. Thyroid function tests were within normal limits, but ultrasound of the neck revealed a 1.5 cm hypoechoic nodule in the right thyroid lobe. Fine-needle aspiration biopsy (FNAB) of the nodule was performed, and histopathological examination revealed features suggestive of papillary carcinoma. Subsequent thyroid imaging demonstrated no evidence of regional or distant metastasis, and the patient was referred to the endocrine surgery team for further management.

Conclusions: Interdisciplinary collaboration and early detection are vital for managing concurrent MG and papillary carcinoma of the thyroid.

Abstract ID: 626: Synapses to Skeleton – A Rare Cause of Early-Onset Dementia

Niveditha C, Sanjana D S, Archana N B, Praveen Kumar S

Bangalore Medical College and Research Institute, Bengaluru (BMCRI Bengaluru), Karnataka, India

Background and Aim: Nasu-Hakola disease, also known as polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL), is a rare genetic disorder. It primarily affects the Finnish and Japanese populations. The disease is characterized by early-onset dementia and multifocal bone cysts caused by mutations in the genes TREM2 and TYROBP.

Methodology: We report a case of young onset dementia who turned out to be Nasu-Hakola disease.

Results: A 45-year-old male security guard presented with progressive behavioral changes for 4 years, urinary incontinence for 3 years, and gait disturbances for 2 years. Symptoms included inappropriate smiling, apathy, impulsive behavior, repetitive speech, distractibility, and difficulty recalling recent events. He developed socially unaware urinary incontinence and a dragging foot, stiff-legged gait. Born of a consanguineous marriage, with no family history of dementia or neuropsychiatric disorders, he was conscious but disoriented to time and place, with alopecia, short stature (160 cm), and a MOCA score of 7/30. He had vertical gaze restriction, limb spasticity, brisk reflexes, and bilateral extensor plantar reflex. MRI showed diffuse cerebral atrophy with periventricular T2 white matter hyperintensity and basal ganglia calcification. X-rays revealed fractures and osteoporosis. Whole exome sequencing identified a homozygous TREM2 mutation, diagnosing him with Nasu-Hakola disease. He is being treated with donepezil, memantine, baclofen, physiotherapy, and orthopedic care, with genetic counseling provided to his family.

Conclusions: This case serves as a reminder to clinicians to consider this rare form of dementia in patients presenting with early-onset dementia accompanied by fracture and bony cysts.

Abstract ID: 627: Stroke Stories: Three Challenges in the Medullary Maze

Vignesh Kumar, Rajagambheeran Venugopal, Jawahar M

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Stroke, an acute neurological syndrome of cerebral vascular origin lasting over 24 h, can stem from either ischemia or hemorrhage. We present three varied presentations of medullary stroke syndromes

Methodology: Patient 1: B/L medial medullary syndrome patient was a 67/M, on treatment for diabetes and hypertension. Patient had sudden onset giddiness and nausea. Four hours later, he developed weakness of all four limbs with difficulty in swallowing both solids and liquids. He was found to have gaze-evoked nystagmus. Hypoglossal diplegia, decreased gag reflex, and power of 1/5 in all four limbs were noted. Plantar was extensor bilaterally. MRI brain showed bilateral medial medullary stroke, typical “heart appearance sign.” Patient expired after a week due to sepsis secondary to aspiration pneumonitis.

Results: Patient 2: Opalski syndrome patient was a 75/M, with background hypertension, COPD, chronic smoker, and alcoholic. He presented with acute onset right hemiparesis, right UMN facial lag, right palate weakness, right hemifacial numbness, and ipsilateral ataxia. There was no contralateral hemi sensory loss and no Horner’s syndrome. MRI was suggestive of lateral medullary syndrome.

Conclusions: Stroke mechanisms in medullary infarction differ between lateral and medial medullary syndromes. Clinical and radiological characteristics, especially perfusion weighted MRI features, are helpful in discriminating the etiologies of stroke in these patients.

Abstract ID: 628: Serum Biomarkers for Differentiating Alzheimer’s Disease from Vascular Dementia – A Cross-Sectional Study from Eastern India

Sidharth Anand, Supriyo Chowdhary, Hrishikesh Kumar, Sattwika Banerjee, Snehashree Neogi, Rebecca Banerjee, Krishnendu Chowdhary

Institute of Neurosciences, Kolkata, West Bengal, India

Background and Aim: AD and VaD are two common types of dementia in the elderly, with overlapping presentation, and clinical course may lead to diagnostic confusion and prognostic difference. Preclinical studies have shown evidence of several peripheral biomarkers which needs extensive validation in clinical samples in diverse race and ethnicity.

Methodology: In the current cross-sectional study, patients diagnosed with AD and VaD as per standard diagnostic criteria recruited along with age- and sex-matched controls without cognitive impairment were selected from the neurology outpatient department. MRI scans were performed to aid diagnosis. Participants underwent cognitive assessments through MoCA, CDR, MDRS, and assessment for depression through BDI-II scale. Blood samples were collected for estimation of human amyloid (A) beta 40/ 42 and human MAP? (microtubule associated protein Tau/ phosphorylated Tau) protein levels from their respective serum samples. The protein levels were measured using Kit based ELISA technique (Elabsciences, China).

Results: We recruited 20 AD and 12 VaD patients and 20 controls of comparable age (64 ± 7, 65 ± 7 years, P = 0.884) and sex. The MOCA scores were not different in AD and VaD groups (16.5 ± 6 and 18.9 ± 4, P = 0.247), and controls have normal MOCA (>25). The A-beta 42 were significantly low in control and AD compared to VaD patients (687.4, 1349.1 pg/mL, P = 0.003). Total Tau/A-beta 42 ratio was increased in in AD compared to control and VaD. Similar positive correlation was observed between phosphorylated Tau and BDI-II in VaD (0.816, P = 0.001).

Conclusions: These preliminary results indicate that increased A-beta 42 and total Tau in VaD can be a promising biomarker for differentiating AD and VaD.

Abstract ID: 629: Navigating Complexities in Cortical Vein Thrombosis: A Case of Recurrence and Resolution

Justin

Madurai Medical College, Tamil Nadu, India

Background and Aim: Cerebral venous thrombosis (CVT) is an uncommon but significant cause of stroke, involving thrombosis of the cerebral veins or dural sinuses. Isolated cortical vein thrombosis (ICVT) is a rare subtype, often presenting diagnostic challenges due to its variable clinical manifestations and imaging findings.

Methodology: A 45-year-old female presented with sudden onset right hemiparesis, upper motor neuron (UMN) facial palsy, and right focal seizures. MRI revealed left superficial cortical vein thrombosis, an acute infarct with hemorrhagic transformation in the left parietal lobe, and subarachnoid hemorrhage (SAH) in the left fronto-parietal sulci. She was treated with anticoagulation therapy and discharged on long-term medication. One year later, the patient experienced numbness on the right side of her face and right upper limb. MRI at this time showed deep cerebral vein thrombosis. She was managed with appropriate medical therapy, including adjustments to her anticoagulation regimen. Coagulation and autoimmune workups were negative for any underlying disorders.

Results: The patient’s initial and subsequent thrombotic events were successfully managed with anticoagulation. Follow-up imaging showed recanalization of the previously thrombosed veins. Throughout her treatment, no underlying coagulation or autoimmune disorder was identified.

Conclusions: Timely diagnosis and individualized management of recurrent ICVT can lead to favorable outcomes despite the absence of identifiable underlying disorders.

Abstract ID: 630: Vacous Smile – Is it Always Wilson’s ?

Ganesh Sreeprakash, Velusami

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: The spectrum of autosomal recessive early-onset parkinsonism is clinically and genetically heterogenous. Here, we report a 16-year-old female presented with asymmetrical upper limb onset dystonia along with oromandibular dystonia and vacous smile with parkinsonism features, and genetic workup showed a novel homozygous likely pathogenic variant in SYNJ1 gene.

Case Report: This is a young female with no known comorbidities born out of third degree consanguineous marriage with a background history of poor scholastic performance presented with insidious onset gradually progressive neurological ailment as involuntary movement in the form of tremulousness and twisting movement of left upper limb followed by left lower limb with intermittent posturing for past one year. She also developed intermittent opening of her mouth with drooling of saliva and difficulty in making a bolus of food in mouth. She also developed difficulty in articulation which progressed to complete anarthria over the past eight months. She also had generalized slowness in activities. On examination, she had a vacous smile with reduced blink rate and anarthria. MMSE was 14/30. Lobar function test showed impaired frontal lobe functions. She had hypometric saccades. Dystonic tremor noted in left upper limb and lower limb with oromandibular dystonia. She had generalized bradykinesia with axial and appendicular rigidity. Glabellar tap was non-accomodative. Examination of cerebellum and autonomic systems was normal.

Discussion: Routine blood investigations, thyroid, serum ceruloplasmin, peripheral smear, and serum ferritin were normal. MRI brain was normal. WES done showed autosomal recessive homozygous mutation in SYNJ1 (novel frameshift variant). She was treated with clonazepam and trihexyphenidyl and had marked improvement in her dystonia.

Conclusions: Screening for SNYJ1 should be considered in patients with early-onset parkinsonism with predominant dystonic symptoms and with associated cognitive impairment. Clonazepam effectively resolves the symptoms of the SYNJ1-related disease. SYNJ1 and most of other PD proteins play a role in vesicle recycling and lipid metabolism at the synapse; the study of SYNJ1 and its associated pathways is crucial for developing a deeper understanding of PD pathogenesis and for finding innovative therapeutic strategies to combat this debilitating disease.

Abstract ID: 631: Emerging Trends in Neuroscience: Role of Artificial Intelligence

Nalin Chaudhury, Randi Venkata Narayana

Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

Background and Aim: AI has been applied in health care for diagnostic assessment, virtual health assistants, treatment of rare diseases, targeted treatment, and drug discovery. The intersection of AI and neuroscience offers transformative potential for understanding and treating neurological disorders. The primary aim of this paper is to explore the advantages and disadvantages of integrating AI into neuroscience.

Methodology: This paper adopts a comprehensive review methodology, synthesizing recent advancements in AI applications within neuroscience. Sources include peer-reviewed journals, conference proceedings, and authoritative texts published over the last decade.

Results: The application of AI within neuroscience enhances diagnosis, improves treatment strategies (neurosurgery and epilepsy surgery), and facilitates the research on brain function and drug discovery for degenerative disorders of brain. However, the integration of AI has many challenges and risks, including data privacy and security, ethical dilemmas, and accessibility and inequality.

Conclusions: The use of AI in neuroscience marks the beginning of a new era in medical innovation. It has the potential to revolutionize diagnostics, treatment, and research in neurology and neurosurgery. However, the ethical and practical challenges associated with AI deployment cannot be overlooked.

Abstract ID: 632: Roger Syndrome – The Seizure Spectrum Unveiled

Ankur Vivek, Vijaya Nath Mishra, Abhishek Pathak

Institute of Medical Sciences, Varanasi (IMS, Varanasi), Uttar Pradesh, India

Background and Aim: Thiamine responsive megaloblastic anemia (TRMA) also known as Rogers syndrome is a rare entity inherited as an autosomal recessive disorder. It consists of diabetes mellitus, megaloblastic anemia, thrombocytopenia, leukopenia, and sensory-neural hearing loss. Here, we report a 17-year-old girl with megaloblastic anemia, sensory-neural deafness, and young onset diabetes mellitus who presented with generalized tonic-clonic seizures.

Case Report: A 17-year-old girl came to neurology department with history of loss of consciousness succeeded by frothing from mouth, abnormal jerking of the body, and sphincter incontinence for the past four years. Upon inquiring her mother, they had a consanguineous marriage. Being a new born, she was noted to have lethargy, and generalized weakness for which help was sought. At that time based on his blood peripheral film and bone marrow biopsy, she was diagnosed to have megaloblastic anemia and received her first blood transfusion at the age of 12 months. Since then, she would require monthly blood transfusions. At the age of 2.5 years, her parents noted hearing impairment and lack of attention when confronted, he was diagnosed to have a sensory-neural deafness. Similar illness was observed in both her younger brothers.

Discussion: Upon investigations, her blood picture showed a hemoglobin of 9 g/dL blood indices, MCV was 106 FL, RBS was 362, and HbA1c was 10.8%. Bone marrow aspiration and trephine biopsy showed normal cellular bone marrow with megaloblastic changes. MRI brain was normal, and EEG was suggestive of 1–2 Hz spike and wave activity. WES was suggestive of SLC19A2 mutation in all affected members.

Conclusions: This clinical entity and its association with epilepsy are extremely rare and must be thought of among the differentials of megaloblastic anemia and epilepsy specially in developing countries.

Abstract ID: 633: A Malady of Two Buoyant Girls Case Reports of SSPE

Kirthika Kannan, Hariharan S, Balaji Gopalan

Chengalpattu Government Medical College, Tamil Nadu, India

Background and Aim: The presentation of SSPE in the adult population seems to be rare with adequate immunization.

Case Report : Two cases presented here had a short duration of symptomatology onset, with response to the management of myoclonus along with a slow course of the disease on follow-up.

Discussion: The observation and follow-up showed that there was an improvement of myoclonus with ASM and adult SSPE progresses rather slowly, with non-worsening of intellectual decline or functional status.

Conclusions: “Adult SSPE progresses slowly” – more evidence is warranted.

Abstract ID: 634: Case Series of Deep Cerebral Venous Thrombosis Patients with Better Prognosis

Siddharthan R, Balaji G, Hariharan S, Thamilpavai N

Government Chengalpattu Medical College, Tamil Nadu, India

Background and Aim: Cerebral venous thrombosis (CVT) is a serious and often fatal medical condition with an increasing incidence, accounting for about 0.5% of stroke patients and a prevalence of 5 per 1 million. Deep CVT is estimated in about 10% of patients with CVT, and it occurs as an isolated event in approximately 1% or accompanies superficial cerebral vein and sinus thrombosis in 15% of the cases. Hence, we present case series of three patients of deep CVT in our institute.

Methodology: Case series of three patients with deep CVT.

Results: With early diagnosis and prompt treatment, three patients of deep CVT improved well.

Conclusions: Because prompt anticoagulant therapy has a great impact on CVT outcome, early diagnosis is essential, especially in patients with involvement of the deep cerebral veins and which plays an important role in prognosis and outcome.

Abstract ID: 635: Acute Ataxic Syndrome in Dengue Fever

M Abirami, Hariharan S, Balaji G, Thamil Pavai N

Government Chengalpattu Medical College, Tamil Nadu, India

Background and Aim: Dengue fever is an arboviral infection mainly found in tropical and subtropical countries. Serotype 2 and 3 are mostly related to CNS manifestations, like encephalopathy, encephalitis, meningitis, ischemic and hemorrhagic stroke, cerebellar syndrome, transverse myelitis, ADEM, GBS, myositis, AIDP, brachial neuritis, and cranial neuropathy.

Methodology: Case reports of the four patients.

Results: Dengue fever is one of the reversible causes of acute ataxia.

Conclusions: India is one of the highly endemic country for dengue, hence all extended complications of dengue especially neurological complications to be anticipated, diagnosed and to be treated in appropriate setting to prevent disability. ADEM is one of the rare CNS complications of dengue fever.

Abstract ID: 636: A Cross-Sectional Study of Assessment of Autonomic Nervous System in Acute Stroke Patients at Three Months Follow-Up

Prem Vaijwade

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim: Post-stroke subjects are likely to have autonomic dysfunction which may predispose them to falls and delay their recovery. If diagnosed appropriate interventions can be provided. To study the prevalence and pattern of autonomic dysfunction (sympathetic and parasympathetic) among patients admitted with acute stroke.

Methodology: A prospective, cross-sectional observational study of acute stroke patients (ischemic and hemorrhagic) was conducted in a tertiary care medical college in Maharashtra from January 2023 to May 2024. Patients were tested at three months follow-up after acute stroke. Patients with recurrent stroke, NIHSS >24, and parkinsonism were excluded. Both arms of autonomic dysfunction were tested – sympathetic (six deep breath tests, standing test, and Valsalva test) and parasympathetic (handgrip test and cold immersion test).

Results: In our study of 43 cases of acute stroke, 72.1% were males. Mean age was 57.12 years. Risk factors were diabetes mellitus (46.5%), hypertension (39.5%), alcohol (32.6%), ischemic heart disease (16.3%), and smoking (14%). Sympathetic dysfunction was observed in 6 (14%), parasympathetic dysfunction was observed in 4 (9.3%), and 4 (9.3%) had both. Cold immersion test and Valsalva tests were the most sensitive tests in diagnosis autonomic dysfunction. Among those with autonomic dysfunction, four out of six (66%) were diabetics, and 1 (16%) had IHD and was not on any alpha blockers nor diuretics.

Conclusions: In our study, the prevalence of post-stroke autonomic dysfunction was found to be 14%. This study highlights the problem of autonomic dysfunction among patients with acute stroke. Further studies need to be conducted to establish post-stroke dysautonomia.

Abstract ID: 637: Prevalence of Electrolyte Abnormalities in Patients with Acute Stroke Presenting to a Tertiary Care Hospital

Nischal Yedla, Naveen Kumar Venigalla, Sudheer Penchala Puttiboyina, Krishnaveni Vanapala, Ananya Krosuri

Kamineni health care, Poranki, Vijayawada, Andhra Pradesh

Background and Aim: The incidence of stroke in India ranged from 105 to 152 per lakh persons per year over the past 20 years. Sodium and potassium concentration disorders are common electrolyte abnormalities in CVA, potentially leading to mortality if not corrected in time. Data on the prevalence of electrolyte disturbances in stroke patients, particularly in developing countries, is insufficient. This study aims to determine the frequency of electrolyte imbalance in patients presenting with acute stroke at a tertiary care hospital.

Methodology: This retrospective cross-sectional study was carried out in the neurology department at a tertiary care hospital in India from December 2023 to April 2024. A total of 107 patients, aged between 30 and 70 years, diagnosed with either ischemic or hemorrhagic stroke via CT scan or MRI of the brain were enrolled. The biochemical analysis of the stroke patients was conducted as routine.

Results: Out of 107 participants, 61 (57.0%) were in the ischemic stroke group, while 46 (43.0%) were in the hemorrhagic stroke group. The mean sodium level was significantly lower in the ischemic group compared to the hemorrhagic group (128.12 ± 4.30 mEq/L vs.137.45 ± 6.15 mEq/L; P value < 0.0001). Potassium levels were significantly higher in the hemorrhagic group compared to the ischemic group (4.65 ± 1.05 mEq/L vs. 4.02 ± 0.6 mEq/L; P value < 0.0001).

Conclusions: Patients arriving at the emergency department with stroke should be screened for electrolyte imbalance as part of routine investigations. Early detection of serum electrolyte imbalances may facilitate prompt intervention and improved outcomes. Monitoring electrolyte imbalances is crucial to prevent complications and improve prognosis.

Abstract ID: 639: Juvenile Sandhoff Disease – A Rare Case of Autosomal Recessive Cerebellar Ataxia

Tejeswara Rao Salihundam, Tejeswara Rao Salihundam, Tejeswara Rao Salihundam, Gopi Seepana, Sateesh Kumar T, Sushma Ch, Manoj Kumar K

Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

Background and Aim: INTRODUCTION: Sandhoff disease is a rare inherited neurodegenerative disease due to hexosaminidase B deficiency causing GM2 ganglioside accumulation. Juvenile Sandhoff disease is much rarer.

Methodology: CASE REPORT: A 10-year-old boy presented with six years duration of insidious onset gradually progressive unsteadiness of gait, followed by 4 years duration of cognitive decline along with speech disturbances, defective eye movements, decreased social interaction, and loss of bowel and bladder control. On examination, child is emaciated, with poor attention, dysarthria, complete ophthalmoplegia, hypotonia, gait, and appendicular ataxia, with SARA score of 20 and dystonia of both lower limbs.

Results: RESULTS – Blood routines were normal. MRI brain showed severe cerebellar atrophy and mild cerebral atrophy with periventricular T2 hyperintensities. Fundoscopy is normal. EEG is normal. NCS showed axonal polyneuropathy. Ultrasonography does not relieve any organomegaly. 2D echo is normal. Thyroid profile, lipid profile, and S.B12 levels were normal. Whole exome sequencing sent revealed homozygous missense mutation in exon 13 of HEXB gene on chromosome 5.

Conclusions: Sandhoff disease is a very rare, particularly juvenile form. This case report suggests that comprehensive genetic testing is important for the diagnosis of unexplained ataxia, and juvenile Sandhoff disease is considered as one of the causes for autosomal recessive ataxia.

Abstract ID: 640: Predictors of Drug-Induced Hepatitis in Patients of CNS Tuberculosis

Ramakant Yadav, Ajay Emani, Abdul Qavi

U.P. University of Medical Science Saifai, Etawah-26 13

Background and Aim: Drug-induced hepatitis (DIH) following anti-tubercular therapy (ATT) in patients with central nervous system tuberculosis (CNS-TB) remains poorly studied. This research aimed to assess the frequency, spectrum, and predictors of DIH in CNS-TB patients.

Methodology: A prospective study enrolled 104 CNS-TB patients without prior liver disease. Clinical, laboratory, and MRI data were collected. DIH was diagnosed based on transaminase elevation after ATT initiation. Predictors of DIH and its impact on outcomes were evaluated.

Results: DIH occurred in 23.1% of patients, with normalization of liver function after ATT cessation. Altered sensorium, elevated erythrocyte sedimentation rate (ESR), and MRI findings (hydrocephalus, cerebrovascular accident [CVA], and myelopathy) were independent DIH predictors. Patients with DIH had a low mortality rate (12.5%) and mostly recovered after ATT discontinuation.

Conclusions: The combination of acquired risk factors predisposed the patients to DILI rather than a single risk factor. It does not seem to be an individual risk factor, but rather a combination of risk factors that renders the liver vulnerable to injury. Early recognition and management of DIH are crucial for optimizing CNS-TB treatment outcomes.

Abstract ID: 641: The Role of Somatosensory Evoked Potentials in Assessing Severity of Non-Compressive Myelopathy

Sohini Ganji

Osmania Medical College, Hyderabad, Telangana, India

Background and Aim: Non-compressive myelopathies, characterized by spinal cord dysfunction in the absence of structural compression, encompass a spectrum of etiologies including inflammatory, infectious, demyelinating, toxic, and metabolic causes. These conditions cause substantial disability and decrease quality of life. Somatosensory evoked potentials (SSEP) represent a non-invasive tool to evaluate somatosensory pathway integrity, offering an initial diagnostic investigation for non-compressive myelopathy.

Methodology: Patients admitted to Neurology Department of Osmania Medical College with clinically confirmed non-compressive myelopathy were included in this study. Exclusion criteria comprised peripheral neuropathy and traumatic brain/spinal cord injury. Detailed history, clinical examination, routine investigations, vitamin B12 levels, CSF analysis, MRI spine, and nerve conduction studies (NCS) were performed. Serum Aquaporin-4 and MOG antibodies were sent when clinically indicated. Median and tibial SSEPs were performed.

Results: Twenty patients (mean age 31 years; 11 females, nine males) suspected of non-compressive myelopathy were analyzed. Diagnoses included NMOSD (7 cases), post-infectious myelitis (4 cases), MOGAD (3 cases), B12 deficiency (3 cases), TB myelitis (1 case), MS (1 case), and Friedrich’s ataxia (1 case). Abnormal latencies, involving N13 and N23 spinal potentials, were observed in all patients. Tibial SSEPs were more frequently abnormal compared to median SSEPs. Five patients with abnormal SSEPs had no evidence of transverse myelitis on MRI.

Conclusions: SSEP’s can be used as a cost-effective and non-invasive indicator of non-compressive myelopathy and can also correlate with severity of disease.

Abstract ID: 642: A Cross-Sectional Study on Clinical Profile and Patient Outcomes in Neurological Emergencies among Pregnant and Peripartum Women Aged Above 18 Years in a Tertiary Care Hospital

Vasavi Biruduraju

Osmania Medical College, Hyderabad, Telangana, India

Background and Aim: Neurological emergencies in pregnancy often have grave prognosis and so must be promptly diagnosed and treated. Significant physiological changes can occur during pregnancy. Owing to these changes, some disease conditions will aggravate, and some will abate during pregnancy. It is important to understand the pathophysiologic mechanism and behavior of each of these disease conditions to ensure timely treatment and reduce morbidity and mortality. Objectives: To study the clinical profile and outcome of neurological emergencies in pregnant and peripartum patients.

Methodology: The study will include pregnant and peripartum women aged above 18 years presenting with neurological emergencies. Participants will be recruited from acute medical care from Osmania general hospital. Study period is from October 2023 to April 2024. They were evaluated by clinical history, detailed neurological examination, and investigated using routine blood work, neuroimaging and other necessary investigations. EEG and NCS were conducted where necessary.

Results: In our study, among 25 patients, majority of cases were multiparous of age group 18 to 30 years. The most common condition seen was PRES (12 patients), six patients had GBS, four had CSVT, two had status epilepticus, and one patient had Wernicke’s encephalopathy. Majority (14) patients had recovery, while six patients had residual neurological deficits, and five cases succumbed to death.

Conclusions: This study gives an insight to the prevalence and presenting symptoms and maternal outcome of neurological emergencies in pregnancy and peripartum.

Abstract ID: 643: Amyotrophic Lateral Sclerosis: Ultrasonographic Assessment of Tongue and Vagus Nerve and Correlation with Speech

P. Arjun Chandrashekar, Seena Vengalil, B K Yamini, Nalini A, Deepak Menon, Saraswati Nashi

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: ALS is a devastating disease which relentlessly progresses to death once the bulbar and respiratory muscles are involved. Ultrasonography of tongue and vagus nerve as well as acoustic analysis of speech are non-invasive, easy, and cost-effective techniques for early detection of these bulbar changes. Primary Objective • To evaluate changes in tongue thickness using ultrasonography in ALS patients. • To study the sonographic changes of vagus nerve size in ALS patients. • Analysis of speech and voice changes in ALS patients. Secondary Objective • To correlate the changes in tongue thickness and vagus nerve size with speech quality.

Methodology: It is a prospective case control study conducted in NIMHANS. A total of 20 ALS patients were included in the study. A total of 20 age- and sex-matched control was included. Ultrasonographic assessment of tongue thickness and cross-sectional area of vagus nerve was assessed. Speech parameters, including fundamental frequency, SD of F0, jitter %, shimmer, and NHR, were assessed for a, i, u. Results were assessed in SPSS software.

Results: Eight were males, and 12 were females. Bulbar onset constituted 35%. Tongue thickness was lower in bulbar onset individuals and bulbar involvement in limb onset patients compared to healthy controls. There was negative correlation between vagus nerve diameter and speech parameters even though not statistically significant.

Conclusions: This is a substudy and preliminary analysis of a larger ongoing study and concludes that speech is negatively impacted by the vagus nerve cross section even though not statistically significant.

Abstract ID: 644: Study on Clinico-Radiological Profile and Outcomes in Patients with Idiopathic Intracranial Hypertension

Molugu Samhitha, Radhakrishna Hari, Ranjith G, Vikram Kishore Reddy Peri, Abhishek Miryala, Srikanth Reddy

Medicover Hospitals, Hitech City, Hyderabad, Telangana, India

Background and Aim: IIH is a syndrome of increased intracranial pressure of unclear etiology seen in obese women of childbearing age and can occur in men, children, and older adults. Aims – To study the clinico-radiological presentations and treatment outcomes in patients of IIH (both medical and surgical).

Methodology: This is a single center, observational, and hospital-based prospective study done over 15 months. Patients presented with symptoms of IIH and fulfilled modified Dandys criteria for IIH, to neurosciences OPD and IPD in xxxx institute are included. Patients with CSVT, history of medications, and systemic disorders associated with raised ICP were excluded. Informed consent is taken, and patients are followed up for 6 months.

Results: Females are commonly affected. The mean age was 35 years, and most common symptom is headache, with obesity being most common comorbidity. B/L papilledema is the most common finding. MRI findings of transverse sinus stenosis, tortuous course of optic nerve, and empty sella correlate with raised ICP s/o IIH. CSF opening pressure raised in 70% patients with mean – 30 cm of H20.50% improved only with medical management. TP shunting was performed in 42% patients. Complete recovery was achieved in 70% of patients with both medical and surgical management.

Conclusions: Main goals of treatment to preserve visual function and alleviate symptoms achieved with combination of weight loss, medical therapies – acetazolamide, topiramate, and surgical interventions depending upon symptoms severity, vision loss, and treatment response.

Abstract ID: 645: Effectiveness of Rituximab Versus Oral Immunosuppressive Therapies in NMOSD

Shivani Jasti, Sadeesh Kumar V, Shobana N, Socrates M, Sadeeshkumar V, Selvakumar C. J.

Coimbatore Medical College Hospital, 1619 A, Trichy Rd, Gopalapuram, Coimbatore, Tamil Nadu, India

Background and Aim: NMOSD is a relapsing autoimmune disorder of CNS characterized by core clinical features of myelitis, optic neuritis, area postrema, or symptomatic brainstem syndrome. Despite its recognition as a relapsing disease, the choice of relapse prevention immunotherapy in NMOSD remains controversial. This study aims to analyze the effectiveness of rituximab versus oral immunosuppressive agents (azathioprine and mycophenolate-mofetil) for relapse prevention in a cohort of NMOSD patients.

Methodology: Ten NMOSD patients fulfilling Wingerchuck criteria were analyzed for demographic information, clinical presentations, and the various treatment strategies employed. All the patients were started on steroids as initial therapy and either rituximab or oral immunosuppressive agents as maintenance therapy. Clinical improvement was determined by EDSS scale, and the patients were followed up for development of relapse.

Results: Our cohort consisted of 10 NMOSD patients with mean age of 36 years with 90% females. The initial clinical presentation was myelitis in 40%, optic neuritis in 30%, and area postrema syndrome in 30%. Relapsing disease was present in 60%, and the mean number of relapses was 2. Among steroid sparing maintenance therapies tried, rituximab was used in 80%, azathioprine in 30%, and mycophenolate-mofetil in 30%. One out of eight patients (12.5%) receiving rituximab experienced a relapse within the first three years of treatment vs three out of 3 (100%) patients receiving azathioprine and two out of three patients (66.6%) receiving mycophenolate-mofetil. All the patients on azathioprine and one out of three patients on mycophenolate-mofetil who experienced a relapse were eventually started on rituximab therapy, none of whom developed a further relapse.

Conclusions: Rituximab significantly lowered NMOSD-relapses when used as initial therapy as well as rescue therapy in individuals who failed to respond to oral immunosuppressive agents.

Abstract ID: 646: A Rare Case of Hereditary Spastic Ataxia

Shivani Jasti, Shobana N, Socrates M, Sadeeshkumar V, Selvakumar C. J.

Coimbatore Medical College Hospital, Trichy Rd, Gopalapuram, Coimbatore, Tamil Nadu, India

Background and Aim: The term spastic ataxia indicates the intersection between combined cerebellar ataxia and spastic paraplegia. Spastic ataxia is the hallmark of hereditary ataxias, such as Friedreich ataxia, ataxia with isolated vitamin E deficiency, ataxia telangiectasia, and autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Because of the rarity and genetic heterogeneity of these conditions, their molecular diagnosis remains challenging and time consuming.

Methodology: A 22-year-old developmentally normal female, born of non-consanguineous marriage with no significant family history, presented with progressive unsteadiness of gait and swaying to either side while walking for five years of age associated with stiffness of both lower limbs. On examination, bilateral pes cavus was observed, spasticity present (LL>UL), bilateral graded sensory loss, impaired JPS, and vibration sense in lower limbs up to knee joint. DTRs right – BJ3+, TJ3+, SJ3+, KJ3+, AJ-; left – BJ3+, TJ3+, SJ3+, KJ3+, AJ-; plantar – bilateral extensor; cerebellar signs – bilateral gaze-evoked nystagmus, bilateral dysmetria, stance, and gait ataxia present. Routine blood investigations were normal; NCS – bilateral lower limb sensorimotor axonal polyneuropathy. OCT revealed bilateral retinal nerve fiber layer thickening suggestive of papilledema. An MRI brain showed diffuse cerebellar atrophy. Sural nerve biopsy was performed which revealed axonal neuropathy.

Results: The possibility of ARSACS was considered in view of typical clinical presentation and supportive investigations. Diagnosis was confirmed by clinical exome sequencing which showed homozygous frameshift mutation in Exon 10 of SACS gene.

Conclusions: Even though it is a rare entity, the possibility of ARSACS should be considered in patients presenting with early-onset cerebellar ataxia combined with spasticity and peripheral neuropathy.

Abstract ID: 647: Anatomical Variation of Intracranial Arteries using Digital Subtraction Angiography: A Retrospective Study

yogesh kaushik, Gourav Goyal, Himanshu Kaushal, Jatinkumar Jain

Mahatma Gandhi Medical College, Jaipur, Rajasthan

Background and Aim: Anatomical cerebral arterial variations n are frequent findings in the general population due to the complex intracranial circulation embryology. Although the majority of normal variations have no major clinical impact, their appreciation may aid in surgical planning and can be useful in preventing complications during endovascular treatment. The present study was conducted to analyze anatomical variation of intracranial arteries using DSA.

Methodology: A total of 429 consecutive patients were included in the study who underwent DSA for any indication by a single neurologist with expertise in stroke and neurointervention. Patients with incomplete imaging, total occlusion of vessel, severe vasospasm, and moyamoya disease were excluded from the study. DSA images were acquired via transfemoral approach with a 5 Fr catheter in a fully equipped DSA unit.

Results: Normal and variation pattern of arteries were found in 45.69% and 54.31% of the subjects, respectively. Fetal PCA was observed in 82 (21%) subjects, and 23% of these subjects had bilateral fetal PCA. Hypoplastic and aplastic ACA was observed in 11.93% and 5.1% subjects, respectively. Azygous and accessory ACA were only observed in 4 and 2 subjects, respectively. MCA trifurcation was observed in 41 subjects (10.4%), among these only four subjects had bilateral MCA trifurcation. Fenestrations were observed in seven subjects (1.77%), most commonly seen in BA (3 subjects). Dolico-ectasia of BA was observed in five subjects. Infundibular PCOM was reported among 3.05% of the subjects.

Conclusions: Fetal PCA, hypoplasia, or aplasia of proximal anterior cerebral artery (A1) were the most common anatomical variations.

Abstract ID: 648: A Cross-Sectional Study to Esitimate Prevalence of Cognitive Impairment in Patients with Heart Failure

Naineesh Gaikwad, Nitin Manohar Gadkari, Shankar Prasad Gorthi

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim : Heart failure (HF) is a major growing health problem. Several cerebral areas and different cognitive domains can be involved in patients with HF developing deficits in cognition. Reduction in cerebral blood flow (CBF) is often referred as a determinant in brain changes affecting people with HF. Thus, it is essential to screen patient of cognitive impairment who are diagnosed and recovering from HF. 2. Aims and Objectives To estimate the prevalence of cognitive impairment in patients with HF.

Methodology: A prospective cross-sectional study was conducted in single tertiary care center. HF patients getting admitted to in intensive care units (ICUs) were screened. Informed written consent was taken of the patients who satisfy the inclusion criteria. The patients were enrolled for the study after stabilization, and patient data form his recodes were noted. Specifically, the serum BNP levels and the 2D-ECHO reports were recorded. The investigator then performs the RCS test and record it. Data are being entered in Excel sheets and analyzed, and results were obtained.

Results: 3. Results: This study included 100 patients, of which 72 were male and 28 were female, with a mean age of 40.5 years ± 14.39. The male to female ratio is 3.6. There were 13 patients who had moderate-severe cognitive impairment – 4 patients with preserved ejection fraction >50, 6 with moderate EF (35–50%), and three patients with severe systolic dysfunction. We have found out that there is no association between systolic dysfunction and cognitive impairment (chi square test 3.72, degree of freedom 2, P value – 0.44) which is not statically significant.

Conclusions: 5. Conclusion: There is no significant cogitative impairment in patient with HF.

Abstract ID: 649: A Study of Cardiac Autonomic Neuropathy in Diabetes Mellitus using Ewing’s Criteria and QTc in Tertiary Care Hospital

Edwin Rathinam

National Institute of Medical Sciences, Jaipur, Rajasthan

Background and Aim: Cardiac autonomic neuropathy (CAN) is one of the major causes of morbidity and mortality in patients with diabetes mellitus (DM). This study aimed to identify autonomic dysfunctions in diabetic patients using simple bedside tests based on Ewing’s criteria and estimation of QTc interval.

Methodology: A descriptive cross-sectional study was conducted by the Department of Neurology over a period of three months which included consenting inpatients and outpatients from Department of General Medicine/Neurology/Cardiology after obtaining clearance from the Institutional Ethical committee to detect CAN with simple bedside tests using Ewing’s criteria and categorized accordingly along with QTc interval estimation. A total of 70 patients were selected for the study considering the inclusion and exclusion criteria. Data was transferred to SPSS 22.0 windows software and analyzed with the help of the statistician.

Results: A total of 70 diabetic patients were assessed, and the observed mean age is 45.14 years, ranging from 24 years to 59 years. A total of 36 were male cases (51.43%), and 34 were female cases (48.57%), with 37.14% presenting with symptoms of CAN. There were 19 type 1 DM patients (27.14%) and 51 type 2 DM patients (72.86%). Notably, 58.57% of type 2 DM patients exhibited CAN.

Conclusions: Patients with diabetes are more likely to have CAN, with higher frequency of CAN in type II diabetes. The severity of CAN is closely associated with age, disease duration and HbA1c. This can be diagnosed using simple bedside tests using Ewing’s Criteria and QTc interval, a reliable prognostic indicator of CAN.

Abstract ID: 650: Effect of Family-Centered Multi-Domain Tele-Rehabilitation Application on Stroke Outcomes: Proposal for a Randomized Control Trial

Pushkraj Marne

Manastik

Background and Aim: Stroke is a leading cause of long-term disabilities globally. India faces a substantial stroke burden, with high incidence rates and mortality. We aim to investigate the effectiveness of Manastik, a family-centered tele-rehabilitation app for stroke patients, which utilizes machine learning to customize routines and healthcare provider feedback for personalized and effective rehabilitation.

Methodology: This prospective randomized controlled trial will include 250 post-stroke patients, randomly assigned to either an intervention group or a control group. The intervention group will undergo Manastik intervention facilitated through an Android-based application for a duration of 12 weeks (approximately three months), while the control group will receive standard care.

Results: The prospective randomized controlled trial involving 250 post-stroke patients will aim to find that the intervention group using the Manastik application will show significant improvements in cognitive function and neurorehabilitation outcomes compared to the control group receiving standard care after 12 weeks.

Conclusions: This study aims to validate the efficacy of family-centered tele-rehabilitation in improving stroke outcomes. The findings will guide the creation of improved stroke services and affirm the effectiveness of family-centered interventions.

Abstract ID: 651: Clinical Profile and Outcome in Patients of Myasthenia Gravis Who Had undergone Thymectomy and Who Received Medical Treatment

Partha Saha, Arun Koul

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi), India

Background and Aim: Myasthenia gravis (MG) is an autoimmune disorder due to production of autoantibodies from the thymus glands, against post-synaptic acetylcholine receptors (AChR) at the neuromuscular junction. Thymectomy has become a widespread treatment option in all MG patients. There is limited evidence of long-term outcomes of treatment modalities among MG cases in North Indian population. The objectives of this study are to describe the clinical profile and determine the clinical outcomes of MG patients who underwent thymectomy plus best medical management and who received only medical treatment.

Methodology: We retrospectively reviewed a total of 35 cases of MG patient’s records. After exclusion, 15 patients of MG were undergone thymectomy with medical treatment, and 15 patients received only pharmacologic treatment. The demographic characteristics and histopathologic results were obtained. The clinical outcome after two years follow-up was also determined and grouped according to the following: (1) complete remission, (2) significant improvement – a) with reduced medications and b) with same medications, (3) no clinical change, and (4) worsening symptoms.

Results: Majority of the patients were females (56.6%) with mean age of 33.4 years and mean duration of myasthenic symptoms of 12 months. Using the MGFA classification, 53.3% and 30% of patients fell under class II and III, respectively, and 93% of patients presented with generalized weakness. In this study, no patient had complete remission in any of the groups. 60% of MG patients who were undergone thymectomy had clinical improvement either with reduced or same medications, compared to 53.3% among the medical treatment group ( P value = 0.90).

Conclusions: This study showed that there is no significant better outcome of thymectomy in comparison with the medical treatment group after two year of treatment.

Abstract ID: 652: A Rare Mimicker of Guillain–Barre Syndrome – Dumb Rabies – A Interesting Case Report

Ramachandran MohanRaj

Thanjavur Medical College, Thanjavur, Tamil Nadu, India

Background and Aim: Acute flaccid paralysis is one of the neurological emergencies, which should be promptly identified and managed to avoid both mortality and morbidity. Acute inflammatory demyelinating polyradiculopathy (Guillain–Barre syndrome) is one of the most common causes for AFP. Not all AIDP is due to post or para-infectious demyelination, other mimickers should be ruled out before making a diagnosis. Here, we are reporting a rare mimicker of GBS – paralytic rabies.

Methodology: A 44-year-old male presented with drooling of saliva and difficulty in swallowing for two days, which progressed as descending paralysis to flaccid quadriparesis with neck and respiratory muscle weakness in next two days. He was referred to our center as? AIDP for further management. On examining, patient was anxious with excessive sweating with flaccid quadriparesis and areflexia clinically fitting into AIDP. But the apprehensive, anxious look of the patient and started as dysphagia made us to think other possibilities. Patient and relatives dined the history of dog bite and aerophobia and hydrophobia. Patient was priest by occupation, who went out early morning for the past one month during which chance of exposure to bat was present. All of these made us to arrive the diagnosis of dumb rabies – paralytic rabies due to bat exposure which mimicked as AIDP.

Results: After getting informed and written consent from the patients, all necessary investigations are carried out. CSF analysis was normal. Neuroimaging showed T2/FLAIR hyperintensities in the basal ganglia and thalamus. Salivary, CSF PCR, and nape of the skin biopsy confirmed the diagnosis of rabies. Patient expired after 11 days of symptom onset.

Conclusions: Paralytic rabies, a differential diagnosis of GBS.

Abstract ID: 653: Marchiafava Bignami Disease – A Rare Neurological Presentation of Chronic Alcoholism

Archana Minimahesan, Anil Kumar Sivan, John K. John, Joban John

Believers Church Medical College Hospital, Thiruvalla, Kerala, India

Background and Aim: Marchiafava Bignami disease is a rare neurological disorder of chronic alcoholism characterized by demyelination and necrosis of corpus callosum.

Methodology: We are reporting the case of 54-year-old gentleman who presented to neurology OPD with c/o slurring of speech and weakness in the left lower limb with unsteadiness while walking with recent worsening for one week. The slurring was gradual in onset and was slowly progressive. Patient experienced swaying of the left side while walking. Patient was a painter by profession who used to climb high for painting which he stopped doing for seven months. He was a chronic alcoholic for 20–30 years, and he stopped one month back. Examination showed cerebellar signs in the form of dysarthria, dysmetria, and gait ataxia.

Results: All blood reports, including vitamin B12 levels, were normal. An MRI brain showed low signal intensity in T1WI and high signal intensity in T2WI in the central portion of genu, body, and splenium of corpus callosum with relative sparing of dorsal and ventral layer which give characteristic sandwich sign.

Conclusions: This disease requires clinico-radiological concordance and a high index of suspicion in relevant clinical setting. There is no consensus for treatment of this disease. They are primarily treated with vitamin B1 supplementation, amantadine (hypothesized to modulate affected dopaminergic pathways), and corticosteroids (by reducing vasogenic edema). Earlier treatment following symptoms will lead to better recovery. Hence, there is a need to identify this disease earlier and earlier intervention.

Abstract ID: 655: Association of Severity of Myasthenia Gravis and Comorbidities with Anxiety, Depression, and Quality of Sleep

Faim Ahamed, Jayantee Kalita, Gutti Nagendra Babu

Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow (SGPGI Lucknow), Uttar Pradesh, India

Background and Aim: Autoimmune myasthenia gravis (MG) may require lifelong treatment with immunomodulating drugs and acetylcholinesterase inhibitors (AChEIs). Patients may face multiple challenges due to disease severity, associated comorbidities, frequent hospital visits, treatment cost, family support which may influence their quality of life, sleep, anxiety, and depression. We report the influence of these factors on anxiety, depression, sleep and quality of life in the patient with MG.

Methodology: This is a cross-sectional study, all the adult MG patients attending our hospital during last two years included. Their demographic details, educational qualifications, occupation, economic status, average cost of treatment per year, MG disease severity, and associated comorbidities were noted. The patients were examined, and his current Myasthenia Gravis Foundation of America (MGFA) score, myasthenia gravis quality of life (MGQoL-15), hospital anxiety and depression-anxiety component (HADS-A), and hospital anxiety and depression-depression component (HADS-D), and Pittsburgh sleep quality index (PSQI) score were assessed.

Results: Overall, 34 patients studied median age of the participants were 47.5 (19–71) years, median follow-up 83.5 (12–320) months, and 38.2% were females. Study revealed poor quality of sleep (global PSQI score >5) in 29.5%, anxiety (HADS-A?11) in 26.5% and depression (HADS-D?11) in 20.6%. HADS-A correlates with MGFA status ( P = 0.007), but HADS-D correlates with MGFA status ( P = 0.001), financial status ( P = 0.00), and family support (0.007). Global PSQI score correlated with MGFA status ( P = 0.004), comorbidities ( P = 0.045), and hospital visits ( P = 0.023). MGFA and MGQoL-15 both were inter-related ( P = 0.00).

Conclusions: The severity of myasthenia is related to HADS-A and HADS-D and PSQI. Financial conditions and family support were related to HADS-D.

Abstract ID: 656: MOGAD Presenting as MRI-Negative Cerebellitis, Area Postrema Syndrome, and Mood Disorder

Archana minimahesan, John K. John, Harikrishnan R, Jerry A. George

Believers Church Medical College Hospital, Thiruvalla, Kerala, India

Background and Aim: Area postrema syndrome is a core feature of NMO-spectrum disorder. However, area postrema syndrome as part of MOGAD is rare.

Case Report: We are reporting case of a 26-year-old gentleman, presented with complaints of swaying while walking for 1 week and multiple episodes of vomiting, fatigue for 5 days. He was being treated for mood disorder for past four months for which he was on treatment with Escitalopram and Clonazepam.

Discussion: His blood investigations were normal. In view of acute onset ataxia with vomiting, possibility of structural lesions was ruled out through MRI brain plain. He was treated symptomatically. As he had persisting ataxia with vomiting and Vertigo, all the antidepressants were withheld in view of possibility of drug-induced ataxia. During the hospital stay, he developed hiccups. Urine toxicology screening was normal. MRI brain with contrast did not reveal any significant abnormality. LP CSF was performed under aseptic precautions. Routine CSF studies, including total counts, protein, sugar, gram stain, AFB stain, culture and sensitivity, cryptococcus antigen, and cytology were normal. His serum MOG was found to be moderately positive. CSF autoimmune encephalitis panel and serum and CSF OCB were negative. His anti-TTG antibodies were also negative, excluding coeliac disease.

Conclusions: There are only few case reports with MOG+/AQP4-negative cases presenting as area postrema syndrome. Possible mechanism considered is the entry of pathogenic MOG antibody due to the lack of blood-brain barrier at the AP and disruption of emesis circuit. MRI-negative MOGAD is also rare but may be a rare phenotype of MOGAD.

Abstract ID: 657: A Rare Presentation of Herpes Simplex Virus (HSV) Encephalitis as Artery of Percheron Infarct

Brijesh J. N., Aravind Gautham V

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: HSV encephalitis is the most common sporadic adult encephalitis with acute/subacute progressive focal or generalized cerebral dysfunction. HSV-related stroke is a rare entity as result of vasculopathy. Here, we report a middle-aged man who presented with focal encephalitis superimposed by sudden worsening of sensorium due to brainstem stroke secondary to HSV induced vasculitis.

Case Report: This is a 53-year-old male with recently diagnosed diabetes/hypertension who presented with subacute onset progressive neurological illness in the form of memory and behavioral disturbances which improved with medications with acute worsening leading to hypersomnolence, drooping of the left eye, and motor weakness. On examination patient was lethargic with left complete ptosis and restricted extraocular movements (vertical > Horizontal) with paucity of movements of the right limbs and extensor plantar, with terminal neck stiffness.

Discussion: Routine blood investigations were normal. MRI brain contrast revealed T1 hypointense and T2/FLAIR hyperintense diffusion restricting lesions in the left temporal lobe with leptomeningeal enhancement suggestive of limbic meningoencephalitis, and patient was started on empirical antivirals for three weeks. Eventually, CSF analysis (PCR) turned positive for HSV DNA, and patient started improving with treatment which was interrupted by sudden worsening in sensorium with focal deficits. Repeat imaging revealed T2/FLAIR hyperintensities in bilateral thalami, left midbrain, and pons with resolving temporal lobe lesions suggestive of artery of Percheron infarct. Repeat CSF study and autoimmune profile were unyielding.

Conclusions: HSV being the most common etiology for acute viral Encephalitis, warrants high degree of clinical suspicion and prompt treatment without which mortality can be as high as 70%. The clinical outcome may be complicated by certain sequelae like cerebral edema, epilepsy, aphasia, cognitive impairments, and motor deficits.

Abstract ID: 658: Comparison of Metoprolol and Amlodipine in Reducing Mortality and Disability of Acute Stroke: An Open-Labeled Randomized Controlled Trial

Jayantee Kalita, Nagendra Babu Gutti

Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow (SGPGI Lucknow), Uttar Pradesh, India

Background and Aim: There is paucity of randomized controlled trials comparing the outcome benefit of beta-blocker and calcium channel blocker in acute stroke. We compare the effect of metoprolol and amlodipine in reducing mortality, disability, and infections in acute stroke.

Methodology: This is an open-labeled randomized controlled trial conducted in a single institution. CT/MRI confirmed stroke patients within three days of onset were included whose age was 18 to 75 years. The patients with secondary intracerebral hemorrhage, organ failure, pregnancy, malignancy, immunosuppressant or already receiving beta-blocker/amlodipine were excluded. Stroke risk factors, Glasgow Coma scale (GCS) score, National Institute of Health Stroke Scale (NIHSS) score, and CT/ MRI findings were noted. The patients with a blood pressure of >160/90 mm of Hg were randomized using 1:1 randomization to metoprolol (25 mg on day 1, 50 mg from next day) or amlodipine (2.5 mg day 1, 5 mg on day 2, subsequent increase was optional). Other standard treatment was continued. Primary outcome was in-hospital death, and secondary outcomes were in-hospital gastrointestinal hemorrhage, pneumonia, sepsis, and three months functional outcome based on modified Rankin scale (mRS). Side effects were noted.

Results: A total of 18 (14%) patients died; 6 (9.8%) in metoprolol and 12 (14%) in amlodipine ( P = 0.20) group. At three months, 45 (80.4%) in metoprolol and 21 (43.3%) in amlodipine group had good outcome ( P < 0.001). The other secondary outcomes were comparable between the two groups. Metoprolol was withdrawn in six patients due to bradycardia and amlodipine in one due to allergic reaction.

Conclusions: Metoprolol is associated with disability benefit in acute stroke compared to amlodipine.

Abstract ID: 659: A Study on Spectrum of Clinical Profile and Outcome After Treatment for Neuro-Tuberculosis in a Tertiary Care Center from Eastern India

Sujoy Kabiraj, Jayanta Ghosal, Tejendra Nath Kundu

Institute of Post Graduate Medical Education and Research, Kolkata (IPGMER Kolkata), West Bengal, India

Background and Aim: Introduction: Although pulmonary tuberculosis is common, neuro-tuberculosis is more severe, with higher morbidity and mortality. Diagnosing it is challenging due to non-specific clinical manifestations, low bacilli density in CSF, and delayed culture recovery of Mycobacterium tuberculosis. Aims: This study’s aim is to identify the clinical spectrum of CNS-TB, including typical and atypical presentations, detect resistant tuberculosis, analyze disease outcomes, and assess treatment response during follow-up.

Methodology: Materials and Methods: The present study was a hospital-based longitudinal descriptive study. This study was conducted from August 2021 to November 2022 at the Department of Neurology, Bangur Institute of Neurosciences, IPGMER. A total of 126 patients were included in this study.

Results: CNS-TB commonly presents with fever, headache, and vomiting (all >41.3%). CSF analysis shows inflammatory markers but rarely confirms M. tuberculosis. MRI is essential for diagnosis (meningeal enhancement, etc.). Empiric anti-tuberculous therapy achieved good outcomes (91.3% recovery at 12 months).

Conclusions: Early CNS-TB diagnosis is crucial; timely anti-TB therapy impacts outcomes most. Diagnosis relies on imaging, CSF tests, clinical suspicion, and INDEX TB criteria due to confirmation challenges.

Abstract ID: 660: A Prospective Observational Study to Compare MMSE and Modified MMSE (3MS) in Detecting Cognitive Dysfunction in Stroke Patients

Shubham Pawar, V. P Singh, S. P. Gorthi, Dulari Gupta

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim: Post-stroke dementia (PSD) occurs in 20–25% patients. There are not many studies from India recording PSD. Aims: To detect cognitive dysfunction by using modified mini mental status examination (3MS) as compared to mini mental status examination (MMSE) in stroke patients (both ischemic and hemorrhagic) at admission and at three months follow-up.

Methodology: This is a single-centered prospective, observational, and cohort study among all stroke patients admitted in University College in Maharashtra. Types of stroke, NIHSS at admission, were recorded. All patients underwent MMSE and 3MS on day zero and day 90. Both scores were compared using student t -test and Pearson’s correlation coefficient.

Results: The study included 76 patients. The mean age of presentation was 51.38 ± 14.39 years. The ratio of male to female was 2:16. Acute ischemic stroke was recorded in 72.36%; while intracranial hemorrhage was observed in 27.64%. A total of 39.47% patients were diabetic, and 59.21% patients were hypertensive. Both MMSE and 3MS scores improved at three months follow-up ( P value = 0.07, P value = 0.002). Pearson’s correlation coefficient between MMSE and 3MS at discharge was found to be highly significant ( r = 0.88, P value ≤ 0.001); at three months, the correlation continued ( r = 0.91, P value ≤ 0.001).

Conclusions: 3MS is a better screening tool as compared to MMSE to detect cognitive dysfunction in stroke patients.

Abstract ID: 661: Automated Systems in the Healthcare: Survey on Ethical Implications of Assistance Robots and Devices in Rehabilitation

Sudhindra Vooturi, Sumanth Panem, Anuja Patil, Sita Jayalakshmi

KIMS Hospital, Hyderabad (KIMS, Hyderabad), Telangana, India

Background and Aim: Advancements in robotics are expected to have broad and important implications in neuro-rehabilitation. However, these advancements will be accompanied by ethical implications like – anonymization to safeguard patient data and privacy. Ethical frameworks may ensure accountability from healthcare institutions and professionals thus making the process transparent to patient. Aim: To understand the expectation of robotic rehabilitation from the stake holders of rehabilitation – Patient, therapists, and physicians.

Methodology: A survey of professionals in area of rehabilitation about their point-of-view on ethics in rehabilitation and assistance robotics was conducted at a tertiary referral center in South India. An electronic survey was designed using Google Forms and in-person interview. One-hundred and seventeen responses were received. The average age of the respondents was 43.72 ± 34 (23–64) years. The respondents included 42 patient/caregivers, 38 rehabilitation therapists, 24 physicians, and 13 device manufacturing companies.

Results: The most frequent reported lacunae in ethical considerations from the various sub-groups of respondents were as follows: • Patients – Lack of sufficient patient education about factors like contraindications and cautions to be taken (78.2%) and concerns about cost (71.3%). • Rehabilitation therapists – Lack of well-defined aims and objectives for rehabilitation robotics (88.7%). • Device manufacturers – limited understanding of requirements (software and safety) to implement ethical guidelines (100.0%). • Healthcare providers/Physicians – staffing issues as robotics require specialist training (64.3%), followed by logistical issues like data security and adaptability (58.2%).

Conclusions: Current survey highlights on position of various stake holders of rehabilitation toward robotics in rehabilitation. While patients report lack of awareness, healthcare providers state logistics and ambiguous objectives as lacunae in ethics.

Abstract ID: 662: Association of ApoE Polymorphism with Stroke in North Indian Population

Rachna Agarwal, Suman Seryam Kushwaha, Chandra Bhushan Tripathi, Rajinder Kumar Dhamija

Institute of Human Behaviour and Allied Sciences, New Delhi, India

Background and Aim: Stroke is the second most frequent cause of mortality and disability worldwide with two-thirds strokes occurring in individuals older than 65 years of age. It is a multifactorial disease with heterogenous etiopathogenesis. Various studies show that the human apolipoprotein E (ApoE) may have an impact on stroke occurrence which is substantiated by the fact that there is strong correlation between ApoE genotyping with cholesterol metabolism, atherosclerosis, ischemic heart diseases, cerebral amyloid angiopathy, and stroke. ApoE gene is located on chromosome 19 coding for apoliporotein E. It is present in three isoforms: ApoE2, ApoE3, and ApoE4 with six genotypes E2/2, E2/3, E2/4, E3/3, E3/4, and E4/4. On this back ground, the present study was taken up to find out the distribution various ApoE genotypes in stroke and association of ApoE4 with stroke.

Methodology: A cross-sectional study was performed on non-diseased and diseased subjects with stroke from outpatient services of Neurology Department of Institute of Human Behavior and Allied Sciences (IHBAS), New Delhi (India). All the subjects with acute onset of persistent neurological deficit were diagnosed as stroke, confirmed by neuroimaging (CT/MRI), whereas in control group, subjects included were attending the department of neurology in same hospital for illness other than stroke and without any memory complaints. The patients of head injury presenting with stroke were excluded. ApoE genotyping was performed in all subjects by ARMS-PCR method.

Results: In stroke group, there were 112 subjects (mean age 57.61 ± 15.21 years; 41 females and 71 males), and control group had 113 patients (mean age: 57.45 ± 14.25 years; 49 females and 64 males). Association study showed that ApoE ?2 emerged out as a risk factor and contributed more than two times risk for stroke (AOR: 2.21, CI: 0.95–5.14).

Conclusions:

Abstract ID: 663: Spectrum of Peripheral Nerve Hyperexcitability Syndrome – An Illustrative Case Study of Three Patients

Purushottam Singh, Aditya Gupta, Anirban Gupta

Army Hospital Research & Referral, New Delhi, India

Background and Aim: Peripheral nerve hyperexcitability (PNH) syndromes encompass muscle stiffness, cramps, and twitching with involuntary abnormal electrical activity on needle EMG. This study aims to analyze the clinical characteristics, immunologic aspects, and treatment outcomes of cramp fasciculation, Isaacs, and Morvan syndromes, which display significant clinical overlap.

Methodology: Between Jan 2023 and Dec 2023, patients exhibiting signs of PNH were assessed at our center. Clinical, electrophysiologic, and neuroimaging features, alongside treatment responses and outcomes, were examined. Autoimmune neuroimmunology antibodies were tested via indirect immunofluorescence assay, and neuroimaging included MRI brain and PET scan. Patients underwent regular follow-ups for symptom monitoring.

Results: Three PNH patients aged 18–63 were evaluated. Identified syndromes included cramp fasciculation with myasthenia gravis, Isaacs, and Morvan syndrome. Common symptoms comprised muscle twitching, cramps, and fasciculations. Distinguishing features included autonomic symptoms in Isaacs and Morvan, and central nervous system involvement in Morvan. Electromyography revealed fasciculation potentials in cramps fasciculation syndrome patient and neuromyotonia in the other two. CASPR2 and LgI1 antibodies in serum were positive in both Isaac and Morvan syndrome patient but was negative in cramps fasciculation syndrome. MRI brain and PET scans were performed in all three patients which were normal. Treatment with IVIG and IV methylprednisolone yielded positive responses. Maintenance immunosuppressants given were azathioprine and rituximab to Morvan and Isaac syndrome patients, respectively. Follow-ups showed remission without relapse.

Conclusions: This study focuses on cramp fasciculation syndrome, Isaacs syndrome, and Morvan syndrome, highlighting their distinct clinical and electrodiagnostic features. Association with VGKC complex antibodies, particularly CASPR2 and LGI1 proteins, underscores the importance of immunologic understanding in managing these syndromes.

Abstract ID: 664: Electroencephalographic Abnormalities and Outcome of Ischemic Stroke Patients

Duvvuru Tharun, S. Saravanan, K. S. Ravi

Tirunelveli Medical College, Tirunelveli, Tamil Nadu, India

Background and Aim: Post-stroke epileptic phenomena have been associated with ischemic stroke (AIS) unfavorable outcome. It is unknown whether EEG activities per se are also associated with stroke prognosis. So, in this study, we set out to analyze early EEG abnormalities in AIS and to correlate raw EEG abnormalities with functional outcome at the time of discharge in patients with AIS.

Methodology: This study is conducted in 120 cases of AIS. Cases severity was assessed by NIHSS score, GCS, and by clinical examination at the time of admission. Baseline information was collected. For all these cases, EEG was performed next day using NATUS EEG 10-20 system and was reported by an independent observer. The various abnormalities like alpha waves, PLEDS, slowing of waves, symmetricity, and other epileptic form activities were studied. Clinical outcome at the time of discharge is studied using mRS scale, and their association was corelated using regression analysis.

Results: In bivariate analysis, unfavorable outcome was more frequent in older patients, and patients with a higher admission NIHSS, a lower ASPECTS. Unfavorable outcome at discharge was independently associated with NIHSS score ( P < 0.05), EEG background activity slowing ( P < 0.05), asymmetry ( P < 0.05), age ( P < 0.05), and remote symptomatic seizures ( P < 0.05). Death after stroke was independently associated with age ( P < 0.05), NIHSS score ( P < 0.001), acute symptomatic seizures ( P < 0.05), and EEG suppression ( P < 0.05).

Conclusions: Early EEG abnormalities were independent predictors and comparable to age and early clinical/imaging severity in stroke functional outcome discrimination, reflecting the concept that EEG is a sensitive and robust method in the functional assessment of the brain.

Abstract ID: 665: Paroxysmal Dyskinesia – Genotype Phenotype Correlation – A Case Series

Rakesh Nagar, Arun Koul

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi)

Background and Aim: Paroxysmal dyskinesias (PDs) represent a rare group of movement disorders characterized by episodic, abnormal, and involuntary movements typically of the limbs, provoked by a trigger, and can present with any combination of dystonia, chorea, and athetosis. PD most often presents earlier in life and tends to decrease in frequency with age. Detailed clinical description of the semiology of the disorder is important in making the diagnosis.

Methodology: We present four cases of genetically proven paroxysmal dyskinesia. Case 1: A 6-year boy presented with clonic movements with twisting of both upper limbs, perioral twitching, lasting approximately 40 to 50 s. His paternal great grandfather had similar complains. Case 2: A 7-year-boy presented with repetitive involuntary left sided deviation of mouth and jerky movements of upper limbs, lasting for few seconds, occurring multiple times without specific trigger. Case 3: A 11-year-girl with normal birth history and normal development six months presented with progressive difficulty in holding neck in a steady state leading to backward drop, posturing of upper limbs followed by lower limb, and difficulty in opening mouth along with excessive drooling of saliva, with sleep benefit. Case 4: A 12-year-boy with delayed milestone presented with progressive gait difficulty, pain in right lower limb, improving on rest, imbalance on uneven ground, smearing of face while eating, and decreased arm swing and extension of both great toes and clumsiness in upper limbs.

Results: All cases worked up for possible etiologies, including metabolic and genetic causes. S. No Genetic testing diagnosis Case 1: Heterozygous pathogenic variant of PRRT 2 paroxysmal kinesigenic dyskinesia. Case 2: Heterozygous mutation of KCNMA1 gene paroxysmal nonkinesigenic dyskinesia 3 with or without generalized epilepsy. Case 3: MR-1 gene mutation paroxysmal nonkinesigenic dyskinesia. Case 4: SLC2A1 gene causing GLUT 1 deficiency syndrome 2 paroxysmal exertional dyskinesia.

Conclusions: Accurate diagnosis leads to proper management, avoiding unwanted medications and prognostication.

Abstract ID: 666: Case Series of Rapidly Progressive Dementia – A Rare Etiology, in a Tertiary Hospital

Kalpana Perumal, Uma Maheshwari E, Kaushik S, Balasubramanian S

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: CJD is a rare and untreatable disease, which has an annual incidence of 1–2 per million worldwide. CJD can be divided into sporadic, familial, iatrogenic, and variant forms. Sporadic Creutzfeldt-Jakob disease (CJD) is the most common human prion diseases. Here, we are presenting a case series of five cases of sporadic CJD.

Case Report: We, hereby, report a case series of five cases of sporadic CJD who presented in the fifth and sixth decades with different manifestations of rapidly progressive dementia, behavioral changes, rigidity of limbs, myoclonus, dystonia, ataxia, and visual hallucinations. All the patients were evaluated for causes of rapidly progressive dementia. Baseline blood investigations, imaging and CSF analysis, were performed. CSF 14-3-3 was positive in two cases. Imaging was suggestive of CJD in all the cases. EEG showed periodic sharp wave complexes at a frequency of about 1 Hz in two cases. All the cases were diagnosed based on the WHO/European consortium criteria and treated symptomatically.

Discussion and Conclusions: CJD must be considered as a potential diagnosis for patients who present with rapidly progressive dementia without any evidence of infection or stroke. A high index of clinical suspicion for CJD, exclusion of differential diagnoses of cognitive decline, and appropriate use of available diagnostic tools, including MRI, cerebrospinal fluid analysis, and EEG, are crucial in accurately and promptly evaluating a patient with suspected CJD.

Abstract ID: 667: To Determine the Prognostic Value of Serum Albumin Levels in Acute Ischemic Stroke by Correlating its Levels with Clinical Outcome

Aakanksh Jain, S. P. Gorthi, Rishi Patel

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim: Acute ischemic stroke (AIS), a debilitating condition resulting from the sudden interruption of blood flow to the brain, is a leading cause of long-term disability and mortality globally. Serum albumin, a key protein found in blood plasma, has emerged as potential predictor of AIS outcomes, particularly in assessing post-stroke disability using the modified Rankin scale (mRS). This study aims to determine the prognostic value of serum albumin levels in acute ischemic stroke by co-relating its levels with clinical outcome.

Methodology: This study has been conducted in a tertiary medical college, western Maharashtra. It is an observational study conducted for 12 months. The patients of acute anterior circulation ischemic stroke were evaluated. NIHSS at admission was calculated and serum albumin levels were measured within 24 h of admission. The functional outcome was evaluated using modified Rankin scale (mRS) at day 30 and day 90.

Results: There were total of 90 patients with the mean age 55.4 + 14.21 years. There were 61 males (67.78%). The comorbidities associated were hypertension (50%), DM (46.67%), ischemic heart disease (18.89%), and smoking (45.56%). The mean albumin was found to be 4.007 ± 0.5524 (range from1.8 to 5.0 mg/dL). Pearson’s correlation test of albumin and mRS at one month was −0.162, and P value was 0.128; correlation of mRS at three months was −0.198, and P value was 0.061.

Conclusions: Our study was a prospective observational study which showed that low albumin levels are associated with poor functional outcome, and better outcome was observed in higher serum albumin levels.

Abstract ID: 668: Clinical and Radiological Profile of Hirayama Disease from a Tertiary Care Hospital

Rashmi Devaraj, Mohan P. Channapanavur

Vydehi Institute of Medical Sciences and Research Centre, Bengaluru (VIMS, Bengaluru), Karnataka, India

Background and Aim: Hirayama disease (HD) is a rare disease predominantly causing distal atrophy of the upper limbs. It mainly affects the male in their second or third decade. It is also called as monomelic amyotrophy. Anterior cervical discectomy and fusion (ACDF) is identified as the best surgical approach for this cervical compressive myelopathy.

Methodology: Patients presenting with insidious-onset distal upper limb wasting between January 2022 and May 2024 were evaluated electrophysiologically. This is an ambispective study. Cervical MRI in neutral position and flexion contrast imaging was performed where feasible.

Results: There were 17 patients who had clinical and radiological features suggestive of HD. A total of 16 patients were male, and one was a female. All patients were less than 30 years of age. Duration of illness was ranged between 3 months and 7 years. All (100%) had oblique amyotrophy, 5 (29%) had fasciculations, and 11 (65%) had minipolymyoclonus. The upper limb reflexes were brisk in 14 (82%), and the lower limb reflexes were brisk in 6 (35%). The plantars were flexors in all patients. Electromyography (EMG) showed chronic denervation in the C7-T1 myotomes. MRI showed loss of cervical lordosis in 15/17 (88%), localized lower cervical cord atrophy in 11/17 (65%), and intramedullary hyperintensity in 2/17 (12%). Flexion study showed loss of dural attachment, anterior displacement of dorsal dura in 4/5 (80%), and enhancing epidural crescent in 5/5 (100%).

Conclusions: There was good corroboration between the clinical features and electrophysiological studies which confirmed features of anterior horn cell disease of lower cervical cord. Routine MRI studies also have a high predictive value, but dynamic MRI contrast study is still preferred. An early diagnosis helps in initiation of cervical collar therapy, and ACDF can be planned accordingly.

Abstract ID: 669: Study on Clinico-Radiological Profile among Patients with Diabetic Striatopathy in a Tertiary Care Hospital

Vidya Sagar Kota, Vidya Sagar Kota, Lulup Sahoo

Institute of medical sciences and SUM Hospital, Odisha, India

Background and Aim: Background –The term “diabetic striatopathy” (DS) was coined as a generic word to describe to a hyperglycemic syndrome associated with chorea/ballism on computed tomography or striatal hyperintensity on T1-weighted (T1-WI) magnetic resonance imaging (MRI). However, the precise pathophysiological pathways, radiographic correlations, and the likely rationale for clinical-radiological discordance remain unknown. AIMS-primary objective: To study the clinical and radiological profile of patients presenting with DS. Secondary objective: To study the predictors of clinical resolution of movement disorders.

Methodology: Inclusion criteria: All patients presenting with movement disorder (chorea/ballisum) with diabetes and striatal changes on CT or MRI (satisfying the definition of DS). Exclusion criteria: Patients with structural intracranial lesion (other than striatopathy), major metabolic perturbation not related to hyperglycemia, history of pre-existing movement disorders, and any other pre-existing neurological disorders were excluded (genetic disorders).

Results: Out of our 12 patients, three patients are diagnosed with concordant DS, four patients are diagnosed with discordant DS. One patient had clinically isolated, and one patient had radiologically isolated syndrome initially.

Conclusions: In view of the correlation between imaging findings and clinical symptoms, the present study demonstrated a higher sensitivity of MRI than that of CT for the detection of hyperglycemic chorea. In addition, the follow-up striatal abnormalities in CT seemed to resolve faster than those of MRI. Regarding imaging presentations, isolated putamen and combined putamen-caudate nucleus involvements were common. Discrepancies between CT and MRI findings were noted in one-fourth of our patients, while chorea and imaging anomalies did not appear concomitantly in another patient.

Abstract ID: 670: Neurological Manifestations in Sjogrens Syndrome

Ramishetty Sandeep

Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai (TNMC, Mumbai), Maharashtra, India

Background and Aim: Primary Sjogren’s syndrome (pSS) is an autoimmune connective tissue disease which affects the exocrine glands and leads to structural and functional derangement. Neurological disorders are one of the most common extra glandular manifestations of pSS.

Methodology: We have retrospectively studied cases who presented to the neurology department with various neurological manifestations and were further diagnosed as Sjogrens syndrome. Patients who were fulfilling the serological criteria of 2016 ACR/EULAR classification criteria for Sjogrens syndrome were included. Exclusion criteria included the patients with past head and neck radiation treatment, hepatitis C infection, AIDS, sarcoidosis, graft versus host disease, IGG4 disease, use of anticholinergic drugs, or other drugs inducing sicca symptoms.

Results: Age group of patients in our study is in the range of 17–58 years. We have seen female preponderance in our study. Male to female ratio in our study is 6:8. Of the14 patients, presented to us with various neurological symptoms, CNS manifestations were observed in six cases, and PNS manifestations were observed in eight cases. Pachymeningitis is found in three cases. Transverse myelitis was observed in three cases, trigeminal neuropathy in three cases, mononeuritis multiplex in three cases, and sensory ganglionopathy in three cases.

Conclusions: In conclusion, Sjogrens syndrome should be considered in patients presenting with various neurologic manifestations, specifically in instances of sensory ganglionopathy, long segment transverse myelitis, cranial nerve palsies (trigeminal neuropathy), and mononeuritis multiplex.

Abstract ID: 671: Whole Exome Sequencing Reveals Novel Candidate Genes in Children with Photoparoxysmal Response in an Indian Population

Mary Iype, Anitha Ayyappan, Bineej B. Jayan, Suresh A. Poovathinal, Ismail Thanseem

Government Medical College Trivandrum, Kerala, India

Background and Aim: The genetic architecture of epilepsy is highly complicated. Specific EEG abnormalities have been observed in epilepsy. Photoparoxysmal response (PPR), often observed in epileptic disorders, is an abnormal cortical response to photic stimulation manifesting as specific EEG changes and is highly heritable. PPR is a potential endophenotype for the mapping of epilepsy genes. Here, we have conducted a whole exome sequencing (WES) study of epilepsy patients with PPR in a South Indian population, in an attempt to identify the genetic causes of PPR.

Methodology: The study design was exploratory and descriptive. Twenty-eight patients with epilepsy, exhibiting Grade-4-PPR, were recruited for the study. WES was performed for all the participants.

Results: Eleven children with primary generalized epilepsy-PPR, 15 with epilepsy with eyelid myoclonia-PPR, and two with focal epilepsy and PPR participated in the study. Among the genes in the cohort that harbored rare and pathogenic variants ( n = 1628), 190 genes have been reported previously in epilepsy. Among the protein products of the candidate genes, IGSF3 is known to bring about hyperexcitability of neurons, CTDSP2 regulates the development of neural progenitor cells, and SETD8 is involved in neural stem reactivation. The PPR candidate genes were enriched in pathways involved in extracellular-matrix-receptor (ECM-receptor) interaction and focal adhesion. The “epilepsy genes,” in particular, were enriched in pathways involved in neurodevelopment and biological processes related to ion transport. The expression of these gene products is also abundant in brain and retina. Apart from epilepsy database, the candidate genes are also enriched in gene-databases associated with intellectual disability, global developmental delay, cognitive delay, and autism spectrum disorder.

Conclusions: This genetic analysis of PPR should trigger studies that finally unravel the genetic etiology of primary generalized epilepsy.

Abstract ID: 672: Genetic Testing in Patients with Refractory Non-Structural Epilepsy – An Ambispective Single Center Study

Gowri Shankar Barathidasan, Suryaprabha Turaga, Shagun Aggarwal, Bhavani Madduluri

Nizam’s Institute of Medical Sciences, Hyderabad (NIMS, Hyderabad), Telangana, India

Background and Aim: Epilepsy affects around 50 million people worldwide. Genetic causes are responsible for seizures in up to 40% of children and 23% of adults with epilepsy. We aim to study the prevalence of pathogenic and likely pathogenic genetic variants and their implications on management.

Methodology: This ambispective study is being conducted in NIMS. The study is being conducted over two years from 2022 to 2024. A sample size of 30 was needed. The study was approved by the Institute Ethics Committee. Whole exome sequencing was performed from the blood samples of patients after taking informed consent. For analysis, we considered only likely pathogenic and pathogenic variants. The prevalence of these variants is the primary outcome. The percentage of patients for whom management was changed based on the genetic test which is calculated as secondary outcome measure. The statistical analysis was conducted using SPSS software.

Results: In our study, 31% of our cases were harboring a significant variant out of 29 total cases. Similar to previous studies, we also got sodium channelopathy as our most common variant (33.3%). Medical management was modified in four out of nine patients (44.4%) in our study.

Conclusions: Our study highlights the importance of genetic testing in the management of refractory epilepsy patients. Unnecessary stereo EEG procedures can be avoided. Management of epilepsy based on precision medicine can be conducted, and prognosis can be explained to patients and their families.

Abstract ID: 674: King Denborough Syndrome – RYR1 Gene-Related Myopathy with Susceptibility to Malignant Hyperthermia

Sangeethameena S, Hariharan S, Balaji G, Tamil Paavai N

Chengalpattu Govt Medical College, Chengalpattu, Tamil Nadu, India

Background and Aim: Background and Aims: The RYR1 gene encodes the skeletal muscle ryanodine receptor that serves as a calcium release channel of the sarcoplasmic reticulum. Dysfunctional RYR1 impairs excitation – contraction coupling, resulting in muscle weakness. Subtypes of RYR1-related disease are based on muscle biopsy, phenotype, and pharmacogenetics.

Methodology: Methodology study design – Case report.

Results: A 17-year-old male, single child of non-consanguineous parents with normal development and no significant family history, presented with insidious onset gradually progressive pure motor proximal weakness of both lower limbs of two years duration. He had lumbar lordosis, waddling gait, hypotonia, and hyporeflexia of all four limbs. Investigations showed elevated CPK levels (2769 u/L), myopathic pattern in EMG. MRI showed moderate to severe fatty atrophy with mild STIR hyperintensity of all muscle groups involving bilateral thigh, pelvis, and gluteal regions. There were also mild to moderate change in bilateral shoulder muscles and mild diffuse atrophy of all muscle groups of both lower legs. Next generation sequencing revealed pathogenic variant in RYR1 gene responsible for both King Denborough syndrome and congenital myopathy 1A with susceptibility to hyperthermia. AD inheritance: His parents were advised to undergo genetic testing. Patient is on physical therapy and N -acetyl cysteine – 2 gm/day.

Conclusions: This case is reported for susceptibility to malignant hyperthermia in RYR1 mutated myopathies. Congenital myopathies can arise by De novo mutation with adolescent onset, elevated CPK levels. Genetic studies are invaluable in accurate diagnosis, management, and prognostication.

Abstract ID: 675: A Study of Etiological and Imaging Profile of Cerebral Venous Sinus Thrombosis Admitted in a Tertiary Care Hospital

Prasanth P, P. K. Murugan, C. Justin, S. Elangovan

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: Cerebral venous sinus thrombosis is an infrequent neurovascular disorder with variable presentations. There are various etiologies causing thrombosis of different segments of veins leading to concordance of signs and symptoms useful in diagnosis and treatment. This study aims to study etiological and imaging profile of CVT admitted in tertiary care hospital.

Methodology: The study includes all patients diagnosed as CVT among inpatients in Govt Rajaji Hospital, Madurai. This was a cross-sectional study conducted from March2023 to April 2024.

Results: Among the total patients, 32 were males, and 68 were females. Puerperium was found to be most common cause of CVT (28%) in the study. A total of 56 patients belong to the age group of 21–40 years. A total of 38% of patient had thrombosis in the superior sagittal sinus.

Conclusions: Vascular imaging in CVT includes MRV and conventional angiography. MRV is preferred in acute stage. Contrast MRV is being most ideal. MRV can also pick out with time-of-flight technique.

Abstract ID: 676: Assessment of Autonomic functions and their Impact on the Quality of Life in Patients of Alzheimers and Vascular Dementia

Anshumalini Panda, Abdul Qavi, Rajani Bala Jasrotia, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshrestha

Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow

Background and Aim: Autonomic dysfunction is a well-studied feature in neurodegenerative dementias, especially in alpha-synucleinopathies like DLB and Parkinson’s disease with dementia, but results are conflicting in Alzheimer’s disease (AD) and vascular dementia (VD). In our study, we aim to assess prevalence of autonomic dysfunction in AD and VD and its impact on functional status and quality of life in patients of AD and VD.

Methodology: Ours is a cross-sectional observational study conducted in department of neurology at Dr RMLIMS Lucknow (Tertiary care level institute in North India). Sample size 40 for cases (AD&VD) and 20 for age matched controls. We applied COMPASS 31 and Ewing’s battery test for assessment of autonomic function and SF-36 and Bristol ADL questionnaire for assessment of functional status.

Results: Ewing’s battery test was abnormal in 22 (54%) patients as compared to eight patients (40%), with a P value = 0.3. The compass 31 score was significantly more in cases ( P value = 0.023). The SF 36 score ( P value = 0.019) and Bristol ADL score ( P value = 0.006) were better in the controls. In cases with autonomic dysfunction by Ewing’s battery test, QoL using Bristol ADL score ( P value = 0.2) and SF 36 (0.051) was worse.

Conclusions: This study confirms our hypothesis that autonomic dysfunction are more prevalent in AD and VD, and they may influence the functional status of patients and contribute to mortality; they also may influence the functional status of patients and contribute to morbidity and mortality.

Abstract ID: 677: The Neurological Spectrum of the CTD Prism!!!

T. Bhanu Chandra Prasad, U. Aruna Kumari, A. Sita Kanthima

Sawai Mansingh Medial College, Jaipur, Rajasthan, India

Background and Aim: Autoimmune diseases have a broad spectrum of clinical manifestations. Among them, neurologic involvement, both from the central nervous system as well as from the peripheral nervous system, is among the most challenging manifestations, regarding diagnosis and treatment. Neurologic involvement in systemic lupus erythematosus, Sjogrens, and antiphospholipid syndrome is frequently observed, and reported and several etiological factors are involved, including immunomediated damage and thrombosis.

Methodology: Two cases of Sjogrens presented as recurrent hyperacute peripheral neuropathy, neck drop, and skin pigmentation. Two cases of SLE presented as pyrexia of unknown origin along with neuropsychiatric manifestations and malignant IIH. Chronic headache turned out be CANCA positive. Two cases HUGHES syndrome presented as foot drop with recurrent abortions and anterior spinal artery thrombosis. Dermatomyositis SLE overlap presented as proximal muscle weakness which is MDA 5+ leukocytoclastic vasculitis (biopsy proven) presenting as acute peripheral neuropathy. Anti-SRP immune-mediated necrotizing myopathy was also noted. IgG4 presented as ptosis and sudden onset ophthalmoplegia. Vasculitic neuropathy presented as painful neuropathy with quadriparesis with high Anti-CCP positive with biopsy showing vasculitis picture most probably PAN. Takayasu arteritis presented as alternating hemiplegia.

Results: An increase in the frequency of neurological disorders related to autoimmune diseases is observed in our study. All the above cases with different neurological presentations, SLE and Sjogrens were the most common autoimmune disease associated with them.

Conclusions: Neurological presentations of connective tissue disorders serve as vital component in the comprehensive assessment and management of these conditions. By recognizing and addressing neurological symptoms, clinicians can improve patient outcomes and enhance our understanding of the underlying mechanisms driving these disorders.

Abstract ID: 678: To Study Congruence and Discrepancy of Interictal and Ictal EEG with MRI Lesions in Suspected Focal Epilepsies

Swati Garg, Arvind Vyas, Bhawna Sharma, Shubham Agrawal

SMS Medical College, Jaipur, Rajasthan, India

Background and Aim: Epilepsy is one of the most common neurologic disorders faced by neurologists worldwide. Aim: This study aims to compare the occurrence and localization of interictal and ictal epileptiform discharges (IEDs) and epileptic seizure patterns (ESPs) in suspected focal epilepsy with the localization of MRI lesions.

Methodology: After informed consent, detailed medical history will be noted. All EEG recordings were performed with electrode placement according to the 10–20 system. All patients underwent epilepsy-protocol cranial MRI. All the parameters will be collected and entered in a tabulated form, and statistical analysis will be conducted using Microsoft Excel™.

Results: In patients with temporal lesions, the localization of interictal epileptiform discharges (IEDs) was most congruent, with 60% of patients showing IEDs exclusively over the lesional lobe. This differed significantly ( P < 0.001) from those with frontal lesions, where only 30% had exclusively frontal IEDs. In cases of parieto-occipital lobe lesions, merely 10% exhibited IEDs exclusively over the lesional lobe, while 48.5% had no parieto-occipital IEDs at all.

Conclusions: The occurrence and localization of interictal and ictal EEG findings vary significantly depending on the brain region affected by lesions. These differences should be carefully considered when interpreting EEG results, especially in patients with extratemporal epilepsies being evaluated for epilepsy surgery.

Abstract ID: 679: Artery of Percheron A Diagnostic Challenge

Maheshpandian Mariappan, Murugan P. K., Venkateshwaran K. J.

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: The blood supply to the human thalami is complex and has multiple variants. The artery of Percheron is one of those variants and is characterized by a solitary arterial trunk that branches from one of the proximal segments of either of the posterior cerebral arteries and supplies blood to the paramedian thalami. This type of variation is present in 4–12% of the population. Occlusion of this artery is rare and results in a bilateral paramedian thalamic infarction that may extend to the midbrain with a multitude of neurologic signs and symptoms, which might prompt the physician to think of an etiology other than stroke.

Case Report: A 55-year-old male presented with acute onset of drowsiness after awakening in the morning, h/o abnormal behavior, and no h/o motor weakness.

Discussion: On examination patient was drowsy and partially responding to oral commands and had b/l ptosis. Pupils were b/l 3 mm SRTL. Impaired adduction on both eyes and abduction nystagmus was present on both eyes. His tone was normal and was moving his limbs to verbal commands. DTR was present, and plantar was flexor bilaterally.

Conclusions: As the patient presented outside the window period for thrombolysis, he was managed in ICU with antiplatelets, statins, and other supportive measures. Patient had aspiration pneumonitis which was managed and his condition improved and got discharged in a clinically stable condition. Had AOP been thought of at the first instance, patient would have benefited of thrombolysis.

Abstract ID: 680: Healthcare Seeking Patterns among Patients with Epilepsy

Saksham Jain, Samhita Panda, Mayank Garg

All India Institute of Medical Sciences, Jodhpur (AIIMS), Rajasthan, India

Background and Aim: A lack of knowledge about epilepsy, coupled with negative attitude and false practices, has been shown to be influenced by sociodemographic parameters and in turn impacts healthcare seeking behavior.

Methodology: An observational single center study was conducted at a tertiary care center in Western India from January 2019 to July 2020. Data on sociodemographic parameters, clinical, and healthcare seeking patterns was recorded from all patients above 18 years with a diagnosis of epilepsy. A pre-validated questionnaire was then administered to assess the knowledge, attitude, and practices related to epilepsy. Data collected was evaluated.

Results: A total of 320 patients with epilepsy were recruited for the study. Majority of the study subjects were young Hindu males from urban and semi-urban areas. Idiopathic generalized epilepsy was the most common diagnosis with significant number of patients showing poor seizure control. The KAP responses showed serious gaps in various aspects. Prevalent misconceptions were that epilepsy is a mental problem (40%), is a hereditary disorder (24.1%), is contagious (13.4%), and is a result of previous life sin (38.8%). A significant proportion of patients (78.8%) feared from the side effects of long-term antiepileptic drug treatment. Almost one-third persons (31.6%) did not know the correct response in terms of first aid measures. The mean KAP score was 14.33 (±3.017 SD), significantly higher in better educated people from urban areas ( P < 0.001 for both).

Conclusions: Despite improved literacy and urbanization, knowledge regarding epilepsy still remains poor with wide prevalence of malpractices. This results in delay in seeking appropriate healthcare after the onset of seizures.

Abstract ID: 681: Primary Progressive Aphasia: A Case Series of Three Patients

Atrikumar Patel, Saurav Raja, Iftikar Khadas, Ashish Kumar Duggal, Jyoti Garg

Ram Manohar Lohia Hospital, Type III, Connaught Place, New Delhi, India

Background and Aim: Primary progressive aphasias (PPAs) are a group of neurodegenerative diseases with early and prominent speech and language impairment, with relative preservation of cognition and without behavioral abnormality. PPA is viewed as a part of fronto-temporal dementia. It consists of three variants – nonfluent/agrammatic variant (naPPA), logopenic variant (lvPPA), and semantic variant (svPPA).

Methodology: We prospectively identified three patients with PPA and report their history, clinical examination, and radiological data.

Results: Patients were diagnosed with PPA as per diagnostic criteria proposed by Tempini et al . and then subclassified into clinical variants based on specific speech and language features characteristic of each subtype. Two patients were diagnosed with naPPA, and one patient was diagnosed with lvPPA. All three patients were females between 55–65 years of age. The patients with naPPA presented with difficulty in speaking with effortful and halting speech, suggestive of apraxia of speech. Patients with lvPPA presented with word-finding difficulties with preserved single-word comprehension. Detailed higher mental function supported the diagnosis of predominant impairment in the language domain with relative preservation of other cognitive domains. Apraxia battery test was conducted in one patient with naPPA which suggested buccofacial apraxia. Brain imaging was suggestive of cortical atrophy predominantly in the fronto-temporal lobe in all three patients. PET-CT of one patient with naPPA showed bilateral fronto-temporal hypometabolism. Histopathological evidence or genetic testing of known pathogenic mutation was unavailable for any patient. All three patients were managed conservatively, including referral for speech therapy.

Conclusions: A high degree of suspicion is required for diagnosis which is based on history and clinical examination, including a detailed higher mental function examination. Patients with PPA should be managed with speech and occupational therapy to enable them to carry out activities of daily living.

Abstract ID: 682: A Rare Case of Woodhouse-Sakati Syndrome, Mimicking Primary Dystonia

Naren Polavarapu, Jawahar Marimuthu, Nanda Kumar Vasudevan, Rajagambeeran V, Balasubramanian Samivel

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Woodhouse-Sakati syndrome (WSS) is a rare autosomal recessive disorder characterized by hypogonadism, alopecia, diabetes mellitus, mental retardation, and progressive sensorineural hearing loss. It is caused by mutations in DCAF17 gene. While neurological symptoms are part of WSS, isolated presentation with dystonia is uncommon.

Case Report: We report a rare case of WSS presenting with predominant dystonia. Neuroimaging and genetic analysis confirmed the diagnosis in our patient. Our patient is a 25-year-old male, born to third degree consanguineous parents, second of three siblings, attained milestones as per age. Presented with involutary posturing of limbs for last 10 years, strained speech for 4 years and dysphagia for 2 years. Symptom onset-asymmetrical, starting in distal left upper limb, progressing to right upper limb, trunk and neck, initially action dystonia, now present even at rest. There were no family h/o similar complaints. On examination, appendicular and axial rigidity present, with flexed posture in upper limbs. Retrocollis and right torticollis in addition to facial dyskinesias and bilateral striatal toe present. Patchy hair loss over scalp was observed. Normal blood workup, including sugars, and Wilson’s workup was negative. MRI brain showed T2 hypointensity in bilateral globus pallidus, substantia nigra, and red nucleus with blooming on GRE, suggestive of neurodegeneration with brain iron accumulation (NBIA). Whole exome sequencing revealed homozygous mutation in DCAF17 gene (c.1422 + 5G>T in intron 13), confirming our diagnosis. The patient was treated with oral anticholinergics, diazepam, and baclofen.

Discussion and Conclusions: WSS can rarely present with severe generalized dystonia, mimicking primary dystonia, highlighting need for multidisciplinary approach and genetic testing in such patients.

Abstract ID: 683: Secondary Efficacy Analysis in ANODYNE Study: A Randomized Double-Blind Placebo-Controlled Trial of Greater Occipital Nerve Block of Methylprednisolone and Lignocaine Compared with Placebo as a Transitional Preventive Treatment for Episodic Cluster Headache

Debashish Chowdhury, Sanjay Rao Kordcal, Rahul Nagane, Ashish Duggal

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi (GBPIPMER, New Delhi), India

Background and Aim: ANODYNE study showed that the greater occipital nerve block (GONB) for episodic cluster headache (ECH) was effective. Here, we report the results of the secondary outcome measures. Objective: To study the efficacy of GONB in improving the pre-specified secondary outcome measures.

Methodology: ANODYNE was a randomized, double-blind, and placebo-controlled trial in ECH patients diagnosed by ICHD-3 criteria who received active GONB (2 mL methylprednisolone [80 mg] and 2 mL lignocaine [2%]) or placebo (4 mL saline injections). The secondary outcome measures included mean change in attack duration, severity, requirement of repeat GONB, verapamil dose, changes in cranial autonomic symptoms (CAS), and severity of restlessness. Efficacy analyzes were performed at week 4 in a modified intention-to-treat population using a generalized linear model. The trial was registered with the Clinical Trials Registry of India (CTRI/2021/21/038397).

Results: Thirty-nine (19 in the active group and 20 in the placebo group) were available for efficacy analysis. The change in attack duration and severity was −18.8 (95% CI: −29.7 to −7.9; P = 0.001) and −2.3 (95% CI: −4.1 to −0.4; P = 0.019), Respectively, favoring the active group. The mean verapamil dose was lower in the active group (−96·6; 95% CI: −191·5 to −1·8; P = 0.046). Five patients receiving placebo and none receiving active GONB required a repeat injection (27.8% vs. 0%; P = 0.022). The mean CAS/attack duration was lower in the active group compared with placebo (−32.8 vs −22.3; −10·5 (95% CI: −15·9 to −5·1); P = 0.001). The active group had fewer attacks between Day 1–3 and Day 1–28 (−0·9 and −11 attacks, respectively) and a significant reduction in the severity of restlessness (−0·4; 95% CI: −0·7 to −0·03; P = 0.036).

Conclusions: GONB resulted in significant improvements in multiple secondary outcomes for ECH patients.

Abstract ID: 684: An Atypical Presentation of Hereditary Sensory Autonomic Neuropathy

Naveed Ahmed Nizamudeen, Jawahar M, Jude Marrian, Vignesh A

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Hereditary sensory autonomic neuropathy is clinically heterogeneous group of neuropathies characterized by prominent sensory loss and variable autonomic features but without significant motor involvement. The pronounced sensory loss in this disease predisposes the patient to unnoticed recurrent trauma, leading to nonhealing ulcers infection and osteomyelitis resulting in acral mutilation.

Case Report: A 14-year-male presented with recurrent ulceration of right big toe with numbness for past 4 years with history of unaware formation of blisters in right hand after holding a hot cup of coffee at 8 years of age. There was a history of amputation of a part of right big toe one year back and history of similar episodes in the left big toe for past four months.

Discussion and Conclusions: The importance of thorough neurological examination is evident from this case as possibility of pure neuritic leprosy overlapping on HSAN would have been missed.

Abstract ID: 685: “Clinical and Etiological Insights into Dystonia” in a Tertiary Care Hospital from South India

Venkatesh Kondle, Murugan P. K., Justin C, Elangovan S

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: Dystonia is a common hyperkinetic movement disorder characterized by abnormal, involuntary, and patterned posturing, which is often sustained or repetitive. We aimed to identify the proportion of various types of dystonias presenting to a tertiary care center in India.

Methodology: This is a cross-sectional study conducted in tertiary care hospital. Information regarding age at onset, duration of illness, distribution of dystonia, additional movement disorders, additional neurological/systemic signs, task specificity and family history, and investigations were collected. Both Axis 1 and Axis 2 classification schemes were applied. Dystonia was considered familial if at least one other family member in a three-generation pedigree had symptoms suggestive of dystonia, tremor, or parkinsonism.

Results: Data from 30 patients were included. Most of the study population had age of onset in second decade. Among 30 patients, 17 are female, and 13 are males. Distribution of dystonia was focal in 16, segmental in 2, multifocal in 1, hemidystonia in 4, and generalized in 7. Isolated dystonia (no clinical signs other than tremor) was observed 18 patients. Positive family history was observed in seven patients. One patient had pseudodystonia.

Conclusions: Isolated focal dystonia was the most common type, among which cervical dystonia was the most common type. Most common associated movement disorders are tremor followed by chorea. Family history is almost positive in one-fourth of study population.

Abstract ID: 686: Impact of delirium on the outcome of stroke

Sai Reddy

Gandhi Medical College

Background and Aim: Delirium is an acute fluctuating disorder of attention and awareness, which often complicates the clinical course of several conditions, including acute stroke. Delirium has been extensively associated with adverse outcomes in terms of length of hospitalization, functional dependence, and mortality in medical and surgery wards. However, limited studies have assessed the impact of delirium on the prognosis of patients with stroke, providing inconsistent results. The aim of the present study was to determine whether delirium occurrence impacts the outcome of patients with acute stroke.

Methodology: It was a prospective study conducted in a tertiary care center, for 6 months from November 2023 and April 2024. All patients presented to our hospital with acute stroke and developed delirium in 72 h, screened using CAM-ICU, were included in the study, and were followed for 90 days for functional disability using the mRS score.

Results: Of 45 patients with acute stroke with delirium, 9 (20%) patients succumbed to death in hospital stay, and 15 (33.3%) patents had functional disability of mRS >_ 3 at 90 days.

Conclusions: In conclusion, our study found that delirium negatively impacts the prognosis of patients with stroke. Patients with post-stroke delirium had a worse functional outcome and a shorter survival.

Abstract ID: 687: Cerebrotendinous xanthomatosis: a rare case of early-onset ataxia

T. Bhanu Chandra Prasad, Sai Lakshmi Perumalla, Aruna Kumari, Sita Kanthima

Panacea Healthcare and Diagnostics, NH-19, Gautam Puri, Sarita Vihar, New Delhi

Background and Aim: Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive disorder resulting from a defective enzyme in the bile acid synthesis pathway, leading to neurological, ocular, vascular, and musculoskeletal symptoms from the deposition of cholestanol and cholesterol in these tissues.

Case Report: We report a case of a 33-year-old female patient born out of second-degree consanguineous parentage who presented with chronic progressive mixed (sensory/cerebellar) gait and limb ataxia, tendon xanthomas, bilateral cataracts, dysarthria, mild cognitive impairment, and febrile seizures during childhood with deranged lipid profile. Magnetic resonance imaging (MRI) of the brain showed cerebellar atrophy and was genetically proven to be CTX.

Discussion: Genetically confirmed CYP27A1 defect.

Conclusions: Due to the low awareness of CTX and the variability of its clinical findings, its diagnosis maybe delayed for years, as in our patient. When diagnosed, most patients may have severe, often irreversible neurological damage. With the early recognition of the CTX and the start of treatment, patients can have a chance to quality life.

Abstract ID: 688: Clinical and Etiological Profile of Pachymeningitis: A Study from a Tertiary Care Center

Kamani Babu, Suryaprabha Turaga, Reshma Sultana

Nizam’s Institute of Medical Sciences, Hyderabad (NIMS, Hyderabad), Telangana, India

Background and Aim: Hypertrophic pachymeningitis is a form of fibrosing chronic inflammatory process, which causes the thickening of the intracranial dura mater. Though the common symptoms are headache and multiple cranial nerve palsies, presentation is non-specific and varied. Aims: To study the clinical and etiological profiles of hypertrophic pachymeningitis and highlight unusual presentations.

Methodology: This was a single-center study where adults between the age groups of 12 and 80 years who had neurologic symptoms and pachymeningeal enhancement on magnetic resonance imaging (MRI) brain/spinal cord retrospectively, over the past 2–3 years, from the electronic database of the institute from the department of neurology, and followed for 5 years from when they were diagnosed. The characteristics of patients, including demographics, clinical features, laboratory data, radiological features, risk factors, and response to treatment were analyzed.

Results: Headache, cranial nerve palsies, and seizures were common symptoms. Cases of hypertrophic pachymeningitis mimicking spinal meningiomatosis, cavernous sinus syndrome, Tolosa–Hunt syndrome, jugular foramen syndrome, meningioma, and hyperostosis are presented.

Conclusions: Ours is an attempt to alert the treating physicians and surgeons about atypical presentations of hypertrophic pachymeningitis in clinical practice to avoid undue complications. In patients with chronic headaches who do not respond to conventional treatment, neuroimaging should be conducted to rule out pachymeningitis.

Abstract ID: 689: Spontaneous Intracranial Hypotension: A Headache that Disappears on Lying Down

Boudhayan Haldar, Dhiman Das

RG KAR Medical College and Hospital 1 Khudiram Bose Sarani Kolkata 74

Background and Aim: Spontaneous intracranial hypotension (SIH) occurs due to abrupt spontaneous leakage of the cerebrospinal fluid (CSF) outside of its anatomical space (vascular/extradural). It is commonly due to disc disease causing rent in the meninges or a fistula formation, leading to the draining of the CSF in the venous network. SIH is an extremely rare presentation that is characterized by orthostatic headache, diffuse pachymeningeal enhancement on magnetic resonance imaging (MRI), with magnetic resonance (MR) myelography identifying CSF leak in most cases.

Case Report: A 49-year-old diabetic hypertensive male patient presented with acute-onset severe dull aching pain in the occipital region radiating to the neck that appeared on the erect posture and disappeared in the supine position. On examination, no neurological deficiency was present. An extensive workup was inconclusive, except for a diffuse pachymeningeal enhancement in the MRI brain and mild sagging. By the second week of presentation, the patient had developed tinnitus and aural fullness.

Discussion: MR myelography of the whole spine was conducted that revealed an epidural CSF collection at 4th to 6th cervical vertebral levels with a focal collection in the posterior paraspinal muscles due to a possible dural defect. The patient was then treated with an epidural blood patch repair that resolved his symptoms and he was discharged without any complications.

Conclusions: SIH is a rare but possible cause of orthostatic headache that may be lethal if early intervention is not done.

Abstract ID: 690: Young Adult with Rapid Cognitive Decline and Parkinsonism

Amlan Mohapatra, Ish Anand

Sir Ganga Ram Hospital, New Delhi, India

Background and Aim: Leukodystrophies are a rare heterogenous group of disorders characterized by central nervous system white matter degeneration. It encompasses a variety of manifestations, including cognitive impairment, psychiatric symptoms, upper motor neuron signs, parkinsonism, and seizures. Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia is common among these. This disease frequently has an autosomal dominant inheritance, which results from a mutation in the CSF1R gene.

Case Report : We present here a 34-year-old female patient who presented with dysarthria, psychiatric symptoms, and cognitive decline . The patient’s detailed history, including family history, clinical characteristics, examination findings, brain MRI, and genetic results, were obtained and analyzed. We thoroughly reviewed the literature available in PubMed and Google Scholar.

Results: Brain MRI revealed b/l periventricular white matter hyperintensities with diffusion restriction along with corpus callous involvement. A heterozygous variant of uncertain significance (c.2546_2548delITCT, p.Phe849del) was detected in exon 19 of the CSF1R gene in whole-genome sequencing. The patient’s brother and father did not show the mutation.

Conclusions: Clinical suspicion and early recognition of clinical features along with radiological and genetic correlation lead to correct diagnosis, which will help in genetic counselling.

Abstract ID: 691: Multifocal Motor Neuropathy without Conduction Block in a Diabetic Patient

Amlan Mohapatra, Amlan Tapan Mohapatra, Ish Anand

Sir Ganga Ram Hospital, New Delhi, India

Background and Aim: Multifocal motor neuropathy (MMN) is an immune-mediated disease entity, characterized by progressive asymmetrical weakness and atrophy without sensory involvement. MMN is diagnosed clinically and nerve conduction studies suggest demyelination and conduction block. However, motor neuropathy without a conduction block is also observed. Here, we present a case report who was a 72-year-old diabetic male patient, who presented with a right-hand finger drop.

Case Report: The patient’s details including history, complete neurological examination, electrodiagnostic studies, and nerve biopsy were obtained and analyzed. Further, we extensively reviewed the literature.

Discussion: The nerve conduction study showed distal asymmetrical, sensory-motor axonal polyneuropathy involving both lower limbs and the left upper limb. Electromyography showed active denervation in the left lower limb distal muscles. A sural nerve biopsy showed axonopathy. Serum ganglioside antibodies were negative.

Conclusions: MMN without conduction block is a treatable entity. Early diagnosis prevents disability. However, further studies are required in co-existing systemic diseases such as diabetes mellitus like in our case.

Abstract ID: 692: To Determine the Predictive Value of Inflammatory Prognostic Index and Atherogenic Coefficient in Acute Ischemic Stroke Patients: A Retrospective Study

Chebolu Harshika, Randhi Venkata Narayana, Puvvada Srikar, Amarapu Sravanthi

Sevenhills Hospital, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Stroke contributes to a significant proportion of mortality and disability globally. There is an increasing need to identify better prognostic indicators. Our study aims to determine the predictive value of the Inflammatory Prognostic Index (IPI) and Atherogenic coefficient (AC) as prognostic indicators in acute ischemic stroke, and correlate these indices with outcomes.

Methodology: A retrospective analysis of 254 patients admitted with acute ischemic stroke between January 2023 and January 2024 was performed. Data including comorbidities, NIHSS score at admission, lipid profile, blood counts, C-reactive protein (CRP), serum albumin, and modified Rankin Score (mRS) at discharge were obtained. IPI= CRP * neutrophil–lymphocyte ratio/serum albumin level. AC = (serum total cholesterol–serum HDL cholesterol)/serum HDL cholesterol. Patients were classified into mild stroke (NIHSS score-5).

Results: Of the 254 patients, 39% had a mild stroke and 96.2% of patients had comorbidities. The IPI and AC values were significantly higher in the moderate-to-severe group ( P- value < 0.05) as compared to the mild stroke group (median values of IPI [1.72 vs. 1.14] and AC [2.5 vs 2.01]). Higher IPI values were shown to be associated with prolonged duration of hospital stay and poor outcomes, whereas AC values did not show such a correlation.

Conclusions: IPI and AC are easily accessible blood parameters, without subjective bias. Including these indices in routine practice helps in risk quantification and prognosis assessment in patients with stroke.

Abstract ID: 694: IV Thrombolysis with Tenecteplase in Pregnancy with Acute Ischemic Stroke and Favorable Outcome-A case

Surekha Dabla, Kiran Bala, Neeraj kumar

LSL Super Specialty Building B block Pt BD Sharma, PGIMS, Rohtak, Haryana

Background and Aim: Pregnancy increases the risk of acute ischemic stroke (AIS) among young women and is responsible for maternal deaths and significant disability. Delay or denial in treatment can significantly worsen outcomes because of concerns of potential adverse events of imaging and reperfusion.

Case Report : We report a 27-year-old lady G2P1A0 with 36 + 2 weeks gestation presented within 3 h of sudden-onset, left-sided weakness with altered behavior, inability to express herself, and facial deviation and gaze preference to the right side with NIHSS 19/42. After a detailed discussion with the family, she was thrombolyzed with IV tenecteplase, she improved over the next 24 h with NIHSS 7/42 and later she completely recovered. She delivered a live healthy baby 8 days of post-thrombolysis.

Discussion and Conclusions: To the best of our knowledge, this is the first case of pregnancy thrombolysis with tenecteplase with a good outcome. We conclude that pregnancy should not be a contraindication for IV thrombolysis.

Abstract ID: 695: A Hospital-Based Study of Ischemic Stroke in Young in a Tertiary Care Center of Northern India

Prerna Dogra, Jyoti Bala Sharma

Fortis Hospital Noida, India

Background and Aim: Young stroke is an emerging problem in India, where the increase in standards of living is leading to an ocean of lifestyle-related disorders. The presence of stroke in the young subset of the population can have a major impact not only on an individual level but can also be crippling for society and the nation at large.

Methodology: We conducted a retrospective population-based study in a tertiary care hospital in North India over 2 years, where we included all patients of ischemic stroke who fell in the age group of 15–44 years of age. A 6-month follow-up and MRS were also documented.

Results: A total of 92 patients were diagnosed with young stroke, out of which 62 (67%) were male and 30 (33%) were female. Dysarthria (42%) and hemiparesis (35%) were the most common presentations. The most common pre-existing comorbidities were hypertension, diabetes, obesity, and pro-coagulant states. Drug addiction and alcohol abuse were also noted. Also, 65% had anterior circulation strokes and 35% had posterior circulation strokes, and 28% were thromolyzed.

Conclusions: Young stroke is an emerging problem in India and a great majority of risk factors may be modifiable.

Abstract ID: 696: Autoimmune Encephalitis: A Tertiary Care Experience from North India

Prerna Dogra, Jyoti Bala Sharma

Fortis Hospital, Noida, India

Background and Aim: Autoimmune encephalitis comprises a group of non-infectious, immune-mediated inflammatory disorders of the brain.

Methodology: We retrospectively studied 14 patients diagnosed with AIE, both seropositive and seronegative for 6 months. Patients were diagnosed on a clinical basis with magnetic resonance imaging (MRI) findings, positron emission tomography (PET) computed tomography (CT) findings, EEG, presence of known antibodies associated with AIE, and response to immunomodulatory therapy.

Results: Baseline EEG was suggestive of generalized slowing in five (35%), focal epileptiform discharges in two (14%), and delta brush in two (14%) patients. The MRI brain showed temporal lobe hyperintensities in three (21%) patients, and thalamic and external capsular hyperintensities in two (14%) patients. Three patients had the presence of anti-Caspr2 antibodies, one had the presence of anti-amphiphysin antibodies, and one had anti-VGKC antibodies. Also, 18 FDG PET CT was conducted for 13 of the 14 patients and showed an abnormality in all-frontotemporoparietal hypometabolism in four (28%), frontotemporal hypometabolism in four (28%), global hypometablism in four (28%), and basal ganglia hypometabolism in one (7%) patient. Cognitive and behavior tests were repeated after 6 months of monthly IVIG. None of the patients had seizure recurrence after treatment. PET CT brain was repeated after 6 months and was found to be suggestive of improvement in FDG uptake in the previously mentioned regions of hypometabolism. EEG normalization was also noted in all patients after completing the course of treatment. MRS score was found to be 0 for all patients post-treatment, except one patient who developed sepsis post-RTX therapy.

Conclusions: AIE should be ruled out before labelling a patient with a diagnosis of dementia with a grave prognosis.

Abstract ID: 697: Neurological Manifestations of Wilson Disease: An Analytical Study

Vikram Khardenavis, Ramu Sekar, P. K. Murugan

Madurai Medical College, Tamil Nadu, India

Background and Aim: Wilson’s disease (WD) presents an autosomal recessive disorder impacting copper metabolism and manifesting hepatic or neuropsychiatric complications. We aim to study demographic, clinical, and investigational features.

Methodology: The study, conducted at Madurai Medical College, gathered patient data using a structured form, emphasizing family history and consanguinity. Collected information included symptom onset, and diagnostic tests such as liver function tests and imaging. Diagnosis of WD required liver disease and meeting specific criteria, including low ceruloplasmin, and KF rings. Statistical analysis presented quantitative data as mean ± standard deviation (SD) and qualitative data as frequency and percentage.

Results: Our study of 14 patients diagnosed with WD revealed a mean age of 21.91 years, with a range from 13 to 51 years, and the majority of male patients (8) compared to female patients (6). All patients exhibited decreased serum ceruloplasmin and KF rings, resulting in a WD diagnostic score of over 4 for each. Treatment included chelating agents and zinc salts for all cases, with 43% showing hepatic involvement. Neurological symptoms were prevalent, with akinetic rigidity, pseudosclerosis with tremor, and dystonia affecting over 40% of patients, whereas cognitive impairment was noted in 29% and seizures in 1 (7%) patients. MRI changes were present in all cases.

Conclusions: WD neurologic symptoms often reverse with anti-copper treatment; yet, many have residual issues. Early treatment improves prognosis.

Abstract ID: 698: Beyond the Surface: Delving into Posterior Reversible Encephalopathy Syndrome through Clinical Cases

Nirosha Metta, Mohan P. Channappanavar, Rashmi Devaraj, Kavya Mala, Nataraju B

Vydehi Institute of Medical Sciences and Research Centre, Bengaluru (VIMS, Bengaluru)

Background and Aim: Posterior reversible encephalopathy syndrome (PRES), first described by Hinchey et al. in 1996, represents a neurological disorder with varied clinical presentation and typical imaging findings of parieto-occipital predominant pattern of vasogenic edema. The aim of this presentation of case series of PRES is to provide a comprehensive overview that integrates knowledge of pathophysiology, clinical presentation, diagnostic evaluation, and prognosis.

Methodology: We conducted a retrospective analysis of eight cases diagnosed with PRES across 8 months at xxxx. Participants were selected based on confirmed radiological findings consistent with PRES and categorized into different age groups. Data on clinical manifestations, radiological features, and underlying etiologies were collected and analyzed descriptively.

Results: We reported eight cases of PRES with variable clinical manifestations of headache, altered sensorium, seizures, and blurring of vision. Out of eight cases, four were females four were males at different age groups with various etiologies (nephrotic syndrome, PSGN, eclampsia, CML blast crisis [TKI tyrosine kinase inhibitors], nivolumab-induced hyponatremia, hypoxia, and SLE). Radiological examination confirmed the presence of posterior-predominant white matter vasogenic edema, involvement of the parietal and occipital lobes, and symmetrical and bilateral findings, indicating typical patterns associated with PRES.

Conclusions: This case series highlights the variable manifestations of PRES across different age groups, and diverse etiologies in its diagnosis and management. Withdrawal or management of offending triggers such as hypertension, impaired renal function, and immunosuppressive therapies improve outcomes.

Abstract ID: 699: Channel into the Clinical Chain of Electric Gain !!! (Spectrum of DEE)

T. Bhanu Chandra Prasad, Sita Kanthima

Sawai Mansingh Medial College, Jaipur, Rajasthan, India

Background and Aim: The term “developmental and epileptic encephalopathy” (DEE) refers to when cognitive functions are influenced by both seizure and interictal epileptiform activity and the neurobiological process behind the epilepsy.

Methodology: First case: A 3-year-old girl born out of consanguineous parentage presented with refractory seizures (GTCS) since the third day of life, infantile spasms since 9th months, exaggerated startle response, global developmental delay, dystonic posture of limbs, EEG showing classic hypsarryhtmia. • Second case: A 13-year-old male child, born out of consanguineous parentage, no h/o birth asphyxia, associated with delay in the global development of milestones, with h/o seizures since the 5th year (semiology of GTCS), h/o startle myoclonus, with behavioral abnormalities in the form of an inappropriate laugh, the excessive beating of legs while sleeping, family h/o younger sister died due to seizures at 3 years of age • Third case: A 2-year-old female child born out of consanguineous parentage, no h/o birth asphyxia, h/o delayed language milestones, febrile seizures, myoclonic jerks since the age of 1 year, now presented with status epilepticus MRI brain: frontotemporal atrophic changes in EEG: generalized polyspike waves.

Results: All three cases underwent whole-exome sequencing, the first two cases were positive for SCN2A (DEE11), and the third case was positive for STXBP1 (DEE4). All children responded to steroids, multiple anti-epileptics; but all of them relapsed and were refractory to other anti-epileptic drugs.

Conclusions: In cases in which encephalopathy is caused by both developmental impairment and epileptic activity, a DEE should be considered. Timely initiation of treatment for people with DEE can result in improved seizure control.

Abstract ID: 700: Hemidystonia and Oromandibular Dyskinesia with Tuberculous Meningitis and Tuberculomas: Atypical Presentation with TBM

Sneh Jain, Shweta Pandey

King George’s Medical University, Lucknow (KGMU Lucknow), Uttar Pradesh, India

Background and Aim: Central nervous system (CNS) tuberculosis is the most severe form of tuberculosis leading to neurological sequalae in up to two-third of survivors. 1 This case report describes generalized dystonia after tuberculous meningitis and tuberculomas. This report emphasizes the movement disorders after infective etiology.

Case Report: A 25-year-old male patient presented with complaints of abnormal posturing movement in the right hand and right leg for the last 7 months and abnormal tongue protrusion with the inability to retract the tongue inside the mouth spontaneously for the last 4 months. So, he started using a finger or a cloth to retract the tongue as a trick, his voice also changed as his labial and alveolar phonetics were not clear (videos are available). He had a history of fever, cough, headache, and unconsciousness in December 2020. He was diagnosed with tuberculous meningitis with multiple tuberculomas, and treated with antitubercular drugs. His condition improved subsequently and started doing his daily routine work independently.

Discussion: His repeat MRI was conducted, in which T2 and FLAIR hyperintensity were present in the right ganglio-capsular region and left thalamic region. Other metabolic work up came negative, and it was correlated from the old MRI and found that he had tuberculomas in the same regions. So, there were some gliotic changes. He was treated with trihexyphenidyl and clonazepam. The doses were titrated and his symptoms were improved.

Conclusions: This report emphasizes the movement disorders after infective etiology. So, if any patient is presenting with any movement disorder, we should do a work up for infective pathology such as tuberculous meningitis (TBM).

Abstract ID: 701: CNS Tuberculosis Masquerading as Central Venous Sinus Thrombosis

Arnab Adhya, Jasmine Parihar

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Central nervous system (CNS) tuberculosis (TB) comprises 1–5% of all TB cases, with a high mortality rate of 15–40%. Diagnosing CNS TB is challenging due to its varied and non-specific presentations, leading to delays in treatment initiation. We present two cases illustrating the elusive manifestations of CNS TB.

Methodology: Two patients with diverse presentations underwent extensive evaluation, including routine biochemistry, cerebrospinal fluid (CSF) analysis, and imaging. Definite diagnoses were established through histopathological examination.

Case Report: The first case involved a 43-year-old female patient with a year-long unilateral moderately severe headache and a seizure after 4 months. Initially suspected of venous sinus thrombosis and started on anticoagulation, her persistent headache led to further evaluation. Imaging suggested an infective etiology with focal meningitis. Normal CT chest and abdomen, along with inconclusive CSF studies, prompted a brain biopsy, confirming tubercular pathology. The second case involved a 45-year-old male patient with a 1-month history of a left-sided headache, followed by progressive right-sided hemiparesis. MRI brain revealed extensive contrast enhancement at the left central sulci and blooming on SWI, suggesting cortical vein thrombosis versus sulcal subarachnoid hemorrhage with significant edema in the left hemisphere. No definite tuberculoma or basal exudates were observed. CSF study, CT chest, and abdomen were all normal. A brain biopsy showed granulomatous pathology, suggestive of tuberculosis. Both patients started on ATT and steroids, showing a good clinical response at a 3-month follow-up.

Discussion and Conclusions: Clinicians should be familiar with varied atypical presentations of such a treatable disease to avoid delay in diagnosis and several dreadful complications.

Abstract ID: 702: Clinical Profile, Neuroimaging Characteristics, and Outcome of MOGAD: A Single-Center Case Series Study

Arya R, Ashraf VV, Abdurehiman KP, Sreevidya LK, Paul J. Alapatt, Smilu Mohanlal, Mubeena

Malabar Institute of Medical Sciences, Govindapuram, Calicut, Kerala, India

Background and Aim: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is the most recently defined inflammatory demyelinating disease of the central nervous system (CNS). The clinical spectrum of MOGAD is expanding with case series and reports. This study aimed to report the clinical profiles, imaging characteristics, and treatment outcomes of MOGAD in our center.

Methodology: This was a single-center case-series study. Clinical and paraclinical data, along with treatment outcomes of patients with MOGAD were analyzed.

Results: A total of 18 patients were included in the study. Of them, 12 were females (66%), and the median age at disease onset was 21.5 years. The most frequent presentation was optic neuritis (61%) with 55% of them being bilateral. Other presentations included focal neurological deficits (39%), acute disseminated encephalomyelitis such as presentations (16%) and myelitis (5.6%). Elevated protein in CSF was observed in two patients. CSF pleocytosis was observed in eight (39%) patients. Neuroimaging with contrast-enhanced MRI brain and spinal cord, optic nerve thickening and enhancement were the most common findings observed in 50%, followed by thalamic involvement (39%). Other findings were focal brain T2/FLAIR hyperintense lesions (9%), cerebellar peduncle lesions (22%), brainstem lesions (27%), periventricular lesions (16.7%), spinal cord lesions (17%), and conus lesion (one patient). All patients were treated with IV methylprednisolone during acute attack and two of them required IVIG along with steroids. Maintenance treatment was administered with oral steroids to all patients. Eight patients were started on MMF, two on rituximab and one patient was administered azathioprine. The mean EDSS score at presentation was 3 ranging between 1 and 8 and the mean EDSS on follow-up was 0–1. On follow-up, five patients had relapse with four of them presenting with optic neuritis and the other with focal neurological deficits.

Conclusions: In conclusion, the clinical characteristics of MOGAD were heterogeneous, ranging from mild optic neuritis to encephalomyelitis and LETM. The long-term prognosis was relatively favorable in most patients. Large-size, multi-center studies are warranted to determine the risks of relapse and optimal treatment strategies.

Abstract ID: 704: Neurological Complications with Primary Varicella Zoster Virus infection: An Observation Study

Nidhi Yadav, Shubhakaran Khichar, Amita Bhargava

DR SNMC Jodhpur, Rajasthan, India

Background and Aim: Worldwide, about 95% of the adult population has been infected with varicella zoster virus (VZV). It can involve various parts of the central and peripheral nervous systems. Neurological complications are rare in primary VZV infection, observed in less than 1% of cases This study was conducted to evaluate the spectrum, clinical course, and outcome of various neurological manifestations in patients with primary varicella-zoster virus infection.

Methodology: It was an observational study of patients with primary VZV infection at the Department of Neurology between January 2023 and March 2024. Patients with neurological manifestations due to VZV reactivation were not included in the study. All primary VZV patients were found to have characteristic chickenpox rash and/or its scar. The course of the disease and clinical outcome after treatment were studied for up to 3 months.

Results: Neuroimaging, either computed tomography or magnetic resonance imaging, cerebrospinal fluid analysis, and electroencephalography were performed in all patients along with other specialized investigations as per the clinical context. The age of presentation varied from adolescents to elderly (13 to 77 years) and the range of clinical spectrum included meningoencephalitis, cerebellitis, cranial neuropathy, and spinal cord involvement in the form of myelitis.

Conclusions: VZV is a common viral illness, and most patients recover without any complications. Although uncommon, neurological complications can be observed and can be life-threatening. So, early diagnosis and management should be prompted.

Abstract ID: 705: Case series: Congenital Myesthenia Syndrome: An Underdiagnosed and Treatable Neuromuscular Disorder

Abhishek Bhargava, Bhawana Sharma, Shubham Agrawal, Drishti Desai

Sawai Mansingh Medial College, Jaipur, Rajasthan, India

Background and Aim: Congenital myasthenia syndrome is an inherited neuromuscular disorder and depending upon the mutation and special clinical features present, it can be categorized into various subtypes.

Methodology: Four cases of congenital myasthenia syndrome were diagnosed in the SMS Neurology Department, Jaipur. Based on the history and clinical findings, congenital myasthenia was suspected and diagnosis was confirmed by whole-exome genetic testing.

Results: Out of the four cases, three patients (average age: 22.4 years) were found to have CHRNE gene mutation, resulting in a post-synaptic type of CMS. All three patients had decreased intrauterine fetal movements; during infancy, they had a poor sucking reflex and feeble cry. Motor milestones were delayed. One out of the four patients also developed respiratory distress and frequent pneumonia during infancy. Patients had fatiguability and fluctuating weakness, which were exacerbated after exercise in hot weather and were relieved by rest and a cold environment. Patients had proximal upper and lower limb weakness along with bilateral ptosis and external ophthalmoplegia. In the view of the onset of symptoms at birth along with the diurnal type of weakness, the possibility of CMS was kept, which was confirmed by a whole-exome gene test. Differential diagnosis of mitochondrial CPEO was kept and ruled out. Three patients were diagnosed with CHRNE mutation and were started on pyridostigmine, which showed dramatic improvement. One patient was diagnosed to be having a slow channel defect, for which fluoxetine was started.

Conclusions: This case series illustrates the importance of correctly diagnosing the mutation-causing CMS.

Abstract ID: 706: Assessing Social Cognitive Impairment in Dementia Patients using a Bedside Novel Cognitive Tool–BASS-D (Brief Assessment of Social Skills in Dementia)

Abhishek Bhargava, Banshi Lal Kumawat, Bhawana Sharma, Rajni Fulwariya

Sawai Mansingh Medial College, Jaipur, Rajasthan, India

Background and Aim: Objective assessment of social skills is rarely undertaken in clinical settings, because there is a lack of appropriate screening tools for quick assessment. The BASS-D provides a brief and informative assessment of social cognition in people with dementia, filling the current gap.

Methodology: The study took place at the SMS Medical College and Associated Hospital, Department of Neurology, Jaipur. It was a hospital-based comparative cross-sectional observational study. The Brief Assessment of Social Skills-Dementia (BASS-D) is administrable bedside and examines emotion recognition, face recognition, face identification, empathy, theory of mind, social disinhibition, and social reasoning. The BASS-D was administered to 25 healthy older adults (mean age = 65.8 years) and 25 age- and sex-matched controls without dementia (mean age: 64.5 years).

Results: Each of the BASS-D domains demonstrated adequate convergent validity ( r = 0.32–0.81), with the exception of face memory. Significant differences in the total BASS-D scores were observed between healthy adults and patients with a diagnosis of dementia.

Conclusions: BASS-D holds promise as a short screening test for assessing the social cognitive domain in dementia patients, which will help clinicians in diagnosing patients with dementia who present with early social cognitive impairment without other domain involvement, such as in frontotemporal dementia. This test will also help in quantifying the extent of social cognitive impairment in patients with dementia according to which counseling of attendants will become simplified, which will assist families and carers in managing social cognition impairments.

Abstract ID: 707: An Uncommon Silver Lining Presentation of a Common Disease: A Case of Isolated Unilateral Hypoglossal Nerve Palsy due to Tuberculous Hypertrophic Pachymeningitis

Sai Gullapalli, L. A. Ravi, V. Prakash

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Case Report: A 36-year-old female homemaker came with complaints of occipital headache, and left-sided neck pain since 2 months. One week after the above complaints, she had difficulty manipulating food in the mouth and propelling it back to the throat. She had no other complaints suggestive of other cranial nerve, motor, sensory, or autonomic dysfunction. On examination, she had right II, III, IV cervical lymphadenopathy. There was atrophy of the left half of the tongue with lower motor neuron (LMN) type of XII cranial nerve palsy. No signs of other cranial nerve, motor, sensory, cerebellar, or autonomic dysfunction. No signs of meningeal irritation. MRI brain with contrast conducted outside was reported as normal. Contrast-enhanced computed tomography (CECT) neck–cervical lymphadenopathy and skull base appeared normal. MRI neck plain with contrast–incidental finding of enhancement of pachymeninges at the left parietooccipital region and pituitary stalk. CSF cartridge based nucleic acid amplification test (CBNAAT)negative, acellular, glucose-76 mg/dL, and protein-25 mg/dL. The vasculitis profile and other auto-immune antibodies were negative. ESR 90 mm/1st h. IgG4-normal. S. ACE-normal. Cervical lymph node biopsy-caseating granulomatous lymphadenitis with lymphocytic infiltrate, Langerhans giant cells, and plasma cells. Tissue RT-PCR-awaited.

Discussion and Conclusions: This case is being presented due to the isolated involvement of pachymeninges by TB without brain parenchymal or leptomeningeal involvement (two to three cases of isolated TPM with cervical lymphadenopathy reported till now). It manifested as isolated unilateral hypoglossal nerve palsy, which is mostly idiopathic and provided a silver lining to the presentation of the patient as it is a treatable cause.

Abstract ID: 708: An Atypical Presentation of Cortical Venous Sinus Thrombosis: Areflexic Quadriparesis with Blindness and Ophthalmoparesis

Senthil Kumar, Marian Jude Vijay A, Ravi L A, Jawahar M, Balasubramaniam S

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Thrombosis of the cerebral venous system presents commonly with headache (~80%) and seizures (45%).

Case Report: We present a case of a 17-year-old female who presented with unusual symptomatology following cortical venous thrombosis.

Discussion: A 17-year-old female patient presented with a past history of a neurological syndrome of double vision and bilateral subacute vision loss with partial recovery 3 years ago. She now presented with further worsening of vision bilaterally with malaligned eyes over 1 week, followed by motor weakness involving the bilateral upper and lower limbs progressed over 3–4 days, without history of headache/vomiting or other positive history. Examination revealed bilaterally absent perception of light, bilateral disc pallor on fundoscopic examination, complete ophthalmoplegia with generalized hypotonia, proximal more than distal weakness of bilateral upper and lower limbs, truncal and neck muscles weakness with areflexia without other positive findings. MRI brain and spine revealed no detectable abnormal findings. MRV revealed features suggestive of cerebral venous thrombosis involving the superior sagittal sinus, straight sinus, and right transverse sinus. Nerve conduction studies revealed features of diffuse radiculopathy. Lumbar puncture showed elevated CSF opening pressure of more than 50 cm of H 2 O with an otherwise normal CSF analysis report.

Conclusions: We report a case of IIH presented with an uncommon syndrome of a rapid diminution of visual acuity, ophthalmoplegia, and flaccid limb weakness due to extensive radiculopathy secondary to elevated ICP. Quadriparesis is a rare presentation of raised intracranial pressure, which may delay the diagnosis and affect the outcome.

Abstract ID: 709: A Rare Case of Diffuse Cerebral and Basal Ganglia Calcification

Deepak Rajendran, Deepak Rajendran, Prakash V, L A Ravi, Balasubramanian Samivel

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Fahr’s syndrome is a rare, genetically dominant, inherited neurological disorder characterized by an abnormal deposit of calcium in the areas of the brain that control movement, including the basal ganglia and the cerebral cortex. Here, we present the case of a 60-year-old female patient with a past history of thyroidectomy, who presented with abnormal twisted posturing of the limbs and seizures.

Case Report : A 60-year-old female patient with a past history of thyroidectomy, presented with abnormal twisted posturing of the limbs and seizures. Semiology was focal onset with impaired awareness. She had dystonic posturing of upper limbs. Biochemical investigations revealed hypocalcemia, hyperphosphatemia, and low levels of PTH, suggesting hypoparathyroidism. MRI brain revealed diffuse calcification throughout the cerebral cortex, subcortical structures, and cerebellum.

Discussion and Conclusions: Fahr’s syndrome should be considered as one of the DDs for basal ganglia calcifications.

Abstract ID: 710: Experience of CSF Biomarkers in Patients with Dementia

S. Sandeep Kumar, Gnanageetha B B, Jaydip Ray Chaudhuri Chaudhuri, Rajashekar Reddy, Mohan Krishna J, Sandeep S, Kamala Priya S, Pavan Kumar K, Swathi Alluri

Vasudha Hospital Hyderabad, Telangana, India

Background and Aim: Dementia is a neurodegenerative disorder characterized by cognitive impairment. Alzheimer’s disease is the most common cause of dementia; however, there are many other causes of dementia. Dementia of Alzheimer’s disease can be differentiated from other neurodegenerative dementia with the presence of CSF biomarkers such as amyloid beta, and phosphorylated tau. Aims: To correlate between CSF biomarkers and clinical manifestations in patients with cognitive impairment.

Methodology: It was an observational study of patients with cognitive impairment who gave consent. All patients underwent clinical and cognitive examination (ACE III), radiological imaging (MRI brain, PET brain), relevant investigations to look for reversible causes of dementia and autoimmune markers, and CSF analysis for biomarkers.

Results: Among the four patients who had given consent, two patients had amnestic MCI, one patient had features suggestive of posterior cortical atrophy, one patient had frontolobar syndrome with parkinsonism. PET brain showed hypometabolism in frontotemporal regions in two patients, mild hypometabolism in B/L caudate nucleus, and parietotemporal cortices in one patient, hypometabolism in the parietotemporal region in other patients. CSF analysis routines were normal, dementia profile revealed reduced beta-amyloid (1-42), raised p Tau/A beta 1-42 ratio. Because two patients had rapid progression, autoimmune, and paraneoplastic panels were positive for anti-titin and anti-YO.

Conclusions: Patients with cognitive impairment may have dual pathology. Various neurodegenerative disorders may have overlapping clinical features. With the advent of new anti-amyloid therapy, biomarker diagnosis of dementia is essential. We should always try to differentiate dementia of Alzheimer’s from other causes.

Abstract ID: 711: Vasculitic Neuropathy: Multitude of Presentations

Raj Gokul Prabhakar, Thamil Pavai A, Ravi L A, Balasubramanian S, Prakash V

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Vasculitic neuropathies are a heterogeneous group of peripheral nerve disorders that can be either systemic or non-systemic. Most patients present with mononeuritis multiplex, requiring high clinical suspicion for proper diagnosis and management.

Case Report: This case series details five patients diagnosed with vasculitic neuropathy. Two patients presented with burning pain in the ankles, palms, and feet, along with walking difficulty and trouble holding slippers for 2 months. Examination showed hip power of 5/5, knee power of 4/5, and ankle power of 1/5, with absent knee and ankle jerks. Sensory examination indicated reduced pain perception. NCS revealed bilateral sensorimotor axonal polyneuropathy. Elevated CRP and ESR were noted; ANA and ANCA were negative. A nerve biopsy confirmed vasculitic neuropathy. Two patients presented with numbness in the left foot, extending to the ankle, and later the right foot. They experienced difficulty holding objects and walking. Examination showed 4+ power in the upper limbs with finger clawing, and 4-/5 in the hip and knee, and 0/5 in the ankle. NCS showed bilateral sensorimotor axonal polyneuropathy. Elevated CRP and ESR; ANA IF and anti-CCP were positive. A 65-year-old male patient reported numbness and weakness in his hands and feet over several months. Examination showed 4+ power in the upper limbs and 4-/5 in the hip and knee, with absent ankle jerks. NCS indicated bilateral sensorimotor axonal polyneuropathy. Elevated CRP and ESR; pANCA was positive. A nerve biopsy confirmed vasculitic neuropathy.

Discussion: All five patients underwent extensive blood panels, autoimmune and serological tests, and NCS. Four patients had biopsy-proven vasculitic neuropathy.

Conclusions: Vasculitic neuropathy should be suspected in patients presenting with mononeuritis multiplex over weeks to months. High clinical suspicion is essential for the diagnosis and management to prevent complications and morbidity.

Abstract ID: 712: Acute Hemorrhagic Leucoencephalitis (AHLE) with Active Hepatitis B Virus Replication in the Central Nervous System (CNS): A Case Report

Shivam Mirg, Jasmine Parihar, Manjari Tripathi, Deepti Vibha, Rajesh Singh

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: AHLE is a rare demyelinating disease characterized by an acute, rapidly progressive, fulminant inflammation of the white matter. Although the exact etiology remains elusive, substantial evidence implicates infectious agents and vaccination as potential triggers.

Case Report: A 27-year-old female patient presented with a 20-day history of new-onset daily persistent headache. A week into illness, the patient had an unknown onset bilateral tonic seizure. Past history was significant for immediate postpartum urosepsis with acute kidney injury and anemia requiring indoor management with hemodialysis and blood transfusion around 6 months ago. Examination of CNS was normal.

Discussion: MRI brain had cortical hemorrhagic lesions at multiple locations with the lesions enhancing on contrast. CSF conducted showed normal opening pressure, 10 lymphocytes, slightly elevated protein, and normal sugar, and was sterile, without any atypical cells. The patient tested positive for hepatitis B surface antigen and hepatitis B envelope antigen with high HBV DNA titers. HBV DNA was also positive in the CSF. The patient was negative for hepatitis B surface antigen tested 6 months ago as a part of her antenatal screening. The patient underwent a brain biopsy, which showed areas of hemorrhage and necrosis with infiltrating histiocytes. HBV DNA was positive in the brain biopsy sample. The patient was treated with systemic steroids and tenofovir. She attained both clinical and radiological resolution after 2 weeks of therapy.

Conclusions: Previous literature suggests hepatitis B association with anti-caspr2 myelo-encephalitis and CLIPPERS. This is the first report of detectable HBV DNA in the brain biopsy specimen of a patient with AHLE, suggesting a possible association.

Abstract ID: 713: “Nine Syndrome-Decode the Symptoms and Crack the Diagnosis”

Dr. Shamili, Justin C, Dr. Murugan P DM Post Graduate; Professor, HOD and Professor Department of Neurology, Madurai Medical College

Shamili Devi, Justin C

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: Nine syndrome is a rare clinical entity with core clinical signs of one-and-a-half syndrome, ipsilateral facial palsy, and contralateral hemiparesis/hemianesthesia and localizing to the dorsal paramedian pontine tegmentum. The awareness of this sign helps in precise localization and consideration of relevant etiologies.

Case Report: A 45-year-old male patient, non-diabetic and non-hypertensive with a background history of alcohol and smoking presented with complaints of acute-onset blurring of vision, slurring of speech, drooling of saliva, and deviation of the angle of the mouth to the right, difficulty in closing the left eye, and swaying while walking.

Discussion: On examination, left eye adduction and abduction, both impaired, right eye abduction nystagmus with impaired adduction in the right eye with normal vertical eye movements and normal pupil along with left LMN facial palsy (left lagophthalmos, deviation of the angle of the mouth to the right) and right pronator drift, impaired tandem walking, and right plantar extensor were observed. Imaging showed a left paramedian pontine infarct. The patient was treated with antiplatelets and statins and the patient improved dramatically.

Conclusions: Nine syndrome is a rare clinical entity with core clinical signs of one-and-a-half syndrome, ipsilateral facial palsy, and contralateral hemiparesis/hemianesthesia and localizing to the dorsal paramedian pontine tegmentum. The awareness of this sign helps in precise localization and consideration of relevant etiologies.

Abstract ID: 714: Double Trouble

Naveen Priyadharsan Ashokan, Balasubramanian S, Ravi LA

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Co-occurrence of myasthenia gravis and amyotrophic lateral sclerosis is very rare. We report one such case.

Case Report: A 46-year-old female patient, who had symptoms of drooping of eyelids and double vision 11/2 years ago. She was found to have antibodies against acetyl choline receptor and hence was managed as a case of myasthenia. The patient presented to us as she had no resolution of her symptoms, as well as she developed dysarthria after 6 months. She had difficulty in the formation of bolus and swallowing. On examination, the patient had both UMN and LMN signs, fulfilling the El Escorial criteria. Secondary causes of motor neuron disease were ruled out. The patient’s RNS revealed a significant decremental response. Repeated serum acetylcholine receptor antibody showed borderline positivity. Computed tomography of the chest revealed no significant abnormalities. The patient’s EMG was suggestive of a neuropathic pattern.

Discussion and Conclusions: This case has been reported to emphasize the consideration of coexistence in worsening patients labeled as myasthenia.

Abstract ID: 715: Comparative Efficacy of DOACs vs. VKAs in Stroke Prevention for Atrial Fibrillation: A Network Meta-Analysis

Pradeep Kumar, Manyata Srivastava, Annu Gulia

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Atrial fibrillation (AF) poses an increased risk of stroke, prompting the emergence of direct oral anticoagulants (DOACs) as a treatment option for stroke prevention. We aimed to systematically evaluate the efficacy of different DOACs compared to vitamin K antagonist (VKA) in preventing strokes among patients with AF through a network meta-analysis.

Methodology: A thorough search on PubMed, EMBASE, and Cochrane databases up to March 31, 2024, for published randomized controlled trials (RCTs) comparing the risks of stroke and systemic embolism among different DOACs with VKA was conducted. A pooled risk ratio (RR) with 95% confidence intervals (CIs) was calculated using a frequentist network meta-analysis.

Results: A total of 22 RCTs that compared the efficacy of various DOACs with VKA in preventing strokes among patients diagnosed with AF were included. No significant variations in major bleeding were observed between each DOAC and VKA (RR = 1.05, 95% CI; 0.97 to 1.13). Dabigatran (RR = 0.78, 95% CI; 0.68 to 0.89), apixaban (RR = 0.81, 95% CI; 0.71 to 0.92), and edoxaban (RR = 0.79, 95% CI; 0.69 to 0.90) exhibited significantly lower risks of intracranial hemorrhage compared to VKA (RR = 1.15, 95% CI; 1.03 to 1.29) and rivaroxaban (RR = 1.13, 95% CI; 1.01 to 1.27) showed similar risks. High-dose regimens were associated with reduced risks of stroke or systemic embolism compared to VKA and low-dose regimens, with both dosage levels presenting similar bleeding risks.

Conclusions: Our findings underscore the potential benefits of DOACs over traditional VKA therapy in the management of AF, particularly in reducing the risk of intracranial hemorrhage. However, individual patient factors and preferences should be considered when selecting the most appropriate anticoagulant therapy.

Abstract ID: 716: Pernicious Anemia Presenting as Cerebral Venous Sinus Thrombosis and Coronary Artery Disease in a Young Man

Punith Bylappa, Ayush Agarwal, Divyani Garg, Achal Kumar Srivastava, Jasmine Parihar, Manjari Tripathi

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: We describe a young patient presenting with cerebral venous sinus thrombosis and coronary artery disease, which paved the way to diagnose hyperhomocysteinemia and pernicious anemia.

Methodology: A 30-year-old male patient with a history of coronary artery disease (LAD-80% occlusion) in the absence of risk factors 4 years ago on irregular medications presented with a history of intermittent weakness of the right upper and lower limbs. He developed a severe headache 3 days later associated with multiple episodes of vomiting and right-sided focal motor seizures. On examination, he was confused with right hemiplegia.

Results: Full blood count was suggestive of moderate anemia with a dimorphic picture, liver and kidney function tests were normal, and viral markers for HIV, hepatitis B and C were negative. MRI brain was suggestive of superior sagittal sinus thrombosis with cortical venous infarcts along the left high frontal lobe. The serum anti-nuclear antibody panel and anti-phospholipid antibody panel were negative. Thrombophilia panel consisting of Factor V Leiden, prothrombin 20210A mutation, and MTHFR mutation were also negative. He had severe hyperhomocysteinemia with significantly low serum vitamin B12. Urine methyl malonic acid levels were elevated. Serum anti-parietal cell antibody was positive and upper GI scopy was suggestive of chronic atrophic gastritis. Lower GI scopy was normal. He was treated with therapeutic anticoagulation and parenteral vitamin B12 supplementation.

Conclusions: Hyperhomocysteinemia secondary to vitamin B12 deficiency as a consequence of pernicious anemia is an important cause of arterial and venous thrombosis.

Abstract ID: 717: Dengue-associated Myelin Oligodendrocyte Glycoprotein (MOG) Antibody-Positive Longitudinally Extensive Transverse Myelitis: A Case Report

Pradeep Lalwani, Shubhakaran Khichar, Amita Bhargava, Nidhi Yadav

DR. S N Medical college, Jodhpur, Rajasthan, India

Background and Aim: Central nervous system complications of dengue include encephalopathy, meningoencephalitis, and intracranial hemorrhage. However, the involvement of the spinal cord is uncommon. Here, we describe a patient who presented with longitudinally extensive transverse myelitis (LETM) with positive anti-myelin oligodendrocyte glycoprotein (MOG) antibody following dengue infection.

Methodology: A 40-year-old male patient presented with acute transverse myelitis with a sensory level at T4. He had a history of fever with myalgia for 10 days and was found to have thrombocytopenia, deranged liver function test, and positive dengue NS 1 serology. CT chest abdomen showed serositis with hepatosplenomegaly and edematous gall bladder wall, suggestive of reactionary changes. MRI brain with spine showed T2W hyperintense bilateral thalami, central pons, and central intramedullary signals between C4 to C7 and D3 to D11 levels. Cerebrospinal fluid showed leukocytosis (76 /dL) with elevated proteins (92 mg/dL). The serum autoimmune antibody was positive for MOG.

Results: Diagnosis of para infectious myelitis associated with dengue virus was kept. He was managed with intravenous methylprednisolone therapy of 1000 mg daily for 5 days, followed by oral steroids. As he had positive anti-MOG antibodies, an immunomodulator, mycophenolate mofetil, 1 g per day, was started. His muscle strength in the lower limbs improved from an MRC grade of 0/5 to 4/5 bilaterally by the day of discharge and was able to ambulate.

Conclusions: Acute transverse myelitis secondary to dengue infection associated with anti-MOG antibody is a rarely reported consequence. Timely diagnosis and treatment can cause a significant reduction in the otherwise resultant morbidity.

Abstract ID: 718: Higher-Order Cognitive Tests Detect Off-The-Radar Cases: Experience from COVID-19

Atanu Biswas, Madhushree Chakraborty, Goutam Das, Piali Chatterjee, Adreesh Mukherjee, Mrinal Kanti Ghosh, Ayanendra Nath Basu, Sankar Pal

Institute of Post Graduate Medical Education and Research, Kolkata (IPGMER Kolkata), West Bengal, India

Background and Aim: Cognitive dysfunction and mental health problems have been frequently reported in research investigating the long-term effects of COVID-19. However, standardized cognitive tests are often not sensitive to subtle cognitive changes, creating a hurdle for early detection of cognitive decline. In this work, we paired standardized tests with higher-order cognitive tests focusing on pragmatic processing.

Methodology: A total of 376 COVID-19 patients were interviewed via telephone. In-person interviews with 99 COVID-19 patients and 31 matched controls were conducted as part of a more thorough examination. Participants were administered regular standardized tests and higher-order cognitive tests. Voxel-based morphometry and vertex analyses were conducted using the FSL software.

Results: In all, 64.89%, 44.95%, and 41.49% of COVID-19 patients reported a deterioration in physical functioning, mental health, and cognitive abilities, respectively. They had an increased risk of depression, anxiety, stress, and insomnia. COVID-19 patients who had depression and anxiety were 8.6 and 19.4 times more likely to have cognitive decline, respectively. Hospitalized COVID-19 patients had reduced gray matter volumes in the frontal mid-orbital R ( P =0.015), frontal superior orbital R ( P = 0.018), rectus R ( P = 0.015), rectus L ( P = 0.025), and right amygdala ( P = 0.047). Most patients who had difficulty in figurative language processing ( P < 0.05) despite scoring above the cut-off on the standardized cognitive tests, reported a considerable decline in their cognitive abilities after a gap of 1 year.

Conclusions: Higher-order cognitive tests might be a marker of early cognitive decline. Incorporating these tests into the dementia assessment protocol might help identify patients who may go unnoticed if we depend too much on standard screening measures.

Abstract ID: 719: Study on Clinical and Radiological Profiles of Cerebral Venous Thrombosis

V. V. N. S. RAVI SANKAR V

Andhra Medical College Visakhapatnam, Andhra Pradesh, India

Background and Aim: Cerebral venous thrombosis (CVT) refers to the thrombosis of dural venous sinuses or the cerebral veins. CVT accounts for 0.5%–1% of all strokes. Epidemiologically, CVT has an annual incidence of 1.16 to 2.02 cases per 100,000. AIMS: To study the clinical and radiological profiles of the application of the cerebral venous sinus thrombosis (CVST) grading scale, CVST score for treatment decisions and prognostication.

Methodology: Sample size: 51, study period: 18 months, study population: all patients >18 years of age with clinical features of CVST subjected to radiology. Statistical analysis: SPSS version 20.

Results: The results indicated that the majority of patients were in the age group of 21 to 40 years, with a higher prevalence in males than females. Chief complaints were headache (100%), convulsions (56%), followed by focal signs (50%), and altered mental sensorium (44%). Subacute onset was observed in 38 cases. The predominant risk factors identified were alcoholism, followed by hyperhomocysteinemia and dehydration. CT, MRI/MRV findings revealed hemorrhagic infarcts in 40%, with a predilection for the parietal (24%) and frontal zones (20%), and 98% involvement of superficial sinuses. Most patients, that is, 94% were treated medically, whereas three patients underwent surgical intervention via decompressive craniotomy.

Conclusions: This study highlights the importance of early detection and appropriate treatment approaches in managing CVST. The findings emphasize the need for targeted preventive measures and rapid therapeutic interventions to improve patient outcomes.

Abstract ID: 720: To Assess Cerebrovascular Blood Flow Dynamics and Reactivity Changes in Primary Headache by Transcranial Doppler

Jai Ganesh K, Balaji G, Hariharan S, Thamilpavai N

Chengalpattu Government Medical College, Tamil Nadu, India

Background and Aim: The aim of the study was to determine whether primary headaches such as migraine with or without aura and trigeminal autonomic cephalgia (TAC) have altered anterior and posterior circulation cerebrovascular hemodynamics and reactivity changes compared with the control group as assessed by transcranial Doppler (TCD) during an interictal period.

Methodology: A total of 25 patients (17 females, 8 males) with a mean age of 34 ± 10 were evaluated during an interictal period: migraine without aura 12 (48%), migraine with aura 10 (40%), and TAC 3 (12%). They were compared with 25 age- and sex-matched controls. Each participant was examined with a 2 MHz probe power mode B TCD of the middle, anterior, and posterior cerebral and vertebral arteries and basilar artery during the interictal period. The indices measured were mean flow velocity, peak systolic velocity, Pulsality Index, and Breath Holding Index.

Results: A significant difference was present between bilateral anterior, middle, and posterior cerebral and vertebral arteries, basilar artery mean flow velocities and peak systolic velocity, Pulsality Index between migraine with or without aura and in patients with a trigeminal autonomic cephalgia versus normal controls ( P < 0.05). There was also a significant increase in vertebral, basilar artery cerebral blood flow velocities relative to controls and migraine with or without aura versus in patients with trigeminal autonomic cephalgia ( P < 0.05).

Conclusions: The possibility of cerebrovascular changes is confirmed in the present study in patients with primary headaches versus healthy controls during the interictal period. The hypothesis of pathophysiology of primary headache with vascular hemodynamics changes was correlated with the non-invasive transcranial Doppler with this study.

Abstract ID: 721: Medial Calcaneal Nerve Conduction Study in Heel Pain

Ishwarya Thiruvuru, P. Philo Hazeena, Deepa Avadhani, Rithvik Ramesh, S. Sundar, R. Lakshmi Narasimhan

Sri Ramachandra Medical College and Research Institute, Chennai (SRMCRI, Chennai), Tamil Nadu, India

Background and Aim: Plantar heel pain can be due to various neurological and non-neurological causes. Of the neglected causes, medial calcaneal neuropathy (MCN) has been implicated as one of the causes of chronic plantar pain. This study aims to compare the sensory conduction of MCN between healthy individuals and patients with heel pain.

Methodology: It was a cross-sectional study that included 75 patients and 75 volunteers who came to the neurology department. Study period-January to June 2023. Ethics approval was obtained. Sensory nerve conduction of MCN was obtained with equipment NIHON KHODEN NEUROPACK in the Electrophysiology Laboratory of Neurology, SRIHER.

Results: Among 75 patients with heel pain, 30 had normal MCN study and 45 had abnormal MCN study with a mean latency, amplitude, and conduction velocity of 1.95 ± 0.46, 16.43 ± 8.85, and 53 ± 11.44 m/s compared to controls latency, amplitude, and conduction velocity, which were 1.58 ± 0.14, 18.55 ± 6.41, and 62.95 ± 4.71 m/s, respectively. The P -values for latency, amplitude, and conduction velocity were 0.0001, 0.0950, and 0.0001, respectively. Among 75 patients, 37 were diabetic with mean latency, amplitude, and conduction velocity of 2.13 ± 0.552, 14.67 ± 6.67, and 49.12 ± 12.05 m/s compared to 38 non-diabetics with mean latency, amplitude, and conduction velocity of 1.78 ± 0.32, 18.07 ± 5.33, and 56.53 ± 9.63 m/s, respectively. P -values for latency, amplitude, and conduction velocity were 0.0007, 0.0170, and 0.0043, respectively. This shows prolongation of latencies, reduction in amplitude, and delayed conduction velocities in diabetics when compared to non-diabetic patients with heel pain.

Conclusions: MCN was significantly affected in heel pain. MCN showed the prolongation of latencies, reduction in amplitude, and delayed conduction velocities in diabetic patients with heel pain.

Abstract ID: 722: Cerebral Amyloid Angiopathy: A Case Series

Manoj Prakash Jeyaseelan, N. Shobana, M. Sacratis, C. J. Selvakumar, V. Sadeesh Kumar

Coimbatore Medical College Hospital, Coimbatore, Tamil Nadu, India

Background and Aim: Cerebral amyloid angiopathy is a type of cerebrovascular disorder characterized by the accumulation of amyloid beta-peptide within the leptomeninges and small/medium-sized cerebral blood vessels. This may manifest as lobar intracerebral hemorrhages (ICH), cognitive impairments, incidental microbleeds, hemosiderosis, and inflammatory leukoencephalopathy. Aim: To study the clinical profile and outcome of the patients diagnosed with cerebral amyloid angiopathy.

Methodology: This was a prospective observational study. Five cases of ICH who satisfied the MODIFIED Boston criteria for the diagnosis of cerebral amyloid angiopathy who attended the Neurology OPD at Coimbatore Medical College Hospital between May 2023 and April 2024 were studied. MRI brain, CT cerebral angiography, complete hemogram, ESR, CRP, LFT, RFT, and ANA profile tests were conducted.

Results: Out of the five patients, the majority were males ( n = 4) and one was female. All of them were above 55 years of age. The mean age was 63.5 years. Four had presented with transient motor weakness, whereas one had presented as a case of dementia. MRI brain revealed at least one lobar ICH in four patients and cerebral microbleeds in three patients. Out of the five patients, four were probable cases, whereas one was a possible case of cerebral amyloid angiopathy as per the modified Boston criteria. Four patients had favorable outcomes at 3-month follow-up, whereas one died. The patient who died had poor GCS (<7) at initial presentation and recurrence of ICH.

Conclusions: Initial Glasgow Coma Scale score and recurrence of ICH could be a marker for mortality in cerebral amyloid angiopathy.

Abstract ID: 723: Cerebral Nocardiosis: An Elusive Foe in the Shadows

Archita Makharia, Anuj Ajay, Chaithra Rajanna, Abhishek Vaingnakar, Aneesh Joshi, Kanwaljeet Garg, Ayush Agarwal, Divyani Garg, Awadh Kishor Pandit, Gagandeep Singh, Manish Soneja, Achal Kumar Srivastava, Divya M. Radhakrishnan

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Cerebral nocardiosis is a rare entity characterized by brain abscesses with non-specific symptoms that often elude timely diagnosis, leading to a high mortality rate.

Case Report: We report the case of a 69-year-old non-diabetic male patient presenting with persistent headache and right eyelid pain, swelling, and proptosis over 4 years, without visual disturbances or a history of an immunocompromised state. Initial fine-needle aspiration cytology suggested nodular fasciitis; however, as symptoms worsened a repeat biopsy was conducted that suggested pseudo papillomatosis hyperplasia of the epidermis. Subsequent magnetic resonance imaging of the brain revealed an inflammatory lesion encompassing the bilateral orbital apex and surrounding sinuses, raising the possibility of IgG4 disease. However, as IgG4 was within normal limits, he did not receive steroids as the diagnosis was doubtful. A year later, another biopsy of the right orbit showed brownish-black refractile material, suggesting a fungal pathology. Despite the administration of voriconazole, the patient’s condition deteriorated. He returned 8 months later with reduced verbal output, altered sensorium, and pus discharge from eyes and was found to have extensive subdural empyema with a right temporal abscess. He underwent decompressive craniectomy and expansion duroplasty, wherein intraoperative samples confirmed nocardiosis. He subsequently developed hydrocephalus, for which an external ventricular drain was inserted. Despite management with trimethoprim-sulfamethoxazole (TMP-SMX), imipenem, and supportive care, the patient succumbed to his illness.

Discussion and Conclusions: Nocardiosis, although rare, should be considered in progressive rhinosinusitis unresponsive to treatment. Differentiating nocardiosis from other pathologies is critical, as each requires distinct management. Employing meticulous microbiological techniques, including acid-fast staining, is crucial for timely and accurate diagnosis, given the high mortality associated with cerebral nocardiosis.

Abstract ID: 724: Rapidly Progressive Dementias: Case Series in a Tertiary Health Center

Vinay Kumar Choppa

Gandhi Hospital Musheerabad, Secunderabad, Telangana, India

Background and Aim: Rapidly progressive dementia (RPD) is an emergency in cognitive neurology, defined as cognitive impairment affecting the daily living activities developed over less than 1 year. This study investigated the profile of patients with rapidly progressive dementia at first presentation.

Methodology: Retrospective case analysis was conducted of 30 patients with rapidly progressive dementia who presented to a tertiary health center from January 20023 to December 2023. Demographic data, time of evolution, radiological, and laboratory findings were collected and analyzed.

Results: Infections presenting with RPD accounted for the most common cause in our series: 8 (26.6%) with progressive multifocal leukoencephalopathy (50%) as the leading cause, followed by SSPE (37.5%) and neurosyphilis (12.5%). Immune-mediated dementias formed the second most common (20%) etiologic cause for RPD. Dementias associated with nutritional and metabolic causes such as Wernicke’s and hypothyroid were the third common cause for RPD (16.6%) in our series, which was followed by neurodegenerative disorders (10%). Prion-associated rapidly progressive dementia was observed in two (6.6%) patients. The most commonly associated additional symptoms were seizures (40%), followed by psychiatry manifestations (33%). Radiology abnormality (MRI + PET) was observed in 26 (86%) patients, which helped with the diagnosis. The mean age of presentation was 45.6667 ± 11. 476 years. There was equal sex predilection with 15 males and 15 females.

Conclusions: Rapidly progressive dementia is an emergency in cognitive neurology. We feel that many patients have treatable or reversible causes. Infectious causes, autoimmune encephalitis/immune-mediated dementias, and nutritional causes are the common causes of RPD in this study. A systematic evaluation can unravel treatable conditions that were doomed as degenerative entities in the past.

Abstract ID: 725: Master Mimicker - A Diagnostic Challenge in a Rheumatoid Arthritis Patient with Quadriparesis

Manoj Kumar N S, Velusamy S, Aravind Gowtham V, Nithyanandam A, Balasubramanian S

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Rheumatoid arthritis is a systemic inflammatory disease characterized by synovitis and the destruction of articular structures in multiple joints. The risk of developing malignant lymphoma is higher in patients with rheumatoid arthritis–2 to 3-fold increased risk. The aim of the case report is to discuss the rare presentation and diagnostic challenges it poses.

Case Report: A 42-year-old female patient with a history of rheumatoid arthritis, initially treated with methotrexate, hydroxychloroquine, and steroids, discontinued treatment for 2 years, opting for native remedies. On April 1, 2024, she presented with giddiness and right hemiparesis, initially diagnosed with cerebrovascular accident. However, subsequent MRI revealed T2/FLAIR hyperintensity in multiple brain regions, indicative of acute brainstem demyelination. Treatment with IV methylprednisolone and IVIg led to symptomatic improvement. By May 7, the patient developed left eyelid drooping, double vision, and nasal regurgitation, prompting another course of IV methylprednisolone. However, her condition worsened, manifesting as quadriparesis, bilateral ptosis, ophthalmoplegia, and cranial nerve palsies. CSF analysis showed elevated protein and decreased glucose levels, whereas imaging indicated bilateral corticospinal tract involvement, raising suspicion of an infiltrative disorder such as lymphoma. Plasma exchange was initiated, and a PET CT scan suggested metabolic activity corresponding to brain lesions observed on MRI. However, due to the family’s reluctance, brain biopsy was deferred.

Discussion: The patient’s aggressive clinical course, characterized by neurological deficits and imaging findings consistent with infiltrative disorder, underscores the challenge in diagnosing the setting of rheumatoid arthritis and managing such cases, particularly when invasive procedures are required for definitive diagnosis.

Conclusions: The case report is presented for its rarity.

Abstract ID: 726: Clinical Spectrum, Therapeutics, and Recurrence of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) in a Tertiary Care Center in Western India

Noel Gomes, Rahul Tyrambak Chakor, Swaleha Nadaf

Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai (TNMC, Mumbai), Maharashtra, India

Background and Aim: MOGAD is an inflammatory disorder of the central nervous system. First described in the 1970s, its clinical presentation includes optic neuritis, myelitis, acute demyelinating encephalomyelitis, and focal encephalitis. Though widely regarded as monophasic illness, recurrence in up to 50% of patients has been reported. Through this study, we aim to explore clinical presentation, recurrence, and our experience with the management of these patients.

Methodology: A retrospective study was conducted at a tertiary care hospital in western India. We examined the characteristics of 32 diagnosed MOGAD cases. Data were collected on patient demographics, clinical and imaging characteristics, acute and chronic treatment, and recurrence.

Results: Our MOGAD cases revealed a female (59%) to male predominance (41%). The mean age of presentation was 28 years (6–62 years). The most common clinical and radiologic presentation was optic neuritis (13, 40%), followed by myelitis (11,35%), ADEM (5,15%), and myelitis + optic neuritis (3,10%). One patient had conus-cauda syndrome, whereas one patient had predominantly temporo-occipital lesions causing higher-order vision loss. For acute treatment, IV methylprednisolone was used in the majority of cases (75%), followed by plasmapharesis (25%). Also, 90% of our patients were treated with chronic therapy post-discharge, out of which 46% were treated with steroids only, 44% were administered a steroid-sparing agent (azathiorpine [79%], rituximab [14%], MMF [7%]). The second immunosuppressant was initiated if patients had recurrence or severe disease during admission. Our cohort had a recurrence rate of 19%.

Conclusions: In this study, we found that MOGAD can have a heterogenous presentation and low recurrence on chronic immunosuppression.

Abstract ID: 727: Fantastic Four: The Medullary Stroke Saga Four Varied Presentation of Medullary Stroke Syndrome

Vignesh Kumar, Rajagambheeran Venugopal, Jawahar M

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Stroke, an acute neurological syndrome of cerebral vascular origin lasting over 24 h, can stem from either ischemia or hemorrhage. We present four varied presentations of medullary stroke syndromes.

Case Report: Patient 1: B/L medial-medullary syndrome patient was a 67/M, on treatment for diabetes and hypertension. The patient had sudden onset giddiness and nausea. Four hours later, he developed weakness in all four limbs with difficulty in swallowing both solids and liquids. He was found to have gaze-evoked nystagmus. Hypoglossal diplegia, decreased gag reflex, and power of 1/5 in all four limbs were noted. Plantar was extensor bilaterally. MRI brain showed bilateral medial medullary stroke, a typical “heart appearance sign.” The patient died after a week due to sepsis secondary to aspiration pneumonitis. Patient 2: Opalski syndrome patient was a 75/M, with background hypertension, COPD, chronic smoking, and alcoholism. He presented with acute-onset right hemiparesis, right UMN facial lag, right palate weakness, right hemifacial numbness, and ipsilateral ataxia. There was no contralateral hemi-sensory loss, and no Horner’s syndrome. MRI was suggestive of a lateral medullary syndrome.

Discussion and Conclusions: This case series elucidates varied presentations of medullary stroke syndromes.

Abstract ID: 728: Posterior Cortical Atrophy – A Descriptive Study from North India

Suman S. Khushwaha, Ashwin Panda, Prateek Babel, Rajinder Dhamija

Institute of Human Behaviour and Allied Sciences, New Delhi, India

Background and Aim: Posterior cortical atrophy is usually considered to be an atypical form of Alzheimer’s disease (although cases of PCA can be associated with DLB, CBD, and rarely prion disease) and has a relatively young age at onset, usually between 50 and 65 years of age and is usually sporadic. Aim: To study the clinical profile of posterior cortical atrophy.

Case Report: We studied eight patients of probable posterior cortical atrophy presenting to our neuro-behavioral clinic. The diagnosis of PCA was made on grounds of history, and higher mental function examination. Neuroimaging included MRI brain, FDG PET-CT brain. All patients fulfilled the consensus criteria of PCA.

Discussion: 1. Out of eight patients based on level 2 categorization, six patients were probable PCA variants of early-onset Alzheimer’s disease and two were probable PCA associated with CBS based on clinical findings and neuroimaging. 2. Visual disorientation reflecting a combination of simultanagnosia, optic ataxia, and apperceptive agnosia were common presenting features. Two patients presented with asymmetric motor features of progressive limb rigidity and myoclonus, and bradykinesia along with these features was diagnosed as probable PCA-CBS (level 2 classification). 3. FDG PETCT brain in these patients revealed hypometabolism within the parietooccipital cortices in all patients.

Conclusions: PCA is an uncommon disorder and needs clinical phenotyping for further management and rehabilitation.

Abstract ID: 729: Two Old Sisters: A Case of Ovarioleukodystrophy in Two Sisters with Novel EIF2B4 Gene Mutation

Vignesh Kumar, Rajagambheeran Venugopal, Jawahar M

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Leukoencephalopathy with vanishing white matter (LVWM) is a disease inherited in an autosomal recessive manner. Ovarioleukodystrophy refers to LVWM in females who exhibit signs or symptoms of progressive ovarian failure. We describe LVWM with secondary amenorrhea and infertility in two sisters.

Case Report: Patient 1, a 25/F presented with a 5-year history of difficulty in walking and stiffness of all four limbs. She also had secondary amenorrhea for the last 3 years. Patient 2, 29/F also had similar complaints from the same age.

Discussion: On examination, both patients had normal cognitive function. Both had spasticity in all four limbs. Power was 4/5 in all four limbs. All deep tendon reflexes were grossly exaggerated. Ankle clonus was present bilaterally. Babinski’s reflex was positive bilaterally. There was mild difficulty in the finger nose test and finger finger test. Spastic gait was noted. The sensory, autonomic, and extrapyramidal examination was normal. Routine blood investigations, TSH was normal. FSH and LH levels were increased and estrogen and progesterone were decreased. USG abdomen showed an atrophic uterus and small ovaries. MRI brain showed diffuse T2/FLAIR hyperintensity involving bilateral subcortical, central, and deep white matter along the frontal and occipital horn of lateral ventricles. Genetic analysis in patient 1 showed homozygous missense mutation in exon 13 of the EIF2B4 gene on chromosome 2:g.27364531A>G, with substitution of proline for serine at codon 481.

Conclusions: The possibility of VWMD needs to be considered in adult patients who present with features of leukodystrophy and associated premature ovarian failure.

Abstract ID: 730: Cortical Clawing - A Case Report

Tamil Kumaran G, Vignesh A, Marian Jude Vijay A, Jawahar M, Balasubramanian S

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Small cerebral cortical infarction can present with isolated hand weakness mimicking peripheral nerve palsy. Here, we report a 42-year-old female patient, a case of stroke who presented with a sfudden onset of isolated claw hand deformity mimicking ulnar nerve palsy.

Case Report: A 42-year-old female patient, known diabetic on irregular treatment, presented with acute onset weakness of the left hand in the form of difficulty in holding objects and difficulty in using the left ring and little finger since that night. There was no weakness in the left proximal arm, left leg, and face. She did not have slurring of speech or any other sensory disturbances. On examination, she had isolated clawing of the left little and ring fingers without any sensory loss and with positive finger flexor and Hoffman reflexes. Nerve conduction studies were normal. MRI brain showed an acute cortical infarct in the right precentral hand knob area. She was treated with anti-platelets, statins, and insulin. MR angiogram, carotid Doppler, and echocardiogram were normal. She recovered completely on follow-up.

Discussion and Conclusions: Cortical infarcts of the hand knob region should be considered in patients presenting with sudden-onset clawing without any sensory deficits. It is also important to identify these small cortical infarcts for further secondary prevention.

Abstract ID: 731: Clinical Spectrum of Dystonia in Parkinsonism

Piyali Bhattacharya, Atanu Biswas

Institute of Post Graduate Medical Education and Research, Kolkata (IPGMER Kolkata), West Bengal, India

Background and Aim: Background: It has been observed that dystonia is often associated with Parkinsonian disorders, both idiopathic and atypical. In this study, we evaluated the presence and type of dystonia in various Parkinsonian disorders and their relationship with levodopa intake Objective: 1. To determine the prevalence of dystonia in different Parkinsonian disorders. 2. To determine the pattern of dystonia in each disorder.

Methodology: We collected data from patients presenting with Parkinsonism in outdoors and indoors of our institution with the help of history, clinical examination, and investigations Tools: 1. Blood investigations 2. Neuroimaging. Inclusion criteria: 1. All patients having idiopathic and atypical Parkinsonism, irrespective of treatment history with exclusion of secondary Parkinsonism and primary dystonia.

Results: In all, 91 patients were included in the study, of which 66 were males and 25 were females. The majority were in the age group of 40 to 69 years. One had progressive supranuclear palsy, one had corticobasal syndrome, and one had idiopathic Parkinsonism. Most were harboring the disease for 1–10 years. Most were taking drugs for 1–5 years. The age of onset was mostly 41–60 years. The prevalence of dystonia was observed more in females and in the age group of 70–79 years. Dystonia prevalence increased with duration of illness and duration of drug intake. Dystonia was slightly more in patients with a younger age of onset. Maximum dystonia prevalence was observed in patients taking levodopa equivalent of drug between 201 and 400. Most patients had neck dystonia followed by upper limb dystonia.

Conclusions: Of the 91 patients, most patients had IPD, most having age of onset 41-60 years. Dystonia was more in females, in the elderly, increasing with the duration of illness and duration of levodopa intake, more in patients with younger age of onset. Most had neck dystonia.

Abstract ID: 732: Anti-Ri Antibody Paraneoplastic Syndrome Presenting as STIFF Person Syndrome: A Case Report

Babitha Sangaraju, Nanda Kumar V, Raja Gambeeran V, Jawahar M, Balasubramanian S

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Paraneoplastic neurological disorders associated with anti-Ri antibodies usually present as opsoclonus–myoclonus-ataxia syndrome. Underlying malignancies associated with anti-Ri Ab are breast cancer, gynecological tumors, and small-cell lung cancer. Herein, we present a case of anti-Ri Ab syndrome presenting as stiff person syndrome, which is rare.

Case Report: A 57-year-old female patient with a history of right breast intraductal carcinoma, s/p modified radical mastectomy, and eight cycles of chemotherapy 2 years ago presented to us with stiffness of all four limbs and trunk finding it difficult to walk, turn around in the bed, to feed herself, and startle response to auditory stimuli, painful spasms in the limbs, neck, mild dysphagia of 4 months duration. On examination, she had ophthalmoplegia in the form of b/l horizontal gaze palsy, upgaze restriction, rigidity involving all four limbs, neck, and axial rigidity with grade 4 tetraparesis, with brisk tendon reflexes with extensor plantar bilateral. She also had startle reflex to auditory stimuli, with involuntary movements in the form of myoclonic tremor in all four limbs with positive head retraction reflex.

Discussion: On investigations, MRI brain, and MRI spine were normal except for spondylotic degenerative changes throughout the spine. CT chest and abdomen were normal. Her serum paraneoplastic profile was strongly positive for anti-Ri Ab. EMG showed co-contraction of agonist and antagonist muscles, supporting evidence for Stiff person syndrome. No evidence of other autoimmune diseases was noted.

Conclusions: Stiff person syndrome spectrum disorder is associated commonly with anti-GAD, anti-amphiphysin antibodies. Anti-Ri Ab syndrome presents usually as brainstem/cerebellar syndrome. To our knowledge, this is the first case of an anti-Ri positive patient presenting as a Stiff person spectrum disorder without opsoclonus–myoclonus-ataxia.

Abstract ID: 733: SRP-Positive Necrotizing Myopathy: Takes More than Just the Muscles

Rajesh Singh

Sir Ganga Ram Hospital New Delhi, India

Background and Aim: Immune-mediated necrotizing myopathy (IMNM) is a type of inflammatory myopathy characterized by acute or subacute severe proximal muscle weakness, oropharyngeal dysphagia, significantly elevated creatine kinase levels, and prominent myofiber necrosis and regeneration with little or no inflammation. We present a case of anti-SRP-associated IMNM presenting with quadriparesis, oropharyngeal dysphagia, cardiopulmonary dysfunction, and high CPK.

Case Report: A 65-year-old female patient presented with rapidly progressive quadriparesis, oropharyngeal dysphagia, cardiopulmonary dysfunction, and high CPK. Early diagnosis and treatment of immune-mediated necrotizing myopathy (IMNM) can lead to significant clinical improvement; however, partially or untreated IMNM can lead to delayed recovery or permanent disability because muscle tissue is rapidly replaced by fatty tissue.

Discussion: The patient was diagnosed with anti-SRP-positive myopathy. Myositis panel was positive for anti-SRP antibody, CPK was > 10,000, whole-body PET showed features of myositis, muscle biopsy from vastus showed foci of active myopathic damage with ongoing prominent regeneration and myofiber necrosis consistent with anti-SRP antibody-mediated necrotizing myopathy.

Conclusions: Necrotizing myopathy is a rare but fatal etiology requiring early diagnosis and treatment is important to prevent severe morbidity and mortality.

Abstract ID: 734: Spectrum of HIV Associated Encephalopathy: A Prospective Observational Study in a Tertiary Care Hospital

Priyanka Pasala, Shobana N, Sacratis M, Sadeesh Kumar V, Selva kumar CJ

Coimbatore Medical College and Government Hospital, Tamil Nadu, India

Background and Aim: HIV-associated encephalopathy is due to encephalopathy either directly caused by HIV perse (or) due to opportunistic infection (or) IRIS. To study the etiology, clinical profile, and management of HIV-associated encephalopathy in our hospital.

Methodology: It was a single-center, prospective observational study. A total of 25 HIV-associated encephalopathy patients admitted to our hospital from 2023 to 2024 were included in this study. Of these, 17 were known patients on ART, and 8 were newly diagnosed patients. Complete hemogram, RFT, LFT, chest X-ray, CT chest, CD4 count, MRI brain, and CSF analysis were conducted.

Results: Of the 25 patients, 15 were males and 10 were females. The main clinical features were headache, seizures, altered sensorium, and focal neurological deficit. The median CD4 count was 167 cells/mm 3 . Nineteen patients had CD4 count < 150 and six patients had CD4 count > 150. MRI brain showed diffuse (or) focal lesions or meningitis-like picture in 23 patients and normal in 4 patients. CSF analysis showed elevated ADA in nine patients, positive India ink stain in four patients, and serum toxoplasma IGg positive in three patients. The etiology of HIV-associated encephalopathy was nine CNS tuberculoma, four cryptococcal meningitis, three primary CNS lymphomas, three cerebral toxoplasmosis, and one PML. The median CD4 count for these cases was 149 cells/mm 3 . Five cases of AIDS-dementia complex were observed. The median CD4 count for these cases was 322 cells/mm 3 .

Conclusions: Early identification of the etiology of HIV-associated encephalopathy is important as appropriate anti-microbial treatment can be started along with ART.

Abstract ID: 735: To Study Predictive Value of the Systemic Immune Inflammation Index for Adverse Outcomes in Patients With Acute Stroke

Sidharth Sharma, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Abdul Qavi

Background and Aim: Post-stroke inflammation is an important factor that can exacerbate brain injury leading to worse outcomes, making it an important prognostic factor. Indices correlating with systemic inflammation can be used for accurate prognostication and possible interventions in the future. Systemic Immune Inflammation Index (SII) is calculated using platelet, neutrophil, and lymphocyte counts. Therefore, the purpose of this study was to evaluate the role of SII in predicting short-term outcomes in patients with acute stroke.

Methodology: It was a prospective study of 103 patients, which will be over by July 2024. Patients were assessed clinically through NIHSS, and SII was calculated and then followed up after 3 months using the MRS score to evaluate the short-term outcomes of patients. This data will be analyzed statistically after the completion of the study period at the end of July 2024.

Results: With this study, we want to evaluate the role of SII in predicting short-term outcomes in patients and find any correlation of SII with NIHSS. This study follow-up period will be over by July 2024, following which the results will be analyzed.

Conclusions: This study looked at the link between SII and clinical outcomes in patients with stroke. The inflammatory response after a stroke is useful for immunoregulatory treatment. As a result, research into SII and the clinical outcomes of patients with stroke is crucial. Nonetheless, further research is needed to better understand the utility of SII.

Abstract ID: 736: Prognostic Role of Hypertriglyceridemia in Patients with Stroke of Atherothrombotic Origin

Ajaya Biswal, S. Saravanan, Birla Pavalam, Sankar Narayanan

Tirunelveli Medical College Hospital, Tamil Nadu, India

Background and Aim: The potential role of hypertriglyceridemia as an independent risk factor for ischemic stroke remains controversial. However, accumulating evidence has shown that hypertriglyceridemia is associated with pathophysiological processes such as endothelial dysfunction, atherosclerosis, and the production of a prothrombotic state, which could contribute to ischemic stroke. Our aim is to assess early in hospital recovery patterns of patients of acute ischemic stroke in relation to triglyceride level.

Methodology: This cross-sectional analytical study was conducted in 110 cases of acute ischemic stroke on patients in the age group 18 to 80 years between August 2023 and March 2024. The severity of stroke was assessed by the NIHSS score, GCS, and clinical neurological examination at the time of admission. Baseline information was collected. All risk factors for ischemic stroke such as hypertension, DM, chronic alcohol intake, and smoking were recorded. Fasting triglyceride (TG) level was conducted on the next day. Hypertriglyceridemia was defined as having a TG level >150 mg/dL. Patients were classified according to TG levels as 1) <150 mg/dL, 2) 150–250 mg/dL, 3) 250–350 mg/dL, and 4) >350 mg/dL. Patients’ neurofunctional status was assessed at the time of discharge using the MRS scale and their relationship with different ranges of TG level was studied by regression analysis.

Results: In all, 110 patients were included with a mean age of 71 years in this study. Also, 69% of patients had hypertriglyceridemia. In the bivariate analysis, an unfavorable outcome was more frequent in older patients, with higher admission NIHSS score, and serum triglyceride levels of more than 250 mg/dL. Unfavorable outcome at discharge was independently associated with NIHSS score ( P < 0.05), age ( P < 0.05), and high serum triglyceride levels ( P < 0.05).

Conclusions: Hypertriglyceridemia is an important modifiable risk factor that negatively affects the recovery in patients with acute ischemic stroke.

Abstract ID: 737: Rural Aspects of Epilepsy: An Experience of Treating People with Epilepsy for 30 Years from a Single Center

Himanshu kaushal, Rajendra kumar Sureka

Mahatma Gandhi Medical College and Hospital, Jaipur, India

Background and Aim: The prevalence rate of epilepsy ranges from 0.48 to 4.96 with a median prevalence of 1.54 as derived from studies conducted in rural suburbs of India. The rural population constitutes about 74% of the total Indian population.

Methodology: Single-center study at the Community Health Centre, Ratan Nagar, Churu District of Rajasthan, India (1994–2024). A total of 8,534 people with epilepsy (PWE) were studied over this period. During this 30-year timeframe, various aspects of the rural population with epilepsy were studied and these observations included 1) Knowledge, Attitude and Practices (KAP) regarding epilepsy amongst PWE and their caregivers, 2) drug therapy, compliance with medication and outcomes, 3) clinico-demographic patterns, and 4) economic burden amongst patients.

Results: Most common type of seizures were generalized tonic–clonic seizures (GTCS), which were found in 82% of PWE. Also, 68% of PWE were well-controlled, 50% were on monotherapy and 15% required more than two drugs. The most common alternative treatment opted for was Ayurvedic treatment. Also, 26.4% and 11.2% of PWE believed that evil spirits or sins in the past were the cause for their seizures (2000 and 2004, respectively). However, with regular health awareness offered at each camp, faith in curative treatment increased from 18% to 59% (from 2000 to 2004). The total cost per month for tablet phenytoin sodium was 1,39,968 INR, phenobarbitone was 1,76,256 INR, levetiracetam was 8,06,400 INR, sodium valproate was 3,69,360 INR, carbamazepine was 1,64,400 INR, and clobazam was 1,53,540 INR. Amongst the total population of 7,200 PWE, 51.2% (3686 PWE) were on monotherapy, 32.6% (2347) were on dual therapy, and 16.2% (1167) required more than two drugs.

Conclusions: Clinicodemographic profile of patients, resources available and knowledge, attitude and practice toward epilepsy, and challenges encountered in remote and rural areas are different from those in urban areas.

Abstract ID: 738: Documenting Reversible Conduction Failure in Guillain–Barré Syndrome: A Compilation of Case Reports

Preethiswary Subramanian, Balaji G, Hariharan S, Thamilpavai N

Government Medical College, Chengalpattu, Tamil Nadu, India

Background and Aim: Guillain–Barre Syndrome (GBS) subtypes can be classified as acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) based on the results of electrodiagnostic studies. Primary axonal variants rarely may produce immune-mediated conduction failure in the nodes of Ranvier, which can only be recognized by serial nerve conduction studies, resulting in reversible conduction failure. Here, we report three such cases of AMAN with reversible conduction failure. AIM: Serial nerve conduction studies were conducted on patients with GBS presenting with AMAN to evaluate the occurrence of reversible conduction failure.

Methodology: We prospectively studied all patients with GBS admitted to our hospital between June 2022 and December 2023. Clinical outcomes and serial NCS were analyzed in all patients. Motor NCSs were performed on bilateral median, ulnar, tibial, fibular nerves with F waves, and sensory NCS conducted on median, ulnar, and sural nerves at the time of admission, within 2 to 3 weeks of the first NCS, and at 8 to 12 weeks interval.

Results: Total GBS cases: 33. Of these, the initial nerve conduction study showed severe amplitude reduction in distal CMAP with conduction blocks in non-compression sites with normal sensory conduction –observed in three cases. Serial nerve conduction studies in all three cases showed an evolving pattern of improvement in proximal amplitude and disappearance of conduction blocks without prolongation of distal latencies or excessive temporal dispersion of CMAPs, suggestive of reversible conduction failure.

Conclusions: Reversible conduction failure has been documented in AMAN and is considered a sign of nodopathy and has a favorable prognosis.

Abstract ID: 739: Prevalence and Outcome of Unilateral Spatial Neglect in Stroke Survivors

Pratik Roy, Atanu Biswas, Gautam Das

Bangur Institute of Neurosciences, Kolkata, West Bengal, India

Background and Aim: Background: Unilateral spatial neglect (USN) is a syndrome in which the patient fails to report or respond to novel or meaningful stimuli presented to the side opposite the brain lesion. Neglect is associated with poorer functional outcomes in stroke survivors with increased risk of falls, longer hospital stays, and caregiver burden. Objective: (1) to explore the prevalence of USN in stroke survivors and (2) to compare the evolution and resolution pattern of USN with respect to location and types of stroke.

Methodology: First-ever acute stroke patients with preserved language function and ability to write with MOCA (Montreal Cognitive Assessment) score ?25 were included. All patients were examined between 72 and 96 h after stroke and re-examined on day 6. For assessment of USN, we used the following tools: (a) Line bisection test, (b) Bisiach–Ogden test (Bisiach and Luzzatti), (c) visual extinction test (Di Pellegrino and colleagues), (d) dichotic listening test (Donald Broadbent), (e) Catherine–Bergego Scale, and (f) Baking tray task (Kerstin Tham and Richard Tegner). We correlated the site and size of stroke with USN.

Results: This is an ongoing study. To date, 42 patients have been included, of whom 25 are male. Four patients were found to have USN, all were male. Moreover, all had suffered from a stroke in the right parietal region. However; all patients with right parietal stroke did not have USN. There was no relation between the USN severity score (Catherine–Bergego Scale) and stroke severity score (NIHSS). Among the modalities of neglect, only visuospatial was involved. USN in all these subjects improved on day 6.

Conclusions: This ongoing study demonstrates USN in acute right parietal stroke, visuospatial remains the most common modality of neglect and it improved over a week.

Abstract ID: 740: Hyperhomocysteinemia with C677T Polymorphism on 5, 10 MTHFR as a Sole Cause of LVO in a Adolescence

Surekha Dabla, Naveen Kumar, Neeraj Kumar

Pt BD Sharma PGIMS Rohtak, Haryana, India

Background and Aim: Hyperhomocysteinemia is an important risk factor for several cardiovascular diseases, including coronary artery disease, peripheral occlusive disease, stroke, and venous thrombosis.

Case Report : We herein report a case of young-onset ischemic stroke with hyperhomocysteinemia caused by the C677T polymorphism in the MTHFR gene causing large posterior circulation stroke. Our patient was unique as at the adolescent age, no other cause except raised levels of homocysteine as a vascular risk factor was found.

Discussion and Conclusions: We conclude that his ischemic event was caused by hyperhomocysteinemia, which independently might progressed to atherosclerosis leading to arterial occlusion and large posterior circulation stroke.

Abstract ID: 741: Atypical Presentation of Rare Disease - Rett Syndrome - A Case Report

Bharath R, Balasubramanian S, Uma Maheshwari E, Kaushik Senthilnathan, Natarajan E

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Rett Syndrome is an extremely rare, post-natal, and serious genetic neurodevelopmental syndrome that occurs mostly in female children. It occurs due to the mutations in the MECP2 gene of the X chromosome. Here, we present a rare case of Rett’s syndrome with atypical features.

Case Report: A 26-year-old female patient born out of a non-consanguineous marriage, with normal birth and developmental history with no previous comorbidities presented with ataxia, involuntary movements (tremors and myoclonus), and seizures of 4 years duration. Clinical examination revealed facial dysmorphism, normal-sized head circumference, cognitive decline with bilateral appendicular and truncal ataxia, bilateral gaze-evoked nystagmus with normal cranial nerves, and sensory examination. Genetic testing with whole-exome sequencing revealed a heterozygous pathogenic variant in the MECP2 gene consistent with Rett’s phenotype detected in the X chromosome exon 3. From the subjective evidence and the performed investigations, the patient was diagnosed with Rett’s syndrome.

Discussion and Conclusions: Our case report highlights a rare case of atypical presentation of genetically confirmed Rett’s syndrome.

Abstract ID: 742: Etiology, Risk Factors, and Outcome of Ischemic Stroke in Young Adults

Tarun Sharma, Shubhkaran Khichar, Amita Bhargava

Sampurnanand Medical College (SNMC) Jodhpur, Rajasthan, India

Background and Aim: Stroke is the second most common cause of mortality and the third most common cause of morbidity globally. In India, the prevalence of ischemic stroke in young adults is 10–15% and common causes are atherosclerosis cardioembolic, and hematological disorders. Common risk factors are hypertension, smoking, alcohol, obesity, and substance abuse. The aim of the study was to identify the etiology, risk factors, and outcomes of stroke in young adults (18–45 years).

Methodology: This was a prospective and observational study, which included 50 patients who were admitted with symptoms suggestive of stroke. The diagnosis was made clinically and radiologically. Etiologically, patients were classified according to the TOAST classification. Patients were stratified according to initial MRS and NIHSS. The main outcome measures at 3 months were based on the final MRS.

Results: The maximum age group affected was 41–45 years. The most common symptom was hemiparesis (88%) followed by giddiness. The most common risk factors were hypertension, alcohol, smoking, dyslipidemia, and ischemic heart disease. Etiology was 34% in atherosclerosis, 22% unknown, and 16% cardioembolic. On imaging, anterior circulation was most commonly involved. Major recovery (MRS-2) was in 36% of patients who had minor to moderate stroke.

Conclusions: Young patient tends to carry manifold social, physical, emotional, vocational, and economic connotations. Modifiable risk factors and treatable causes are also common in young adults which can be identified to prevent stroke.

Abstract ID: 743: An Observational Analytical Study to Determine the Neurological Deterioration in Intracerebral Hemorrhage by GCS At 72 Hrs

Faye Shaikh, Sreehari Dinesh, V. P. SINGH, S. P. GORTHI

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim: In patients with primary hypertensive intracranial hemorrhage (ICH), 70% of patients develop neurological deterioration within the first 24 h of onset, whereas 22% of patients deteriorated in the first 72 h. Hematoma expansion is defined as an increase of more than 6 mL from the initial CT scan. In this study, we aim to estimate neurological outcomes in patients with ICH at 72 h using the Glasgow Coma Scale (GCS).

Methodology: In this single-center observational study, all patients above 18 years were included. Traumatic ICH and ICH of venous origin were excluded. Hematoma volume was calculated by the ABC/2 formula and GCS was assessed at admission, 24, and 72 h. The ICH score was calculated for each patient.

Results: Out of 60 patients, 45 (75%) were male patients. The mean age was 55.4 years. ICH SCORE > 4 at presentation was observed in 13 (21.6%) patients. Thirty percent of patients showed a drop in the GCS from baseline to 24 h and 24 (40%) patients showed a drop in the GCS from baseline to 72 h. Nine (15%) patients showed hematoma expansion from baseline to 24 h, whereas 10 (13%) patients showed hematoma expansion from baseline to 72 h. Thirteen (21.6%) patients required surgical intervention. The overall mortality was 9 (15%).

Conclusions: Elderly age, poor GCS on admission, and hematoma expansion are the most consistent predictors of death and poor outcomes.

Abstract ID: 744: Sjogren Syndrome Cases Presenting with Bilateral Foot Drop

Shakthi Govindarajan, Nithyanandam A, Aravind Gautham Visweshwaran, Velusamy S, Jawahar M, Balasubramanian S

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Sjögren’s syndrome, an autoimmune disorder primarily affecting the exocrine glands, is frequently associated with peripheral neuropathy. This neurological complication results from immune-mediated damage to the peripheral nerves, leading to symptoms such as pain, numbness, and weakness. The precise mechanisms linking Sjögren’s syndrome to peripheral neuropathy involve a complex interplay of autoimmune responses, highlighting the need for comprehensive diagnostic and therapeutic management. Sjogren cases diagnosed by EULAR/ACR criteria with complete neurological examination, electrophysiology tests, neuroimaging, and routine tests.

Case Report: Case 1: A 42-year-old female patient with Sjögren’s syndrome presented with subacute onset of pins and needles sensation in the bilateral foot, followed by bilateral foot drop. Case 2: A 52-year-old female patient, a known case of rheumatoid arthritis with secondary Sjögren’s syndrome presented with an acute presentation of numbness of the dorsum and plantar surface of the left foot one morning, followed by a foot drop that evening itself. Three days later, she developed similar symptoms in the right limb also. Case 3: A 29-year-old female patient with secondary Sjögren’s syndrome presented with pins and needles sensation in the bilateral lower limbs, followed by difficulty in using bilateral lower limbs. Also, this patient presented with asymmetric numbness of the face.

Discussion and Conclusions: The association between Sjögren’s syndrome and bilateral foot drop highlights the need for awareness of its neurological complications. Prompt diagnosis through comprehensive neurological evaluation and appropriate immunosuppressive treatment can significantly improve outcomes and enhance the quality of life for affected individuals.

Abstract ID: 745: Study on Clinico Radiological Profile and Outcomes in Patients with Normal Pressure Hydrocephalus (NPH)

Anugu Rao, Hari Radhakrishna

Medicover Hospitals, Hitech City, Hyderabad, India

Background and Aim: Background-Idiopathic NPH has a slowly progressive course, main symptoms are gait disturbances, cognitive impairment, and urinary incontinence. Aims-To study clinicoradiological profile and outcomes in patients with NPH.

Methodology: Single-center, observational, hospital-based prospective study over 15 months. Inclusion criteria-patients who presented met with international evidence-based diagnostic criteria for NPH to both neurology and neurosurgery OPD and IPD in xxx institute are included in this study. Exclusion criteria-patients with other dementias and secondary NPH were excluded from the study. Informed consent was taken and patients were followed up for 3 months to learn regarding the improvement of symptoms in surgically treated patients and to watch for worsening in other patients.

Results: Males were commonly affected, the mean age was 60 in males and 52 years in females with HTN and DM being the commonest comorbidities. MRI-ventriculomegaly, increased Evan’s index > 0.3, Sylvian fissure and insular cistern widening and crowding at the vertex S/o NPH. In all, 58% of people underwent VP shunt. Remarkable recovery in gait disturbances was observed in patients who underwent surgery.

Conclusions: The main goals of treatment are alleviation of symptoms so that a morbidity-free life can be achieved. Patients who underwent surgery had significant improvement in gait disturbances, non-interventional patients did not have much progression in 3 months.

Abstract ID: 746: Endovascular Treatment After Surgical Clipping and Endovascular Coiling of Intracranial Aneurysms

Anil Behera, Vikram Kumar Huded, Tanaya Mishra, Anush S. Rangrajan

Narayana Health City Bangalore, Karnataka, India

Background and Aim: As highlighted in the BRAT study, the outcome of treatment modalities depends strongly on the location of the aneurysm. The sustained advantage of endovascular treatment in posterior circulation aneurysms is well-proven. Regrowth and remnant of aneurysm lead to re-rupture, which is associated with significantly higher morbidity and mortality. The rate of de novo aneurysm appearance in previously clipped ruptured aneurysm could be 0.09%. Young individuals and patients with multiple aneurysms are more prone to de novo aneurysm formation during the follow-up. In this study, various characteristics of aneurysms were analyzed, which underwent clipping. An effort was made to understand the importance of angiographic follow-up of aneurysm after clipping.

Methodology: In this single-center retrospective study, we included patients whose intracranial aneurysms were initially treated surgically, identified to have re-growth, remnants, or de novo aneurysm on follow-up and subsequently addressed by endovascular treatment at our center between 2014 January and 2024 January. The study protocol was approved and consent was waivered by the institution review board. Data were entered in Microsoft Excel and analyzed using IBM-SPSS. Continuous variables are summarized as mean with standard deviation and median and interquartile ranges based on data distribution. The Chi-square test was used to analyze categorical and t -test for continuous variables. A P -value less than 0.05 was considered significant.

Results: Eleven patients underwent endovascular treatment after post-clipping re-growth/remnant. The mean age was 44.55 ± 8.64 years. Five females and six males were included The time interval between clipping and check DSA ranged from 1 to 75 months with a mean of 9.77 ± 21.89 months. Only one patient underwent check DSA after re-rupture. Almost all de novo aneurysms were treated endovascularly along with the re-grown aneurysms in view of anatomical proximity between de novo and re-grown aneurysms. Flow diverter (FD) stenting was conducted in eight patients. Stent-assisted coiling was performed in two patients.

Conclusions: Interval angiographic follow-up of aneurysms after clipping is necessary to prevent a fatal outcome of rupture. Difficult-to-clip aneurysms need short interval follow-ups, that is, 1 week to 1 month to identify residual aneurysm, which can be treated endovascularly. Awareness among the patients must be raised regarding angiographic follow-up as re-rupture is associated with significant morbidity and mortality. De novo aneurysms can arise during the follow-up, which need to be treated based on size and morphology.

Abstract ID: 747: Spectrum of Cognitive Changes following Subarachnoid Hemorrhage

Joydeep Ghosh, Debarup Das, Kuntal Biswas, Uddalak Chakraborty, Biman Kanti Ray, Atanu Biswas

Institute of Post Graduate Medical Education and Research, Kolkata (IPGMER Kolkata), West Bengal, India

Background and Aim: Background: Subarachnoid hemorrhage carries a high risk of early mortality and long-term disability in survivors. It comprises 5% of all vascular events and may lead to mortality in 48% of cases if not treated early. A staggering number (2.6 times) of aneurysmal SAH develop dementia in the next 10 years. Many patients of SAH develop impairment in executive function, verbal memory, and processing speed. Aims and Objectives: 1. Incidence of cognitive dysfunction in patients with SAH. 2. To assess the predictors of cognitive dysfunction in SAH.

Methodology: A cross-sectional observational study was conducted in a tertiary care hospital after considering proper inclusion and exclusion criteria and using the following questionnaire, ACE III (Bengali version illiterate and literate battery), CDR (Clinical Dementia Rating Scale), HAM-D (Hamilton’s Depression rating scale) along with domain specific tests such as forward digit span, backward digit span, clock drawing, trail making B, verbal learning test, verbal fluency test, picture naming, dot counting, fragmented letter test, and cookie theft test.

Results: Of the 24 patients studied, 14 were aneurysms and 10 were non-aneurysmal, gender ratio (M:F) was 10:14. Memory was impaired mostly in interhemispheric, with attention scores low in all regions, and suprasellar region showing impairment in language domains. Visuospatial was relatively spared.

Conclusions: Cognitive dysfunction was observed in our cohort, with memory impairment observed in patients with interhemispheric involvement. Attention span was low in 20 out of 24 patients of SAH both aneurysmal and non-aneurysmal. The language was affected in suprasellar involvement. The visuospatial domain was largely unaffected in our study. Though long-term follow-up may give newer involvement, which needs to be explored.

Abstract ID: 748: Autonomic Function Test in Patients with Fibromyalgia

Mekala Anusha, Deepa Avadhani, P Philo Hazeena, Rithvik Ramesh, S. Sundar, R. Lakshmi Narasimhan

Sri Ramachandra Medical College and Research Institute, Chennai (SRMCRI, Chennai), Tamil Nadu, India

Background and Aim: Fibromyalgia is a disorder of musculoskeletal pain accompanied by fatigue, sleep, memory, and mood disturbances. The pathophysiology of fibromyalgia includes abnormalities of neuroendocrine, and autonomic nervous systems, genetic factors, psychosocial variables, and environmental stressors causing pain. In a recent study, it was found that ANS abnormalities have been correlated with fibromyalgia. The significance noted was a decrease in sympathetic and parasympathetic activities. Hence, the aim of the study was to determine the changes in the autonomic function test (SSR and RRIV) in patients with fibromyalgia.

Methodology: It was a cross-sectional study that included 20 patients and 20 volunteers who came to the neurology department. The study period was from January to March 2023. Ethics approval was obtained. Disease severity was evaluated using the Fibromyalgia Impact Questionnaire. SSR and RRIV were carried out in the Electrophysiology Laboratory of Neurology, SRIHER, with NIHON KHODEN NEUROPACK.

Results: The mean latency and amplitude of SSR in the median nerve of patients with fibromyalgia were 0.7215 and 0.6235 when compared to controls latency and amplitude were 1.449 and 2.452, respectively. The P -values for latency and amplitude were P = 0.0006 and P = 0.0001 respectively. The mean latency and amplitude of SSR in the tibial nerve of patients with fibromyalgia were 0.807 and 1.035 when compared to controls, latency and amplitude were 1.787 and 2.93, respectively. The P -values for latency and amplitude were P = 0.0001 and P = 0.0001, respectively. The RRIV in resting and hyperventilation among patients with fibromyalgia were 7.32 and 17.95 compared to controls, resting and hyperventilation were 9.13 and 20.13, respectively. The P -values for resting and hyperventilation were P = 0.4800 and P = 0.4936, respectively.

Conclusions: The results showed patients with fibromyalgia have ANS dysfunction compared to controls.

Abstract ID: 749: A Case Series of Artery of Percheron Infarct

Aruna Kotteti, Aruna Kumari Kotteti

SRM Medical Collegemand Hospital and Research Centre Kattankulathur, Chennai, India

Background and Aim: We are presenting four cases of artery of Percheron infarct with varied clinical manifestations

Case Report: Case 1: A 49-year-old male patient with diabetes mellitus presented with giddiness and vertical diplopia. Examination revealed partial ptosis of the right eye and vertical gaze palsy. MRI brain showed acute infarct in bilateral medial thalami and right upper midbrain suggestive of artery of Percheron infarct. His ocular findings resolved within 3 weeks. Case 2: A 69-years-old male patient with diabetes mellitus; and hypertension presented with sudden-onset giddiness and memory disturbances. Neurological examination was normal, except for impaired recent memory. MRI brain showed diffusion restriction in the bilateral paramedian thalamus, suggestive of the artery of Percheron infarct. His memory improved after 1 month. Case 3: A 30-year-old male with no comorbidities presented with sudden-onset of loss of consciousness, followed by diminished consciousness for 2 days after that he had excessive sleepiness for 7 days. Neurological examination was normal, except for reduced alertness. A prothrombotic profile was sent as he was young; reports are awaited. ECHO was normal. MRI brain showed acute infarct in bilateral paramedian thalami. He was discharged after 7 days with no neurological deficit and with a normal mental state. Case report 4: A 60-year-old female patient with hypertension since 15 years presented with a sudden onset of giddiness, followed by weakness of the right upper and lower limbs with vertical diplopia. On examination, she had hemiparesis and hemiataxia right side; WII vertical gaze restriction, and impairment of recent y. MRI brain showed acute infarct in bilateral medial thalami and left hemi mid-brain.

Discussion and Conclusions: The artery of Percheron infarct is often underdiagnosed or misdiagnosed due to its complex clinical manifestations. MRI brain is the diagnostic modality of choice.

Abstract ID: 750: Unveiling the First Case Report of Autosomal Dominant Nocturnal Frontal Lobe Epilepsy with CHRNB2 Mutation in an Indian Family: Clinical and Genetic Insights

Sidharth S, Manjari Tripathi, Jasmine Parihar, Deepti Vibha, Rajesh Singh, Animesh Das

All India Institute of Medical Sciences, New Delhi (AIIMS, New Delhi), India

Background and Aim: Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is a genetic epilepsy syndrome, characterized by clusters of nocturnal motor seizures. This condition is linked to heterozygous pathogenic variants in genes encoding subunits of the nicotinic acetylcholine receptor, such as CHRNA2, CHRNA4, CHRNB2, CABP4, DEPDC5, CRH , and KCNT1 . Mutations in the CHRNB2 gene, encoding the beta-2 subunit, are extremely rare. This case report presents the first documented instance of ADNFLE with a CHRNB2 mutation in an Indian family.

Case Report: We describe an Indian family with two siblings (brother and sister) diagnosed with ADNFLE and identified with a pathogenic variant in CHRNB2 . Both siblings had normal birth and developmental milestones without a previous seizure history. Seizure onset occurred at the age of 12 years in the brother and age 14 years in the sister, characterized by sudden nocturnal awakenings 1.5 to 2 h into sleep, exhibiting confused speech, and bilateral tonic–clonic seizures lasting 30 s to 1 min. They experienced three to four such episodes during sleep, including daytime naps, with no neurological deficits and preserved cognition.

Discussion: Video EEG recorded seizures originating during sleep, with a head turn to the left and bilateral tonic–clonic activity accompanied by focal slow waves in FP2. MRI brain epilepsy protocol results were normal. MEG localized seizure activity to the right posterior frontal and right mid-frontal regions. PET imaging revealed hypometabolism in the right inferior frontal region.

Conclusions: We report the first Indian family diagnosed with ADNFLE harboring a rare pathogenic mutation in the CHRNB2 gene, contributing to the understanding of this rare genetic epilepsy and highlighting the importance of genetic testing in diagnosis and management.

Abstract ID: 751: Varying Presentations of Primary Mitochondrial Disorders-MELAS- Case Report of Two Cases

Rosy Thomas, Ravi L A, A. Marian Jude Vijay, A. Vignesh, M. Jawahar, S. Balasubramanian

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Primary mitochondrial disorders (PMDs) constitute the most common cause of inborn errors of metabolism in children and frequently affect the central nervous system. MELAS is a prototypic PMD, affected patients have a maternal pattern of inheritance that is due to pathogenic variants in mtDNA with variable heteroplasmy. The most common mutation observed in MELAS is the m.3243A>G mutation in the MT-TL1 gene. Here, we present two cases of genetically proven MELAS with varied manifestations.

Case Report: The first case is a 15-year-old girl who presented with headache, vomiting, and decreased responsiveness with a background history of seizure disorder since 3 years before presentation. MRI brain conducted at that time revealed acute infarct in the left occipital lobe and a chronic infarct in the left peritrigonal area. On examination, there were no focal neurological deficits. MRI brain revealed acute infarcts in the left intermediate temporal region and right parieto-occipital region. Serum lactate and pyruvate levels were elevated, and genetic analysis revealed MTL1-m3243A>G pathogenic mutation.

Discussion and Conclusions: Here, we present the varying presentations of a single disease and the need to look out for mitochondrial diseases in young patients presenting with neurological complaints with or without a family history.

Abstract ID: 752: Lymphoma Hiding Behind Cloak of Peripheral Neuropathy

Ankita Sharma, Ish Anand, Anuradha Batra

Sir Ganga Ram Hospital, New Delhi, India

Background and Aim: Paraneoplastic syndromes are defined as a group of systemic disorders that most commonly develop in association with solid organ tumors and lymphomas. Central nervous system (CNS) lesions and peripheral neuropathy are rare among patients with non-Hodgkin’s lymphoma (NHL). The incidence of peripheral neuropathy was 5%. Guillain–Barré syndrome (GBS) is rare and may occur in less than 0.3% of patients with NHL. We report a case of peripheral neuropathy, initially kept as acute inflammatory demyelinating neuropathy, which soon turns out to be glutamic acid decarboxylase (GAD) 65-related peripheral neuropathy secondary to diffuse large B cell Lymphoma.

Case Report: A 65-year-old male patient presented to us as a case of subacute onset peripheral neuropathy with no bladder or bowel, and cranial nerve involvement. We took the detailed history and physical examination and investigated over lines of peripheral neuropathy.

Discussion: His serum paraneoplastic panel showed anti-GAD 65 strong positivity. Whole PET CT showed multiple osteolytic skeletal lesions and thickening of the duodenum. Duodenal biopsy suggested diffuse large B cell lymphoma, which was also affirmed by bone marrow biopsy. He was then started on chemotherapy.

Conclusions: A keen and detailed evaluation of peripheral neuropathies is essential. Paraneoplastic syndromes secondary to treatable malignancies must not be missed.

Abstract ID: 753: A Study on the Clinical Significance of Diffusion-Weighted MR Imaging in Infratentorial Strokes

Raiz Iqbal, Sriramakrishnan V, Jason Ambrose

Tiruvannamalai Medical College, Tamil Nadu, India

Background and Aim: In posterior circulation stroke, the DWI lesion detection rate is significantly lower than in anterior circulation stroke. It is unclear whether DWI provides clinically useful information about stroke etiology or prognosis in patients with infratentorial strokes. The aim is to study the association between DWI characteristics and stroke etiology, stroke severity, and functional outcome in patients with infratentorial strokes.

Methodology: Study population: All patients with infratentorial stroke who attended the Neurology Department, TVMCH, from June 2023 to May 2024. Study design: Cross-sectional study, sample size: n = 100. All patients received standard stroke treatment. We used blinded comparison of DWI features (number, distribution, and volume of lesions) with clinical characteristics, namely, stroke etiology ([TOAST] classification), severity (NIH Stroke Scale [NIHSS]), length of stay [LOS], and functional outcome using modified Rankin Scale and Barthel Index.

Results: The number of DWI lesions was correlated with underlying stroke etiology, which showed cardio-embolic strokes had more DWI lesions ( P < 0.001). No significant correlations were detected between the number of DWI lesions and other clinical characteristics. The distribution of DWI lesions showed a correlation with stroke etiology. Cardioembolic stroke had clinically silent, acute DWI lesions in the anterior circulation in addition to their infratentorial lesions ( P < 0.001). DWI lesion volume did not show a correlation with clinical characteristics ( P > 0.05).

Conclusions: DWI is a more reliable imaging method than MRI in detecting lesions in infratentorial strokes. The positive correlations between stroke etiology and the number and distribution of DWI lesions require confirmation by future studies before these findings can be generalized.

Abstract ID: 754: Adolescent Onset Hereditary Neuropathy with a Novel Mutation

Avni Apte, Anshu Rohatgi, Sunita Bijarniya Mahay, Laxmikant Ramsingh Tomar

Sir Ganga Ram Hospital, New Delhi, India

Background and Aim: PHARC (polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, and early-onset cataract) is an acronym for rare, neurodegenerative disease caused by biallelic variants in the ABHD12 gene. Located on chromosome 20, it encodes the alpha/beta hydrolase domain-containing protein 12 and plays a vital role in lipid metabolism, especially in the central nervous system. Patients demonstrate clinical variability with regard to disease onset, severity, and progression. Not all clinical findings are necessarily present initially. Patients may be misdiagnosed with other neurodegenerative diseases with roughly similar phenotypes, such as Charcot–Marie–Tooth, Usher type 3, and adult Refsum’s disease. We describe the case of a 15-year-old boy who presented with paraparesis.

Methodology: A 15-year-old boy presented with a complaint of insidious onset, slowly progressive, symmetrical paraparesis, and high-arched feet. Examination showed bilateral thenar and extensor digitorum brevis muscle wasting, weakness at bilateral hip and knee joints, absent deep tendon reflexes in both lower limbs, and broad-based gait. Family history was positive for third-degree consanguinity, and the younger brother was found to be suffering from hearing loss and progressive visual disturbances. Nerve conduction studies suggested polyradiculoneuropathy. Optical coherence tomography of the younger brother revealed retinoschisis and abnormal central auditory pathway on brainstem-evoked auditory response.

Results: Molecular genetic analysis revealed homozygous mutation for c.(749+1_750-1)_ (950+1_951-1)del at exons 8–10 of the ABHD12 gene on chromosome 20 in both siblings and heterozygous deletion in the ABHD12 gene in their father.

Conclusions: We wish to contribute to various genotype–phenotype presentations of PHARC syndrome and emphasize the importance of molecular genetic diagnosis in the clinical approach to a case.

Abstract ID: 755: A Study on Altered Functional Connectivity in Idiopathic Parkinson’s Disease Patients with Cognitive Impairment

Manali Chandra, Moulibrata Sarkar

Institute of Post Graduate Medical Education and Research, Kolkata (IPGMER Kolkata), West Bengal, India

Background and Aim: Parkinson’s disease (PD) is the second most common neurodegenerative disorder. Among multiple non-motor symptoms of IPD, cognitive impairment is common. PD is known to cause brain network dysfunction. fMRI is an imaging technique to characterize the changes in such networks including DMN (default mode network). Several studies have shown disrupted functional connectivity in DMN in patients with PD by resting fMRI. Aim: To find any difference in functional connectivity in DMN in healthy controls from patients with IPD with cognitive impairment.

Methodology: This was an observational cross-sectional analytical study, starting from August 2023 and continuing. Any IPD patient, from our neuromedicine OPD having cognitive impairment was included and those with cognitive impairment with contribution by encephalopathy, major depression, or vascular etiology were excluded. Data were collected from patients by taking history and conducting clinical examination, followed by neuroimaging in the form of fMRI. Findings are being compared with healthy controls using clinical and imaging findings by statistical methods.

Results: 4 IPD patients with cognitive impairment were compared with four healthy controls. The average UPDRS motor score and ACE III score for patients were 21 and 72. Seed-to-voxel correlation analysis of resting state fMRI (with seed ROI placed in the posterior cingulate gyrus, MNI coordinates x = 0, y= −53, z = 26) revealed the strongest functional connectivity of PCC (posterior cingulate cortex) with the precuneus in the DMN maps. A paired t -test revealed significant differences in the strength of connectivity between human controls and patients with PD.

Conclusions: The results obtained show patients of IPD with cognitive impairment have abnormal functional connectivity in DMN at the precuneus.

Abstract ID: 756: Mapping of Cerebral Collaterals and Vascular Risk Factors to Functional Outcome in Subjects with Acute Ischemic Stroke in Anterior Cerebral Circulation: An Observational Analytical Study

Sreehari Dinesh, Sankar Prasad Gorthi

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim: Cerebral collaterals of patients with acute anterior ischemic stroke are of prime importance as this holds the key to successful reperfusion of the injured brain. Determining associations between modifiable risk factors such as hypertension and cerebral collateral status to functional outcomes will help streamline stroke care. Aim: To estimate the prevalence of good functional outcomes in acute anterior ischemic stroke subjects using CT angiography scores of cerebral collaterals and assess if chronic hypertension provides robust cerebral collateral circulation in patients with acute anterior ischemic stroke.

Methodology: This was an observational analytical study of patients with acute anterior ischemic stroke who presented within 48 h of symptom onset. Cerebral collaterals were classified based on various scores to identify the best to predict functional outcome at 3 months of follow-up. We also assessed hypertension and its relation to cerebral collateral.

Results: A total of 121 patients of acute anterior ischemic stroke were enrolled. The mean age of patients was 55.88 ± 18 years, 70.2% being males. The average NIHSS of patients was 6.6 ± 3.6. Hypertension was the most common risk factor (57.0%), followed by diabetes mellitus (40.5%). There was no significant correlation between chronic hypertension and good cerebral collateral status ( P -value = 0.68). Also, 19.8% of cases underwent IV thrombolysis and 5.8% endovascular thrombectomy. CT ASPECTS had the best sensitivity in predicting the functional outcome with an area under the curve of 0.918, followed by the MAAS collaterals scoring system with an area under the curve of 0.637. Good outcomes (modified Rankin Score at 3 months ≤ 2) were observed in 86% of cases.

Conclusions: Patients with poor collateral status had poor functional outcomes.

Abstract ID: 757: Clinical Spectrum of Multiple Sclerosis and the Response to Treatment in South Indian Population in A Tertiary Care Hospital: A Prospective Study

Uma Maheshwari E, Balasubramanian Samivel S, Jawahar M, Boopathy R M

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Multiple sclerosis (MS) is a demyelinating disease of the central nervous system that exhibits marked clinical heterogenicity. The clinical and epidemiological heterogenicity and the response to treatment were studied in our population.

Methodology: A total of 44 patients with MS (18 men and 26 women) who satisfied the diagnostic criteria for definite MS and who had a remitting and a relapsing course were followed prospectively in our hospital which is a tertiary care hospital.

Results: The age of onset for MS were 20–29 years in 77.2% ( n = 34) and 30–44 years in 22.8% ( n = 10). The initial clinical manifestations were visual impairment (63.3%), other cranial nerve dysfunction (9%), impairment of sensory functions (9%), and motor impairment (18.7%). The interval between the first symptom and relapsing symptom ranged from 4 to 48 months with a mean of 23.3 months, and the mean number of relapses was five. Positive rates of CSF oligoclonal bands, IgG index, and the number of patients with characteristic brain MRI lesions were low. However, the visual evoked potential abnormality was relatively high (64.4%). The patients were treated with interferon beta 1a, and appropriate escalation to monoclonal antibody (natalizumab) was conducted as per the Canadian MS working group updated recommendations. The treatment outcomes were assessed using EDSS (Expanded Disability Status Scale) and MMSE (Mini-Mental State Examination).

Conclusions: Early institution of the disease-modifying therapy in remitting relapsing MS can prevent the disabling residual neurological deficits.

Abstract ID: 758: Spectrum of Leukoencephalopathies: A Case Series

Durairaj Arumugam, Devi Varadharaj, Suresh Thirunavukarasu, B B V Ramanan, Jayasri P, Viswatha Hari Baskar

Indira Gandhi Government General Hospital and Post Graduate Institute, Puducherry, India

Background and Aim: Leukoencephalopathy disorder involves the white matter of the central nervous system. Such disorders may result from mutations in genes encoding the components of myelin or may be acquired manifestations of vascular, inflammatory, infectious, traumatic, toxic, nutritional, or neoplastic diseases.

Case Report : Herewith, we report a case series of six patients with varied clinical and radiological presentations of leukoencephalopathies.

Discussion and Conclusions: .

Abstract ID: 759: Neuro Melioidosis Masquerading as Stroke-Like Syndrome

Anil Behera, Tanaya Mishra, Vikram Huded, Anush S. Rangarajan, Anush S. Rangarajan

Narayana health city Bangalore, Karnataka, India

Background and Aim: Melioidosis, which is caused by Burkholderia pseudomallei , is predominantly a disease of tropical climates and is especially widespread in southeast Asia and northern Australia. Melioidosis affecting the central nervous system has a low incidence but a high mortality. A recent systematic review found 120 cases reported worldwide with only 16 cases from India. Only 1.5 to 5% of cases present with craniospinal manifestations. However, mortality in neuromelioidosis remains high. CNS melioidosis has varied manifestations, including encephalomyelitis, cerebral abscess, cranial nerve deficit, and isolated meningitis.

Case Report: We presented a case of a 38-year-old patient presenting with a fever with seizure with multiple cranial nerve palsies, diagnosed to have neuromeloidosis via pus culture through brain biopsy. Patient, a 38-year-old male patient, with nil comorbidities presented with complaints of subacute onset loss of dexterity in the right hand, which progressed to right-side hemiparesis. The patient was admitted to a local hospital for the evaluation of a stroke. The patient had high-grade fever with seizure with worsening of weakness and right UMN facial palsy with lateral gaze palsy with bulbar symptoms, dysphagia. MRI brain plain and contrast with vessel wall imaging showed foci of increased signal intensities on T2-weighted and FLAIR images involving the left fronto-parietal lobes, left ganglio-capsular region, thalamus and brainstem, with areas of patchy diffusion restriction in left fronto-parietal lobes, thalamus, and left cerebral peduncle, which was reported to have CNS infectious and neuro-melioidosis with less likely possibilities of CNS vasculitis.

Discussion: Neurosurgery consultation: Brain biopsy from the frontal region was taken and sent for tissue culture. Tissue culture showed Burkholderia pseudomallei , which was previously suspected and was susceptible to meropenem and cotrimoxazole. The patient was treated with inj. meropenem and inj. cotrimoxazole and other supportive medications.

Conclusions: There are very few case reports of neuromeliodosis in India, with some authors describing its presentation as cerebral abscesses. This case proves that in tropical countries such as India, a high index of suspicion along with knowledge of varied presentations and unique imaging findings are key to the management of neuromeliodosis.

Abstract ID: 760: Cerebral Collaterals in CT Angiography in Predicting Functional Outcome of Anterior Ischemic Stroke Patients: An Observational Study

Siri Komalla, Sreehari Dinesh Kumar, S.P. Gorthi, V. P. Singh

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim: To assess the predictors of clinical outcome in acute anterior ischemic stroke patients in reference to cerebral collaterals on CT angiography.

Methodology: Patients with acute anterior circulation ischemic stroke were evaluated. CTA was performed and evaluated for intracranial collaterals according to the predefined criteria: mCTA score at baseline. The functional outcome of patients was evaluated using a modified Rankin scale (MRS) and Barthel Index at day 90.

Results: The mean age of the population was 54.58 ± 14.62 years with the majority of patients between the age group of 40 and 60 years. There were 19 males (73.08%) and 7 females (26.92%). The Comorbidities associated with the patients were hypertension (38.46%), diabetes mellitus (30.77%), ischemic heart disease (11.54%), and CVA (3.85%). No comorbidities were observed in nine patients (34.62%). Mann–Whitney U test was conducted to determine whether there was a difference in the outcome. The results indicated a significant difference between mCTA and Barthel Index with a negative correlation with z-score = −5.30 and P < 0.00001 (statistically significant). There was a positive correlation between mCTA and MRS with z-score = 4.01 and P < 0.00001 (statistically significant).

Conclusions: It can be concluded that good cerebral collateral status results in good functional outcomes in patients with acute anterior ischemic stroke.

Abstract ID: 761: A Comparative Study of Risk Factors Across Young and Old Acute Ischaemic Strokes: A Hospital-Based Study in North India

Pranav Gupta, Neeraj Bhutani, Surinder Sharma, K. K. Singh, Priya Agrawal

Santokba Durlabhji Memorial Hospital & Medical Jaipur, Rajasthan, India

Background and Aim: To compare established risk factors of ischemic strokes across young and old.

Methodology: The study was conducted at SDMH Jaipur from June 2015 to December 2016. It was a hospital-based analytical type of observational, cross-sectional study. In all, 150 stroke patients above 18 years of age were included in the young (<50 years) or old age group (> 50 years).

Results: Of 150 patients, 75 patients were in the young stroke group and 75 patients were in the old stroke group. Out of these 150 patients, 66.67% were males and 33.33% were female. The mean age of young patients was 41.64 years, whereas in old stroke patients, it was 65.8 years. Also, 38.67% in the young group and 72% in the old group had hypertension. Diabetes was found in 16% and 32% of the young and old groups, respectively. High serum homocysteine was observed in 64% of young and 73% of old patients. Low serum vitamin B12 was found in 25% of young and 32% of old patients. VDRL was reactive in 1.3% of young and 4% of old patients. ANA was positive in 6.6% of young and 2.6% of old patients. From those tested, APLA was positive in 3.64% of young and 4.16% of old patients.

Conclusions: Hypertension, diabetes mellitus, inadequate physical activity, dyslipidemia were significant risk factors associated with the old group. RHD was significantly associated with the young group. Risk factors such as low vitamin B12, high homocysteine, vasculitis, and APLA syndrome, which are traditionally regarded as risk factors in the young were similarly found in the old age group. Serum vitamin B12 and homocysteine levels should be conducted routinely in the evaluation of stroke irrespective of age of onset.

Abstract ID: 762: A Rare Case Report of Madras Variant MND from North India

Deepika Dalal, Suman Kushwaha, Aparna Aparna Thomas

Institute of Human Behaviour and Allied Sciences, Delhi, India

Background and Aim: Madras motor neuron disease (MMND) is a rare neurodegenerative disorder of young onset motor neuron disease. The clinical spectrum includes weakness and wasting of limb muscles, pyramidal signs, lower cranial nerve involvement, sensory neural hearing loss, optic atrophy, cerebellar signs, and chin fasciculations. MMND was first described in 1970 by Meenakshisundaram et al. from Madras.

Case report : A 17-year female patient born from a non-consanguineous marriage with normal developmental milestones, resident of Delhi, presented to us with a history of difficulty in walking for the last 2 months, difficulty in swallowing, and change in voice for the last 1 month with background history of bilateral hearing loss since last 4 years. On examination, the patient had a normal cognitive function, bilateral sensorineural deafness, dysarthria, tongue atrophy, fasciculation, weak gag reflex, bilateral hand muscles atrophy, polyminimyoclonus, brisk DTR, and extensor plantar. Gait was cautious with right foot drop. The patient did not have any other sensory cerebellar, or vestibular symptoms. Audiometry was suggestive of bilateral moderate SNHL. NCV was suggestive of axonal neuropathy. EMG revealed a denervation pattern with reduced recruitment and incomplete interference. All other laboratory routines and imaging were normal.

Discussion and Conclusions: MMND is a very uncommon motor neuron disease with variable clinical presentation. So, for patients who present very early in the disease course, close follow-up is necessary to better understand the natural history and clinical progression of this disease, especially in young patients.

Abstract ID: 763: The Electroclinical Features and Seizure Outcome of Cingulate Epilepsy patients undergoing resective surgery in a tertiary care center in South India

Kiren Koshy, Ramshekhar N. Menon, Ashalatha Radhakrishnan, Adarsh Anil Kumar, Bejoy Thomas

SCTIMST, Thiruvananthapuram, Kerala, India

Background and Aim: Cingulate epilepsy is difficult to characterize electrophysiologically due to the deep location, scarcity of interictal epileptiform discharges (IEDs), and pseudolateralization due to the medial location. In this retrospective study, we aim to describe the electroclinical features and seizure outcomes of patients with cingulate epilepsy who underwent resective surgery in a tertiary epilepsy center.

Methodology: The surgical database maintained in xxx center from 1995 to 2023 was reviewed, and patients who had undergone cingulate or cingulate plus resections were included. Clinical data were obtained from the discharge summaries and EEG/MRI data from the EMR.

Results: Video EEG showed IEDs in 22 out of 23 patients (95.65%). IEDs were temporal in 6 patients (26.09%), parieto-occipital in 4 patients (17.39%), and frontal in 19 patients (82.61%). The ictal onset was diffuse in 7 cases (30.44%), temporal in 3 cases (13.04%), and frontal in 10 cases (43.48%). A structural lesion was observed in MRI in 22 patients (95.65%)-tumor (in 6 cases), focal cortical dysplasia in 15 cases, and tuber in 1 case. Postoperative complications were noted in 8 patients (30.77%)-monoparesis or hemiparesis in five patients and postoperative meningitis in 1 patient. After 1 year of follow-up after surgery, 18 patients (78.26%) had an Engel 1a outcome, whereas 4 patients had an Engel class II outcome and 1 patient had an Engel class III outcome.

Conclusions: The diverse clinical, interictal, and ictal features observed in cingulate epilepsy need to be understood to diagnose this condition early and maximize the surgical success rate.

Abstract ID: 764: Unlocking the Hand Knob to Heart: A Case of Peri Partum Cardiomyopathy Initially Presenting as Wrist Drop

Nageswaran bala Ramachandran, Velusamy S, Nithyanandam A, Aravind Gautham V, Balasubramanian S

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Peripartum cardiomyopathy (PPCM) is one of the rare causes of stroke in young females. We report a case of acute cardioembolic stroke in a postpartum female as the initial presenting feature of peripartum cardiomyopathy.

Case Report : A 31-year-old, previously healthy woman with a history of preeclampsia, presented 8 weeks after the delivery with left-hand weakness, unable to extend her wrist and fingers, slurring of speech, and deviation of the angle of the mouth to the right side. On neurological examination, she had left-sided upper motor neuron type facial nerve palsy and weakness of the left wrist and finger extensors with preserved synkinetic wrist extension. The magnetic resonance imaging showed acute infarct of the right pre-and post-central gyri. Her transthoracic echocardiography showed global hypokinesia of the left ventricle with an ejection fraction of 27% and no intracardiac thrombus.

Discussion: She was started on anticoagulants, and heart failure medications and followed up. After 8 months, she discontinued nicoumalone and developed left hemiparesis with right MCA territory infarct. Her echocardiogram showed global left ventricular hypokinesia, and right ventricular dysfunction, with an ejection fraction of 25% and no intracardiac thrombus. She was restarted on anticoagulation and heart failure medications.

Conclusions: We should consider PPCM in any patient presenting with stroke in the puerperium. Early diagnosis, optimization of heart failure medication, and prompt anticoagulation prevent further complications.

Abstract ID: 765: A Sleeping Beast-Stroke and Sleep

Gani Mozhi, Kishore R, Rajasekaran M, Kannan N

Mahatma Gandhi Memorial Medical College, Tamil Nadu, India

Background and Aim: Sleep-related breathing disorder (SRBD) is one of the common consequences after stroke and neglected risk factor too. The purpose of the study was to identify the incidence of SRBD after stroke.

Methodology: A prospective observational study of 42 patients with stroke admitted to the Government KAPV Medical College hospital over 6 months were assessed at the time of admission. Details such as age, gender, sex, risk factors, comorbidities, body mass index (BMI), obesity, waist–hip ratio, STOP BANG QUESTIONNAIRE, NIHSS and MRS scores on admission and discharge, size, the pattern of strokes, type, and treatment options were collected. Polysomnography (PSG) was conducted, Apnea–Hypopnea Index was assessed. The patient was followed up during the hospital stay for the outcome.

Results: Of the 42 patients studied, males (71%) had more incidence of OSA than women (29%). Even patients with low BMI (54%), and normal neck circumference (63%) have a high incidence of OSA after stroke. Sleep apnea or hypopnea was a common finding in our patients. Also, 53% had AHI > 5/h, 25% had AHI between 5 and 15. OSA was present in 30%, central apnea in 11%, and mixed in 9%. The incidence of OSA in MCA territory infarct (73%), ACA territory infarct (18%), and PCA territory (2%). Among MCA territory infarct, large stroke was present in 55% and small vessel stroke was present in 45%. The size of the lesion does not predict the risk of SRBD.

Conclusions: As our findings showed the highest diagnostic outcome efficacy, it is strongly requested to conduct PSG routinely. Further studies regarding early diagnosis and treatment will significantly improve the quality of life and reduce the reinfarction size.

Abstract ID: 766: Clinical, Etiological, and Imaging Profiles of Posterior Reversible Encephalopathy Syndrome: A Single-Center Experience from a Tertiary Care Hospital in Coastal Andhra Pradesh

Deepthi Kanuganti, Kumaravelu Somasundaram, Anita Nelakuditi, Varaprasad Devabhaktuni

Aster Ramesh Hospitals Nagarampalem, Guntur, Andhra Pradesh, India

Background and Aim: Posterior reversible encephalopathy syndrome (PRES) is a state coupled with a unique clinical and radiological appearance. Various conditions such as preeclampsia/eclampsia, autoimmune diseases, chemotherapy bone marrow, and organ transplantation are foreseen. The aim is to review the clinical, etiological, and radiological findings and outcomes of patients diagnosed with PRES.

Methodology: In all, 30 patients with clinical and neuroimaging findings consistent with PRES were included in this prospective and retrospective study.

Results: The study included 30 individuals ranging in age from 17 to 83 years. A total of 19 patients had eclampsia, 2 patients had SLE, 1 patient had SLE with eclampsia, 1 patient had severe acute tubular necrosis with eclampsia, and 1 patient had shock post-LSCS, 5 patients had chronic kidney disease. Out of 19 patients with eclampsia, 11 patients had acute surge in blood pressure, 6 patients had normotension and 1 patient had hypotension at the time of presentation. Seizures were the most common symptom. MR imaging showed vasogenic edema at 100% with typical locations (parietooccipital cortex) in 13% and atypical (frontotemporal cortices, basal ganglia, cerebellum, brainstem, and thalamus) plus a typical location in 86% of patients and findings such as restricted diffusions at 9.6% and hemorrhage at 9.6% additionally. Overall, 96.7% of our patients recovered without sequelae in the short-term.

Conclusions: In this study, atypical regions were frequently included along with typical locations. Restricted diffusion and hemorrhages were two examples of unusual imaging properties. It was observed that not only the rapidly developing hypertension disrupting cerebral autoregulation but also normotension and even hypotension causing cerebral hypoperfusion and endothelial dysfunction cause PRES.

Abstract ID: 767: Clinico-Radiological Profile of MOGAD: Insights from a Tertiary Care Center

Sharanya Raj, Archana N B, Vibhav M S, Shreyas Kumar Lamani, Praveen Kumar S, Pramod K, Janardhan D C, Thejus B, Raghu Patil, Anilkumarr Swammy

Bangalore Medical College and Research Institute, Bengaluru (BMCRI Bengaluru), Karnataka, India

Background and Aim: Anti-MOG antibody-associated disease is a new entrant in the spectrum of CNS demyelinating disorders. We aim to describe the clinical, demographic, and radiological features and treatment received by patients with MOGAD and identify factors that could predict relapse and hence indicate the need for long-term immunomodulatory therapy.

Methodology: All patients admitted to the Department of Neurology at our institution, with a diagnosis of MOGAD, from 2018 to 2023, were included in this descriptive study. The clinical picture, investigations, treatment received, response to therapy, and relapses were noted. Serum MOG and AQP4 antibodies were tested in all patients by a cell-based assay.

Results: The 34 patients in this study had a total of 41 events (initial presentation and relapses). The female-to-male ratio was 1.8:1. The age of patients ranged from 5 to 72 years. Common symptoms were blurring of vision (75%), orbital pain (58.5%), headache (17%), sensory symptoms (17%), limb weakness (14.6%), bowel and bladder disturbances (4.8%), ataxia and diplopia (2.4%), altered sensorium (2.4%). Six patients had a fever before symptom onset. On MRI, 19 events demonstrated B/L optic neuritis, 13 were unilateral, 6 had spinal cord, and 7 had brain involvement. CSF analysis was available in 25 of the 34 patients. Abnormal CSF findings were observed in 10 patients. All patients received pulse steroids during the acute phase. Two patients, in addition, received IVIg. Relapses were noted in 7 patients. Commonly used agents for long-term immunomodulatory therapy were monthly pulse steroids, rituximab, and azathioprine.

Conclusions: This study highlights the advantage of early initiation of long-term immunomodulatory therapy in patients with MOGAD to reduce relapse and accumulation of neurological deficits.

Abstract ID: 768: Blood and Serum Tests Should be Included in Chronic Migraine Work Up to Get Better Results

Amarapu Sravanthi, Randhi Venkata Narayana, Chebolu Harshika, Kakarala Pradeep, Bandaru Balakrishna, Jogi Pranathi, Gontu Viswanadha Reddy

Sevenhills Hospital, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Migraine headaches are common neurological disorders throughout the globe. Correct diagnosis and treatment are challenging tasks for the physicians. Women experience migraines three times more often than men. Worldwide, migraine is considered one of the most disabling diseases among young people. Aim: Migraine headache patients need to be assessed for their symptom relief and help them to go back to work and be less disabled. Co-morbid conditions should be assessed. Uncontrolled migraine headache patients should be enquired about their health issues and loss of work hours.

Methodology: A total of 50 chronic migraine patients between the ages of 18 and 45 years who fulfilled the ICHD-3 criteria were evaluated at our outpatient clinic between January 2023 and April 2024. Apart from neuroimaging, all patients were advised to undergo the following blood tests (hemogram, random blood glucose, renal function tests, liver function tests, serum uric acid levels, serum calcium, serum B12, and vitamin-D levels) after prior consent.

Results: All patients clinical and neurological examinations were normal. In all, 34 patients were women and 16 were men and their neuroimaging showed no significant abnormalities. Among 50% of patients (25), blood and serum tests showed vitamin B12 deficiency in 10 patients, 6 patients had vitamin-D deficiency, and uric acid levels were high in 5 patients, and hemoglobin was low in 4 patients.

Conclusions: Co-morbid conditions such as vitamin deficiency and abnormal accumulation of metabolic products should be identified and treated in migraine patients to get better relief of their symptoms.

Abstract ID: 769: Association of Stress, Depression, and Anxiety in Patients Presenting with Migraine

Sonam Saxena, Prerana Gupta, Jigar Haria, Harshdeep Singh, Kuljeet Singh Anand

Background and Aim: Headache frequently presents as a primary complaint in outpatient settings, particularly in psychiatry departments, often serving as a somatic expression of underlying psychiatric disorders such as depression, anxiety, and stress. This study aims to pinpoint gaps in understanding and assist psychiatrists and neurologists in identifying stress, depression, and anxiety in migraine patients while evaluating the efficacy of various screening tools in assessing symptom severity.

Methodology: Conducted at Teerthanker Mahaveer Medical College and Research Center, Moradabad, this study involved 188 outpatient migraine patients from the Departments of Psychiatry and Internal Medicine or Neurology. Written informed consent was obtained from participants and caregivers. The demographic information, psychiatric history, and relevant medical data were collected using a semi-structured proforma. Assessment of depression and anxiety symptoms employed scales such as HAM-A and HAM-D, whereas stress severity and types were evaluated using the PSS and Scale for Stress Domain Questionnaire (SSDQ).

Results: Analysis revealed a significant association between depression and throbbing/stabbing headache types ( P = 0.039) and between anxiety and pounding headaches ( P = 0.035) based on PSS and SSDQ. Similarly, HAM-D showed a significant correlation between depression and throbbing/stabbing headache types ( P = 0.020), whereas HAM-A indicated higher anxiety in pounding headache cases ( P = 0.017).

Conclusions: In the present study, various scales were compared in the patients of migraine, a significant difference was observed in Hamilton depression, and Hamilton anxiety but no significant difference in PSS and SSDQ scales. With the use of HAM-D and HAM-A, a significant number of cases were observed with depression followed by anxiety.

Abstract ID: 770: Beyond Movements: Exploring Cognitive and Motor Interactions in Parkinson’s Disease

Harshdeep Singh, Kuljeet Singh Anand

JSS Medical College and Hospital, Mysuru (JSSMCH, Mysuru), Karnataka, India

Background and Aim: Parkinson’s disease (PD) alters muscle function and control, impacting movement timings and inhibition. Muscle tone, strength, and tendon reflexes play crucial roles in this cognitive–motor network, that may necessitate interventions for PD management and neurorehabilitation. This study explores the influence of muscle tone, strength, and tendon reflexes on movement timings and response inhibition in subjects with PD. The primary goal was to examine the impact of these muscle aspects on finger-tapping timings and Go/No-Go and Flanker task performances while assessing their relationships. The secondary objective was to understand how changes in muscle functions interact with movement timings and inhibition, providing insights into the integrated nature of motor and cognitive functions in PD.

Methodology: Ninety-two subjects with PD underwent assessments of muscle tone, strength, and tendon reflexes in both upper extremities. Subsequently, finger tapping, Go/No-Go, and Flanker tasks were performed. Statistical tests were applied for the final analysis to determine the impact of muscle functions on movement timings and inhibitory control and evaluate relationships.

Results: The study revealed significant associations between finger tapping timings and muscle tone, strength, and tendon reflexes. Reaction timings increased with higher muscle tone scores and lower muscle strength scores. Tendon reflexes significantly influenced the dominating (right) side, with non-significant results for the non-dominant (left) side. Muscle functions also significantly impacted response inhibition performance in various ways.

Conclusions: Understanding the cognitive-motor network in PD suggests targeted rehabilitation strategies addressing hypertonia, muscle strengthening, and reflex optimization for effective management.

Abstract ID: 771: Neurological Complications following COVID-19 Vaccine

Kuljeet Anand, Jyoti Garg, Rahul Jain, Abhishek Juneja, Rakesh Kumar Mahajan

National Institute of Mental Health and Neuro-Sciences, Bengaluru (NIMHANS, Bengaluru), Karnataka, India

Background and Aim: During the COVID-19 pandemic, predominantly two vaccines, that is, ChAdOx1n Cov-19 (Covishield) and BBV-152 (Covaxin), were approved in India. This is a report of neurological complications following the COVID-19 vaccine from June 2021 to March 2022.

Case Report: A total of seven patients (M:F = 5:2; age range: 20–50 years) who developed neurological complications following the COVID-19 vaccine within 3–10 days were encountered at the tertiary level. All patients had received the Covishield vaccination.

Discussion: Neurological complications include three cases of Gullian–Barre syndrome (M:F = 2:1) presented within days 4, 6, and 10 of receiving the vaccine. Nerve conductions revealed acute motor axonal neuropathy. Two patients had acute ischemic strokes on days 3 and 4. The MRI brain showed medullary infarction in one and MCA infarction in the other. One patient had LETM on day 3 post-vaccination. The MRI spine showed involvement of the cervical and dorsal regions. One patient had Bell’s palsy following the first as well as the second dose of vaccine within 10 days of receiving vaccination.

Conclusions: Though COVID-19 vaccines are safe and efficacious, rare occurrences of serious neurological syndromes are observed following ChAd Ox1n Cov-19 vaccination.

Abstract ID: 772: Ethambutol-Induced Loss of Vision in a Patient on Antitubercular Drugs

Santosh Trivedi, Jyoti Kiran, Ashis Datta, A. Shobhana

Institute of Neuroscience Kolkata 6B, A J C Bose Road Kolkata, West Bengal, India

Background and Aim: Ethambutol-induced toxic optic neuropathy (EON) is a well-known potential side effect of ethambutol. Longer duration as well as higher doses predispose the patients to develop drug toxicity. Early recognition and discontinuation of the drug may prevent further deterioration. This study was taken up to create awareness among physicians about the vision-threatening side effects of ethambutol and the need to carefully assess the risk–benefit ratio while continuing this drug.

Methodology: A cross-sectional observational study including patients clinically diagnosed to have EON with a history of intake of anti-tubercular drugs including ethambutol within 1 year of symptom onset. Ocular and other neurological causes for visual loss were excluded meticulously. Demographic profile, clinical features, dose, and duration of ethambutol intake as well as latency for onset of clinical symptoms were recorded for all patients.

Results: A total of 12 patients (6 males and 6 females, mean age of 45 years) were included in our study, who had bilateral irreversible severe DOV over the course of 45 to 60 days, except 1 patient whose vision improved. The total duration of ethambutol intake ranged from 6 months to 18 months at doses varying from 800 to 1200 mg/day. MRI brain and orbit were normal in most, except one having chiasmal signal change.

Conclusions: Ethambutol toxicity may occur even with a standard dose and extended duration of ethambutol intake. All newly diagnosed TB patients must have a baseline ophthalmological examination and a periodic evaluation thereafter. The prescribing doctor and patient need to be well aware of EON.

Abstract ID: 773: Is Autoimmunity a Trigger or an Association in Motor Neuron Disease? Two Case Reports

Jenu Santhosh, Uma Maheshwari, Bala Subramanian, Natarajan S, Balasubramanian S

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Motor neuron diseases refer to a group of chronic sporadic and hereditary neurological disorders characterized by progressive degeneration of motor neurons. The possibility of autoimmunity in the pathogenesis of motor neuron diseases has been debated for years with little consensus. Here, we present two cases of amyotrophic lateral sclerosis with associated autoimmune diseases.

Case Report: Case 1: A 34-year-old woman presented with limb weakness and speech disturbances. The neurological assessment revealed facial weakness, dysarthria, thinning of the bilateral thenar and hypothenar muscles, bilateral dorsal interossei wasting, clawing of fingers of both hands, weakness of intrinsic muscles of both hands, bilateral mild lower limb weakness, brisk reflexes, and bilateral plantar extensor with muscle fasciculations with decreased touch, temperature sensation over both the hands-on dorsal and palmar aspects, with preserved vibration sense. Case 2: A 52-year-old female patient with no previous comorbidities presented with weakness of both upper limbs with speech disturbances with strained quality of voice along with dysphagia, which was more for liquids than solids. On examination, there was asymmetric weakness of the upper limbs with right more than left with bilateral mild lower limb weakness with spasticity of all four limbs along with wasting and fasciculations with exaggerated deep tendon reflexes and extensor plantar with spastic dysarthria and tongue fasciculations.

Discussion: Lip biopsy performed in case 1 revealed Sjogren’s syndrome. Electromyography revealed neurogenic damage. The patient received a course of high-dose intravenous corticosteroids, which was followed by an oral preparation. Evaluation of case 2 revealed elevated anti-thyroperoxidase antibody with features of thyroiditis in the ultrasonogram neck and elevated thyroid stimulating hormone (TSH levels. The patient was started on riluzole and thyroxine.

Conclusions: Autoimmunity as an association or trigger in motor neuron diseases needs further studies.

Abstract ID: 774: An Interesting Case of Pregnancy-related Tuberculous Meningitis Flare with Delayed Postpartum Paradoxical IRIS (Immune Reconstitution Inflammatory Syndrome)

Brijesh J. N., Nithyanandam A., Velusamy S.

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: TB meningitis accounts for 1–2% of all tuberculosis (TB) cases in immunocompetent individuals with a mortality as high as 30%. Pregnancy, being a state of systemic immunomodulation, leads to flare up of rheumatologic/infectious diseases such as TB. Here we report a young female in the postpartum period who presented with subacute meningoencephalitis with further clinical and radiological worsening after brief improvement in spite of anti-tubercular medications but began to improve after high-dose steroids, thereby suggesting delayed postpartum paradoxical immune reconstitution inflammatory syndrome (IRIS).

Case Report: This 29-year-old female patient in the postpartum period presented with subacute onset progressive and improving neurological illness in the form of altered sensorium with low-grade fever, weight loss, and secondary headache with visual disturbances, which improved with medications briefly followed by worsening that improved after the correction of communicating hydrocephalus with a ventriculoperitoneal (VP) shunt. The clinical course worsened further with increasing unsteadiness and altered sensorium. On examination, the patient was drowsy with bilateral gaze evoked nystagmus, and terminal neck stiffness with truncal and appendicular ataxia.

Discussion: Routine blood investigations revealed anemia, elevated ESR, and hyponatremia. Computed tomography (CT) brain showed diffuse cerebral edema with hydrocephalus. Initial magnetic resonance imaging (MRI) brain with contrast showed leptomeningeal enhancement in the basal cisterns. CSF analysis revealed low sugars, high protein, and high ADA with lymphocytic predominance with negative CBNAAT and fungal stains. The patient was started on ATT, after which there was improvement but followed by worsening. Repeat contrast imaging showed T2/FLAIR hyperintense lesions in the brainstem with multiple conglomerate ring-enhancing lesions—tuberculoma with TB meningitis. Repeat CSF analysis was consistent with TB. After ruling out MDRTB, the patient was continued on ATT with the addition of high-dose steroids with a slow taper, resulting in good clinical improvement without further deterioration and hence she was discharged with residual but improving neurological deficits.

Conclusions: Therefore, a high degree of clinical suspicion and timely initiation of appropriate treatment are necessary to prevent severe neurological morbidity and mortality. One has to consider screening for latent TB during pregnancy in endemic areas such as India to prevent devastating complications peripartally.

Abstract ID: 775: Longitudinal Study of Cognitive Profile after Status Epilepticus

Suman S. Khushwaha, Himanshu Shakya, Siddharth Maheshwari, Chandra Bhushan Tripathi, Ashwin Panda, Prateek Babel, Rajinder Dhamija

Institute of Human Behaviour and Allied Sciences, New Delhi, India

Background and Aim: Status epilepticus (SE) has potential wide-ranging after-effects, including cognitive dysfunction. The study aimed to assess the cognitive profile of patients after SE at three follow-up intervals, and also to identify the clinical predictors of cognitive functions in status epilepticus.

Methodology: A total number of 50 patients suffering from SE admitted to neurology were included in the study. Patients already having an additional alternative cause of cognitive impairment were excluded. They were evaluated 24 hours after the last seizure event, at 7 days, and 1 month. A set of cognitive tests including mini mental state examination (MMSE), Montreal Cognitive Assessment (MoCA), frontal assessment battery (FAB) and others were used for assessment.

Results: The study is currently under process, interim analysis found cognitive dysfunction initially in all patients. All patients (100%) had impairment in attention, calculation, recall, set-shifting, cognitive flexibility, processing speed, and lexical fluency. The majority of patients (>70%) had disorientation, executive dysfunction, and poor abstract thinking. The language part of the test was preserved in most of the cases (>80%), and deficits if any were mild. In the follow-up assessment, the majority of patients (>95%) had complete improvement in orientation, attention, calculation, and recall and partial improvement in visuospatial skill deficits, lexical fluency, abstract thinking, set-shifting, and processing speed. A small proportion of patients (<5%), out of the super-refractory SE group had severe and persistent cognitive deficits.

Conclusions: Status epilepticus causes cognitive dysfunction. The deficits improve gradually in most of the cases, but some long-term deficits are present in a minority of them. In status epilepticus, such as in stroke and myocardial infarction (MI), time is of utmost importance.

Abstract ID: 776: Prognostic Scores in Prediction of Outcome of Guillain-–Barre Syndrome in a Tertiary Care Hospital

Tintu Thomas, Prasanth C Varghese, Fiju Chacko

Jubilee Mission Medical College, Thrissur, Kerala, India

Background and Aim: Guillain–Barre syndrome (GBS) is an acute-onset immune-mediated monophasic polyneuropathy. Various prognostic models were needed to identify the patients with poor outcomes. The Erasmus GBS Respiratory Insufficiency Score (EGRIS) and the modified Erasmus GBS Outcome Score (mEGOS) are prognostic models used in the prediction of mechanical ventilation and functional outcome, respectively. The aim is to study the use of mEGOS in determining the poor functional outcome at 6 months and use of EGRIS in predicting the need for mechanical ventilation and to determine the various parameters predicting the outcome in GBS.

Methodology: This study was a prospective observational study in patients with GBS for determining the utility of prognostic models such as modified EGOS and EGRIS in determining the poor outcomes at a tertiary care hospital. In addition to the above, the clinical, demographic, investigational, and treatment parameters affecting the outcome were studied. All patients with a diagnosis of GBS fulfilled by the National Institute of Neurological Disorders and Stroke (NINDS) criteria were admitted within 2 weeks of onset of weakness to the Department of Neurology within a period of 18 months from the date on which institutional ethics committee clearance was obtained. A total of 58 patients were enrolled in the study. All patients underwent complete neurological examination, including Medical Research Council (MRC) sum score, nerve conduction study, and CSF examination. Informed consent was taken for each subject. Outcome was assessed by Hughes disability Scale (HMS). HMS is a functional rating from 0-6 (0-asymptomatic, 1- mild signs and symptoms but able to run, 2- able to walk unaided for 5 meters, 3- able to walk with support, 4- bed ridden or wheel chair bound, 5- requiring ventilator assistance and 6- death). The mEGOS and the EGRIS were conducted for all patients at admission. The mEGOS predicted the risk of being unable to walk independently within the first 6 months of disease based on age, muscle strength, and preceding diarrhoea. The EGRIS corresponds to an estimated risk of respiratory failure within the first week based on the time from the onset to admission, MRC sum score, and the presence of facial/bulbar weakness. GBS outcome score is predicted as a good outcome when the score is less than or equal to 2 and a bad outcome when it is more than or equal to 3. Enrolled patients were followed up for 6 months. Statistical analysis was carried out using SPSS version 21.0. Descriptive analysis was reported as the mean and standard deviation of continuous variables. Independent sample t -test and chi-square test were used to establish the association, P < 0.05 was considered as significant.

Results: In all, 58 patients (43 males; 15 females; mean age of 47.1 ± 18.6 years) were enrolled. Amongst these, 4 (6.9%) patients died during their in-hospital stay. Antecedent infection was present in 21 (36%), areflexia in 54 (93.1%), autonomic dysfunction in 6 (10.3%), facial and bulbar weakness in 23 (39.7%), neck flexor weakness in 14 (24.1%), albuminocytological dissociation in 52 (89.7%), respiratory insufficiency/mechanical ventilation in 10 (17.2%) patients. The mean mEGOS at admission was 4.9 ± 3.1 and EGRIS was 3.1 ± 1.39. The mean MRC sums core at admission was 42.2 ± 10.4. 49 cases (84.5%) were demyelinating and 9 cases (15.5%) were axonal patterns. On analysis, predictors associated with poor outcome at 6 months were age ( P = 0.001), female gender ( P = 0.016), MRC sum score at admission ( P = 0.021), autonomic dysfunction ( P = 0.030), mechanical ventilation/respiratory insufficiency ( P = 0.001), and demyelinating pattern ( P = 0.007). Higher mEGOS at admission was a predictor of poor outcome ( P = 0.035) and EGRIS at admission of 4.8 ± 0.7 predicted the need for ventilation ( P = 0.001).

Conclusions: EGRIS and mEGOS are good prognostic models for determining the need for mechanical ventilation and functional outcome. Detailed evaluation of clinical and electrophysiological parameters may help in predicting the outcome.

Abstract ID: 777: Clinicoradiological Profile and Outcome of Acute Ischemic Stroke Patients Undergoing IV Thrombolysis with Tenecteplase in a Tertiary Care Center

Navya Jyothi V, Fiju Chacko, Harisuthan T

Jubilee Mission Medical College and Research Institute, Thrissur, Kerala, India

Background and Aim: Tenecteplase is an approved engineered recombinant tissue plasminogen activator for the treatment of acute ischemic stroke with molecular and practical advantages. More studies from developing countries such as India will help determine the ethnicity-associated clinical-radiological profile, improve the outcome of IV thrombolysis, and counter the stroke epidemic in our country. The study aims to determine the clinico-radiological profile and outcome of patients with acute ischemic stroke (AIS) undergoing IV thrombolysis with tenecteplase.

Methodology: This was a prospective observational study of stroke patients treated with tenecteplase. On admission, clinical characteristics, temporal, epidemiological, imaging parameters, outcome measures including baseline National Institutes of Health Stroke Scale (NIHSS), 24 hours, at discharge, and modified Rankin Scores (mRS) at 0, discharge, and 3 months were recorded in a structured proforma.

Results: Out of the 107 patients, 72% were males. The mean age of the study population was 62.4 years. Hypertension (52.3%) was the most common risk factor. Time parameters such as the mean onset-to-door time and door-to-needle time were 120.3 min and 49.6 min, respectively, which were confirmed with the international standards. The mean NIHSS was 8.1 ± 4.8 and serial improvement was noted in the post-treatment mean NIHSS scores at 24 h and discharge were 7.6 and 6.4 respectively. The mean mRS score was 3.54 ± 1.4 and the follow-up mRS at discharge and 3 months were 2.9 and 1.22, respectively. Haemorrhagic complications occurred in 14%.

Conclusions: Tenecteplase is a safe and efficacious agent for stroke thrombolysis, suitable for stroke thrombolysis in resource-limited settings, considering its cost-effectiveness and ease of administration.

Abstract ID: 778: Toxic Neuropathies Presenting Like GBS: Two Case Reports of Lead and Tacrolimus Toxicity

Patibandla Sipra, Raja Gambeeran, M. Jawahar, Velusamy Subramanian

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: Toxic neuropathies result from substances such as drugs, heavy metals, and toxins damaging the peripheral nerves. Most toxic neuropathies are axonal, and sensory predominant, but can present acutely with motor involvement. Here we present two cases of toxin-induced neuropathy-lead and tacrolimus.

Case report: Case 1- A 16-year-old female patient presented with weakness in both lower limbs and then over the next 10 days developed weakness in both upper limbs, which was ascending and reached nadir for the last 2 weeks. O/E- The patient was found to have hypotonia in all four limbs, quadriparesis, with areflexia. History of intake of herbal medicine for 30 days prior is present. Case 2- A 29-year-old female patient, known case of hypertensive, chronic kidney disease (CKD), underwent renal transplant in August 2023 and started on mycophenolate mofetil (MMF) and tacrolimus. Three months later, she developed weakness in both lower limbs followed by both upper limbs over 2 days and breathlessness. O/E -The patient was found to have hypotonia in all four limbs, quadriparesis, and areflexia with neck weakness.

Discussion: Case 1- Nerve conduction study (NCS) showed a demyelinating pattern. Her blood samples were sent for toxicological analysis and found to have elevated levels of lead (25 µg/dL). The patient was treated with five cycles of plasmapheresis and showed significant improvement. Case 2- NCS was suggestive of demyelinating polyradiculopathy. She was given five cycles of plasmapheresis with no significant improvement and persistent weakness. Tacrolimus toxicity was suspected, levels were found to be elevated and it was withheld with the patient showing good recovery.

Conclusions: Toxic neuropathy should be considered as a differential diagnosis in patients presenting like Guillain–Barré syndrome (GBS) with herbal medicine use/on tacrolimus.

Abstract ID: 779: Management of Refractory Trigeminal Neuralgia

Praveen Kumar, Nagalaxmi Patnala

KIMS Hospital, Hyderabad (KIMS, Hyderabad), Telangana, India

Background and Aim: Trigeminal neuralgia is one of the most painful conditions affecting people and patients seek relief from this dreaded condition in all ways possible; even surgery may not offer solace in many patients. This retrospective study explores the various minimally invasive measures that can help such patients. The aim is to study the minimally invasive procedures in the management of refractory trigeminal neuralgia.

Methodology: All patients presenting to the Neurology and Pain Medicine OPD with refractory trigeminal neuralgia were included in the study. Various procedures performed were left to the discretion of the pain physician who performed them after explaining all the treatment options. The visual analog scale (VAS) was used to assess the pain relief. Due consent was taken before each procedure.

Results: A total of 26 patients were included in the study over 1 year. Peripheral nerve block and radio-frequency ablation were conducted in 14 patients and Gasserion ganglion radio-frequency ablation for 7 patients. Botulinum toxin administration locally was done for 5 patients. Complications- Two patients needed repeat radio frequency ablation. Five patients had mild facial swelling, which subsided in a few hours. VAS scores decreased by 50% in all patients. Medication could be reduced to a single dose of oxcarbazepine.

Conclusions: Minimally invasive pain relief procedures are simple, durable, efficient techniques to manage refractory trigeminal neuralgia.

Abstract ID: 780: A Rare Association of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) and Coronary Artery Disease

Sankavi K, Shobana N, Socratis M, Selvakumar C. J., Sadeeshkumar V

Affiliation

Background and Aim: The cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a heritable small-vessels angiopathy caused by mutations in the NOTCH3 gene located on chromosome 19. Neurological manifestations of CADASIL are well-established; however, there is a growing recognition of the importance of coronary artery disease (CAD) in CADASIL. Patients with this condition are at higher risk for developing premature and extensive coronary artery disease. The study aims to highlight the rare association of CADASIL and CAD.

Case report: We report a case of a 55-year-old male patient who presented with recurrent migrainous headaches for 25 years of age with a history of dysarthria and emotional incontinence for 32 years of age. He had a history of memory disturbance for 36 years of age. Now, he was admitted with acute anginal symptoms. Family history revealed extensive similar symptoms. Lobar function examination showed dysfunction of the bilateral frontal cortex and bilateral temporal lobe with subcortical dementia. Neurological examination revealed a pseudobulbar effect with bipyramidal signs.

Discussion: All routine investigations were within limits. Electrocardiogram (ECG) showed acute ST elevation in anterior leads. Urgent coronary angiogram revealed diffuse narrowing of the proximal left anterior descending artery showing 95% type A lesion and deployed with a drug-eluting sirolimus stent. The magnetic resonance imaging (MRI) brain T2 and fluid-attenuated inversion recovery (FLAIR) showed bilateral symmetrical hyperintensity involving periventricular and subcortical white matter extending to the anterior temporal pole. The background of positive family history and clinical findings prompted us to evaluate inheritable arteriopathy of small vessels and we proceeded with the NOTCH 3 gene test, which was consistent with CADASIL. The patient was treated with dual antiplatelets, angiotensin-converting enzyme inhibitors (ACEIs), high-dose lipid-lowering agents, and speech therapy.

Conclusions: Further insights into extracranial vasculopathy of CADASIL may help in the development of new targeted therapies.

Abstract ID: 781: Thinking Out of the Box: Case Report of a Rare Disease

Shreyasi Banerjee, Amrit Chattopadhyay

Medical college, Kolkata, West Bengal, India

Background and Aim: Lipoid proteinosis (Urbach–Wiethe disease) is a rare autosomal recessive disorder characterized by abnormal hyaline deposition in the skin, mucous membranes, brain, and other body parts. Here we like to report a case of lipoid proteinosis admitted to our hospital.

Case report: Necessary data were collected from the patient and care giver by adequate history taking, and routine general and neurological examination. Evaluation of the case was done by routine blood examination, magnetic resonance imaging (MRI) of brain, fibre-optic laryngoscopy (FOL), and skin biopsy. Finally, confirmation of the diagnosis was made with the help of genetic testing (whole-exome sequencing). Our patient, a 13-year-old male patient, born out of a consanguineous marriage presented to us with h/o progressive hoarseness of voice, slurring of speech, dysphagia to solid, and poor cognitive development.

Discussion: Examination revealed a scar of a previous vesicular skin lesion, papules along the eyelid margin, and gross thickening of the tongue and oral mucosal membrane. FOL revealed a thickening of the vocal cord. Skin biopsy showed periodic acid-schiff (PAS)-positive deposition. MRI brain showed b/l basal ganglia calcification. Whole-exome sequencing was sent but the report is awaited.

Conclusions: Here in this case, though neurological symptoms were there, the diagnosis was made by approaching the case with non-neurological clues. So, while evaluating cases, especially the rare ones, we must focus on signs and symptoms beyond the neurological purview that may help us to reach the diagnosis.

Abstract ID: 782: Vasculitis as a Cause of Peripheral Neuropathy in Sjogren’s Syndrome: A Case Report

Shiny Thomson, Jawahar M, Balasubramanian S, Nandhakumar V

Madras Medical College, Chennai (MMC, Chennai), Tamil Nadu, India

Background and Aim: Sjögren’s syndrome is a systemic autoimmune condition affecting exocrine glands and various organs, with peripheral neuropathy prevalence ranging from less than 2% to over 60%. The clinical spectrum of peripheral neurologic involvement in Sjögren’s syndrome is wide and all segments of the peripheral nervous system motor, sensory, or autonomic alone or in combination may be affected. The origins of this type of neuropathy, whether autoimmune or non-immune, remain unclear.

Case report: We report a case of a 42-year-old woman with primary Sjögren’s syndrome, presenting with progressive numbness and pins-and-needles sensation in her feet, followed by a burning sensation. After 1.5 months, she developed worsening distal lower limb weakness and polyarthralgia. Clinical examination showed no overt signs of systemic vasculitis, but significant distal lower limb weakness and complete sensory loss were present below both ankles. Laboratory tests revealed positive indirect immunofluorescent antinuclear antibodies (IIF-ANA) and extractable nuclear antigens (ENA) screening for anti-SSA/Ro 60-3+ and Ku antibodies. A nerve biopsy indicated vasculitis suggestive of vasculitic neuropathy.

Discussion: The patient was treated with steroids and intravenous cyclophosphamide.

Conclusions: This case highlights the varied presentations of primary Sjögren’s syndrome and its possible pathogenesis.

Abstract ID: 783: Cracking the Code: Unveiling the Clinical Mysteries and Triumphs of Arterial Dissections and Dissecting Aneurysms

Pranav Mehta, Sankar Prasad Gorthi, SreeHari Dinesh, Neha Mohite, Sumit Kharat, Chetna Patil, Dulari Gupta

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

Background and Aim: Arterial dissection presents diagnostic and therapeutic challenges, requiring a nuanced understanding of its clinical spectrum. This single-centre observational study aimed to explore the clinical profiles of patients diagnosed with dissecting aneurysms within the broader context of arterial dissection, focusing on demographic characteristics, clinical presentations, treatment modalities, and outcomes.

Methodology: From January 2023 to January 2024, we collected data on patients diagnosed with arterial dissection, with a subset identified as having dissecting aneurysms. Comprehensive data encompassing demographics, presenting complaints, comorbidities, vessel involvement, treatment regimens, and functional outcomes using the modified Rankin Scale (mRS) were analysed. Brain and vessel imaging were conducted as per the institute protocol, with financial constraints limiting uniform testing.

Results: Out of a total of 16 patients identified, 14 were males with a mean age of 45.8 years. They commonly complained of giddiness and vomiting. Nine of them involved posterior circulation (2 basilar, 1 posterior inferior cerebellar artery (PICA), and 6 involved the vertebral artery), and seven anterior (internal carotid artery-ICA). Four cases were those of dissecting aneurysms. Diabetes and hypertension (4 each) were common risk factors. Eleven patients were given dual antiplatelet therapy (DAPT) and five received anticoagulation, followed by DAPT. At 3 months of follow-up, 13 cases had modified Rankin scale (mRS) of 0 with 1 each of mRS 4, 5, and 6.

Conclusions: Dissections are a rare cause of stroke, often presenting atypically. Successful outcomes achieved through precise interventions underscore the importance of considering this entity in the differential diagnosis of stroke, particularly in cases with unusual clinical presentations. Pseudoaneurysms is a rarer entity and there is a need to profile these patients.

Abstract ID: 784: Diagnostic Features of Nerve Conduction Studies in Patients with Systemic and Non-systemic Vasculitic Neuropathy as Compared to Other Non-Inflammatory Axonal Polyneuropathies: A Retrospective Observational Study

Hamsa B T, Archana B. Netto, Praveen Kumar, Pramod K, Janardhan D C

Bangalore Medical College and Research Institute, Bengaluru (BMCRI Bengaluru), Karnataka, India

Background and Aim: In vasculitic neuropathy (VN), a 50% side-to-side difference in the amplitude of compound muscle action potentials has been proposed but the evidence is lacking. The aim of this study was to characterize electrodiagnostic features that best distinguish VN from other non-inflammatory axonal polyneuropathies.

Methodology: We conducted a retrospective observational study between September 2016 and April 2024. We reviewed the records of patients with VN who had bilateral nerve conduction studies (NCS) and evaluated different electrodiagnostic models to help distinguish VN from other non-inflammatory axonal polyneuropathies. Inclusion criteria: Cases- patients aged above 18 years with biopsy-proven VN. Controls- Polyneuropathy secondary to diabetes mellitus, ethanol, toxin-induced neuropathy, vitamin B12 deficiency with baseline nerve conduction study.

Results: We identified 25 cases and 25 controls with non-inflammatory axonal neuropathies. The amplitude percent difference Z score model did not show discriminatory capability between cases and controls. The conduction velocity (CV) percent difference Z score of posterior tibial nerve (PTN) showed the best discriminatory capability between cases and controls (area under the curve [AUC]: 0.789; 95% confidence interval [CI]: 0.606, 0.972). The CV absolute difference Z score of PTN showed the second-best discriminatory capability between cases and controls (AUC: 0.781; 95% CI: 0.595, 0.968).

Conclusions: As proposed by multiple studies, VN has a 50% difference in side-to-side amplitude. This study did not show any significant discriminatory capability between cases and controls for the proposed hypothesis.

Abstract ID: 785: MRI Features, Clinical Profile, and their Relationship with Functional Outcomes in Posterior Circulation Stroke

Pazhaniyandi K, Sriramakrishnan V, Jason Ambrose

Tirunelveli Government Medical College, Tirunelveli, Tamil Nadu, India

Background and Aim: Posterior circulation stroke is an important neurological condition and its most common features are ataxia, fluctuating sensorium, and cranial nerve involvement. Posterior cerebral artery (PCA), anterior inferior cerebellar artery (AICA), and posterior inferior cerebellar artery (PICA) are the common arteries involved. The etiology is atherosclerosis followed by cardioembolism. The study aims to throw light on radiological and clinical features of posterior circulation strokes and correlate with functional outcomes.

Methodology: Cross-sectional study with samples being patients who attended the neurology department with posterior circulation stroke from 2023 to 2024. Based on previous studies, the sample size was computed as 93 using the formula: n= Z 2 * p* (1-p) *1/e 2 . The patients with symptoms of posterior circulation stroke with age 18 to 80 years confirmed by magnetic resonance imaging (MRI) brain studies were included and those with recurrent stroke were excluded. The National Institutes of Health Stroke Scale (NIHSS) score at admission, symptomatology, risk factor status, and radiological features were observed. Functional status was implied by modified Rankin scores at the time of discharge.

Results: Among 93 patients, 59 were males and 34 were females. The maximum age group- 6th decade. In all, 42 had hypertension and 30 had diabetes mellitus. Common symptoms were giddiness and vomiting followed by headache, altered sensorium, and cranial nerve deficits. Large artery stroke was the commonest observed in 65, lacunar stroke in 20, and cardioembolic in 8 patients. In functional outcomes, no disability was observed in 18, minor in 62, major in 9, and 4 patients died.

Conclusions: Males were affected more and the age commonly involved was above 60 years. Hypertension, diabetes, and smoking contributed as the major risk factors. Giddiness was the commonest symptom. Most patients had cerebellum and brainstem lesions. The territory commonly involved was mid-brain, cerebellum, thalamus, and occipital and temporal lobes.

Abstract ID: 786: Perampanel as an Add on Drug in Adults with Uncontrolled Seizures

Amarapu Sravanthi, Randhi Venkata narayana, Chebolu Harshika, Bandaru Balakrishna, Kakarala Pradeep

Sevenhills Hospital, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Nearly 25–30% of patients with epilepsy are refractory to treatment due to various factors. Anti-seizure drugs with novel anti-epileptic mechanisms are always welcome to the treatment armamentarium. Perampanel was launched in India in August 2017 as an add-on for focal onset seizures with or without secondary generalization in adults. Perampanel is the first and only non-competitive α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptor antagonist specifically engineered to block glutamate activity at postsynaptic AMPA receptors. The AMPA receptor is the predominant mediator of excitatory neurotransmission in the brain, implicated in the generation and spread of epileptic activity. Perampanel is tried as an add-on in some of our patients with refractory epilepsy for its effectiveness and tolerability.

Methodology: Forty patients (22 males/18 females) of 18 to 65 years of age with refractory epilepsy presented to the neurology out-patient clinic between January 2018 and December 2023 and were treated with perampanel as an add-on were analysed.

Results: Perampanel was added as a second add-on starting with 2 mg once a day and the dose was built up to 4 mg after 3 weeks. Patients were followed at regular intervals and analysed at the end of 3 months, 6 months, 1 year, and 2 years.

Conclusions: Perampanel is tolerated well and reduces seizure frequency in adults with refractory epilepsy.

Abstract ID: 787: Demographic and Clinical Profiles of Immunologically Mediated CNS Demyelinating Diseases in a Tertiary Care Hospital

Tuhin Santra, Sandip Pal, Debsadhan Biswas, Indranil Datta, Ankur Banik

Kolkata Medical College, Kolkata, West Bengal, India

Background and Aim: To study clinical and radiological profiles of patients with immunologically mediated central nervous system (CNS) demyelination.

Methodology: An observational cross-sectional study over a period of 6 months was conducted in patients admitted with immunologically mediated CNS demyelinating disorder.

Results: In all, 36 patients (10 males & 26 females) were included. Optic neuritis was detected in 24 patients (66.7%), among which 16 were females (66.7%). Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) & neuromyelitis optica spectrum disorder (NMOSD) were diagnosed in 11 (45.8%) and 5 (20.8%) patients, respectively, the rest were seronegative (33.3%). Myelopathy was found in 12 patients, 10 of whom were females (83.3%). Three patients were positive for neuromyelitis optica (NMO) and myelin oligodendrocyte glycoprotein (MOG) each (25%), four were seronegative (33.3%) and one each was diagnosed with relapsing-remitting multiple sclerosis (RRMS) and spinal clinically isolated syndrome (CIS). Optico-spinal involvement was detected in one NMO and seronegative patient each. Cerebral involvement was noted in four patients, two of which were of RRMS, one had acute disseminated encephalomyelitis (ADEM), and one had NMOSD.

Conclusions: MOGAD is the commonest demyelinating disorder in this part of India and optic neuritis is the commonest mode of presentation. However, the pattern of optic nerve involvement is probably not helpful in differentiating various types of CNS demyelinating disorders.

Abstract ID: 788: Clinico-etiological Profile of Patients with Movement Disorder Emergencies: A Case Series of 26 Cases

Dinesh Manna, Ankur Banik

Kolkata Medical College, Kolkata, West Bengal, India

Background and Aim: Although acute movement disorders are often encountered, the literature is sparse, especially in our socio-demographic background. In this study, we describe 26 cases of movement disorder emergencies.

Case report: Patients attending the Medical College, Kolkata, with movement disorder as the sole or predominant presentation within 4 weeks of disease onset were enrolled in our study after taking proper consent. Detailed history, examination, and relevant investigations were obtained.

Discussion: Amongst 26 patients, 21 had hyperkinetic disorders, whereas there were only three cases of hypokinetic and two mixed movement disorders. Among the hyperkinetic group, phenomenologically, tremor was most common, followed by chorea, dystonia, and myoclonus. Tardive dyskinesia, opsoclonus myoclonus ataxia, hemiballismus, and ataxia were also noted. Of the three patients with hypokinetic presentation, two had catatonia and one had drug-induced Parkinsonism. The two cases with mixed phenomenology were all drug-induced acute parkinsonism with cranio-cervical dyskinesia and oculogyric crisis. Etiologically, we encountered eight drug-induced conditions, four patients with stroke, three cases of post-infective immunologically mediated movement disorders, two patients each with hyperglycemia, functional movement disorder, neuropsychiatric disorder, idiopathic, and one case each of post-hypoxic myoclonus, status dystonicus presentation of pantothenate kinase-associated neurodegeneration (PKAN) and autoimmune encephalitis.

Conclusions: Movement disorder emergencies vary widely in phenomenology and etiology. Vascular and metabolic causes predominantly have hyperkinetic phenomenology, whereas neuropsychiatric causes have hypokinetic presentation. Drug-induced causes have a mixed picture with preferential cranio-cervical involvement.

Abstract ID: 789: A Study of Clinical Presentation and Etiology in Patients with Nontraumatic Convexal Subarachnoid Haemorrhage

Santhosh Arepalli, Velusamy Subramanian, Jawahar Marimuthu, Nanda Kumar Vasudevan, Nithyanandam Allimuthu, Aravind Gautham V, Balasubramanian Samivel

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: Convexal subarachnoid haemorrhage (cSAH) represents an infrequent type of spontaneous, non-traumatic, and non-aneurysmal SAH distinguished by the bleeding into the convexities of the brain sulci without parenchymal involvement; the etiology varies. The aim of our study pertained to the analysis of the etiology and clinical manifestations observed in individuals who present with cSAH.

Methodology: All consecutive patients admitted to our hospital with non-traumatic non-aneurysmal subarachnoid hemorrhage, from September 2023 to April 2024 were included in the study. Demographic features, clinical characteristics, complementary investigations, etiology, and mortality were assessed. A total of 15 patients were identified and analysed.

Results: The mean age was 46 years (range, 23-76 years). Out of 15 patients, 6 (40%) were females. Symptoms on admission: headache (87%), sensory and/or motor symptoms (33%), and seizures (33%). The commonest causes were cerebral venous sinus thrombosis (73%), posterior reversible encephalopathy syndrome (13%), and arteriovenous malformation (7%). One patient died.

Conclusions: Convexal subarachnoid haemorrhage may be associated with a broad range of etiologies. Within our patient population, a higher incidence of cerebral venous sinus thrombosis was noted. The mortality rate was minimal and not directly linked to the haemorrhage itself.

Abstract ID: 790: CT Volume and Biomarker (Carcinoembryonic Antigen) Level Correlation with Severity of Acute Ischemic Stroke

Archana Verma, P. A. Hemash, Ashutosh Mishra

All India Institute of Medical Sciences, Raebareli (AIIMS, Raebareli), Uttar Pradesh, India

Background and Aim: There is evidence that has shown that carcinoembryonic antigen (CEA) levels are increased in atherosclerosis. To the best of our knowledge, there is no published literature where it has been used to assess the correlation between the severity of acute ischemic stroke (AIS) with infarct volume on computed tomography (CT) scan and CEA level. The aim is to study the correlation between CEA levels and infarct volume and severity of AIS.

Methodology: We conducted a hospital-based, observational study, and all the consecutive patients of AIS (>18 years of age) presenting within 1 week were enrolled and detailed clinical examination, investigation, and CEA levels were done within 7 days. We had also done CEA in healthy subjects between 45 and 75 years of age with no chronic diseases.

Results: Fifty-seven consecutive AIS cases were recruited in the study. The age group of cases enrolled in the study ranged from 30 to 85 years with a mean age of 61.56 ± 13.17 years. Among them, 39 (80.6) cases were male. The mean infarct volume on CT scan was 15.73 ± 20.86 cm 3 . Baseline Glasgow Coma Scale (GCS) was good, GCS (13–15) in 28 (77.8) cases and the mean modified ranking scale (mRS) was 3.94 ± 0.583 and the National Institutes of Health Stroke Scale (NIHSS) score was 6.86 ± 4.89. The mean serum CEA levels in AIS cases and healthy controls were 5.09 ± 3.275 and 2.02 ± 0.743 ng/mL, respectively ( P < 0.01). A significant difference in CEA levels was observed between cases and controls when the test was applied. No significant association was observed between CEA levels and NIHSS scores at admission, mRS, or with GCS. No significant association was observed between the infarct volume on CT scan and CEA levels.

Conclusions: The CEA levels are higher among AIS patients than in the normal population. However, no significant association was observed between CEA levels and NIHSS score at admission, mRS, or with GCS and infarct volume on CT scan.

Abstract ID: 791: Sanskrit Vedic Chants Intervention is Associated with Beneficial Behavioral and Biological Changes in Children with Autistic Spectral Disorders

Sunil K. Narayan, Priyadarshini K, Sivanand Kattimani, Venkatesh Chandrasekhar, Susrut Badhe

SCTIMST, Thiruvananthapuram, Kerala, India,

Background and Aim: Autism is a neurodevelopmental disorder resulting from neurohumoral and synaptic aberrations. Effective interventions are scanty and empirical therapies continue as desperate measures to manage the condition. Grosswald and Travis (2011) have shown that reciting Sanskrit, an ancient and phonetically pure language improves brain functions. We aimed to investigate the behavioral and biological effects of Vedic chants interventions in children with autism.

Methodology: Baseline demographic and risk factors, Childhood Autism Rating Scale (CARS) II scores, Sleep Quality Scores (SDSS), and Parental Anxiety Scores (PSS) were recorded. All cases underwent exposure to the Vedic chants intervention program (VCIP) 1 h/day x 6 days a week x 4 weeks. In all cases, post-exposure CARS scores, SDSS, and PSS were documented. In all, 32 cases had pre-and post-exposure salivary enzyme linked immunosorbent assay (ELISA) for cortisol, adrenaline, dopamine, serotonin, melatonin, and oxytocin. In all, 26 cases had pre-and post-exposure digital spectral electroencephalogram (EEG) evaluation. Paired t -tests between pre-and post-exposure values were used to evaluate the interventional effects. In all, 49 cases underwent magnetic resonance imaging (MRI) brain studies and 50 had genetic tests.

Results: There was a modest but significant beneficial improvement in all CARS domains, and SDSS scores but no significant differences in PSS. There was a trend for a fall in salivary cortisol, dopamine, melatonin, and serotonin levels, and rise in oxytocin, and significant for serotonin. Post-intervention spectral EEG showed less variability of slow rhythms.

Conclusions: Sanskrit VCIP induced behavioral and biological changes in autistic children calling for more robust controlled studies.

Abstract ID: 792: Autosomal Recessive Axonal Motor Neuropathy Caused by HINT1 Mutation: New Association of a Psychiatric Disorder to Neurology Phenotype

V. V. Ashraf, Paul Johny, Divya Pachat, Suresh Kumar PN, Paul J Alapatt

Aster MIMS Hospital Kottakkal, Malappuram, Kerala, India

Background and Aim: Recessive loss of function mutations in the HINT1 gene is associated with predominantly motor neuropathy with neuromyotonia (NMAN). Around 19 variations in the HINT1 gene are reported in around 100 patients worldwide.

Case report: We report a HINT1 neuropathy with neuromyotonia from South India identified by whole-exome sequencing. A 17-year-old adolescent male presented with a 2-year history of progressive weakness of lower limbs, mainly involving the proximal muscles. He also reported muscle cramps. He was the second born of consanguineous parents and had a global developmental delay. He had behavioural issues and hyperactivity and was on psychiatric medications from the age of 5 years. On examination, he was intellectually disabled (IQ-73), had proximal weakness of lower limbs, and absent deep tendon reflexes. Blood investigations showed elevated creatine phosphokinase (1123 IU). Electroneuromyography (ENMG) revealed motor axonal neuropathy of all limbs and neuromyotonia in upper and lower limb muscles. A whole-gene exome sequencing revealed a mutation in the HINT1 gene (c242G>A p.(Gly81Asp).

Discussion and Conclusions: In a patient with young-onset neuropathy with features of hereditary neuropathy with neuromyotonia, one should keep the possibility of HINT1 gene mutation, which can be easily missed on routine investigations. This is not a pure peripheral nervous system disease, and patients can have behavioral and developmental problems.

Abstract ID: 793: Benefits of Bhramari and Prana Mudra in Reducing Awakening During Sleep: Study of Sixty Patients

Prof. R. Pazhani, Bavani Pazhani

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: Bhramari Pranayama and Prana Mudra are yoga methods, which have multiple benefits. The aim of this study is to find out its benefits in people with frequent awakening at night with no underlying co-morbid conditions.

Methodology: Sixty patients with a minimum of two awakenings at night in the age range of 30 to 50 years were taken for this study. Thirty-five were males and 25 were females. All were of normal built, with no snoring at night, no other sleep disorder symptoms, and no co-morbid conditions including anxiety and depression. Due to awakening at night, they felt not fresh in the morning. All 60 patients were taught Bhramari Pranayama and Prana Mudra. After 1 month and after 3 months, they were reviewed. They preferred not to be on sedatives.

Results: All sixty patients were reviewed after 1 month and after 3 months. After 3 months, all of them felt no sleep awakening. They were fresh in the morning with no fatigue and dullness like before after the intervention with the above yoga methods.

Conclusions: The Bhramari Pranayama and Prana Mudra benefitted all patients in this study. There was no awakening during sleep at the end of 3 months in this study patients. Bhramari Pranayama and Prana Mudra are beneficial to patients with frequent sleep awakenings.

Abstract ID: 794: Risk of Atherosclerosis among Patients with Epilepsy on Anti-Seizure Medications

Abhinav Ankur, B K Bajaj

Vardhman Mahavir Medical College (VMMC), New Delhi, India

Background and Aim: Epilepsy is one of the commonest neurological disorders affecting approximately 70 million people worldwide. The prevalence of epilepsy in India in 2020 was 4.7 per 1,000 population. The commonly used anti-seizure drugs are phenytoin, valproic acid, carbamazepine, and levetiracetam. This study investigates the risk of atherosclerosis in patients taking anti-seizure medications for over 2 years. Atherosclerosis is a potential side effect of these medications, and this study uses biochemical markers and carotid artery intima-medial thickness (CA-IMT) to assess this risk.

Methodology: The study was carried out in the Department of Neurology in collaboration with the Department of Radiology, Vardhman Mahavir Medical College, and Safdarjung Hospital. Fifty patients of epilepsy aged 12–40 years and on anti-seizure medications (ASM) and 50 healthy controls were recruited in the study. Detailed medical history and physical exams were conducted on both groups. Blood tests assessed general health and cholesterol levels. Ultrasound scans measured the thickness of the carotid artery walls, a marker of atherosclerosis risk.

Results: The mean age of patients and controls were 22.58 (±6.97) years and 23.5 (±5.79) years respectively ( P = 0.475). In the patient group, 28% were females and 72% were males, whereas in the control group, these percentages were 34% and 66%, respectively. There was a significantly higher frequency of overweight individuals among the cases ( P = 0.019). Patients had significantly higher levels of total cholesterol ( P = 0.001), non-HDL cholesterol ( P = 0.001), and LDL:total cholesterol ( P = 0.001) compared to controls. There was no significant difference in triglyceride level, or LDL cholesterol between the patient group and the control group. The examination of carotid artery intima-media thickness (CA-IMT) among patients treated with various anti-seizure medications (levetiracetam, phenytoin, carbamazepine, and valproate) in comparison to the control group revealed significant differences ( P = 0.0001). The mean±SD of CA-IMT was 0.65 ± 0.12 mm for levetiracetam, 0.61 ± 0.19 mm for eptoin, 0.68 ± 0.15 mm for carbamazepine, and 0.7 ± 0.15 mm for valproate.

Conclusions: We concluded that there is an increased risk of atherosclerosis in patients on ASM monotherapy for more than 2 years as compared to age- and sex-matched healthy controls of age 12 to 40 years as there was greater CA-IMT and dyslipidemia in the patient group as compared to the control group.

Abstract ID: 795: A Comprehensive Review of Myasthenia Gravis Cases in Tamil Nadu: Clinical and Etiological Perspectives from a Tertiary Hospital

Arvind Visweshwar, Murugan, Ramu

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder characterized by varying degrees of skeletal muscle weakness. This study aims to analyse the etiological factors and clinical presentations of MG in 15 patients treated at a tertiary care hospital. The results highlight the diversity in clinical manifestations and underlying causes, underscoring the need for individualized diagnostic and therapeutic approaches.

Case report: A retrospective analysis was conducted on 15 patients diagnosed with MG at a tertiary care hospital from May 2021 to December 2023. Data collection: patient records were reviewed for demographic information, clinical presentation, diagnostic tests, treatment received, and outcomes. Data on the presence of autoantibodies (anti-AChR, anti-MuSK), thymic abnormalities, and other associated autoimmune conditions were also collected. Diagnostic criteria: Diagnosis of MG was based on clinical features, serological tests for specific antibodies, and electrophysiological studies including repetitive nerve stimulation (RNS).

Discussion: In this case series of myasthenia gravis at a Tamil Nadu tertiary hospital, 66.7% had anti-AChR antibodies, 20% had anti-MuSK antibodies, and 13.3% were double seronegative. Thymic pathology included thymoma (13.3%) and thymic hyperplasia (33.3%). Associated autoimmune disorders were found in 40%, with thyroid disorders most common. Treatment involved acetylcholinesterase inhibitors, prednisone, steroid-sparing agents, intravenous immunoglobulin (IVIG)/plasmapheresis, and thymectomy in selected cases. Diagnostic tests included RNS and chest imaging.

Conclusions: Understanding the diverse etiological factors and clinical presentations of MG is crucial for optimizing patient management. This study emphasizes the importance of a comprehensive diagnostic approach and personalized treatment plans to improve patient outcomes in MG.

Abstract ID: 796: Antibody-Associated Autoimmune Encephalitis: A Prospective Single Center Study

Degapudi Sruthi, Sruthi. Degapudi, Uma Maheshwari E, Nithyanandam A, Aravind Gautham, Jawahar M, Velusamy S, Balasubramanian S

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: Autoimmune encephalitis is being increasingly recognized as a cause of encephalitis, in which antibodies target neuronal antigens, leading to a range of symptoms including neuropsychiatric disorders, seizures, and/or abnormal movements.

Methodology: Patients with antibody-positive autoimmune encephalitis, between June 2022 and May 2024 from Rajeev Gandhi Government General Hospital (RGGGH) were recruited. Patients were treated according to the current practice guidelines and data regarding clinical features, and outcomes were gathered.

Results: Nineteen patients were identified with autoimmune encephalitis at presentation ranging from 13 to 55 years. The most common type of encephalitis was N-methyl-D-aspartate (NMDA) receptor encephalitis ( n = 11). Others included leucine-rich glioma-inactivated 1 (LGI1) ( n = 2), ma-2 ( n = 3), contactin-associated protein (CASPR) ( n = 1) and yo ( n = 2).

Conclusions: This study found that paraneoplastic encephalitis has a poor prognosis in keeping with previous studies and that NMDA encephalitis is most amenable to first-or second-line immunotherapy.

Abstract ID: 797: Clinical and Radiological Characteristics and Outcomes in New-Onset Refractory Status Epilepticus: A Prospective Study in a Tertiary Care Center

Chadalawada Anuhya, Rithvik - Ramesh, Deepa - Avadhani, P.Philo - Hazeena, S.Sundar - -, R Lakshmi Narasimhan

Sri Ramachandra Medical College and Research Institute (SRMCRI), Chennai, Tamil Nadu, India

Background and Aim: New-onset refractory status epilepticus (NORSE) is a neurological emergency with a mortality rate of approximately 22%. NORSE refers to status epilepticus in patients without a previous diagnosis of epilepsy or other relevant neurological disorders, lacking clear acute/active structural, toxic, or metabolic causes. Febrile infection-related epilepsy syndrome (FIRES) is a subtype of NORSE that occurs following a febrile infection between 2 weeks to 24 h before onset. This study aims to describe the clinical, laboratory, and neuroimaging characteristics, etiology, treatment, and outcomes of adults with new-onset refractory status epilepticus.

Methodology: This prospective observational study included 20 patients. The study period was from June 2023 to March 2024 at Sree Ramachandra Institute of Higher Education and Research (SRIHER). Multivariate logistic regression analysis was performed to identify variables affecting outcomes along with descriptive analysis. Patients above 18 years of age with no previous history of seizure disorders were included and we excluded those with a previous seizure history or acute/active toxic, metabolic, or structural causes.

Results: In all, 60% were males with a mean age of 47 years. Demographic factors and co-morbidities did not affect outcomes. Variables such as the time from prodrome to seizure/status onset, seizure semiology progression, time from seizure onset to treatment initiation, presence of inflammatory cerebrospinal fluid (CSF) profile, or abnormal imaging did not have a significant impact. Also, 55% had febrile prodrome, 55% had an inflammatory CSF profile, and 35% had normal imaging. Four tested positive for herpes simplex virus (HSV)-1, 2 for central nervous system (CNS) tuberculosis, and 1 had an autoimmune etiology. Nine patients received immunotherapy. Also, 55% progressed to super refractory seizures; 10 died. Among survivors, 8 had favourable outcomes with mRS score 0-2.

Conclusions: In our study, no significant differences in outcomes were observed based on variables such as demographic profile, CSF profile, magnetic resonance imaging (MRI) findings, or immunotherapy usage, warranting further research.

Abstract ID: 798: Auto-Immune Nodopathies- The Masquerader

Vyshnavi Jamalapuram, Sita Jayalakshmi S, Anuja Patil

KIMS Hospital, Hyderabad, Telangana, India

Background and Aim: In recent years, antibodies to NF155, CNTN1, CASPR1, and NF186 have been implicated in the pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) via the functional impairment of the node of Ranvier. Herewith we present a case series– clinical characteristics of three patients with chronic neuropathy, who were tested neurofascin-positive.

Case report: The clinical details of three cases with anti-neurofascin antibodies were collected. Case 1: A 60Y/M, no previous comorbidities with complaints of cramps, right upper limb distal weakness with mild wasting, fasciculations, postural and kinetic tremors over 6 months. Case 2: 56/M, previous chronic axonal neuropathy–quadriparesis with bulbar palsy received intravenous immunoglobulin (IVIG), 1 year ago, with minimal recovery, developed new-onset ptosis with restricted extraocular muscles (EOM). Case 3: A 27/M, known drug resistant epilepsy (DRE)/ temporal lobe epilepsy (TLE), post right anterior temporal lobectomy/amygdalohippocampectomy (ATL/AH)- H/o acute inflammatory demyelinating polyneuropathy (AIDP)– descending quadriparesis, was treated with intravenous immunoglobulin (IVIg), and weakness significantly improved. Four months later, the patient developed on-and-off paresthesias, with residual bilateral hand weakness and postural tremors in bilateral little and ring fingers.

Discussion: Case 1: Evaluation showed motor axonal neuropathy. Electroneuro-myography (ENMG) showed active denervation with chronic reinnervation changes and fasciculations. The serum autoimmune neuropathy panel revealed NF-186 and anti-MAG antibodies. He received rituximab 2 pulse doses along with symptomatic treatment. Case 2: Serum autoimmune neuropathy panel was positive for NF-140. Case 3: Repeat nerve conduction study (NCS) was s/o B/L ulnar neuropathy. The serum autoimmune neuropathy panel was positive for NF-140.

Conclusions: Patients with atypical presentations of chronic neuropathy, or patients with partial response to IVIg, need to be evaluated for autoimmune etiology, as they may respond to rituximab.

Abstract ID: 799: Atypical CNS Presentations, Radiological Features, and Treatment Outcomes of Scrub Typhus: Insights from 18 Cases from a Quaternary Center

Sahil Mathur, Farhan Mohammed Ansari, Debayan Dutta, Nikhilesh Pradhan, Maherafsha Hundekari, Seena Vengalil, Girish B. Kulkarni, Pooja Mailankody, Faheem Arshad, Saraswati Nashi, Hansashree Padmanabha, Nagarathna Chandrashekhar, Karthik K

National Institute of Mental Health and Neuro-Sciences (NIMHANS), Bengaluru, Karnataka, India

Background and Aim: Scrub typhus is a zoonosis caused by Orientia tsutsugamushi , whose spectrum of presentation is broad, encompassing constitutional symptoms and diverse systemic manifestations. We evaluate the clinical and radiological features, as well as the outcomes, of neurological disorders associated with scrub typhus.

Methodology: This was a retrospective, cross-sectional study from South India, from January 2020 to January 2023. Patients presented with meningoencephalitis, cerebellitis, rhombencephalitis, vasculitis, opsoclonus myoclonus ataxia syndrome (OMAS), and moyamoya syndrome. The investigations included cerebrospinal fluid (CSF) examination and magnetic resonance imaging (MRI) brain.

Results: Eighteen patients with scrub typhus-associated neurological disorders were included, with a mean age of 31.6 ± 13.1 years and two-thirds were males. The majority of patients had lymphocytic pleocytosis in the CSF and raised protein levels. Brain MRI changes included meningeal enhancement, acute infarcts, and alterations in the brainstem and cerebellum. All patients received doxycycline and the majority showed improvement at follow-up.

Conclusions: Scrub typhus-associated neurological disorders commonly present as meningitis, encephalitis, or meningoencephalitis, with lymphocytic pleocytosis and raised protein levels in the CSF. We expanded the spectrum to include central nervous system (CNS) vasculitis-like picture, rhombencephalitis, OMAS, and a hitherto unreported complication of moyamoya syndrome. Early diagnosis and prompt treatment with doxycycline and supportive care can lead to favourable outcomes.

Abstract ID: 800: Wide Spectrum of Critical Care Emergencies in Neurointensive Care Unit

Akkireddy Kanthima, Nagarjunakonda Venkata Sundarachary, Aruna Kumari Upputuri, Gottipati Bindu Narmada, Bhargavi Devi M

Guntur Medical College, Guntur, Andhra Pradesh, India

Background and Aim: Emergencies in neurology comprise a wide variety of disorders ranging from status epilepticus, movement disorders, autoimmune encephalitis, infections, rheumatological overlapping disorders, endocrine, neuropathies, channelopathies, and vascular events. The aim was to find the etiology and outcomes of various neurological emergencies admitted in the Neurology intensive care unit.

Methodology: This was a prospective study conducted from 2022 to 2024 and the patients were admitted to the intensive care unit (Pediatric and Adult Neuro ICU). We present a spectrum of 50 different neurological emergencies; Guillain–Barre syndrome with autonomic dysfunction, autoimmune encephalitis (6-NMDA, 2-LGI1, 1-GABAB), neuroleptic malignant syndromes (3 cases), myasthenic crisis (3 cases), status epilepticus (NORSE, FIRES, genetic epileptiform encephalopathies, neural migration disorders, NMDA encephalitis), nonconvulsive status epilepticus (NCSE) (post electrocution, autoimmune encephalitis), autoimmune autonomic ganglionopathies, status dystonicus (ACTH induced, antipsychotic induced), meningo encephalitis, fulminant subacute sclerosing panencephalitis (SSPE) in HIV patients, systemic lupus erythematosus (SLE) with cerebral venous sinus thrombosis (CSVT) with malignant idiopathic intracranial hypertension (IIH), tuberous sclerosis with status epilepticus and pneumothorax secondary to lymphangiomyomatosis (LAM), acute hemorrhagic leukoencephalitis (Hurts disease), Sjogrens myopathy with respiratory failure, anterior spinal artery (ASA) thrombosis, Hughes syndrome, internal carotid artery dissection presenting as ptosis and ophthalmoparesis, catastrophic antiphospholipid antibodies (APLA), Lercihe syndrome presenting as paraparesis, cerebral venous thrombosis (CVT) secondary to thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), lysergic acid diethylamide (LSD)-induced stroke in young, methanol induced striatopathy, infections such as tetanus, diphtheria, Creutzfeldt-Jakob disease (CJD), Lymes, Cryptococcomas, neurosyphilis and Varicella zoster presenting as stroke, pituitary apoplexy, catatonia secondary to depression (ECT done), chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CIPPERS), Wernicke’s encephalopathy, alternating hemiparesis secondary to steroid responsive encephalopathy associated with autoimmune thyroiditis (SREATS), monoclonal gammopathy of undetermined significance (MGUS), neuropathy complicating myocardial infarction (MI), fibromuscular dysplasia, hypokalemic periodic paralysis, levodopa-induced severe dyskinesia, krait bite with neurological manifestations, parkinsonism hyperpyrexia syndrome, necrotizing inflammatory myopathy, post-traumatic cerebrospinal fluid (CSF) leak causing intracranial hypotension, diffuse cerebral edema secondary to urea cycle defect, diabetic striatopathy (Hemiballismus, paraballismus), pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS), inborn errors of metabolism (maple syrup urine disease, pyruvate dehydrogenase deficiency), moyamoya disease, Takayasu arteritis, vestibular neuronitis, nodopathies, drug reaction with eosinophilia and systemic symptoms (lamotrigine induced), arsenic-induced neuropathy.

Results: Total mortality: 7 cases. Mortality maximum was observed in infectious cases and immunological disorders. Total male cases: 23, female cases: 27, pediatric cases (≤14 years)- 14, adults-36.

Conclusions: Our case series covers a wide spectrum of neurological emergencies and prompt intervention, leading to early diagnosis and management.

Abstract ID: 801: Acute Neurocritical Management of Severe Dextromethorphan Poisoning: A Case Report

Naveen Ranga, Naveen Kumar Ranga, Surekha Dabla

Pt BD Sharma PGIMS, Rohtak, Haryana, India

Background and Aim: An overdose of dextromethorphan (DXM) can cause significant central nervous system issues, such as coma. This case study seeks to demonstrate how severe DXM poisoning is managed clinically, underscore the critical role of neurocritical care in enhancing patient recovery, and track the patient’s progress and functional improvement.

Case report: This report describes a 28-year-old male patient who ingested an entire bottle of DXM-containing antitussive and was found comatose at home. On admission, he exhibited poor respiratory effort and a Glasgow Coma Scale (GCS) score of 3/15, necessitating immediate mechanical ventilation. Initial metabolic tests revealed severe metabolic acidosis and acute kidney injury, which required hemodialysis. Magnetic resonance imaging (MRI) scans at admission were unremarkable, but toxicology confirmed opioid exposure. Despite remaining comatose and experiencing a seizure, the patient’s metabolic profile gradually improved with supportive care. Follow-up MRI a week later showed extensive bilateral symmetrical damage to the centrum semiovale and basal ganglia. By the second week, he was weaned off the ventilator, though he remained akinetic and mute. By the fourth week, he regained consciousness, displayed purposeful movements, and required minimal assistance for walking. This case underscores the critical role of neurocritical care in managing severe DXM poisoning.

Discussion: The focused, supportive care provided in a neurocritical setting significantly enhances recovery prospects and functional restoration in patients suffering from severe acute brain injury due to DXM overdose.

Conclusions: The focused, supportive care provided in a neurocritical setting significantly enhances recovery prospects and functional restoration in patients suffering from severe acute brain injury due to DXM overdose.

Abstract ID: 802: Assessing Stroke Care Standards in Rural India: Pre-SMART INDIA Training Evaluation and Future Improvement Metrics: Interim Analysis

Biswamohan Mishra, Aprajita Parial, Sakshi Sharma, Gulafshan Iqbal, Zubina Khan, Ayush Agarwal, M V Padma Srivastava, Venugopalan Y Vishnu, Smart India Investigators

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Healthcare delivery in rural India faces significant challenges due to a shortage of specialists, particularly in managing acute conditions such as stroke. This study is part of an ongoing multi-centric SMART INDIA project, which aims to compare two innovative solutions: training physicians in district hospitals to manage stroke (“Stroke physician model”) versus using a low-cost Telestroke model. We evaluated the current stroke care standards at district hospitals and nodal centers using a 24-point stroke care bundle, with the objective of leveraging these data in the future to quantify the enhancements following the implementation of SMART INDIA training.

Methodology: The study is currently ongoing and the available patient data from eleven district hospitals and six nodal centers were analyzed. Two neurologists independently assessed the extent of stroke care bundle components administered before SMART INDIA training.

Results: Interim analysis revealed that of 268 records screened (nodal centers 201, district hospitals 67), the mean age of the cohort was 59.83 ± 14.94 years, with 164 males [61.19%] and ischemic stroke n = 183 [68.3%] as the major subtype. The mean percentage of stroke care bundle components administered overall was 37.12 ± 17.90%. Nodal centers performed better (42.14 ± 16.92%) than district hospitals (20.99 ± 11.26%), ( P < 0.00001).

Conclusions: Preliminary results highlight the suboptimal acute stroke care delivery in rural district hospitals, with less than 50% adherence to standard care protocols, underscoring the stark gaps in rural healthcare, particularly in managing stroke. The final results will be presented in the IANCON 2024.

Abstract ID: 803: Asymmetric Bilateral Chronic Progressive External Ophthalmoplegia with False-Positive Acetylcholine Receptor Antibody: A Rare Case Report

Chinmayee Swain, Ashok Kumar Mallick, Kali Prasanna Swain

Srirama Chandra Bhanja Medical College and Hospital, Cuttack, Odisha

Background and Aim: We present a rare case with atypical presenting features of asymmetric bilateral chronic progressive external ophthalmoplegia (CPEO) with a false positive acetylcholine receptor antibody test resulting in diagnostic delay. The case was treated initially for myasthenia gravis due to asymmetric ptosis and ophthalmoplegia.

Case report: A 26-year-old female patient presented with a 13-year history of slowly progressive extraocular motility restriction with ptosis in the right eye, followed by a 5-year history of similar presentation in the left eye with a 5-year history of diplopia. Clinical examination showed extraocular motility restriction in all gazes with asymmetric ptosis in the right eye more than the left eye. On diplopia charting, there was diplopia in superotemporal and downward direction.

Discussion: Serum acetylcholine receptor antibody (AchR Ab) was positive on serum enzyme-linked immunosorbent assay (ELISA); however, following 4 months of oral pyridostigmine therapy, there were no signs of clinical improvement. The serum sample was sent for AchR Ab by radio-immunoassay (RIA), which came back negative. The magnetic resonance imaging (MRI) brain and orbit were normal. Whole-exome sequencing with mitochondrial sequencing revealed a heterozygous variant in exon 1 of the TWNK gene (c.1070 G>A mutation).

Conclusions: Sometimes, CPEO may be difficult to diagnose, especially when the symptoms are asymmetric, lack other systemic signs, have diplopia, or have no family history of CPEO. Thus, practitioners should keep CPEO in their differential diagnosis to avoid unnecessary delay in diagnosis.

Abstract ID: 804: Rare Case of Neurosarcoidosis Presenting as Longitudinal Extensive Transverse Myelitis

P. Harshitha, P. Sri Harshitha, Prashant Bhatele, Aparna Pai

Manipal Academy of Higher Education (MAHE), Manipal, Karnataka, India

Background and Aim: Sarcoidosis is a multisystem immune-mediated disorder characterized by granulomatous inflammation of affected organs. Neurological involvement accounts for 5% to 10% of cases, of which, longitudinal extensive transverse myelitis (LETM) is very rare.

Case report: We present a case of a 53-year-old lady, with leg numbness, slippage of chappals, cotton wool sensation in both feet and swaying while walking. Examination revealed hypertonia in all limbs, exaggerated deep tendon reflexes, and bilateral extensor plantar response. The distal sense of joint position and vibration was impaired in both feet. Laboratory tests showed increased inflammatory markers and a high serum angiotensin-converting enzyme level. The brain magnetic resonance imaging (MRI) was normal. Spine MRI revealed a long segment of T2/ fluid attenuated inversion recovery (FLAIR) hyperintensity along the posterior portion of the cervicothoracic spinal cord and post-contrast enhancement. A chest X-ray and computed tomography (CT) scan of the chest showed features of sarcoidosis. Bronchoscopy, and bronchoalveolar lavage results were normal. The fine needle aspiration biopsy of the subcarinal lymph node yielded no granulomatous changes.

Discussion: Therefore, elevated serum angiotensin converting enzyme (ACE) levels and typical CT chest and MRI spine results led to the diagnosis of neurosarcoidosis, which presented as LETM. We started the patient on steroids and her symptoms completely resolved during the 1-month follow-up. She was then initiated on mycophenolate mofetil.

Conclusions: In neurosarcoidosis, isolated spinal cord involvement is rare and presents significant challenges in diagnosis. Glucocorticoids are the cornerstone of treatment, especially in the acute stage. The most effective steroid-sparing agent is mycophenolate mofetil.

Abstract ID: 805: Study of Clinical Phenotypes Linked to Cerebral Small Vessel Disease: A Single Center-Based Observational Study

Chebolu Harshika, Randhi Venkata Narayana, Kakarala Pradeep, Amarapu Sravanthi, Puvvada Srikar

SevenHills Hospital, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Cerebral small vessel disease (cSVD) affects the smaller caliber vessels of the brain, including arterioles (perforating, diameter < 0.1 mm), capillaries, and venules, and results in chronic and often progressive/accumulating grey and white matter injury in the brain. Small subcortical infarcts (SCI), lacunes, cerebral microbleeds (CMBs), cortical microinfarcts, white matter hyperintensity (WMH), perivascular spaces (PVS), and cortical superficial siderosis are the major neuroimaging markers of cSVD. Our study aims to describe the various clinical phenotypes associated with cSVD.

Methodology: An observational retrospective study was performed between January 2024 and July 2024. In all, 109 patients with cSVD on magnetic resonance imaging (MRI) brain, clinical phenotypes, and neuroimaging markers were studied.

Results: Of the 109 patients, 45 (41.2%) patients had acute ischemic stroke, 25 (22.9%) had hemorrhagic stroke, 10 (9.17%) had cognitive defects, 9 (8.25%) patients had cardiac abnormalities, 7 (6.42%) patients had parkinsonism, 3 (2.75%) patients had bladder disturbances, 5 (4.58%) patients had gait ataxia, 4 (3.66%) patients presented with mood disturbances, 2 (1.8%) patients had obstructive sleep apnea (OSA). WMHs were the most common abnormality- 82%, SCIs- 76%, cortical microinfarcts- 60%, and PVS- 45% cases.

Conclusions: cSVD can present with multiple neurological phenotypes such as stroke, mood disturbances, parkinsonism, gait and bladder disturbances, and cognitive defects. Non-neurological phenotypes such as cardiovascular abnormalities or respiratory (obstructive sleep apnea) have also been associated with cSVD. Tobacco, hypertension, and diabetes are modifiable risk factors for cSVD.

Abstract ID: 806: Genetic Profile of Muscle Diseases in a Tertiary Care Center

Akkireddy Kanthima, Sundarachary Nagarjunakonda, Aruna Kumari U, Gottipati Bindu Narmada, Mandarapu Bhargavi Devi

Guntur Medical College, Guntur, Andhra Pradesh, India

Background and Aim: Niemann–Pick disease type C (NPC) is an uncommon, autosomal recessive lysosomal storage disorder characterized by progressive neurodegeneration. This report details the clinical presentation, diagnostic workup, and genetic confirmation of NPC in a 23-year-old female patient born to second-degree consanguineous parents.

Case report: A 23-year-old female patient, presented with a history of unsteadiness while walking for 3 years, tremulousness of both upper limbs for 2.5 years, slurred speech for 2 years, and progressive weakness of both lower limbs for 1 year. Her symptoms began insidiously and progressively worsened, leading to significant functional impairment requiring assistance for daily activities. Symptoms included difficulty writing due to dystonic posturing of the index fingers and experiencing falls, particularly in low-light conditions. Neurological examination revealed a wide-based gait with unsteadiness, intention tremor, and scanning speech. Spasticity in the lower limbs with brisk reflexes was observed. Coordination tests showed dysdiadochokinesia and impaired finger-to-nose and heel-to-knee performance. Ophthalmological examination identified a cherry-red spot in the macula, and the patient exhibited loss of vertical saccades with normal horizontal saccades. Nerve conduction studies indicated mild demyelinating sensory-motor neuropathy in all four limbs.

Discussion: Given the progressive ataxia, cherry-red macular spot, and consanguineous parentage, NPC was highly suspected, and subsequently confirmed through genetic testing.

Conclusions: This case underscores the importance of considering NPC in young patients presenting with progressive ataxia, intention tremor, and cerebellar atrophy, especially in the context of consanguinity. The presence of a cherry-red spot in the macula and loss of vertical saccades are key diagnostic indicators. Early genetic testing is crucial for the timely diagnosis and management of NPC, potentially improving patient outcomes.

Abstract ID: 807: A Cross-Sectional Study of Neurophysiological and Neuroradiological Changes in Chronic Kidney Disease Patients Aged < 18 years in a Tertiary Care Center

Krishna Chaithanya Reddy Bollipalli

Osmania Medical College, Hyderabad, Telangana, India

Background and Aim: Chronic Kidney Disease (CKD) has a significant impact on all systems including the nervous system. The nervous system involvement can be the central nervous system in the form of altered sensorium, seizure disorder, delirium, strokes, coma, or the peripheral nervous system involvement in the form of sensory and motor neuropathies. Detection and treatment of these disorders are very important and they reduce the quality of life and increase disability or may cause early mortality. This study aims at detecting these disorders early so that early intervention can be undertaken. For detecting cerebral cortical involvement, imaging studies such as computed tomography (CT)/magnetic resonance imaging (MRI) are done and electrophysiological studies such as electroencephalogram (EEG), for peripheral nerve disorders, nerve conduction disorder (NCS) is performed.

Methodology: The study will include patients aged below 18 years diagnosed with CKD. Participants were recruited from the nephrology and medical departments at the Osmania General Hospital. History, complete neurological examination, nerve conduction studies, CT brain/MRI, and EEG were conducted for all patients.

Results: In all, 20 patients of both sexes (12 females and 8 males) with a mean age of 11 years were studied. Two (10%) patients developed polyneuropathy, mostly of axonal polyneuropathy pattern, whereas 18 (90%) patients showed normal NCS. Abnormal EEG was detected in four (20%) patients mostly generalized epileptiform activity with background slowing. Abnormal CT/MRI findings were detected in four (20%) patients mostly of encephalomalacia changes.

Conclusions: Early demonstration and management of uremic neurological conditions may decrease the physical disability of patients with CKD.

Abstract ID: 808: Histoplasmosis Presenting as a Space-Occupying Lesion in the Brain: Case Series

Kiran Bhagwat, Jitesh Goel, Manoj Gopal Madakshira, K. P. Sengar, G. Madhusudhan, Vikalp Jain, K. V. Padmaprtakash, Faiz Ahmed

Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

Background and Aim: Histoplasmosis is a respiratory–systemic mycosis, developing from an auto-limited and benign respiratory infection to a life-threatening disseminated disease form. This infection flares up in the setting of immunosuppression. Overall, individuals develop an infected benign form; however, under some circumstances, the infection evolves to dissemination to involve the lungs, spleen, and liver, contrasting with infrequent reports for that of the central nervous system (CNS) in patients who present progressive disseminated histoplasmosis. Our study attempts to highlight a narrow relationship between H. capsulatum infection and the risk of developing CNS involvement. Here we aim to discuss the rare but treatable cause of a space-occupying lesion of the brain in an immunocompromised state, with complete clinico-radiological remission after successful administration of appropriate antifungal therapy.

Case report: Single-center observational study. We present a case series of three retro positive middle-aged patients, who presented as symptoms of headache, altered sensorium, and focal neurodeficit. Diagnosed as radiologically + histopathologically proven cases of CNS histoplasmosis. It was treated appropriately, which resulted in good clinical outcomes on follow-up.

Discussion and Conclusions: Histoplasmosis should be kept as one of the differentials in a ring-enhancing lesion in the brain parenchyma apart from other common mimickers such as tuberculosis, neurocysticercosis, cryptococcoses, and toxoplasmosis. Hence, a high index of suspicion with prompt treatment of the pathogen may lead to good clinical outcomes.

Abstract ID: 809: Unveiling the Paralyzing Veil: Recurrent Hypokalemic Paralysis in a Case of Conn’s Syndrome Caused by Adrenal Adenoma

Aparna Thomas, Ashwin Kumar Panda, Shimpy Priya, Suman Kushwaha, Aldrin Anthony Dung Dung, Rajinder K. Dhamija

Institute of Human Behaviour and Allied Sciences, Delhi, India

Background and Aim: Hypokalemic periodic paralysis is a rare disorder characterized by transient attacks of flaccid paralysis of varying intensity and duration. Although mostly familial in etiology, several sporadic cases of different etiologies have been reported, including rare causes such as primary hyperaldosteronism. The secondary hypokalemic periodic paralysis is less common and the clues for investigating a secondary cause include the lack of family history and the time of onset of symptoms, that is, those who have their first attack of hypokalemic periodic paralysis in adulthood. Hypokalemic periodic paralysis as a presenting manifestation of Conn’s syndrome is rare with only a few cases reported in the literature.

Case report: A 48-year-old male patient, with a known case of hypertension for 1 year, on irregular medications, with recurrent episodes of quadriparesis (4-5 episodes) for 1 year, no other significant family history or past history, presented with acute flaccid quadriparesis without sensory, bowel, and bladder symptoms. O/E- Blood pressure (BP)- 170/90, extraocular movements-full, normal deep tendon reflexes, plantar-b/l flexor, sensory system-intact. Nerve conduction velocity (NCV)- normal. S K+-2.9 mmol/L, arterial blood gas (ABG)- metabolic alkalosis, serum creatine phosphokinase (CPK)- 1686, electrocardiogram (ECG)- sinus bradycardia, PR prolongation with U waves, serum creatinine-1.4 mg/dL. The patient was treated with potassium replacement with subsequent improvement in muscle power. Due to delayed onset, underlying uncontrolled hypertension, and high CPK levels, he was evaluated for secondary reversible causes. USG abdomen with renal artery Doppler was a normal study. Serum aldosterone and aldosterone/direct renin ratio were elevated with suppressed levels of serum renin. Serum cortisol levels and thyroid function test-normal. He was further evaluated for Conns’ syndrome with contrast enhanced CT (CECT) abdomen, which showed (L) lipid-rich adrenal adenoma. He was started on spironolactone, BP was controlled with normalization of potassium levels, and being referred for surgical management.

Discussion and Conclusions: Patients with the first attack of hypokalemic periodic paralysis in adulthood should be screened carefully for a secondary cause

Abstract ID: 811: Unraveling the Enigma of Peripheral Neuropathy in POEMS Syndrome in a Young Male

Aparna Thomas, Ashwin Kumar Panda, Ankita Bansal, Suman Kushwaha, Aldrin Anthony Dung Dung, Rajinder K. Dhamija

Institute of Human Behaviour and allied sciences, New Delhi, India

Background and Aim: Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein elevation, and skin changes (POEMS) syndrome is a rare paraneoplastic disorder. It was first described by Crow in 1956 and then by Fukase in 1968. The POEMS syndrome is associated with excessive plasma cell proliferation. It is mostly observed in 60s with a slight male predominance. We describe a rare case of a young patient who presented classically with all the criteria necessary for the diagnosis of POEMS syndrome.

Case report: A 25-year-old male patient with no past medical history presented with symmetric weakness involving bilateral lower limbs, distal > proximal associated with burning pain, and paresthesias for 2 months. It was associated with thinning both feet and high stepping gait. There was a history of abdominal distension with significant weight loss and night sweats, for which he was started on antituberculous treatment (ATT) from outside for the past 3 months. O/E-patient was emaciated with hyperpigmentation of the skin and loss of buccal pad of the fat. Cervical lymphadenopathy, hepatosplenomegaly were present, grade 1 papilloedema+b/l foot drop present. Investigations showed thrombocytosis with raised erythrocyte sedimentation rate (ESR). Nerve conduction velocity (NCV) showed demyelinating polyneuropathy. Although renal function test (RFT) and serum electrolytes were normal, 24 hours urine examination showed macroalbuminuria. The magnetic resonance imaging (MRI) showed multiple lytic bony lesions. USG’s abdomen showed hepatosplenomegaly with ascites and pleural effusion. There was endocrinopathy involving the adrenal, thyroid, pituitary, and gonadal axis. Serum electrophoresis was normal. Immunofixation showed an M band in the lambda region and normal immunoglobulin levels. The excision biopsy of the cervical lymph node was consistent with Castleman’s disease. As per Dispenzieri (2023), the patient was satisfied the criteria for POEMS syndrome and was referred further for bone marrow biopsy and autologous stem cell transplantation.

Discussion and Conclusions: We present a case report of a 25-year-old patient with classic POEMS syndrome with Castleman’s disease. Early diagnosis and treatment can prevent irreversible damage.

Abstract ID: 813: Hirayama Disease-”ALS Mimic”

Apsara P S, P. K. Murugan, Justin C

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: Hirayama disease is a relatively unusual cause of cervical myelopathy characterized by uni-or bilateral asymmetric weakness, fasciculations, and atrophy of the distal upper limbs, mostly observed in young males. The incidence of Hirayama disease is low, and it is rarely encountered in clinical settings.

Case report: A 16-year-old male patient with no relevant medical history presented with a 3-year duration of weakness and wasting of the hand and forearm on the left side. His weakness and atrophy started initially in the small muscles of the left hand and gradually progressed to involve the left forearm muscles. Motor examination showed atrophy and weakness (G4/5) of the thenar, hypothenar, interosseous, and forearm muscles with sparing of the brachioradialis muscle on the left side. Deep tendon reflexes and sensory examination were normal.

Discussion: In flexion magnetic resonance imaging (MRI) cervical spine, enhancing crescent-shaped posterior epidural lesion was noted at C5-D2 level, anterior displacement of the posterior thecal sac, and focal decreased AP diameter of cord at C6-C7 level. Routine laboratory investigations were within normal limits. The vasculitic workup was negative. A nerve conduction study showed a low compound muscle action potential (CMAP) amplitude in the abductor digiti minimi.

Conclusions: We presented a rare case report of “Hirayama disease,” which is rarely encountered in clinical practice. It should be suspected in male patients presenting with unilateral or asymmetrical bilateral lower motor weakness of hands and forearms. It is a benign entity and no treatment other than a cervical collar is usually needed in most cases.

Abstract ID: 814: Clinical and Radiological Improvement in Fulminant Subacute Sclerosing Panencephalitis Treated with Intravenous Immunoglobulin

Rekha Patil, Archana N B, Thejus B, Archana Netto, Praveen S Kumar

Bangalore Medical College and Research Institute (BMCRI), Bengaluru, Karnataka, India

Background and Aim: Fulminant SSPE (subacute sclerosing panencephalitis) has been defined as the development of at least 66% neurological disability within 3 months of the first neurological symptom and death or a severe neurological disability (exceeding 90%) within 6 months of onset. The most common age of onset is 5–15 years, usually 5–8 years after measles infection. Myoclonus, cognitive decline, poor scholastic performances, and behavioral abnormalities are the most common presenting features. We report a rare case of fulminant SSPE who was treated with intravenous immunoglobulin.

Case report: We report a rare case of a 17-year-old male patient with fulminant SSPE who was treated with intravenous immunoglobulin with clinical and radiological response. A 17-year-old male patient presented with a progressive history of psychiatric features such as social isolation, auditory and visual hallucinations, disinhibition, excessive laughter, memory disturbances, and psychomotor regression over a period of 8 months. At the time of examination, the patient was found to have major neurocognitive impairment in all domains and also had akinetic mutism. Investigations were conducted. Magnetic resonance imaging (MRI) brain with contrast showed T2/fluid-attenuated inversion recovery (FLAIR) white matter hyperintensities in the entire left cerebral hemisphere, right frontal, and bilateral temporal lobes. The cerebrospinal fluid (CSF) IgG anti-measles antibody by enzyme linked immunosorbent assay (ELISA) was 625. The electroencephalogram (EEG) showed generalized symmetric spontaneous periodic high amplitude complexes. Anti-HIV antibodies were negative. Based on these findings, a diagnosis of fulminant SSPE was made and the patient was given intravenous immunoglobulin (IVIG), clinical improvement was noted as the patient was able to ambulate and over time MRI brain changes decreased.

Discussion and Conclusions: IVIG can be a therapeutic option for fulminant SSPE for temporary clinical improvement and to decrease morbidity.

Abstract ID: 815: Ataxia, Peripheral Neuropathy, Uterine and Ovarian Agenesis and Hypergonadotropic Hypogonadism- A Diagnostic Conundrum

Alvee Saluja, Shahbaz Anees, L. H. Ghotekar, Anul Haque

Lady Hardinge Medical College, New Delhi, India

Background and Aim: The PNPLA8 gene encodes for a mitochondrial membrane-associated calcium-independent phospholipase A2. Mutations in PNPLA8 have been associated with microcephaly, myopathy, lactic acidosis, seizures, cerebellar ataxia, and peripheral neuropathy. However, gonadal agenesis and hypergonadotropic hypogonadism along with neurodegeneration have not been reported with PNPLA8 mutations. We report the first case of PNPLA8- associated neurodegeneration from India and describe gonadal agenesis as a clinical phenotype associated with PNPLA8 mutation for the first time.

Case report: The clinical phenotype, biochemical investigations, radiology, and genetic testing of the index case are described. An 18-year-old female patient born out of a non-consanguineous marriage at term had normal developmental milestones. At 12 years of age, she was amenorrheic and did not develop secondary sexual characteristics. At 14 years of age, she started having progressively worsening imbalance and gait difficulty. One year afterward, there was slippage of footwear without awareness, difficulty getting up from squatting position, and she would often fall while washing her face with water. Examination revealed pes cavus, hammer toes, cerebellar and sensory gait ataxia, decreased hand grip, proximal and distal lower limb weakness, distal lower limb sensory loss below the knees, and generalized areflexia. Her estradiol levels were low, follicle stimulating hormone (FSH) and luteinizing hormone (LH) levels were elevated. Magneti resonance imaging (MRI) pelvis showed absence of the cervix, uterus, ovaries, and upper 2/3rd of the vagina. The nerve conduction study suggested demyelinating sensorimotor neuropathy affecting all four limbs. MRI brain plus spine was unrevealing.

Discussion and conclusions: PNPLA8-associated mitochondrial dysfunction can present with gonadal agenesis and neurodegeneration in the form of cerebellar ataxia and peripheral neuropathy.

Abstract ID: 816: Anti-CV2/CRMP5 Antibody-Mediated Autoimmune Encephalitis: A rare type of Autoimmune Encephalitis- A Case Report

Shishirakumar Goudar

Apollo Hospital Seshadripuram, Bengaluru, Karnataka, India

Background and Aim: The anti-CV2/collapsin response mediator protein 5 (CRMP5) antibody is associated with paraneoplastic neurological syndromes. It clinically manifests as limbic encephalitis, chorea, retinitis, cerebellar ataxia, myelopathy, and peripheral neuropathy. CV2/CRMP 5-mediated paraneoplastic syndrome is rare and there are a few reports worldwide and we could not find any case reports from India.

Case report: A 67-year-old male patient, presented with generalized tonic–clonic status epilepticus of 2 h duration, on a background of longstanding diabetes with retinopathy, neuropathy, and hypertension.

Discussion and conclusions: We present the clinical features, investigation reports, the course of the case in the hospital, and follow-up details of the case after the discharge.

Abstract ID: 817: An Unusual Etiology of Status Epilepticus

Shreyashi Jha, Santosh Kumar Pendyala, Mona Tiwari, Sumedha Singh

Institute of Post Graduate Medical Education and Research, Kolkata (IPGMER Kolkata), West Bengal, India

Background and Aim: L-2 Hydroxyglutaric aciduria is a rare, progressive, autosomal recessively inherited metabolic disorder exclusively affecting the central nervous system. Seizures were reported in less than one-third of the cases with two reported cases of childhood-onset status epilepticus. We report the first case of an adult-onset status epilepticus, which was diagnosed with L2 hydroxyglutaric aciduria.

Case report: A 22-year-old female patient presented to the emergency with status epilepticus. She had no previous history of seizures. She was born of non-consanguinous parentage without any perinatal insult. She had psychomotor regression in early childhood progressive imbalance while walking and mild postural tremors. The magnetic resonance imaging (MRI) brain showed T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities in the subcortical white matter involving subcortical “u” fibers, basal ganglia, and dentate nuclei suggestive of L2 hydroxyglutaric aciduria. Urine showed elevated 2 hydroxy glutaric acid levels. Whole-exome sequencing showed a missense mutation in the L2HGDH gene.

Discussion and conclusions: L2HGA should be suspected even in adult-onset status epilepticus in light of characteristic MRI findings and clinical history.

Abstract ID: 818: Blended Phenotype of Mitochondrial DNA Depletion with Hypomyelinating Leukodystrophy: A Rare Case Report

Praveen Kumar Mopuri, Agadi J B, Priya Ranganath, Sujit Kumar, Pramod M N

Apollo Hospital Seshadripuram Bengaluru, Karnataka, India

Background and Aim: Mitochondrial DNA depletion syndrome-6 (MTDPS6) and hypomyelinating leukodystrophy-18 (HLD18) are severe autosomal recessive disorders with an early infantile onset.

Case report: We present a case of a 4-year-old male child born to third-degree consanguinity, presented with a history of hypoglycemic events, global developmental delay (GDD), neuroregression, hypotonia, generalized wasting, and poor weight gain noticed since 2 years of age. No history of seizures. Vision and hearing normal. Vitals: within normal limits for his age. Dysmorphism: broad nasal bridge, myopathic facies, cupid bow upper lip. Central nervous system (CNS): Higher mental functions intact, cranial nerves–normal, no nystagmus bulk normal, tone-hypotonia with diminished reflexes per-abdomen-soft, hepatomegaly 5 cm below right costal margin (RCM). No splenomegaly INV. Magnetic resonance imaging (MRI) brain, nerve conduction study (NCS), and genetic studies were done.

Discussion and conclusions: We report a child from a consanguineous marriage exhibiting a rare, blended phenotype with features of both MTDPS6 and HLD18. Ours is the first case report of this blended phenotype worldwide. Details of the MRI, clinical image of the case, and genetic report are presented and discussed.

Abstract ID: 819: When Scorpions Strike-“Pres following Scorpion Bite”

Ankur Vivek, Vijaya Nath Mishra, Abhishek Pathak, Pranjali Batra, Arpan Mitra

Institute of Medical Sciences, Varanasi (IMS, Varanasi), Uttar Pradesh, India

Background and Aim: A clinic-radiological illness known as posterior reversible encephalopathy (PRES) can occur in a variety of settings, including an immunological response, a systemic infection, or a rise in blood pressure. Symptoms of PRES include headache, disorientation, loss of consciousness, and headache. A blood–brain barrier lesion that results in reversible vasogenic edema explains its process.

Case report: We report a case of a 9-year-old girl who was stung by a scorpion and a few seconds after that, she developed a headache that was holo cranial, heaviness, severe in intensity with a visual analogue scale (VAS) score of 9/10, associated with nausea and vomiting. A few minutes after this, she had a diminution of vision from both eyes which was painless, both for far and near vision, in all gazes of vision with no history of diplopia or congestion. Within a few minutes, she could not see anything. Then, 1-2 hours after that, she had multiple episodes of abnormal tonic–clonic movements of bilateral upper and lower limbs with frothing from the mouth, uproling of eyes, and loss of consciousness. The duration of each event was 2-3 min with a total of 5-6 episodes with no regaining of consciousness in between. She came to the hospital with a Glasgow Coma Scale (GCS) of E1V1M5.

Discussion: The magnetic resonance imaging (MRI) brain was conducted, which was suggestive of vasogenic edema in the bilateral parietooccipital area. The electroencephalography (EEG) was s/o generalized slowing. All routine investigations were within normal limits.

Conclusions: This is the first instance of PRES in India after a scorpion sting. According to the available and recent data, we attempt to discuss the potential mechanisms leading to this neurologic disorder.

Abstract ID: 820: The Engima of IIH in CML: A Medical Mystery Unveiled

Ankur Vivek, Vijaya Nath Mishra, Abhishek Pathak, Pranjali Batra, Arpan Mitra

Institute of Medical Sciences (IMS), Varanasi, Uttar Pradesh, India

Background and Aim: The myeloproliferative disease known as chronic myeloid leukemia (CML) is typified by an excess of myeloid cells. About 15 to 20% of adult leukemias are caused by CML. At the presentation, 50 years is the median age.

Case report: A female patient, age 18 years, diagnosed with CML, had headaches on the right side and blurred vision for 4 to 5 days before the presentation. Despite receiving CML treatment, the patient never experienced a hematological remission. The patient saw an ophthalmologist, who diagnosed bilateral optic disc edema and suggested a brain magnetic resonance imaging (MRI). Her headache and impaired eyesight worsened, so she went to the emergency room. Significant bilateral papilledema was observed on funduscopic examination. A leukocytosis of 240 × 10 3 /uL and a platelet count of 1,202 × 10 3 were found during the laboratory evaluation. Together with myelocytes and meta-myelocytes, 17% of blasts were observed in the white cell differential count. Neuroimaging showed a nonspecific cerebrospinal fluid (CSF) flair signal. Upon lumbar puncture (LP), opening pressures were noticeably higher. However, the makeup of the CSF was normal. After the LP, the patient’s symptoms significantly improved.

Discussion and conclusions: Papilledema in a patient with leukemia with normal CSF and neuro-imaging is most likely due to benign intracranial hypertension. The most likely pathogenesis of the elevated intracranial pressure (ICP) in such cases is that the elevated white cell count in leukemia causes hyperviscosity, which decreases the absorption of CSF from the arachnoid villi.

Abstract ID: 821: Genetic Threads: Unraveling the Mystery of ARSACS in One Family

Ankur Vivek, Vijaya Nath Mishra, Abhishek Pathak, Pranjali Batra, Arpan Mitra

Institute of Medical Sciences, Varanasi (IMS, Varanasi), Uttar Pradesh, India

Background and Aim: Biallelic mutations in the SACS gene result in the rare neurological disease known as autosomal recessive spastic ataxia of Charlevoix–Saguenay (ARSACS). Formerly believed to be exclusive to the Charlevoix–Saguenay area of Quebec, new research suggests that this condition is widespread globally. The three main characteristics that define it traditionally are axonal demyelinating sensory neuropathy, pyramidal involvement, and ataxia. Nonetheless, a variety of clinical characteristics have been linked to this illness. This study describes an Indian family with ataxia, hypokinesia/bradykinesia, and rigidity, all of which are indicative of a new deletion mutation in the SACS gene that causes ARSACS.

Case report: We describe a patient who presented with complaints of difficulty in walking along with imbalance while walking with scanning speech along with tremulous movement of both upper limbs with pseudobulbar affect for the past 2 years. There were 12 members in the family who had similar disease phenotypes over the three generations. On examination, there were slow saccades with broken pursuits with restriction of vertical extraocular movements with scanning speech and cerebellar signs positive. The patient had generalized hyporeflexia. Nerve conduction velocity (NCV) was suggestive of sensorimotor demyelinating neuropathy. Magnetic resonance imaging (MRI) brain was suggestive of cerebellar atrophy. The whole exome sequencing (WES) was done, which was suggestive of SACS gene mutation. A similar mutation was also observed in his two elder brothers, son, and father.

Discussion and conclusions: Our findings expand the genetic and phenotypic spectrum of this disorder.

Abstract ID: 822: Unveiling the Silent Link: Paraneoplastic ALS and Bronchogenic Carcinoma

Ankur Vivek, Vijaya Nath Mishra, Abhishek Pathak, Pranjali Batra, Arpan Mitra

Institute of Medical Sciences (IMS), Varanasi, Uttar Pradesh, India

Background and Aim: The deadly and uncommon neurodegenerative disease known as amyotrophic lateral sclerosis (ALS) is typified by the loss of both upper and lower motor neurons in the brain and spinal cord. The purpose of this case was to increase awareness of paraneoplastic ALS.

Case report: A 50-year-old male patient presented with 2 years of progressively lower and upper extremity weakness along with fasciculations. He had been diagnosed with chronic bronchogenic carcinoma 18 months ago and was on chemotherapy (cisplatin-based). One year ago, he had noticed atrophy of the quadriceps femoris, hamstrings, deltoid, biceps, triceps, and intrinsic muscles of the hands. At the same time, the patient also had speech difficulty in the form that it was harsh, effortful, strained, and strangled and he had a choking sensation while eating solid food. On neurological examination, he had diffused muscle weakness, amyotrophy, and fasciculations in lower and upper limbs, exaggerated tendon reflex, and a bilaterally positive Babinski sign.

Discussion : The electromyography (EMG) showed low compound muscle action potentials (CMAPs) in all tested nerves of the upper and lower limbs, with normal motor conduction velocity (MCV). Additionally, needle examination revealed spontaneous activity (fibrillations and positive sharp waves) and widespread chronic neurogenic suffering in multiple muscles of four regions (bulbar, cervical, thoracic, and lumbar regions). Brain and spine magnetic resonance imaging (MRIs) were unremarkable. The cerebrospinal fluid (CSF) was unremarkable. The tests for paraneoplastic antibodies in serum and CSF were negative. He received pulse steroid therapy, after which the distal muscle strength of the lower and upper limbs improved slightly. At 1-year follow-up, his clinical symptoms were not exacerbated.

Conclusions: It is important to correctly identify ALS patients with paraneoplastic origin.

Abstract ID: 823: Seizing Clues: Exploring the Epilepsy Connection in Pendred Syndrome

Ankur Vivek, Vijaya Nath Mishra, Abhishek Pathak, Arpan Mitra, Akansha Jain, Nayana Bhuyan

Institute of Medical Sciences (IMS), Varanasi, Uttar Pradesh, India

Background and Aim: One prevalent autosomal recessive condition that causes deafness is pendred syndrome. Symptoms include enlarged vestibular aqueducts (EVA), goiter, sensorineural hearing loss, and in rare cases, Mondini dysplasia.

Case report: The patient was a 13-year-old boy. He was born to healthy, non-consanguineous parents after a physiological pregnancy. The patient has been deaf since birth. At the age of 6 years of age, the patient had abnormal tonic–clonic movement of bilateral upper and lower limbs with uproling of eyes, frothing from the mouth, and loss of consciousness. This episode lasted for 4–5 min. The patient was started on tab levepil 500 mg BD along with tab clobazam 5 mg HS. Over the next 4 years, he had 5–6 episodes per year. At the age of 10 years, the patient had a change in seizure semiology, he had abnormal twitching movements in his perioral area and picking movements in both his upper limbs, followed by neck drop and loss of posturing leading to fall. This episode lasted for 20–30 s. The patient had 10–12 episodes per day. Tab valproate 500 mg BD and tab lacoset 100 mg BD were added and dose of clobazam was increased to 10 mg HS. The patient was additionally treated with three courses of methylprednisolone at 10 years old. After three courses, seizures were controlled.

Discussion and conclusions: The magnetic resonance imaging (MRI) brain was done which was normal. The electroencephalogram (EEG) was suggestive of generalized slow-wave activity. Proper history and genetic analysis are essential in neurology.

Abstract ID: 824: Genetic Profile of a Cohort of Indian ALS Patients

SEENA V, Gautham Arunachal Udupi, Keerthipriya M S, Saraswati Nashi, Deepak Menon, Atchayaram Nalini

National Institute of Mental Health and Neuro-Sciences (NIMHANS), Bengaluru, Karnataka, India

Background and Aim: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with progressive degeneration of upper and lower motor neurons. In all, 10% of the cases were familial, the rest were sporadic. Common genetic forms are C9ORF72, SOD1, VCP, TDP42, and OPTN. The genetic spectrum of ALS patients in India is unknown and this study was undertaken to know the genetic profile of Indian ALS patients.

Methodology: Retrospective analysis of genetic data of 700 patients with ALS observed in NIMHANS, Bangaluru, from 2015-2022.

Results: In all, 113 patients had a confirmed genetic mutation. The mean age at presentation was 51.9 ±11.2 (19-72) years. The male:female ratio was 71:42. The most common genetic variants identified were - SETX (13) and SOD1 (12). Eight patients, each had mutations in DCTN1, FUS, NEFH, and OPTN. Other less common mutations were in FIG4, UBQLN2, TARDBP, ATXN2, ANXA11, UNC13A and ERBB4. Rarely mutations were found in C9ORF72, MATR3, CHMP2B, VAPB, GRN, SQSTM1 and TBK1. The majority were heterozygous variants, except for two cases of OPTN with homozygous mutations. The majority were missense variants (90.2%), except two in-frame deletions in NEFH, FUS, stop gain mutations in OPTN, FIG4, splice regions in SETX, OPTN, splice acceptor defect in OPTN, splice donor defect in FIG4, initiator codon defect in OPTN, in-frame insertion in NEFH and deletion in SPG11. Novel variants were identified in NEFH, SOD1, OPTN, DCTN1, UNC13A, FUS, TARDBP, UBQLN2, ANXA11, ATXN2, SETX, FIG4, MATR3, ERBB4, and TBK1.

Conclusions: This study unravels the genetic spectrum of patients with ALS from an Indian cohort. SETX and SOD1 mutations were the most common. Missense and heterozygous variants were identified in 90% of the cases.

Abstract ID: 825: Smartphone Use Restriction as a Treatment of Primary Headache: A Randomized Controlled Clinical Trial

Adila Jawaid, Deepti Vibha

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Primary headaches, such as migraine and tension-type headaches, are highly prevalent conditions. Smartphone use has been associated with an increased risk of brain tumors and may also affect headache outcomes. We aim to conduct a randomized controlled trial to examine the effects of smartphone restriction in patients with primary headaches as a potential non-pharmacological treatment.

Methodology: We recruited patients with primary headaches and assessed their smartphone use by screen time and safety attitudes questionnaire (SAQ) score. We assigned non-smartphone users to two external control groups (Arm A and B), and high smartphone users to either standard treatment (Arm C) or intervention group (Arm D). The intervention group was instructed to reduce their smartphone use by one-third for 4 weeks. We used statistical methods to compare the groups and identify the mediators and moderators of the intervention effect.

Results: The expected outcome of acute pain medications, headache frequency, and the number of prophylaxis medications used for headaches in the 1st, 3 rd , and 6 th months after the start of the study period, between the intervention group and the control group. We expect that the intervention group used all three measures significantly less than the control group at the end of the study period.

Conclusions: This study was the first to investigate the longitudinal association of smartphone use and headache characteristics in patients. The study documents smartphone uses in various aspects in patients with headaches and analyzes the evolution of time to study the temporal relationship of smartphone use in patients with primary headaches.

Abstract ID: 826: Prevalence of Neurological Disorders Across Urban Population in Guwahati, Assam, India

Manash Ghose, Nomal Chandra Borah, Rupjyoti Das, Amit Ranjan Barua, Aparajita Barman, Ananya Barman

Kha lane, Usha nagar, Byelane 2, PO Dispur, Guwahati, India

Background and Aim: Neurological disorders have a significant impact on health globally. They pose a large economic burden worldwide and also contribute to mortality and morbidity. This study was thus designed to understand the prevalence of different neurological disorders in the city of Guwahati.

Methodology: The study was carried out in the urban population of Guwahati, Assam. A house-to-house survey was conducted to include individuals who were found eligible and gave consent for participation in the study. All subjects underwent a screening phase followed by a clinical evaluation phase.

Results: A total of 1,727 (out of 8,010) individuals were found positive for different neurological disorders. The crude prevalence rate was 21.6%. Patients were classified exclusively on the basis of clinical evaluation. The cohort comprised 4,130 males (52.18%) and 3,830 females (47.82%). The age range varied from 7 to 81 years. A high prevalence of neurological disorders was noted in the age group of 11–20 years (n = 2,166; 27%). The literacy rate was 90.4% (n = 7,241). Ethnicity-wise 92.55% (n = 7,413) of the populations were Assamese, 5.69% ( n = 456) were Bengali and 1.39% (n = 111) were Nepalese. Religion-wise, 87.63% (n = 7,019) of the population were Hindus, whereas the remaining 12.33% (n = 988) were Muslims. The most common neurological disorders were headache (n = 1,079; 62.5%) followed by head and neck pain (n = 330; 19.1%), and epilepsy (n = 80; 4.63%).

Conclusions: Thus, healthcare professionals need to develop a multifaceted comprehensive approach for the control of these neurological disorders.

Abstract ID: 827: A Case of CIDP Associated with HIV

Sangeetha Nair, Sakthi Velayutham S, Haritha S, Aubin Mathew Varghese, Ramgiri Kamlesh, Mugundhan K

Stanley Medical College Chennai, Tamil Nadu, India

Background and Aim: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by proximal and distal weakness of all four limbs, progressive over more than 8 weeks, with hypo/areflexia. It is an aberrant immune response directed toward the components of myelin sheath, and occurs in the setting of HIV, as are other immune-mediated neurological disorders.

Case report: A 46-year-old gentleman came with distal followed by proximal lower limb weakness, then distal upper limbs weakness and truncal weakness, associated with the numbness of both lower limbs, gradually progressing over 1 year. Examination revealed hypotonia of all four limbs, distal weakness of both upper limbs and distal more than proximal weakness of both lower limbs, truncal weakness, global areflexia, and graded loss of all sensations over both lower limbs.

Discussion: Routine hematological and biochemical investigations and thyroid profile were normal. The nerve conduction study (NCS) showed demyelinating neuropathy of all four limbs with secondary axonal changes and polyradiculopathy. He was detected to be HIV (+) and his CD4 count was 219. The cerebrospinal fluid (CSF) study showed albuminocytological dissociation. He was managed with intravenous immunoglobulin (IVIG) 0.4 g/kg/d for 5 days with a diagnosis of CIDP associated with HIV.

Conclusions: HIV-infected patients with CIDP have a benign course with a few relapses and respond rapidly to corticosteroids. CSF protein levels are slightly higher in patients with HIV reflecting an immune-mediated disruption of blood–nerve barrier at spinal roots. They have higher CSF lymphocyte counts due to CSF viremia. A decline in T-regulatory-CD4 cells potentiates autoimmune phenomena in HIV. Antiretroviral treatment (ART) results in immune reconstitution and contributes to remission. Some patients with low baseline CD4 and high viral load have CIDP as an immune reconstitution inflammatory syndrome (IRIS). Concomitant use of ART with steroids may curtail IRIS.

Abstract ID: 828: A Case of Reversible Visual Loss

Alex J, Kingsly Jeba Singh, Sethuram A, Shankar Ganesh

Kanyakumari Government Medical College Kanyakumari, Tamil Nadu, India

Background and Aim: 60/M, came with chief complaints: vision loss in both eyes x 1 day with a Background history of chronic alcoholism with low nutritional status consumes alcohol daily, smoked 20 beedis/day. He was apparently normal 1 day ago and on the previous night before his fishing job had an alcohol binge with four members of his fishing crew and he was fishing. On the next day early morning around 3 am, he developed sudden painless progressive loss of vision in both eyes. Initially, he was able to identify his fellow fisherman with difficulty, and at 6 am on reaching shore, he was not able to perceive light.

Case report: Built-thin, poorly nourished, clubbing + nicotine stains + arcus senilis+, higher mental function (HMF): conscious, oriented to time, place, person. Speech: normal in fluency, repetition, naming, and comprehension. II (Optic): V/A not able to perceive light, visual field-centro-caecal scotoma + color vision could not be assessed, pupil (direct/indirect)-3 mm, sluggishly reacting to light, fundus-temporal pallor+.

Discussion: Blood workup: Hb: 13 g/dL, total count (TC): 4000, platelet (PLT): 2.1, peripheral smear– no megaloblast, serum protein level: 5.5, viral markers were negative, C-reactive protein (CRP): negative, erythrocyte sedimentation rate (ESR): 3, Also, rheumatoid arthritis (RA) factor- negative. Mean corpuscular volume (MCV)-93 fl, mean corpuscular hemoglobin (MCH)- 29 pg, mean corpuscular hemoglobin concentration (MCHC)- 34 g/dL, visual evoked potential (VEP)- not obtained, intraocular pressure (IOP)- normal.

Conclusions: Toxic optic neuropathy (amblyopia)-alcohol, tobacco, nutritional.

Abstract ID: 829: Seizure Disorder-Diagnosis Unwrapped

Anisha Ashraf, Shankar Ganesh

Kanyakumari Government Medical College, Tamil Nadu, India

Background and Aim: A 31/M from Valliyur with no occupation presented to the casualty in a confused state following involuntary movements involving all four limbs associated with unrolling of eye movements that day morning The patient was apparently normal since that day morning; then, he developed C/O one episode of seizures–involving all four limbs, lasting for 1 min, associated with tongue bite, unrolling of eye movements, frothing from the mouth, and postictal confusion lasted for 5 min, not associated with level of consciousness (LOC), urinary incontinence, headache, post-ictal paralysis. No precipitating factors or aura preceding the episode. H/o skin lesions over the face and trunk since childhood. H/o poor scholastic performance since childhood. K/C/O seizure disorder since childhood treatment for 1 week.

Case report: Vitals- normal, conscious- oriented, Ash Leaf Spots+ Shagreen Patch+ mild intellectual disability cranial nerve examination – normal, tone- normal, power -5/5 5/5, plantar- bilateral flexor, no cerebellar/meningeal Signs.

Discussion: Blood Investigations- normal, Echo- normal, computed tomography (CT) chest- normal, CT brain- multiple subependymal calcifications noted, magnetic resonance imaging (MRI) brain- enhancing nodular lesion with calcification at Foramen of Monro at aubependymal location suggest subependymal giant cell astrocytoma, subependymal calcified nodules along lateral ventricle with cortical tubers.

Conclusions: Tuberous sclerosis with seizure disorder.

Abstract ID: 830: A Case of Issac Syndrome with Double Seropositivity

Krishna Chaithanya Reddy Bollipalli

Osmania Medical College, Hyderabad, Telangana, India

Background and Aim: Issac syndrome, is a peripheral nerve hyperexcitability (PNH) disorder marked by muscle twitching, myokymia, neuromyotonia, and dysautonomia. Many patients have voltage-gated potassium channel (VGKC) complex antibodies. It is often considered a paraneoplastic syndrome due to its association with thymoma and small-cell lung cancer. Clinical overlaps are common in patients with leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) antibodies, the primary VGKC complex antibodies.

Case report: A 38-year-old male patient presented with muscle twitching, cramps, increased sweating, and insomnia for 7 days. There was no limb weakness, sensory complaints, cranial nerve involvement, hallucinations, psychotic behavior, tremors, agitation, seizures, fever, vomiting, or headache. There was no history of heavy metal exposure, herbal medicine intake, weight loss, appetite loss, fever, night sweats, thyroid disorders, or other autonomic symptoms. He had similar symptoms 1.5 years ago, managed conservatively.

Discussion: His complete blood picture (CBP), liver enzymes, creatine phosphokinase (CPK), thyroid profile, chest computed tomography (CT), and nerve conduction study (NCS) were normal. Needle electromyography (EMG) showed myokymic discharges and neuromyotonia. Anti-VGKC antibodies were positive for CASPR2 and LGI1. He was treated with intravenous immunoglobulin (IVIG) (2 g/kg over 5 days), phenytoin (100 mg BD), and azathioprine. Symptoms significantly reduced, and there were no symptoms on a 1-year follow-up.

Conclusions: Double seropositivity can lead to more severe symptoms, and recurrent attacks, and may or may not be associated with thymoma.

Abstract ID: 831: From Seizures to Surprise: A Case Series of Three Patients

Ibrahim Hussain, Deepika Joshi, Anand Kumar

Banaras Hindu University, Varanasi (BHU, Varanasi), Uttar Pradesh, India

Background and Aim: Many conditions mimic seizures. Here, we discuss three cases that were diagnosed as seizures from the periphery but were not true seizures after evaluation.

Case report: We described three cases that mimicked seizures. Case 1: A 42-year-old man presented with a history of multiple episodes of abnormal twitching toward one side along with a brief episode of loss of consciousness lasting for about 30 s. It was not controlled despite five antiseizure medications (ASMs). The video electroencephalogram (VEEG) and magnetic resonance imaging (MRI) brain were normal. The pulse rate was found to be 36/min. The electrocardiogram (ECG) monitoring showed a complete heart block with intermittent pauses. The patient became asymptomatic after the permanent pacemaker implantation (PPI) implant. Case 2: A 20-year-girl with right focal seizures in the past presented with multiple episodes of loss of consciousness lasting about 2 to 3 h. The non-contrast CT (NCCT) showed a left parietal neurocysticercosis (NCC). The seizures were uncontrolled with five ASMs. Admitted and observed- abnormal thrusting, cycling, and bipedal movements suggestive of psychogenic seizures. Ictal EEG movement artifacts. Better with psychotherapy. Case 3: A 24-year-old patient presented with a history of abnormal tonic posturing of both upper and lower limbs. Treated as epilepsy elsewhere with levetiracetam 1000 mg/day. History reviewed-abnormal dystonic posturing following sudden getting up from rest. Paroxysmal kinesthetic dyskinesias triggered by the movements. Started on oxcarbamazepine with symptom relief.

Discussion and conclusions: Syncope, psychogenic seizures, and kinesthetic dyskinesias may mimic seizures. Simple clinical assessments such as pulse rate assessment, often omitted in the busy OPDs, may be life-saving. History taking and home video may help in the diagnosis.

Abstract ID: 832: The Orgy of HSV Encephalitis in Immunocompetent Young Patients

Abhinav Ankur

Vardhman Mahavir Medical College (VMMC), New Delhi, India

Background and Aim: Herpes simplex virus (HSV) encephalitis accounts for up to 20% of all identified viral encephalitis. A 2014 study showed with acyclovir, mortality has reduced from 38% to around 5.5–11% with the majority of mortality in immunocompromised individuals. We present two immunocompetent patients who presented with gastrointestinal symptoms, developed late neurological symptoms, and despite an adequate dose of acyclovir did not improve.

Case report: Case 1: A 19-year-old immunocompetent female presented with hiccups for 10–15 days. After admission, she developed an altered sensorium. On further questioning, a family member revealed a history of low-grade fever on and off for the past 1 month. Routine cerebrospinal fluid (CSF) parameters were within normal limits. The electroencephalogram (EEG) showed generalized slowing. The magnetic resonance imaging (MRI) showed asymmetrical hyperintensity in the left infero-frontal and insular cortex and thalamus. HSV polymerase chain reaction (PCR) was positive and despite 21 days of acyclovir therapy, the patient did not improve and died. Case 2: A 15-year-old immunocompetent boy presented with abdominal pain with a low-grade fever for 15 days. Ultrasonography (USG) was suggestive of necrotic lymphadenopathy. CSF parameters were normal. The patient developed new-onset refractory status epilepticus on the 7th day of admission. A repeat CSF study revealed normal routine parameters. EEG showed very low amplitude delta activity. MRI showed symmetrical hyperintensities. HSV PCR was found to be positive and despite 21 days of acyclovir therapy, the patient did not improve and died.

Discussion and conclusions: It emphasizes the possibility of HSV encephalitis even if a patient presents with gastrointestinal symptoms with chronic onset. There should be a low threshold for testing of HSV PCR and consideration for acyclovir therapy even with normal CSF routine parameters if there is either clinical or electroencephalographic finding of seizure.

Abstract ID: 833: A Study on Awareness and Health-Seeking Behavior of Migraine Patients in a Recently Established Tertiary Care Center in Assam

Hemanga K. Dhing, Hemanga Kumar Dhing, Masaraf Hussain

All India Institute of Medical Sciences, Guwahati, Assam, India

Background and Aim: Migraine is a common type of headache leading to significant disability. Yet, there is a significant lag in seeking treatment due to a lack of awareness. The aim is to study the awareness and health-seeking behavior of patients with migraine in a semi-urban population in Assam.

Methodology: This was a retrospective study conducted in the Neurology OPD in All India Institute of Medical Sciences (AIIMS) Guwahati from December 2023 to May 2024. Data were collected using a questionnaire filled out by the patients with consent, as per the routine OPD procedure.

Results: A total of 172 patients were included, and diagnosed according to the international classification of headache disorders (ICHD-3) criteria. The mean age was 33.3 (±11.4) years. The mean duration was 5.5 (±7.35) years. Hemicranial headache was present in half (51%) of patients. Headache was pulsatile in nature in 55% of patients. Photophobia and phonophobia were the most common associated features (78%). Imaging was conducted by 33 (19%) patients. Migraine was transformed to chronic in 46 (26.7%) patients. The mean migraine disability assessment (MIDAS) score was 29 (±23). Disability was severe in 84 (47.2%) patients. Only 16 (9%) patients had consulted neurologists previously. Half (49%) of the patients had sought consultation of other speciality, the most common being ophthalmologists (44%), followed by ENT (8.7%). Multiple specialty consultation was taken by 11% of patients. Only 10% of patients have taken prophylactic drugs. The maximum duration was 2 years taken by single patients. The average duration was 3 months. The reason for discontinuation of the drug was either non-remission or unawareness of the chronicity of the disease.

Conclusions: The majority of the patients though suffering from severe disable migraine, were unaware of the disease or its chronicity. Adequate awareness measures are needed for proper management

Abstract ID: 834: Unraveling a Decade-Old Mystery of Recurrent Seizures and Recurrent Strokes in a Young Adult

Aishwarya Jirwankar, Aditya Gudhate, Mayur Thakkar, Neeraj Jain, Sangeeta Rawat

Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

Background and Aim: Focal epilepsy with recurrent strokes in a young adult has several differentials and needs further evaluation based on the clinical profile. Atypical presentation of genetic disorders such as mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is not uncommon. We highlight the same in our case.

Case report: We evaluated a case of a 30-year-old man who presented in our OPD with a 10-year history of recurrent focal seizures with recurrent attacks of transient neuro deficits in the form of diminution of vision and ataxia for 5 years and now having episodic slurring of a speech lasting for < 24 hrs.

Discussion: He was evaluated elsewhere and all his routine investigations, including vasculitis workup, were normal. He was subjected to a magnetic resonance imaging (MRI) brain on several occasions, which showed acute ischemic stroke at various locations every time not conforming to a particular vascular territory. His digital subtraction angiography (DSA) was normal and so were serum lactate levels. Suspecting a mitochondrial disorder, his mitochondrial genome was studied. His genetic testing revealed a rare MTND 5 mutation detectable in less than 10% of MELAS cases.

Conclusions: MELAS can mimic various disorders presenting with recurrent episodic transient neuro deficits such as demyelination and vasculitis. All cases may not show typical features of MELAS and serum lactate levels can be normal. A high index of suspicion should prompt genetic analysis to help in diagnosing and prognostication.

Abstract ID: 835: Case of Cockayne Syndrome

Sangeetha Nair, Kuralarasi Annadurai, Sownthariya R, Sowmini P R

Stanley Medical College Chennai, Tamil Nadu, India

Background and Aim: Cockayne syndrome is a rare autosomal recessive genetic disorder resulting from a DNA repair defect, leading to increased sensitivity of cells of affected individuals to the harmful effects of ultraviolet (UV) light. Patients display unique facial features including sunken eyes, beaked nose, and prominent ears, cachectic dwarfism, intellectual disability, microcephaly, progressive neurological dysfunction, gait defects, retinal degeneration, hearing impairment, and photosensitivity.

Case report: Two siblings, a 12-year-old girl, and a 10-year-old boy, born out of third-degree consanguineous marriage, without a family history of neurological illnesses, were brought with a 5-6-year history of progressive unsteadiness while walking and clumsiness of hands, associated with short stature. On examination, the 12-year-old girl had a height of 118 cm, a weight of 18 kg, a head circumference of 48 cm, sunken eyes, a beaked nose, prominent ears, and malaligned teeth. Neurological examination revealed mild cognitive dysfunction, scanning-type dysarthria, rigidity of all four limbs, appendicular, and axial ataxia. The nerve conduction study (NCS) showed sensory motor axonal neuropathy of all four limbs. The magnetic resonance imaging (MRI) brain showed mild cerebellar atrophy. On examination, the 10-year-old boy had a height of 113 cm, a weight of 18 kg, a head circumference of 48 cm, a beaked nose, prominent ears, and mal-aligned teeth. Neurological examination showed appendicular and axial ataxia. NCS showed sensory motor axonal neuropathy of all four limbs. MRI brain showed bilateral basal ganglia calcifications.

Discussion: Ophthalmologic, ENT, and cardiac evaluation were normal for both children. Routine hematological and biochemical investigations, thyroid profile, and growth hormone levels were normal for both children.

Conclusions: Annual eye and ear checkups, blood sugar and blood pressure (BP) checkups, liver function test (LFT) and renal function test (RFT), and periodic reviews with physiotherapists are advised. Clinicians should prognosticate the illness and provide parental counselling.

Abstract ID: 836: A Multicenter, Observational Study to Assess Clinical Effectiveness and Safety of Levetiracetam Extended Release (XR) Formulation in Outpatient Settings of India (RESOLVE Study)

Krishnaprasad Korukonda, Shalin Shah, Umang Patel, Girish Kulkarni, Rasika Deshpande, Yakshdeep Dave, Girish Kulkarni, Rasika Deshpande

Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India

Background and Aim: A significant proportion of people with epilepsy (PWE) shows inadequate response due to poor treatment adherence and compliance on long term. Levetiracetam (LEV) is widely used as first-line anti-seizure medication (ASM) in range of PWE. The current study was explored to foster understanding about safety and effectiveness of LEV extended release (XR) formulation as maintenance strategy in real-world settings.

Methodology: This was a multicenter, retrospective, observational study performed at 80 centers across India. The study was approved by central ethics committee. Change in seizure frequency, clinical global impression of improvement (CGI-I) and adverse events (AEs) at 12 weeks were analyzed using descriptive statistics using SPSS version 29.0.1.0.

Results: 1255 PWE from 80 centers were included, gender: 793 (63.18%) male and 462 (36.81%) female with mean age 40.66 years ± 15.70 SD. 689 (54.90%) were diagnosed with focal onset seizure (FoS); 468 (37.29%) with primary generalized tonic-clonic seizures (PGTCS), 65 (5.18%) with juvenile myoclonic epilepsy (JME), whereas 33 (2.63%) with focal to bilateral tonic–clonic seizures (FBTCs). LEV XR was used as an adjuvant and as monotherapy in 997 (79.44%) and 258 (20.56%), respectively. For Focal onset seizures, median monthly seizure frequency was substantially reduced from 3 to 1 at 12 week (p <0.0001), responder rate and seizure freedom were 64.22% and 24.86%, respectively. CGI-I score showed 35.70% as “very much improved” and 40.08% as “much improved”. Treatment emergent adverse events (TEAEs) were dizziness (24.22%), somnolence (21.22%) and behavioral adverse events (BAEs) (20.32%) at the dose of 1000 mg of LEV XR, which was not associated with treatment withdrawal.

Conclusions: LEV XR is safe and efficacious ASM for the treatment of partial as well as generalized seizures in Indian context.

Abstract ID: 837: Parry Romberg Syndrome – A Unique Neurocutaneous Disorder

Pavithra Ravi, Sangeetha J Nair, Malcolm K Jeyaraj, K. Mugundhan, Sakthi S. Velayutham, P. R. Sowmini, V. Kannan, R Viveka Saravanan, K. Mugundhan

Stanley Medical College Chennai, Tamil Nadu, India

Background and Aim: Parry–Romberg syndrome (PRS) is a rare disorder which is characterized by unilateral facial atrophy affecting skin, soft tissue, muscles and also extending to the bone with associated systemic manifestations. There is a close relationship between Parry–Romberg syndrome and linear scleroderma en coup de saber. Around 15% patients present with neurologic manifestations. We report a 14-year-old girl with progressive hemifacial atrophy with migraine and occipital seizures with characteristic imaging.

Case report: A 14-year-old girl presented with progressive atrophy of left side of face with patchy hair loss on left frontal region of scalp of 3 years duration. Initially, patient was treated as localized scleroderma. Patient had left-sided migraine type headache for 3 years. She had 3 episodes of seizures in past 4 months, starting with severe throbbing left-sided headache followed by seeing orange/yellow round spots moving from right to left visual field lasting for 5-10 minutes followed by tonic-clonic movements of right upper and lower limb with angle of mouth deviation to right with loss of awareness lasting for 2 minutes with post-ictal confusion for 15 minutes. On examination, patient had left eye telangiectasia, left frontal alopecia, left focal atrophy of forehead, face and ear and no other focal neurological deficits.

Discussion: The magnetic resonance imaging (MRI) brain showed ill-defined non-enhancing T2/ fluid-attenuated inversion recovery (FLAIR) hyperintensities with multiple foci of calcification and microhemorrhages involving left parieto-occipito-temporal cortex and white matter and left capsuloganglionic region. Mild leptomeningeal enhancement noted in left parieto-occipital sulcal spaces. The video electroencephalogram (VEEG) showed left occipito-temporal focal slowing. Antinuclear antibodies (ANA) and extractable nuclear antigen antibodies (ENA) profiles were negative. Patient is diagnosed to be a case of Parry–Romberg syndrome with migraine and left occipital seizures. She has been started on anti-epileptic drugs (AEDs) and also in dermatology follow-up.

Conclusions: The recognition of PRS is essential for its multidisciplinary treatment approach. Immunosuppressive drugs can be used in patients with cerebral involvement.

Abstract ID: 838: Stroke as Initial Presentation of Systemic Lupus Erythematosus Without Other System Involvement

Rajasekara Pandian Thiruvettai, Sowmini P R, Kannan V, Viveka Saravanan, Mugundhan Krishnan, Sakthi Velayudham, Malcolm Jeyaraj

Stanley Medical College, Tamil Nadu, India

Background and Aim: Systemic lupus erythematosus (SLE) is an autoimmune illness with multisystem involvement, and usual neuropsychiatric manifestations of SLE are accompanied by other system involvement also. Isolated central nervous system (CNS) vasculitis secondary to SLE presenting as stroke is not common. We present a case of stroke in young women as initial manifestation of SLE.

Case report: A 28-year-old female mother of a single child was admitted with right hemiparesis with speech disturbance for 1-day duration, with no risk factors and no recurrent pregnancy loss. Initial diagnosis of stroke in young was made

Discussion: Basic investigations and computed tomography (CT) brain plain were normal, magnetic resonance imaging (MRI) brain showed, few microbleeds in midbrain, T2-weighted fluid-attenuated inversion recovery (T2 FLAIR) hyperintensity with diffusion restriction in left medial hemi medulla, T2 FLAIR hyperintensity without diffusion restriction in pons, with leptomeningeal enhancement in pontomedullary junction, mesencephalo pontine junction and superior aspect of pons and her laboratory reports were positive for antinuclear antibody (ANA)-3+ positive (homogeneous pattern with fine granules). Her ANA profile showed SS-A –strong positive RO -52- strong positive, SS-B -strong positive. Other procoagulant workup including antiphospholipid antibody (APLA) was negative, and viral markers (HIV 1 and 2, HBsAg, antiHCV antibody- negative, aquaporin antibody and myelin oligodendrocyte glycoprotein (MOG) antibody) were negative (normal cardiac status and carotid vertebral doppler study normal). Patient also underwent cerebrospinal fluid (CSF) analysis to rule out infectious causes of leptomeningeal enhancement (cartridge based nucleic acid amplification test for tuberculosis was negative).

Conclusions: Systemic lupus erythematosus can also present as isolated CNS vasculitis, presenting as stroke in the absence of other system involvement was unique and unusual

Abstract ID: 839: Loss of Ambulation Due to Calcific Myonecrosis: A Rare but Reversible Complication of Wilson’s Disease

Abhishek Vaingankar, Divyani Garg, Ayush Agarwal, Divya M. Radhakrishnan, Awadh Kishor Pandit, Madhavi Tripathi, Achal K. Srivastava

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Wilson’s disease (WD) is a rare disorder of copper metabolism due to autosomal recessive mutation of ATP7B gene on chromosome 13q14-21. Here, we report two cases of Wilsons’s disease presenting with calcific myonecrosis of the pelvic girdle, leading to loss of ambulation.

Methodology: A search was conducted in PubMed using the following keywords ‘Wilsons disease’, ‘Wilson’s disease’, ‘bone’, ‘osteomuscular’, ‘osseus’, ‘osseomuscular’ and ‘skeletal’ in varying combinations. The search yielded 159 results. Titles and abstracts of the articles were screened, and full texts of 109 papers were analyzed. Seventeen articles were included in the final review.

Results: We report two established cases of Wilson’s disease with extensive heterotopic ossifications of the muscles around the pelvic girdle associated with pelvic fractures, leading to loss of mobility. Both patients were already on treatment for Wilson’s disease which included chelation and rehabilitation. However, despite an initial period of improvement, both patients developed lower limb and pelvic pain and grew wheelchair-bound, leading to a consideration for possible neurological deterioration. With prompt recognition of calcific myonecrosis and intensive rehabilitation, both patients showed improvement.

Conclusions: Osseomuscular manifestations are underrecognized manifestations of Wilsons’s disease. This case report highlights calcific myonecrosis as very rare complication of Wilson’s disease. Timely recognition may prevent further disability

Abstract ID: 840: Not so common Ripple In Tubercular Meningitis: Facial Myokymia in Tubercular Meningitis

Pranjali Batra, Varun Kumar Singh, Rameshwar Nath Chaurasia

Banaras Hindu University (BHU), Varanasi, Uttar Pradesh, India

Background and Aim: Tubercular meningitis may affect the persons of all ages. Movement disorders have been described in tubercular meningitis.

Case report: A 60-year-old female presented with low-grade fever for two months and altered sensorium for the past 20 days. She also had neck rigidity, decreased movement on right side of the body and facial myokymia.

Discussion and conclusions: Movement disorders have been reported in Tuberculous meningitis (TBM). Facial myokymia is rare in TBM with multifactorial pathomechanism.

Abstract ID: 842: Mixed Connective Tissue Disease (MCTD): A Rare Presentation of CNS Vasculitis

Arpan Mitra, Abhishek Pathak, Abhishek Pathak

Institute of Medical Sciences (IMS), Varanasi, Uttar Pradesh, India

Background and Aim: The absence of an etiology within 72 hours of de novo refractory status epilepticus is the sine qua non-condition for new onset refractory status epilepticus (NORSE). NORSE is a challenging syndrome to diagnose and manage, and the outcome is generally poor with high mortality rate. One major challenge with NORSE is ascertaining the underlying etiology, a key determinant of outcome. The most common etiology of NORSE is cryptogenic followed by autoimmune. In this study, our aim is to identify clinical and radiological profile of NORSE patients and also to look for treatment response and outcome.

Methodology: It is a prospective study where all the patients of NORSE admitted in neurology ward/ICU of IMS, BHU from June 2022 to May 2024 are consecutively included. Detailed history and clinical examination done with all necessary investigation including magnetic resonance imaging (MRI)brain, video electroencephalograph (VEEG), cerebrospinal fluid (CSF) analysis, autoimmune and infection workup. Treatment is given as per standard protocol. After discharge, all survived patients were followed up for 3 months.

Results: A total of 19 NORSE patients were included in our study. Mean age of patient is 26.6 ± 12.8 years, male: female= 8:11. Mean duration of hospital stay is 25.6 days. Most common cause was found to be cryptogenic (n = 6) followed by seronegative autoimmune encephalitis (n = 4). Hospital mortality is 26.3% (n = 5), and total mortality on three-month follow-up is 36.8% (n = 7)

Conclusions: NORSE is rare clinical situation where diagnosis and treatment are challenging. Further research is needed to answer various questions encountered in routine clinical practice.

Abstract ID: 843: Movements and the Smear

Sri Sai Venkata Haritha Putrevu, Gopi Seepana

Andhra Medical College Visakhapatnam, Andhra Pradesh, India

Background and Aim: Neuro-acanthocytosis is a group of disorders characterized by movement disorders, neuropathy, cognitive and psychiatric disturbances and seizures with acanthocytes on peripheral blood smear.

Case report: A 36-year-old man, born out of second-degree consanguineous parentage, presented with seizures— 4 years duration, involuntary movements— 2 years duration. Seizures— left focal tonic to bilateral tonic-clonic seizures. Involuntary movements—excessive bending at the knees with intermittent dragging and inward turning of right foot, stamping of left foot and leaning to either side. the posturing of neck— bending backward while sitting, talking, opening of jaw. Random movements in the form of fidgeting, crossing legs while lying or sitting. Slurred speech— unintelligible. Difficulty swallowing— choking spells with pushing of bolus out of mouth while eating. Lip bites present. Diabetes mellitus— 4 years duration. On examination, generalized wasting— more in hands and feet. Bilateral pes cavus. Hypotonia of all the limbs. Grade 4 weakness in left upper limb. Reflexes absent. Plantar- flexor. Involuntary movements— retrocollis, increasing on walking, intermittent opening of jaw, while walking— dragging of right foot, stamping of left foot and leaning to left. On outstretched hands— posturing of fingers. Feeding dystonia present. Chorea present— shrugging, fidgeting, crossing and uncrossing legs. Sensory examination— loss of joint sense in all limbs. Romberg’s test positive

Discussion: Investigations: lipid profile- normal. Serum creatinine phosphokinase- 1115 U/L. acanthocytes in peripheral smear, nerve conduction study (NCS)- normal, echocardiography-cardiomyopathy, magnetic resonance imaging (MRI) brain- bilateral caudate atrophy. Genetic analysis- homozygous mutation of VPS13A. Suggestive of chorea-acanthocytosis.

Conclusions: Clinical suspicion is needed. Chorea-acanthocytes can be diagnosed with a routine investigation like peripheral blood smear.

Abstract ID: 844: A Case of Vasculitic Neuropathy

Molugu Samhitha, Radhakrishna Hari, Irfan Mohammed

Medicover Hospitals, Hitech City, Hyderabad, India

Background and Aim: Vasculitic neuropathy is a dreaded complication of many diseases. Non-systemic vasculitic neuropathy accounts for 30-35% cases of vasculitic neuropathies. The remaining systemic vasculitic neuropathies are divided into primary and secondary.

Case report: A 74-year-old female, with no comorbidities presented with numbness of both feet for 10 days, gradually progressive associated with difficulty in walking. Symptoms were more severe in left lower limb (LL), and left foot was totally immobile. Mild intermittent sensory impairment in both LL for one month. Tingling sensation in right hand involving 1, 2 and 3 fingers for 3 days. Burning sensation over soles at nights, disturbing her sleep. Requires a walker for ambulation. Past H/o joint pains involving knees, shoulders and wrists for 2 years, without treatment. Later, diagnosed with fibromyalgia, treated with corticosteroids and leflunomide for 4 months and treated for osteoarthritis of knees. O/E- hypotonia and foot drop in both LL (left>right). Power at knees-3/5. Bilateral LL- areflexia. Bilateral sensory loss for all modalities below knees (left>right). No sensory loss or motor weakness in upper limbs.

Discussion: Nerve conduction study (NCS)- no compound muscle action potentials (CMAPs) and no sensory nerve action potential (SNAPs) from both lower limbs. Mild decrease in SNAP amplitude from Rt.ulnar nerve. Magnetic resonance imaging (MRI) lumbar spine-spondylotic changes. Erythrocyte sedimentation rate (ESR) 30mm/1st hour, C-reactive protein (CRP) 76.3 mg/dl, anti-cyclic citrullinated peptides (CCP) antibody 464U/L, rheumatoid arthritis (RA) factor - 377.3U/ml. Left sural nerve biopsy s/o vasculitic neuropathy. She was treated with intravenous (IV) methylprednisolone 500 mg for 5 days and Inj. Rituximab. On review, she was able to walk independently.

Conclusions: Peripheral neuropathy may be the first manifestation of vasculitic disorder. Thus, prompt recognition is required to initiate appropriate workup and start treatment to reduce risk of irreversible neurologic damage.

Abstract ID: 845: Woe of Two Brothers: The Partington Syndrome

Sravya Ravi, A. Thomas Edwin Raj, Kannan Balakrishnan, Rachel packiyaseeli, Jitendra Kumar

Thoothukudi Command Hospital

Background and Aim: Among the multitude of causes leading to mental retardation, Partington’s syndrome is a very rare entity (incidence <1/1000000) that should be considered in a male child with positive family history, after the more common Fragile-X syndrome. It has numerous peculiar syndromic presentations, knowledge of which, helps in clinching the diagnosis. The aim is to evaluate and diagnose two brothers who presented with features of this rare, yet unique syndrome.

Case report: Two male siblings with normal birth and developmental history presented with regression of attained milestones, mental retardation and intractable epilepsy. Routine investigations, neuroimaging, electroencephalography, screening for inborn errors of metabolism and genetic analysis were performed as a part of workup.

Discussion: In both siblings, clinical examination revealed regression of milestones, spasticity and mental retardation and routine investigations were normal. Screening for inborn errors of metabolism was negative. Magnetic resonance imaging (MRI) brain of elder sibling revealed T2-weighted fluid-attenuated inversion recovery (T2/FLAIR) hyperintensities with restricted diffusion in both cerebral hemispheres and leukodystrophy, while the MRI brain of second child is normal. Genetic analysis of both siblings revealed ARX mutation ARX.C.596C>A on X chromosome.

Conclusions: Although uncommon, Partington’s syndrome is a potential cause to suspect in male children with a positive family history and features of intractable epilepsy, mental retardation, focal dystonia, autism spectrum disorder (ASD), etc. Accurate diagnosis and subsequent genetic counseling could be offered to avert the anguish in these families.

Abstract ID: 846: Leber’s Hereditary Optic Neuropathy Misdiagnosed as Post-Viral Papillitis

Naresh Chinthala, Atri Kumar Patel, Sagarbhai Hemabhai Choudhari, Ashish Kumar Duggal, Jyoti Garg

Thanjavur Medical College, Thanjavur, Tamil Nadu, India

Background and Aim: Leber’s Hereditary Optic Neuropathy (LHON) is a rare genetic condition caused by mitochondrial DNA mutation. It affects young adults, causing subacute onset painless sequential vision loss over weeks to months. It is caused by a mutation in mitochondrial DNA and is often misdiagnosed.

Case Report: A 16-year-old male presented with gradual onset of vision loss in both eyes following a recent episode of conjunctivitis in both eyes. He experienced difficulty in discerning fine details of objects, which progressively worsened over 15 days and then stabilized. His visual acuity was finger counting at 3 meters in the right eye and 1 meter in the left eye. Color vision was impaired in both eyes. Fundus examination showed bilateral disc pallor with peripapillary dilated and tortuous vessels. The Contrast-enhanced MRI (CE-MRI) brain with orbit and cerebrospinal fluid (CSF) was normal and both neuromyelitis optica (NMO) and myelin-oligodendrocyte-glycoprotein (MOG) antibodies were negative. Initially, he was diagnosed with post-viral papillitis and was treated with steroids, but his vision didn’t improve. Optical coherence tomography (OCT) showed a thinning peripapillary retinal nerve fiber layer. The dilated vessels did not reveal any leakage on fluorescein angiography. In view of disc pallor with peripapillary dilated tortuosity and normal fundus fluorescein angiography (FFA), LHON was suspected, and mitochondrial DNA analysis showed the presence of a G3460A mutation.

Conclusions: LHON may mimic other causes of optic neuritis. Failure to improve with immunosuppression the presence of circumpapillary telangiectasia, which does not show leakage on FFA, are important clinical clues for diagnosis, which can be confirmed by genetic analysis.

Abstract ID: 847: Looking Beyond Sjogren’s Syndrome in Treatment-Resistant CIDP: A Case of Anti Neurofascin Antibody 186 Associated Demyelinating Neuropathy In Sjogren’s Syndrome

Pritam Majumdar, Thomas Mathew, Surabhi Garg, Sucharitha Vasu, Sindhu V Nambiar, Shagun Bhardwaj, Sai Kanth, Sharath Kumar GG, Raghunandan Nadig, Sagar Badachi, Sonia Shivde, Akshata Huddar, Sudheeran Kannoth, Gosala Raja KukkutaSarma, Vineeta Shobha, Anusha Jawahar

St. Johns Medical College, Bengaluru

Background and Aim: Sjogren’s syndrome can present with various neurological syndromes, including chronic inflammatory demyelinating polyneuropathy (CIDP). Typically, Sjogren-related CIDP responds well to corticosteroids but can coexist with other autoimmune diseases, such as ANFA 186-related disease. We present a case of Sjogren-associated CIDP that was resistant to steroids and immunotherapy, and further evaluation revealed co-existent ANFA 186 and responded well to rituximab.

Case report: ANFA155, 186 and 140 were measured by competitive enzyme linked immunosorbent assay (ELISA) technique using human neurofascins, as per the manufacturer’s guidelines of the Blue gene kit. A 55-year-old woman with sicca symptoms and joint pain was diagnosed with Sjogren’s syndrome based on a positive Schirmer test and anti-SSa antibodies. She started treatment with mycophenolate mofetil and oral steroids. Later, she developed bilateral proximal lower limb weakness and asymmetrical sensory symptoms in her hands. Examination showed moderate proximal muscle weakness in the lower limbs, absent reflexes and loss of vibration sense. Nerve conduction velocity tests revealed asymmetric prolongation of distal and proximal F latencies in demyelinating ranges with relatively preserved amplitudes. Magnetic resonance (MR) neurography showed bilateral thickening of lumbar nerve roots. Tests for monoclonal gammopathies were negative, but the autoimmune neuropathy panel was positive for anti-neurofascin 186 antibodies with a titer of 532 ng/mL (normal <232 ng/mL). The patient then received Rituximab and remained symptom-free at a six-week follow-up.

Discussion and Conclusions: In treatment-resistant CIDP associated with Sjogren’s syndrome, considering nodo/paranodopathies is crucial. A low threshold for testing these antibodies can lead to the correct treatment choice at a quick time.

Abstract ID: 848: Unveiling the Genetic Quandary: Exploring Early Onset Familial Alzheimer’s Disease within a Novel Presenilin Mutation-Afflicted Family

Panda Kumar, Aparna Thomas, Suman Kushwaha, Rajinder K. Dhamija

Institute of Human Behaviour and Allied Sciences, Delhi, India

Background and Aim: Early onset Alzheimer’s Disease (AD) characterized by onset before 65 years. Three genes were verified as causative factor for early onset familial Alzheimer’s disease (EOAD): Amyloid precursor protein (APP), Presenilin 1 (PSEN1) and Presenilin 2 (PSEN2). Approximately 300 mutations of PSEN1, PSEN2 and APP in 635 affected individuals or families have been reported in the Dementia Mutation Database ( https://www.alzforum.org/mutations )

Case report: Case 1—our index case was 40-year-old housewife who presented with symptoms of episodic memory loss—5 years, executive dysfunction and behavioral issues—4 years, slowness in activities—2 years, myoclonic jerks—4 months with 1 episode of generalized tonic-clonic seizure (GTCS), inability to do activities of daily living (ADLs) and bedbound since 2 months. O/E- multifocal myoclonus+, generalized rigidity+, Montral cognitive assessment (MoCA)-0. Routine investigations-normal. Magnetic resonance imaging (MRI) brain showed generalized cortical with predominant temporal atrophy. Family history was significant. Case 2—2 weeks later patient’s elder sister 46-year-old housewife admitted with forgetfulness and behavioral issues—2 years, slowness in activities—1 year, GTCS multiple episodes—1-year, myoclonic jerks—4 months with altered sensorium and signs of aspiration pneumonia. Laboratory investigations showed raised total leukocytes, CXR-b/l infiltrates, in view of worsening sensorium and desaturation patient was intubated, later succumbed to death. Case 3—20-year-old male, son of index case, cognitive issues and seizures since 3 years. Case 4—18-year-old daughter of index case with episodes mania. Case 5—father of index case, cognitive decline, behavioral issues, myoclonus started since 35 years, died at 43 years. Case 6—elder brother of father of index case, with cognitive decline, behavioral issues started at 40 years, died at 45 years. Case 7—younger brother of father of index case with cognitive decline, behavioral issues, seizures died at 35 years. Case 8—daughter of younger brother of father of index case with cognitive decline, behavioral issues and seizures. Case 9—Paternal Grandfather of index case, cognitive decline and behavioral symptoms started at 42 years, died by 45 years. Case 10—Son of elder sister of index case, cognitive decline, behavioral issues, seizures since 12 years.

Discussion and conclusions: Here we describe an Indian family with varying symptoms with a novel presenilin mutation.

Abstract ID: 849: Horizontal Gaze Palsy with Progressive Scoliosis: A Case Report from Eastern India

Ande Mihira Chakra Varthy, Sandip Pal, Debsadhan Biswas

Kolkata Medical College, Kolkata, West Bengal, India

Background and Aim: Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare congenital disorder characterized by absence of conjugate horizontal eye movements and progressive scoliosis developing in childhood and adolescence. It occurs due to mutation in ROBO 3 gene (chromosome 11q23-q25). Here, we report a case of a 12-year-old girl who presented with complaints of difficulty in looking sideways since early childhood.

Case report: Detailed history, clinical examination, magnetic resonance imaging (MRI) brain and whole exome sequencing were done in this patient who presented to our neuro-ophthalmology clinic in Medical College, Kolkata. This patient from rural Bengal without any family history presented to us with difficulty in looking to sideways and oscillatory eye movements since early childhood. General examination revealed scoliosis at dorso lumbar spine with convexity to right. On neurological examination, there is convergence on looking sideways suggestive of horizontal gaze palsy with preserved vertical gaze. MRI brain showed midline cleft in medulla with absence of facial colliculus Whole exome sequencing revealed homozygous likely pathogenic ROBO3 mutation which confirmed our diagnosis of HGPPS.

Discussion and conclusions: In patients with congenital cranial dysinnervation disorder predominantly affecting horizontal gaze, we should look for scoliosis.

Abstract ID: 850: An Uncommon Silver Lining Presentation of a Common Disease -A Case of Isolated Unilateral Hypoglossal Nerve Palsy due to Tuberculous Pachymeningitis

Sai Gullapalli, L. A. Ravi, S. Balasubramaniam

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: A 36-year-old female homemaker came with complaints of occipital headache, left-side neck pain since 2 months. One week after above complaints, she had difficulty in manipulating food in mouth and propelling it back to throat. She has no other complaints suggestive of other cranial nerve, motor, sensory or autonomic dysfunction.

Case report: On examination, she has right cervical II, III, IV lymphadenopathy. There is atrophy of left half of tongue with lower motor neuron (LMN) type of XII cranial nerve palsy. No other signs are suggestive of cranial nerve, motor, sensory, cerebellar or autonomic dysfunction and no signs of meningeal irritation.

Discussion: Magnetic resonance imaging (MRI) brain with contrast done in a private hospital was reported as normal. Contrast-enhanced computed tomography (CECT) neck-cervical lymphadenopathy and skull base appear normal. MRI neck plain with contrast - incidental finding of enhancement of pachymeninges at the left parieto-occipital region and pituitary stalk. Right cervical and supraclavicular lymphadenopathy. Cerebrospinal fluid Gene XPERT (CBNAAT)—negative, acellular, glucose -76 mg/dl, protein -25 mg/dl. Vasculitis profile and other autoimmune antibodies- negative. Erythrocyte sedimentation rate (ESR)- 90mm/1st hr. IgG4- normal. Serum angiotensin converting enzyme (ACE)- normal. Cervical lymph node biopsy-caseating granulomatous lymphadenitis with lymphocytic infiltrate, Langerhans giant cells and plasma cells. Tissue reverse transcription polymerase chain reaction (RT PCR) awaited.

Conclusions: This case is being presented due to isolated involvement of pachymeninges by Tuberculous (TB) without brain parenchymal or leptomeningeal involvement (2-3 case reports of isolated tuberculous pachymeningitis till now). It manifested as isolated unilateral hypoglossal nerve palsy which is mostly idiopathic and provided a silver lining to the presentation of patient as it is treatable cause.

Abstract ID: 851: A Case Series of Opsoclonus Myoclonus Syndrome in a Tertiary Care Centre

Bilia Aipu

Grant Medical College and JJ Hospital, Mumbai, Maharashtra, India

Background and Aim: Opsoclonus myoclonus (dancing eye, dancing feet) syndrome is a rare syndrome characterized by opsoclonus, myoclonus and ataxia. Half of all cases occur in children with neuroblastoma. Among various etiologies of opsoclonus myoclonus syndrome (OMS), paraneoplastic, paraviral or idiopathic encephalitis are the most common causes, and an autoimmune mediated brain stem dysfunction is the suggested underlying pathomechanism. Treatment targets the etiology and employs steroids, intravenous immunoglobulin (IVIG), plasmapheresis or other anti-inflammatory therapies. The aim of the study emphasizes the importance of early recognition, findings out the etiology and appropriate treatment can have positive impact on quality of life.

Case report: It is a prospective observational case series study of three patients who presented with opsoclonus, myoclonus, ataxia who were investigated and treated. Informed written consent was taken.

Discussion: All three cases have parainfectious cause as etiology. Workup has been done to rule out other causes. All patients were treated with immunosuppression (i.e., steroids followed by IV immunoglobulin) and showed complete improvement in all patients.

Conclusions: Adult cases of opsoclonus myoclonus with idiopathic or infectious etiologies have a more favorable prognosis than those with neoplastic origin.

Abstract ID: 852: Neuroleptic Malignant Syndrome in Antibody Negative Autoimmune Encephalitis: A Coincidence or Causation?

Manish Bhartiya, Shivank, Vinny Wilson, Arindam Mukherjee

Command Hospital, Southern Command, Pune

Background and Aim: Autoimmune encephalitis (AE) refers to the pathophysiological process characterized by immune-mediated inflammation against neuronal antigens and synaptic proteins. It has varied clinical manifestations which include neuropsychiatric symptoms, seizures and fresh neurological deficits. Neuroleptic malignant syndrome (NMS) is a dreaded complication post-consumption of certain psychiatric medications like antipsychotics. We describe a case of antibody-negative AE in a previously healthy female who presented as NMS.

Case report: A 31-year-old previously healthy female presented with complaints of intermittent episodes of fever and abnormal behavior (excessive crying, decreased verbal output) of two weeks duration. During the course of the stay in the hospital, she developed dystonic posturing, hyperthermia and reduced verbal output.

Discussion: Her examination revealed intermittent episodes of fever, dystonic posturing of the upper limbs, rigidity and akinetic mute state. Her laboratory reports revealed raised total leukocyte count along with raised creatine phosphokinase (CPK) (>2000). Her cerebrospinal fluid (CSF) studies revealed lymphocytic pleocytosis with no other abnormality. She was managed as a case of NMS and started on bromocriptine. She showed gradual recovery; however, she continued to have abnormal behavior. Possibility of autoimmune encephalitis was considered. Positron emission tomography (PET) brain revealed decreased F18 fluorodeoxyglucose (FDG) intake in bilateral motor cortices and occipital lobes. Her autoimmune encephalitis panel was negative. She was managed with high-dose pulse steroids and intravenous immunoglobulin. She responded well to treatment and has presently completely recovered. Repeat FDG PET of the brain after 12 weeks is normal.

Conclusions: A high index of suspicion should be kept to diagnose NMS. Autoimmune encephalitis should always be considered in young individuals presenting with neuropsychiatric manifestations. Rarely NMS may herald autoimmune encephalitis.

Abstract ID: 853: Connecting the Dots: Forgetfulness, Short Stature, Backache, and Alopecia in a Young Patient

Niveditha C, Thejus B, Archana N B, Praveen Kumar S, Janardhan D C, Pramod K

Bangalore Medical College and Research Institute (BMCRI), Bengaluru, Karnataka, India

Background and Aim: Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is characterized by baldness, recurrent ischemic strokes, lower backache, headache and dementia, linked to homozygous mutations in the high-temperature requirement serine peptidase A1 (HTRA1) gene on the long arm of chromosome 10 (10q26). CARASIL is the second known genetic form of non-hypertensive cerebral small-vessel disease, with cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) (autosomal dominant) being the first.

Case report: We report a genetically proven case of CARASIL in a young woman with classical clinical and neuroimaging findings. A 23-year-old non-diabetic, non-hypertensive female presented with memory disturbances for four years, struggling with recent events. Her medical history included lumbar spondylosis surgery 6 years prior and postpartum cerebral venous thrombosis with seizures 8 years ago. General examination revealed alopecia and short stature. Neurological examination showed a Montreal cognitive assessment (MoCA) score of 6/36, with deficits in executive functioning, visuospatial orientation, attention, delayed recall and normal motor function but absent knee jerk reflex. Brain imaging- contrast enhanced magnetic resonance imaging (CE-MRI) suggested symmetrical periventricular small vessel ischemic changes with multiple microbleeds involving the bilateral temporal lobes, basal ganglia and pons. Whole spine screening MRI revealed cervical and lumbar spondylosis. Considering CADASIL and CARASIL as a differentials genetic testing was sent, which revealed a homozygous mutation on exon 4, variant c.830_831del (p.Glu277ValfsTer61), suggesting a diagnosis of CARASIL.

Discussion and conclusions: This case reminds clinicians to connect the dots whenever such findings are seen in a young patient and underscores the importance of broadening the understanding of the genetic variations linked to this disorder

Abstract ID: 854: A Comprehensive Evaluation of the Non-Motor Symptoms, Quality of Life and Caregiver Burden in Patients with Idiopathic Dystonia

Nishanth Gowda, Nitish Kamble, Vikram V. Holla, Babylakshmi Muthusamy, Ravi Yadav, Pramod K. Pal

National Institute of Mental Health and Neuro-Sciences (NIMHANS), Bengaluru, Karnataka, India

Background and Aim: Dystonia is a hyperkinetic movement disorder. A proper understanding of these non-motor symptoms aids in developing appropriate therapeutic strategies. Hence, we aimed to study non-motor symptoms and their impact on the quality of life and caregiver burden.

Methodology: Sixty idiopathic dystonia patients participated in a cross-sectional study at tertiary center. Approved by the Institute ethics committee, the study required written consent and adhered to inclusion/exclusion criteria. Demographics were noted, and motor severity assessed using Burke-Fahn-Marsden dystonia rating scale-motor part (BFMDRS) and unified dystonia rating scale (UDRS). Various validated scales measured non-motor symptoms, including rapid eye movement (REM) sleep behavior disorder screening questionnaire (RBDSQ), Epworth sleepiness scale (ESS), Pittsburgh sleep quality index (PSQI), Hospital anxiety and depression scale-anxiety subscale (HADS-A), Hospital anxiety and depression scale depression subscale (HADS-D), Montreal cognitive assessment (MoCA), World health organization quality of life (WHO-QoL). Caregiver burden was gauged using the Zarit scale.

Results: The mean age was 28.63 ± 11.96 years. Men (n = 41) outnumbered women (n = 19). The mean age of men (30.5 ± 11.9 years) was higher than women (26.3 ± 8.5 years), (P = 0.24). The mean age at disease onset was 21.01 ± 13.89 years. Mean disease duration was 8.55 ± 7.45 years. Subjectively the most common non-motor symptoms reported were pain, anxiety, reduced sleep and depression. Geste occurred in 29 patients without age, onset or dystonia severity correlation. Generalized dystonia prevailed in infancy/childhood onset. Segmental/multifocal dystonia in adolescence/adulthood onset. Other movement disorders (chorea, parkinsonism, myoclonus, ataxia) coexisted in 30%. Levodopa responded in 26.6% (16 patients). UDRS: 28.67 ± 15.65, BFMDRS: 30.53 ± 20.99. RBD was not observed in any of the patients with a mean RBDSQ score of 2.33 ± 1.48. The ESS score was 3.00 ± 2.11. The WHO-QoL score was 78.44 ± 10.21. The HADS-A score was 6.03 ± 3.08 and HADS-D score was 7.08 ± 2.95. There was a positive correlation between BFMDRS score and the Zarit caregiver burden score that was not significant (p = 0.089).

Conclusions: Our study provides a detailed account of the various non-motor symptoms in patients with idiopathic dystonia.

Abstract ID: 855: Choroid Thickness in Patients with Migraine: A Preliminary Study

Chinmayi Seemikeri, Rajani Chandrakant Battu, D. V. Seshagiri, Girish Baburao Kulkarni, Pooja Mailankody

National Institute of Mental Health and Neuro-Sciences (NIMHANS), Bengaluru, Karnataka, India

Background and Aim: Migraine is the second most disabling disease and has a neurovascular basis. Choroid is a vascular layer of retina and can be measured using optical coherence tomography (OCT). This study aims to evaluate the choroid thickness in patients with migraine and compare with that of healthy controls to gain insights regarding migraine pathophysiology.

Methodology: We recruited 16 patients with migraine (as per ICHD3 criteria) (M:F = 3:13) and 16 age and gender-matched controls (M:F =7:9) after written and informed consent. After detailed neurological and ophthalmological evaluation, all underwent OCT. The peripapillary choroid thickness (PPChT) was measured in temporal (T), temporosuperior (TS), temporoinferior (TI), nasal (N), nasal-superior (NS), nasal-inferior (NI) and global (G) segments, while subfoveal choroid (SFChT) was measured beneath fovea manually with calipers. The comparisons were carried out using Student’s t-test.

Results: The mean age of patients was 37.06 (±9.57) years, and the controls were 32.06 (±5.77) years (p = 0.08). The mean duration of illness in patients was 9 (±2.52) years and monthly migraine days were 8 (±4.31). The PPChT (global) in patients (RE-240.88 ± 32.29 µm, LE-239.88 ± 28.07µm) as compared to healthy controls (RE- 295.69 ± 18.47 µm, LE- 307.63 ± 19.61 µm) and SFChT in patients (RE-234.38 ± 28.64 µm, LE-241.81 ± 32.58 µm) as compared to controls (RE-301.56 ± 17.13 µm, LE-305.19 ± 22.48µm) were significantly thinner (p < 0.001).

Conclusions: The findings indicate significant thinning in choroid in patients with migraine as compared to controls. Assessment of choroid thickness may be a potential biomarker in migraine. The same needs to be evaluated by a larger sample size and longitudinal studies.

Abstract ID: 856: CNS Burkitt Lymphoma Mimicking Demyelination

Harvy Parikh, Ajit Sowani

Zydus Hospitals Ahmedabad, Gujarat, India

Background and Aim: Central nervous system (CNS) lymphoma and demyelination may be difficult to differentiate based on clinical and radiological grounds.

Case report: A 59-year-old female with dysarthria, right lower motor neuron (LMN) facial palsy and right-sided cerebellar signs, was investigated and found to have T2-weighted imaging (T2WI) hyperintense enhancing oval lesions with leptomeningeal contrast enhancement on magnetic resonance imaging (MRI) in midbrain, pons, right superior cerebellar peduncle and right cerebellum. Routine investigation, cerebrospinal fluid (CSF) analysis, neuromyelitis optica with myelin oligodendrocyte glycoprotein antibody profile (NMO MOG), paraneoplastic panel, autoimmune encephalitis panel and angiotensin-converting enzyme (ACE) were negative. The possibility of malignancy vs demyelination was considered, and a trial of steroids was given. She responded in the form of improvement of speech and gait. She was discharged with the plan of brain biopsy in case of worsening. After 15 days, she presented with recurrence of similar symptoms with the brainstem lesions mildly increased in size and was treated with intravenous immunoglobulin (IVIG) in view of previous response to steroids. Her gait and speech improved significantly with treatment. A month later, she presented with progressive severe dysarthria, left hemiparesis and deteriorating sensorium. The magnetic resonance imaging (MRI) was suggestive of fresh homogenous enhancing lesion in right basal ganglia with increase in size of previous lesions. Hence, a brain biopsy was done, and a diagnosis of Burkitt’s lymphoma was reached.

Discussion: She was treated with Rituximab, cranial radiotherapy and temozolamide. At 5 months follow-up, she has improved partially and is conscious, oriented, dysarthric, with improving left hemiparesis and improving radiological lesions.

Conclusions: This case highlights how the MRI findings of lymphoma can mimic demyelination initially.

Abstract ID: 857: Cerebellum - An Under-Recognized Cause of Acute Cervical Dystonia

Pritam Majumdar, Siddhartha Sekhar Anand, Durjoy Lahiri, Aniruddha Kundu, Shruti Bhandari

St. Johns Medical College, Bengaluru, Karnataka, India

Background and Aim: Cervical dystonia is the most common focal dystonia in adulthood. The primary causes attributed to it are genetic factors, trauma and drug-induced effects, but the role of the cerebellum remains unknown. In this study, we present three cases to explore and unravel this potential association

Case report: After obtaining informed consent, clinical, demographic, examination, radiological and discharge condition details were recorded. Case 1: A 55-year-old hypertensive female presented with acute encephalopathy and recurrent vomiting. Her Glasgow coma scale (GCS) was 9/15, and blood pressure (BP) was 200/110 mm Hg. A non-contrast computed tomography (NCCT) scan of the head revealed a deep right cerebellar bleed mildly abutting the fourth ventricle. She was managed conservatively with blood pressure control and was noticed to have developed left-sided torticollis, which persisted until discharge. Case 2: A 72-year-old female with hypertension presented with sudden onset right-sided hemiplegia, gait imbalance and recurrent vomiting. Her GCS was 15/15, and National institutes of health stroke scale (NIHSS) score was 7. An NCCT head scan showed a left cerebellar infarct in the posterior inferior cerebellar artery (PICA) territory, involving the cerebellum and medulla. She developed right-sided torticollis, which partially improved with physiotherapy by discharge. Case 3: A 72-year-old male with a 3-year history of hypertension presented with acute quadriplegia, recurrent vomiting and drowsiness. His GCS was 5/15, and he was immediately intubated. An NCCT head scan showed a left cerebellar and bilateral parieto-occipital infarct, confirmed by magnetic resonance imaging (MRI). He was managed conservatively and developed right-sided torticollis on the second hospital day, which partially improved with physiotherapy by discharge.

Discussion and Conclusions: Our study expands on the above theory clinically and suggests that the cerebellum should be investigated in cases of unexplained cervical dystonias where the workup is negative.

Abstract ID: 858: Fair is not Always Fair

Priyanka Jangam, Aruna Kumari U

Guntur Government Hospital, Guntur, Andhra Pradesh

Background and Aim: A five-year-old girl born of second-degree consanguineous parentage, cried immediately after birth, normal delivery, no ICU admissions, no developmental delay, at birth she had fair skin and hair which got gradually pigmented with age. Now her hair is light brown in color with dark skin complexion, otherwise active and playful child. She didn’t have any history of recurrent fevers. History of presenting illness: now presented with sudden onset vision loss inform of difficulty in seeing letters on board and later she was not able to grasp objects which progressed rapidly, and she was dependent on attenders. one month later she had complaints of pain and tightness of legs with difficulty in walking. Family history: second-degree consanguineous parentage, first pregnancy intrauterine device (IUD), her elder brother normal.

Case report: On examination, frontal bossing is seen, and scalp hair, eyebrows, hair on body are light colored. Visual acuity—PL absent both eyes, tone spastic, power 4/5 all limbs, deep tendon reflex (DTR) exaggerated, plantar bilateral extensor. Fundus: atypical retinitis pigmentosa with macular dystrophy.

Discussion: Investigations: Magnetic resonance imaging (MRI) showed demyelination or white matter disease and cord edema from cervicomedullary junction to c4. Cerebrospinal fluid (CSF) analysis-normal. Her routine investigations are normal.

Conclusions: Griscelli syndrome is rare disorder with only 150 cases reported to date. Three types: type 1- neurological manifestations, type 2- immunological defects, type 3- dermatological manifestations. Our patient presented with neurological manifestations through genetically diagnosed type 2.

Abstract ID: 859: Turning Head Against Hyperglycemia: A Case Series Highlighting the Clinic-Radiological Profile Including Arterial Spin Labelling (ASL) of Hyperglycemia-Associated Seizures

Vishal Sharma, Akshata Huddar, Gosala Raja Kukkuta Sarma, Raghunandan Nadig, Thomas Mathew, Sindhu V. Nambiar, Sagar Badachi, Sonia Shivde, Gosala Raja Kukkutta Sarma, Raghunandan Nadig, Thomas Mathew, Sagar Badachi, Sonia Shivde

St. Johns Medical College, Bengaluru, Karnataka, India

Background and Aim: Hyperglycemia-associated seizures with their clinical heterogeneity and atypical brain involvement can complicate diagnosis and management. We present three cases exemplifying the spectrum of hyperglycemia-associated seizures and their radiological profile.

Case report: Case series of three patients with clinical and radiological details taken after informed consent. 1. 62-year-old-known diabetic experienced, intermittent episodes of head turning to right, lasting one minute with intact awareness. Neurological examination was normal. Elevated blood sugar levels (422 mg/dL) and high HbA1C (10.6%) were noted, although magnetic resonance imaging (MRI) was normal, arterial spin labeling (ASL) showed hyperperfusion in the left occipital lobe. The electroencephalogram (EEG) was normal, and patient was started on levetiracetam. A repeat ASL after seizure control showed resolution of hyperperfusion. 2. A newly diagnosed 32-year-old diabetic male presented with recurrent transient vision blurring, hallucinations of fire in the right visual field and episodes of head deviation to right. Examination confirmed right hemianopia, consistent with left parieto-occipital T2 hypointensity showing gyral enhancement on contrast study on MRI. Patient showed improvement in hemianopia with sugar (410 mg/dL, HbA1C 11.4%) and antiseizure management. 3. A 50-year-old female, known diabetic, presented with episodes of posturing of the left upper limb with head deviation to left with intact awareness. Patient was found to have sugars of 324, with HbA1C-11. MRI showed gyral hyperintensity in the right frontal lobe with contrast enhancement, and ASL showed hyperperfusion. EEG and lumbar puncture were normal. Patient was started on levetiracetam and sugars were controlled.

Discussion and conclusions: Considering hyperglycemia and associated imaging findings is crucial to avoid unnecessary investigations. The ASL sequence may be a useful marker and can sometimes show early changes even before they appear in other imaging sequences.

Abstract ID: 860: PLA2G6 Associated Neurodegeneration: Rare Case Report of a Novel Mutation in Two Siblings

Chinmayee Swain, Ashok Kumar Mallick, Ashok Kumar Mallick, Kali Prasanna Swain

Srirama Chandra Bhanja Medical College and Hospital, Cuttack, Odisha

Background and Aim: Phospholipase-associated neurodegeneration (PLAN) caused by PLA2G6 mutations is a recessively inherited disorder. We report two siblings with neurodegeneration with brain iron accumulation (NBIA2) in a tertiary care hospital in eastern India.

Case report: A 21-year-old man was asymptomatic until age 10 years, when he begun to drag his left lower limb, gradually developed generalized dystonia over 5 years. At the age of 20, he was bradykinetic with hypomimia, upper extremity myoclonic jerks, dysarthria and dysphagia. He had two episodes of generalized tonic-clonic seizure in last 2 months. He was confined to wheelchair since 1 year. He lost his speech since 7 months. His younger brother, 17-year-old male, was asymptomatic until age of 10 years, when he begun to drag his both lower limbs with gait impairment.

On examination both had spastic speech, slow pursuit, vertical gaze restrictions (upward), slow saccade, optic atrophy, increased deep tendon reflex (DTR), bilateral ankle and patellar clonus, extensor plantar, spastic gait. Also, elder brother had masked facies, anterocolis, generalized dystonia and younger brother had cerebellar signs. All laboratory tests including serum biochemistry, liver function test (LFT), thyroid function test (TFT), serum copper and ceruloplasmin levels and cerebrospinal fluid (CSF) were normal. Kayser–Fleischer rings were not detected. Electroencephalogram (EEG) showed generalized epileptiform discharges in elder brother and normal in younger brother. Magnetic resonance imaging (MRI) showed generalized cortical atrophy, iron deposition in globi pallidi and substantia nigra in both, cerebellar atrophy in younger brother. Whole exome sequencing revealed a homozygous missense variant in exon 13 of PLA2G6 gene (c.1744G>T mutation) in elder brother.

Discussion and conclusions: NBIA is a very rare case, and it is important to make a diagnosis as early as possible so that patients can get treated. It will also reduce the mortality rate and improve quality of life.

Abstract ID: 862: The Usefulness of 131I Radionuclide Imaging in a Case of Atypical Parkinsonism with Syncope

Ragavendar B, Ramkumar Sugumaran, Sunil K Narayan, Rajeswari Aghoram, Sai Kishore

Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India

Background and Aim: Parkinson’s disease (PD) is a neurodegenerative disorder characterized by dopaminergic neuron loss. Accurate diagnosis of PD and differentiation from other Parkinsonian syndromes is challenging but crucial for appropriate management. 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy has emerged as a promising tool for diagnosing PD due to its ability to detect early cardiac sympathetic denervation, a common feature in PD. Since 123I-MIBG availability is limited in India and costly, we used 131I-MIBG as a viable alternative to detect early cardiac sympathetic denervation.

Case report: A 73-year-old patient exhibited symptoms of orthostatic hypotension, insomnia, urinary urgency and recurrent syncopal episodes. Clinical examination revealed subtle limb rigidity and severe dysautonomia without tremors. He was diagnosed with atypical Parkinsonism. Laboratory tests and imaging studies, including 131I-MIBG myocardial scintigraphy, were conducted to distinguish between Pure autonomic failure (PAF) and multiple system atrophy (MSA) by assessing cardiac sympathetic innervation.

Discussion: 131I-MIBG imaging revealed a reduced Heart/Mediastinum uptake ratio of 1.15 (<1.7), indicating severe postganglionic cardiac sympathetic denervation. This finding confirmed the diagnosis of Pure Autonomic Failure (PAF) over Multiple System Atrophy (MSA). The patient was treated with lifestyle changes, abdominal binders, fludrocortisone and pyridostigmine for orthostatic hypotension and supine hypertension, showed significant improvement in symptoms and is under regular follow-up.

Conclusions: 131I-MIBG myocardial scintigraphy is a feasible and cost-effective method for assessing cardiac sympathetic activity in Parkinsonism, aiding in early and accurate diagnosis. Further studies are needed to validate its clinical utility and establish it as a reliable alternative to 123I-MIBG in PD diagnosis.

Abstract ID: 863: Comparison of Simultaneous Plasma Exchange (PLEX) and Intravenous Methylprednisolone (IVMPS) Versus IVMPS with Add on PLEX in the Treatment of Acute Severe Myelitis of Neuromyelitis Optica Spectrum Disorders (NMOSD): A Prospective Randomized Open Label Blinded Endpoint (PROBE) Study

Md Mohsin Raza, Rajesh Kumar Singh

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system that causes optic neuritis and myelitis. Relapses of NMOSD are severe and lead to residual disability. The current treatment of acute myelitis is intravenous methylprednisolone (IVMPS) for 5 days, followed by plasma exchange (PLEX) if there is no response. This may delay the recovery of the damaged cells. We aim to compare the efficacy of simultaneous PLEX and IVMPS versus IVMPS with add-on PLEX for acute myelitis of NMOSD at 3 months of randomization.

Methodology: We will conduct a prospective, randomized, open-label, blinded endpoint (PROBE) study. We will enroll patients with acute severe myelitis who fulfill the criteria of NMOSD. We will randomly assign them to receive either simultaneous PLEX and IVMPS or IVMPS with add-on PLEX. We will assess the primary outcome of improvement in the Kurtzke Expanded Disability Status Scale (EDSS) at 3 months.

Results: Based on previous studies, we expect a beneficial effect of simultaneous PLEX and IVMPS on the outcome of acute myelitis of NMOSD. We hypothesize that simultaneous PLEX and IVMPS will result in a greater improvement in EDSS than IVMPS with add-on PLEX at 3 months.

Conclusions: This study will provide evidence on the optimal treatment of acute myelitis of NMOSD. It will test the hypothesis that simultaneous PLEX and IVMPS will improve the recovery of the damaged cells and prevent further disability.

Abstract ID: 864: GFAP Astrocytopathy Presenting as Pseudo-Foster-Kennedy Syndrome

Atrikumar Patel, Kumari Archana, Ashish Kumar Duggal, Jyoti Garg

Ram Manohar Lohia Hospital, Type III, Connaught Place, New Delhi, India

Background and Aim: GFAP (Glial Fibrillary Acidic Protein) is an intermediate filament protein in astrocytes. Antibody to GFAP in Serum/cerebrospinal fluid (CSF) causes a clinical entity known as autoimmune GFAP astrocytopathy. Clinical spectrum includes meningitis, encephalitis and myelitis with monophasic or relapsing course. Visual symptoms in GFAP astrocytopathy can occur in the form of papilledema or papillitis. We report a case of GFAP astrocytopathy with asymmetric disc edema.

Case report: A 25-year-old female presented with a complaint of seeing bright visual objects in the visual fields of both eyes, transient visual obscuration for 4 months and subacute onset blurring of vision in the right eye for 2 months. Fundus examination suggested right-side optic disc pallor and left-side optic disc oedema. Magnetic resonance imaging (MRI) brain was normal, but there was subtle T2 hyperintensity in the right optic nerve’s intraorbital part. Lumbar puncture revealed normal opening pressure with 2 cells/mm 3 and normal protein and sugar. Optical coherence tomography (OCT) revealed increased retinal nerve fiber layer thickness in the left eye and reduced retinal nerve fiber layer (RNFL) in the right eye. The patient tested negative for Neuromyelitis optica (NMO) and myelin oligodendrocyte glycoprotein (MOG) antibodies, but the serum GFAP antibody was positive. The patient was treated with intravenous (IV) pulse steroids followed by oral steroids in a tapering dose. Intravenous immunoglobulin (IVIG) was given after a pulse dose of steroids, with minimal improvement in visual acuity.

Discussion and conclusions: GFAP astrocytopathy is an important cause of optic neuropathy, and GFAP antibody testing should be considered in patients with isolated optic neuropathy, especially when NMO and MOG antibodies are negative.

Abstract ID: 865: A Case of Quadriparesis- Intramedullary and Extramedullary Lesion

Manojkumar G

Thanjavur Medical College, Thanjavur, Tamil Nadu

Background and Aim: A 30-year-old admitted with rheumatic heart disease- acute rheumatic fever (RHD-AR) admitted with subacute onset asymmetric weakness lower limb followed by upper limb proximal followed by distal for 3 months with urinary urgency without cranial and cerebellar involvement MRI shows c2c3 compressive lesion and c5 intramedullary lesion.

Case report: After informed consent, a detailed history was taken and thoroughly examined. Relevant investigations were done. Imaging of brain and spinal done. A 30-year-old with neurofibromatosis presented with extramedullary and intramedullary lesion.

Discussion and conclusions: Neurofibromatosis commonly presents with extramedullary intradural dumbbell lesion causing compressive myelopathy. This is rare presentation of neurofibromatosis where both intramedullary and extramedullary lesion coexisting, so always look for intramedullary lesion also.

Abstract ID: 866: Clinical Profile, Treatment Strategies, and Comparative Long-Term Outcomes of Pediatric Autoimmune Encephalitis: A Single Center Study In South India

Mohinish S, Muhammed Shadique, Neeraj Elango, Jered Livingston, Leema Pauline, Sonia Rosalind Martina, Shree Shruthi Badrinath, Neeraj Elango, Jered Livingston, Leema Pauline

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: In pediatric autoimmune encephalitis (AIE), neurological manifestations are prominent initial presentation as compared to behavioral or psychiatric symptoms. Early diagnosis and prompt treatment lead to improved outcomes with reduced relapses. The aim of this study was to analyze the clinical profile and treatment options in seropositive and seronegative patients and long-term motor and neurocognitive outcomes in both groups.

Methodology: Materials and methods: An ambispective analysis of AIE patients admitted in the center from January 2021 to December 2023.

Results: A total of 36 children were included. 25 (70%) were positive for cerebrospinal fluid (CSF) autoimmune panel (NMDAR), in which 5 (20%) were herpes simplex virus (HSV) triggered, 10 (28%) probable, 1 (2%) possible AIE. The mean age of onset was 6.1 years (1.2 years-12 years). The clinical features were seizures (83%), encephalopathy (72%), movement disorders (52%), behavioral disturbances (48%), sleep disturbances and speech disturbances (43%) and autonomic (16%). Mean duration of paediatric intensive care unit (PICU) stay was 23 days. Neuroimaging was normal in 66.7% of cases. Mean time to initiate immunotherapy is 13 days. 23 (96%) seropositive cases and 3 (28%) sero negative cases received 2nd line immunotherapy. Among seropositive cases, 78% received Rituximab, 18% both Rituximab and Cyclophosphamide. Overall mortality was 2 (5.5%). Mean duration of follow-up 18 months (6 – 42 months). Three cases had relapsed during follow-up, and two cases were noted to have autoimmune epilepsy. 60% of seropositive and 18% of seronegative cases had residual deficits in terms of modified Rankin Scale, cognitive and behavior outcome scales. All HSV-triggered cases had residual defects and poor outcomes. No underlying tumor was identified in our cohort.

Conclusions: Though autoimmune encephalitis has a stormy acute phase, it usually remains monophasic in children. Outcomes are favorable in the large majority of affected children. Those with HSV encephalitis and evidence of structural brain lesions fare poorly.

Abstract ID: 867: Case Series of Spontaneous Intracranial Hypotension

Neha Kadam Duke, Rahul Vitthal kulkarni, Shripad Suryakant Pujari, Vishal Deshpande, Rushikesh Deshpande, Radhika Lotilikar

Deenanath Mangeshkar Hospital and Research Center, Pune, Maharashtra, India

Background and Aim: Spontaneous intracranial hypotension (SIH) occurs due to low cerebrospinal fluid (CSF) volume within central nervous system, due to spontaneous leak of CSF into extradural space presenting with varied symptoms. SIH is often misdiagnosed due to vague clinical features and lack of diagnostic consensus among the medical field practitioners. Longer time to diagnosis and treatment can lead to deleterious consequences and delayed recovery. The aim is to study clinico-radiological profile and management of patients diagnosed as SIH.

Case report: A retrospective case series of 13 cases of SIH diagnosed in our hospital from Aug 2020 to April 2024 were included. Demography and clinical characteristics were noted. Imaging findings and treatment given were documented. Functional outcomes in the form of treatment responses and recurrences were noted.

Discussion: A total of 13 cases (males 6; female 7) with diagnosis of SIH were identified. The mean age at onset was 37 years (range: 26-53 years). The most common clinical presentations were postural headache (100%), nausea or vomiting (77%), neck pain (37%) and visual complaints (7%). Magnetic resonance imaging (MRI) demonstrated subdural hygroma (61.53%), pachymeningeal enhancement (76.92%), prominent venous sinuses (61.53%). Twelve patients underwent magnetic resonance (MR) myelography which demonstrated cerebrospinal fluid (CSF) leak. Sites of leak were cervical (84.61%), thoracic (15.38%), lumbar leak (7%) while 23% had >1 site of leak. Twelve patients who progressed on conservative treatment were treated with epidural blood patch. Recurrence was 41%.

Conclusions: Timely diagnosis and treatment are required for early functional recovery and to prevent further complications in SIH patients.

Abstract ID: 868: Glitch in Twitch! A Case of Bilateral Hemi Facial Spasm

Mohinish S, Muhammed Shadique, Sonia Rosalind Martina, Shree Shruthi Badrinath, Neeraj Elango, Jered Livingston, Leema Pauline

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: Hemi facial spasm (HFS) is characterized by paroxysmal, involuntary twitching of facial muscles on one side of the face innervated by the ipsilateral facial nerve. The abnormal involuntary firing of the peripheral facial nerve triggers short or longer contractions of muscles of facial expression. As the name suggests, the disorder is almost always unilateral except in some rare incidences, with a reported prevalence of 2.6% of all HFS cases, during which one can see the bilateral involvement of facial muscles.

Case Report: A 11-year-old male child presented with abnormal twitching movements of both eyelids and twitching movements of left angle of mouth for past 6 months duration which was intermittent in nature, initially less than 10 episodes/day, each episode lasting few seconds. Movement is noted all through the day with no diurnal variation and is noted in sleep also. These movements get aggravated during stress and emotions and not able to suppress it voluntarily. O/E: Eyelid twitching noted with characteristic brow lift associated with twitching around angle of moth on both sides, suggestive of bifacial spasm. Workups for movement disorder including serum ceruloplasmin, antistreptolysin O (ASO) titer, electroencephalogram (EEG) were normal. The magnetic resonance imaging (MRI) Axial T1 Spin echo, fast Imaging employing steady-state acquisition (FIESTA) sequence showed bilateral type II AICA loop (vascular loop) is crossing and indenting the 7th nerve at its root exit zone. Diagnosis: Bifacial Spasm: Primary type due to neuro-vascular conflicts at root exit zone. Started on Oral Carbamazepine initially at 10 mg/kg/day, and dose was titrated and monitored closely. During follow-up after 3 weeks, twitching stopped completely.

Discussion and conclusions: Differentials for movement disorders of face include Motor tics, Blepharospasm, Focal seizures and Eyelid myoclonus, which need to be differentiated for appropriate management. “Other Babinski sign” is pathognomonic of HFS and helps distinguish from eye twitching/closure due to blepharospasm, in which brow-lift is characteristically absent.

Abstract ID: 869: A Case Series of Multifocal Motor Neuropathy

Harshil Bagadia

Grant Medical College and JJ Hospital, Mumbai, Maharashtra, India

Background and Aim: Multifocal motor neuropathy (MMN) is a rare immune-mediated motor neuropathy characterized by asymmetric weakness that preferentially affects distal upper limb muscles. The diagnosis of MMN is based on clinical, laboratory and electrophysiological characteristics demonstrating conduction block. Steroids and therapeutic plasma exchange are ineffective in MMN and may lead to worsening of the disease. However, more than 80% of patients with MMN experience improvement after intravenous immunoglobulin (IVIG). The aim of the study is to emphasize the importance of early recognition and appropriate treatment can have positive impact on quality of life.

Case report: It is a prospective observational case series study of three patients who presented with asymmetrical onset pure motor distal predominant weakness who were investigated and treated. Informed written consent was taken.

Discussion: All three cases were either having conduction block on nerve conduction study (NCS) electromyography (EMG) study or positive IgM ganglioside panel or both. All patients were treated with IV immunoglobulin therapy and 2/3 patients showed significant improvement.

Conclusions: Early intravenous immunoglobulin is the only therapy with proven efficiency in MMN patients by providing improvement of muscle strength.

Abstract ID: 870: Anti-MDA5 Associated Juvenile Dermatomyositis & NMO Overlap A Case of Poly Autoimmunity

Neeraj Elango, Mohinish S, Muhammed Shadique, Sonia Rosalind Martina, Shree Shruthi Badrinath, Jered Livingston, Leema Pauline

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: Poly autoimmunity is defined as the presence of more than one autoimmune disease in a single patient. Clinically amyopathic form of juvenile dermatomyositis (JDM)represents less than 2% myositis. Neuromyelitis optica (NMO) in children accounts for 3 to 5% of all cases, and 10 to 40% of cases have other coexisting autoimmune disease.

Case report: A 10-year-old boy presented with history of fever, arthritis, skin rash, no weakness for 6 months. Serum creatine phosphokinase (CPK) level was normal. Clinically diagnosed as a case of amyopathic form of JDM and was started on steroids. Had acute onset of vomiting, seizures, encephalopathy within 1 week. O/E: severe wasting noted. Patchy non-scarring alopecia. Heliotrope rash presents with bilateral scarring plaque over both helixes and gottron papules over both MCP, IP joint. Pitted scar over digits, fingertip ulcers in hands, feet with Lip erosions. Visual acuity of only light perception in both eyes. Pupils—equal, sluggishly reactive, with optic atrophy noted. Generalized wasting with painful restriction of movements with preserved tendon reflexes. Serum CPK—47 U/L, erythrocyte sedimentation rate (ESR)—88 mm/hour. Magnetic resonance imaging (MRI) brain showed hyper-intense lesions involving corpus callosum, right midbrain, pons, cerebellar peduncles, left parietotemporal region, ganglio-capsular regions, thalami, hypothalami, optic chiasm and bilateral optic tracts. We proceeded with myositis panel and serum for NMO and myelin oligodendrocyte glycoprotein (MOG). Both NMO and anti-MDA5 antibodies were strongly positive, suggestive of poly autoimmunity. Clinical improvement was noted with immunotherapy, as he was able to stand and take few steps with support. Visual loss improved from just light perception to 6/12 in both eyes at discharge. He was put on oral cyclosporine and currently under follow-up.

Discussion and Conclusions: Association of JDM with neuromyelitis optica spectrum disorder (NMOSD) only 3 cases have been reported so far The course of pediatric form of this overlap seems to be monophasic and has a better prognosis after treatment with steroids. Presentation includes seizures, psychiatric symptoms and visual loss. Visual loss in JDM due to retinal vasculitis/NMO.

Abstract ID: 871: Clinical and Etiological Profile of New Onset Seizures in a Tertiary Care Centre

Santhosh S, Fiju Chacko

Jubilee Mission Medical College, Thrissur, Kerala, India

Background and Aim: Seizure is a medical emergency and about 1 in 10 persons will experience a seizure in their lifetime. There are only few studies on the clinical and etiological profile new-onset seizure. This present study attempted to explore the clinical and etiological profile of new-onset seizure in adults above 18 years of age. Aims and objectives: 1) To determine various clinical type of seizures in adults. 2) To study various causes of new-onset seizures. 3) To find out the most common cause of new-onset seizures in various age groups

Methodology: This cross-sectional observational study was conducted in the department of neurology in a tertiary care hospital between 2022 July and 2023 July after getting Ethical Committee approval. Nature, methodology and risks involved in the study have explained to the patients and their relatives, and informed consent was obtained. The sample size calculated from the parent study was 106. All patients who qualified the inclusion criteria were evaluated following a systematic procedure. They were interviewed as per the proforma, following which, a complete clinical examination was done. Cases of new-onset seizures (first seizure ever experienced by the patient) were diagnosed with clinical history, examination, laboratory, electroencephalogram (EEG) and radiological studies. Seizures were classified according to the International League Against Epilepsy (ILAE) latest classification. Causes of seizures are evaluated depending upon the clinical history and examination. All patients with new-onset seizure underwent clinical examination, blood investigation, EEG and magnetic resonance imaging (MRI) brain (epilepsy protocol-specific brain MRI with 1-3 mm slices with additional coronal FLAIR sequences). For those patients on whom MRI cannot be performed (in case of any contraindication), computed tomography (CT) scan was performed alternatively. Statistical analysis will be carried out using SPSS version 21.0. Descriptive analyses were reported as mean and standard deviation of continuous variables. Chi-square test and Fischer exact test will be used to establish association, p < 0.05 was considered significant.

Results: A total of 106 patients with new onset seizures were enrolled on our study. Their age ranged from 18 years to 85 years. Highest incidence of new onset seizure was in the age group of 21-30 years followed by 31-40 years. Male to female ratio was 1.3:1. Among the new onset seizures most were generalized tonic-clonic seizures (71%) followed by focal to bilateral tonic-clonic seizure. All seizure types were common in males except focal aware seizure. In participants with alcohol withdrawal, generalized tonic-clonic seizures (GTCS) was the predominant seizure type. Among the patients studied only 38% had findings on neurological examination. The most common clinical finding was neck stiffness (60) followed by hemiplegia/hemiparesis (30%). 27% new-onset seizures were caused by cerebrovascular accidents, which included ischemic and hemorrhagic stroke. Next common cause was central nervous system infection, which include tuberculous (TB) meningitis 38%, viral (25%), bacterial (11%), 2 cases of neurocysticercosis and one case of cryptococcal meningitis. Sixteen patients with metabolic seizures were seen in our study, among which most common was hypoglycemic seizure. Five cases of seizure due to space occupying lesion were found in our study. Two cases of organophosphorus poisoning and 1 case of camphor poisoning-induced seizures were seen in our study. Among the pregnancy-associated seizure, cerebral venous thrombosis (CVT) was the commonest cause, followed by eclampsia. Imaging abnormality was seen in 54%. EEG was done for 49 patients, among 26 were abnormal, 23 were normal.

Conclusions: New-onset seizures had a male predominance with male-to-female ratio of 1.3:1. First episode of seizures was most common in 20-30 years age group. Most common type of seizure in our study was GTCS. Cerebrovascular accidents were the most common etiology followed by central nervous system (CNS) infections. Among cerebrovascular accident, most common was ischemic stroke, while among infections TB meningitis was attributed to maximum cases. In our study, 8.4% of cases no cause have been identified.

Abstract ID: 872: Rare Case of Giant Axonal Neuropathy in a Family

Jitendra Kumar, Thomas Edwin Raj, B. Kannan, C. Rachel, Sravya Ravi

Thoothukudi Government Medical College Tamil Nadu, India

Background and Aim: Giant axonal neuropathy is a very rare disorder; only about 50 affected families have been described in the medical literature. It is an inherited neurodegenerative disorder caused by mutations in the giant axonal neuropathy (GAN) gene. It affects both the central and peripheral nervous systems. We discuss clinical, electrophysiological, radiological and genetic features-related patients with GAN.

Case report: Two patients of same family with suspected GAN were included in this study. The diagnosis was considered in patients with early onset polyneuropathy and characteristic hair with central nervous system involvement or suggestive neuroimaging findings. Biochemical, metabolic and electrophysiological investigations were performed. Diagnosis was confirmed by targeted sequencing of the GAN gene.

Discussion: The patients were found to have gene mutations in GAN gene. Peripheral neuropathy, characteristic hair, cerebellar dysfunction and electrophysiological and radiological findings suggestive of GAN.

Conclusions: GAN is a clinically, radiologically and genetically heterogeneous disease with an expanding clinical and genetic spectrum. Patients classically present with central as well as peripheral nervous system involvement but either of them may be subclinical

Abstract ID: 873: Occipital Seizures Unveiling Hyperglycemia: A Diagnostic Challenge

Vikram Khardenavis, P. K. Murugan, Senthur Raja Pandiyan

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: Occipital seizures are a unique presentation of hyperglycemia. The focal character of the seizures described during hyperglycemia has been reported consistently over the years. They have an excellent prognosis when glucose levels get controlled.

Case report: A 52-year-old male with no comorbidities presented to casualty in Government Rajaji Hospital, Madurai, with history of acute onset. Severe intensity continuous right-sided headache for 3 days. During headache, he noticed that his wife was short, brother was taller, saw multiples of objects like cup and noted vividly colored objects. He was evaluated locally and found to have hyperglycemia with fasting blood sugar (FBS) 231 and postprandial blood sugar (PPBS) 348 mg/dl. On 3rd day, he had 3 episodes of non-versive head turning to left with awareness, followed by stoppage of headache. He was treated with anti-epileptic-levetiracetam and carbamazepine with anti-diabetic treatment

Discussion: He had no further episodes. His neurological examination was normal. The magnetic resonance imaging (MRI) brain showed minimal T2 Fluid-attenuated inversion recovery (FLAIR) hyperintensity in right parieto-occipital lobe. Electroencephalogram (EEG) normal. All routine investigations were normal except for elevated blood sugars and HbA1C of 9.7. His serum osmolality was 276. No urine ketone bodies were seen.

Conclusions: Recognition of hyperglycemia in occipital seizures is vital as they tend to be refractory to antiepileptic drugs (AEDs) and respond well to antidiabetic treatment

Abstract ID: 874: A Rare Case of Neurodegenerative Langerhans Cell Histiocytosis Presenting as a Spastic Ataxic Syndrome Treated with Intravenous Rituximab

Sai Apoorva Reddy, Thejus B, Archana Netto, Praveen Kumar, Sharath G. G. Kumar

Bangalore Medical College and Research Institute, Bengaluru (BMCRI Bengaluru), Karnataka, India

Background and Aim: Langerhans cell histiocytosis (LCH) is a rare disease of the monocyte-macrophage system. Neurodegenerative LCH (ND-LCH) is a distinct subtype which can present with cerebellar ataxia, ataxic dysarthria, cognitive impairment, pseudobulbar palsy. MRI brain imaging shows symmetric lesions in the dentate nucleus, cerebellar white matter, pons or basal ganglia. We report a rare case of ND LCH in an 18-year-old male.

Case report: We report a rare case of an 18-year-old male who presented with a spastic ataxic syndrome with ND LCH who was treated with Intravenous Rituximab. An 18-year-old male born out of 3rd degree consanguineous marriage presented with progressive tremulousness in bilateral upper limbs, gait ataxia, spastic ataxic dysarthria, cognitive impairment, spastic weakness of lower limbs, hypermetric saccades, limb ataxia in all 4 limbs, Montral cognitive assessment (MoCA) score was 16. Magnetic resonance imaging (MRI) brain with spinal cord imaging showed symmetric T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities in bilateral dentate nuclei, cerebellar white matter, ventral pons, bilateral globus pallidus, superior cerebellar peduncle, midbrain volume loss, reduced height of D10 vertebra was noted. Computed tomography (CT) studies showed sclerosis of sphenoid bone, maxillary bone, lytic lesion in the midline mandible, centrilobular nodules with diffuse cystic changes in the bilateral lungs. With these findings, a diagnosis of ND-LCH was made and he was treated with Intravenous Rituximab and showed clinical improvement.

Discussion and conclusions: A high index of suspicion should be present in young patients who present with spastic ataxia and distinctive MRI brain findings of ND-LCH for early initiation of immunosuppression with agents such as Rituximab for better outcome.

Abstract ID: 875: Silent Storm: Unraveling Leptomeningeal Carcinomatosis Linked to Gastrointestinal Cancer

Vishal Sharma, Akshata Huddar, Raghunandan Nadig, Thomas Mathew, Gosala Raja Kukkuta Sarma

St. Johns Medical College, Bengaluru, Karnataka, India

Background and Aim: Carcinomatosis meningitis is complication of systemic malignancies characterized by the spread of cancer cells to the meninges. This case report details the clinical course of a young male with persistent headaches and neurological deficits, ultimately diagnosed with leptomeningeal carcinomatosis secondary to gastrointestinal adenocarcinoma, emphasizing the need for thorough diagnostic evaluation.

Case report: A 32-year-old male presented with an intermittent headache for four months, starting at the vertex which worsened to involve the holocranium. Subsequently, the patient developed left-sided facial deviation and blurred vision with diplopia. Examination revealed papilledema and bilateral facial palsy. MRI brain with contrast and venogram indicated left transverse, posterior sagittal sinus stenosis and sulcal hyperintensities in the parieto-occipital region due to venous congestion. Lumbar puncture showed pressure of 27cm H2O, zero cells, low protein and normal cultures, and atypical presentation of IIH was considered. The patient improved with anti-edema measures, resolving his symptoms and facial palsy. Three months later, patient discontinued medications, experiencing a recurrence of headaches, multiple vomiting episodes and bilateral facial palsy. He had reduced sensation in right trigeminal nerve, bilateral sensorineural hearing loss and vestibular ataxia. Magnetic resonance imaging (MRI) with contrast showed leptomeningeal enhancement in the cerebellar hemisphere and enhancement of bilateral trigeminal, vestibulocochlear nerves. The patient reported intermittent blood in stools and a weight loss of 9 kg in four months. Lumbar puncture showed six cells with normal proteins but revealed atypical cells and opening pressure of 55cmH2O. 18F fluoro-deoxy-glucose positron emission tomography (FDG-PET) showed thickening at the recto-sigmoid junction, colonoscopy revealed noduloproliferative growth. Biopsy confirmed adenocarcinoma with signet ring cells and was scheduled for chemotherapy.

Discussion and conclusions: This case highlights the importance of thorough diagnostic evaluations in patients presenting with neurological deficits and persistent headaches, as these symptoms can sometimes indicate serious conditions like leptomeningeal carcinomatosis.

Abstract ID: 876: An Interesting Case of Secondary Headache

Tino Baby, Lakshmanan S

Abstract

Background and Aim: Among various causes of secondary headaches, giant cell arteritis should be considered over the age of 50 years particularly in the setting of an elevated erythrocyte sedimentation rate (ESR) and/or C-Reactive protein (CRP) with visual disturbances especially transient/permanent monocular visual loss, jaw claudication unexplained fever or other constitutional symptoms, temporal artery, tenderness to palpation and a current or prior diagnosis of polymyalgia rheumatica.

Case report: A 63-year-old female came with complaints of easy fatigability for 2 months, fever for 1 month, headache for 1 month predominantly in bilateral temporal region, associated with tender cord-like swelling in the bilateral temporal region and jaw pain which got aggravated while chewing. On physical examination, both temporal arteries felt thickened, nodular and tender with more pronounced thickening on the left side with diminished pulses on right side and absent pulses on left side. The rest of the general and systemic examinations and basic laboratory investigations were within normal limits. The ESR and CRP levels were elevated. Non-contrast computed tomography (NCCT) of the brain was normal. Because of high clinical suspicion of giant cell arteritis (GCA), color doppler ultrasound of bilateral temporal arteries was done which revealed enlargement of both superficial temporal arteries with luminal irregularity, narrowing with hypoechoic wall thickness known as ‘halo sign’. Conventional tomography (CT) angiogram showed left superficial temporal artery attenuation with surrounding cuffing.

Discussion: Provisional diagnosis of GCA was made according to the American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR 2022) criteria with score of ≥ 6 points.

Conclusions: If color doppler ultrasound demonstrates halo sign, then the diagnosis is confirmed without need for a biopsy.

Abstract ID: 877: Huntingtons Disease – Great Mimicker – Masquerading as Progressive Myoclonic Epilepsy

Hemanth Kalakuntla, Veena Narisetty

Osmania Medical College, Hyderabad, Telangana, India

Background and Aim: Huntington’s disease (HD) is an autosomal dominant inherited neurodegenerative disease characterized by neuropsychiatric symptoms, a movement disorder (most commonly chorea) and progressive cognitive impairment. Our aim is to present a unique case of HD, which has presented as progressive myoclonic epilepsy with mild cognitive impairment and no mental disorder and fewer CAG repeats.

Case report : A 38-year-old male born of 3rd degree consanguineous parentage with normal birth and developmental history presented with sudden brief jerky movements—myoclonus since 12 years, which were intermittent and brief to start with, they were more in the lower limbs compared to upper limbs, the jerks were present even at rest. Later he developed facial myoclonus, prominent when speaking, leading to unintelligible speech, these movements were absent in sleep, 7 years after the start of illness he developed involuntary, sustained, abnormal posturing of both upper limbs, right more than the left, with no significant behavioral, cognitive, autonomic symptoms, with no significant past history.

Discussion: After the above history, whole exome sequencing was done, which showed no significant gene and analysis showed 37 CAG repeats, confirming the diagnosis of Huntington’s disease and magnetic resonance imaging (MRI) showed caudate atrophy.

Conclusions: Through our case, we would like to impress upon the varied presentations of most common neurodegenerative movement disorder which every clinician has to be aware of in order to avoid misdiagnosis and waste of time and resources.

Abstract ID: 878: Once Bitten, Twice not so Shy

Aditya Ganguly

Kolkata Medical College, Kolkata, West Bengal, India

Background and Aim: Delirium is a common problem in neurology and is mostly caused by a few common causes. Here we present a case of delirium which developed due to an unusual cause and under unusual circumstances. This will help clinicians make a quicker diagnosis and provide better outcomes.

Case report: Detailed history was taken, general and neurological examination done and neuroimaging performed for this patient admitted under the Department of General Medicine, Medical College Kolkata as a referral case. A 52-year-old lady with a h/o stable ischemic heart disease (SIHD) presented with subacute onset, progressively worsening holocranial headache followed by delirium after traveling to Darjeeling from Kolkata. Her symptoms started within 1 day of reaching her destination. The patient has a history of similar headache and slight drowsiness when she visited Darjeeling around 2 years back. She was advised not to visit the place but she refused to follow that advice. The patient was quickly brought back to Kolkata within 3 days of her symptoms and admitted to our institution. Routine blood investigations were normal. Magnetic resonance imaging (MRI) brain showed diffuse T2/ fluid-attenuated inversion recovery (FLAIR) hyperintensities in the subcortical white matter and corpus callosum with a cytotoxic lesion of the corpus callosum (CLOCC)—these were suggestive of high-altitude cerebral edema. The patient was managed in the ICU with supplemental O2 therapy and dexamethasone and made a complete recovery over several weeks.

Discussion and Conclusions: One should keep a close watch to ensure that high-altitude cerebral edema as a cause of delirium is not missed.

Abstract ID: 879: A Case of Undiagnosed Silent Syndrome

Shailina Ali, Rishikesh Joshi, Mayur Takkar, Neeraj Jain, Dean Sangeeta Ravat

Seth G S Medical College & KEM Hospital, Mumbai, Maharashtra, India

Background and Aim: Lithium, a mood stabilizer, can cause both acute and chronic neurotoxicity. Here, we present an exceptional sequela of lithium toxicity, the Syndrome of Irreversible lithium-effectuated neurotoxicity (SILENT). Usually, full recovery follows lithium discontinuation, but in rare instances, permanent neurological sequelae occur.

Case report: Case description of a patient with subacute onset static cerebellar dysfunction after lithium therapy. A 50-year-old female presented with a four-year history of subacute onset gait and limb ataxia with scanning speech without involvement of pyramidal, extrapyramidal system and cognition. Four years earlier, she was started on lithium therapy for bipolar disorder. After 2 months, she developed fever, gastroenteritis, renal failure and altered sensorium. Her lithium level was 2.6 mmol/L (normal range: 0.6–1.2 mmol/L). She was intubated and underwent hemodialysis. After metabolic parameters normalized, her sensorium improved but developed persistent cerebellar dysfunction. Her recent routine labs, Vitamin B12, thyroid-stimulating hormone (TSH), nerve conduction study (NCS) and whole-body positron emission tomography (PET) study were normal. The magnetic resonance imaging (MRI) showed extensive cerebellar atrophy and normal basal ganglia.

Discussion and conclusions: This case report highlights SILENT as a potential cause of cerebellar ataxia and underscores the need for pretreatment counseling about irreversible toxicity of lithium therapy.

Abstract ID: 880: Young Man with Droopy Face: An Interesting Case of Bilateral Facial Paresis

B. Hyndav Kumar, B. Hyndav Kumar Kumar

Vydehi Institute of Medical Sciences and Research Centre, Bengaluru (VIMS, Bengaluru), Karnataka, India

Background and Aim: In this poster, we present a case of bilateral facial palsy occurring as first clinical manifestation of human immunodeficiency virus (HIV) infection.

Case report: A 29-year-old heterosexual male with no significant past medical history presented with a 7-day history of headache with altered behavior, high-grade fever with chills and rigors, 5 days history of bifacial weakness and 1 episode of vomiting the before night. General examination revealed bifacial lower motor neuron (LMN) type of weakness, neck rigidity and drowsiness.

Discussion and conclusions: Peripheral facial palsy can occur at any stage of HIV infection; however, it is more likely to be present in healthy HIV carriers than in patients with acquired immunodeficiency syndrome (AIDS). Peripheral palsy can be divided into two types according to etiology; an early stage-related palsy and a late stage-related palsy. In initial stages of HIV infection, patients may develop either Bell’s palsy or Guillain–Barre syndrome.

Abstract ID: 881: Supranuclear Gaze Palsy – PSP or Atypical PKAN? – A Case Report

Vaibhav Bhandari, Animesh Das

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Pantothenate kinase-associated neurodegeneration (PKAN) is an autosomal recessive heredodegenerative disease caused by a mutation in the PANK 2 gene. It is characterized by progressive neurodegenerative disorder and iron deposition in the central nervous system. PKAN presents with motor symptoms of parkinsonism, dystonia and tremors. The disease has two main phenotypes: typical PKAN, which manifests in the first decade of life with rapid progression leading to loss of ambulation within 10-15 years, and atypical PKAN, which has a later age of onset, slower progression and a better prognosis.

Case report: A 40-year-old female presented with bilateral heel dystonia, bilateral upper limb distal postural tremors and difficulty in walking. Symptoms also included freezing of gait, postural instability, bradykinesia, memory impairment and palilalia. On examination, vertical gaze palsy with slow and hypometric saccades was present. Positive family history, T2 fluid-attenuated inversion recovery (FLAIR) hyperintensity in globus pallidus and above symptoms suggested heredodegenerative parkinsonism. Whole exome sequencing suggested PANK2 gene positivity.

Discussion: Given the symptoms, late onset of disease, slow progression, magnetic resonance imaging (MRI) finding and positive whole exome sequencing (WES) with PANK 2 gene, the patient was diagnosed with atypical PKAN. Treatment included syndopa, trihexyphenidyl and baclofen for Parkinsonism symptoms. Deferiprone, an iron chelator, was also started.

Conclusions: Atypical PKAN with supranuclear gaze palsy was observed in a patient, contributing to the phenotypic variability of the disease. The report highlights the importance of imaging and genetic studies in diagnosing atypical symptoms and diseases.

Abstract ID: 882: Acute Transverse Myelitis following ASV in South Tamil Nadu

Maheshpandian Mariappan, Justin C, Ramu S

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: The Russell’s viper is a frequently encountered venomous snake in India, often leading to significant morbidity and mortality. This case report highlights an infrequently observed complication following the treatment of a viper bite.

Case report: A 36-year-old male hotel worker received 20 vials of polyvalent anti-snake venom after a viper bite on his left leg. Patient initially presented with compartment syndrome of left leg managed with fasciotomy. Initial improvements were noted in homeostasis and circulatory shock, but subsequent acute renal failure necessitated regular hemodialysis. Upon admission, no neurological abnormalities were detected.

Discussion: However, starting on the third day, the patient’s neurological status progressively deteriorated, presenting with weakness of left lower limb associated with numbness and subsequently involving the opposite limb the next day ascending upward till the umbilicus associated with complete loss of sensation below the umbilicus. Magnetic resonance imaging (MRI) of the spine confirmed acute intramedullary signal changes suggestive of transverse myelitis. High-dose methylprednisolone administration resulted in a partial recovery till date.

Conclusions: This study contributes insights into the complexities of managing neurological complications arising from snake envenomation treatment.

Abstract ID: 883: Unraveling the Conundrum: Ischemic Stroke IN Left Ventricular Non-Compaction

Manvitha G, Johann Christopher, Umesh T, Umesh T, Johann Christopher, Umesh T

Care Hospital Banjara Hills, Hyderabad, Telangana, India

Background and Aim: Left ventricular non-compaction (LVNC) is characterized by a thin, compacted epicardial layer and an extensive non-compacted endocardial layer with prominent trabeculation and deep recesses that communicate with the left ventricular (LV) cavity leading to less ejection fraction which in turn leads to intracardiac thrombus formation and systemic embolization.

Case Report: A 5-year-old child born out of third-degree consanguineous marriage came with chief complaints of sudden onset weakness of right upper and lower limb with deviation of mouth to left side since 1 day. Neurological examination revealed right upper and lower limb pyramidal distribution of weakness with right upper motor neuron facial palsy. Magnetic resonance imaging (MRI) brain was done which showed acute infarct in the left middle cerebral artery (MCA) territory and cerebral angiogram revealed abrupt cut off of left M1 segment suggestive of thrombotic occlusion. 2D echo showed left ventricular (LV) non-compaction with dilated left ventricle and left ventricular dysfunction, left atrial appendage (LAA) aneurysm with thrombus and spontaneous echo contrast. Cardiac computed tomography (CT) which showed aneurysmal Left atrial appendage with one-third thrombotic occlusion. N-terminal pro–B-type natriuretic peptide (NT-pro BNP) was elevated. She was treated with anticoagulants. 2D-ECHO at the end of 1 year revealed complete resolution of the thrombus from the left atrial appendage. Cardiac MRI was done for quantification of the non-compaction and thrombus evaluation which revealed non-compaction volume equal to 82 ml, non-compaction mass equal to 86 grams and resolution of the thrombus. Family screening revealed a younger sister having 2D echo which revealed LV non-compaction with left atrial appendage aneurysm with no thrombus and a first cousin sister who succumbed 8 years ago. Genetic evaluation was unremarkable.

Discussion and Conclusions: Coexisting of left ventricular non-compaction (LVNC) and left atrial appendage (LAA) aneurysm is accompanied by a high risk of thrombosis, and anticoagulation needs to be instituted. Comprehensive workup for the etiology of stroke is warranted as it modifies the treatment.

Abstract ID: 884: Rare Flavor of a Common Cerebellar Ataxia: Chorea and Wing-Beating Tremor

Nilanjana Patra, Ashis Datta

185/1 A.J.C Institute of Neurosciences, Kolkata, India

Background and Aim: Spinocerebellar ataxia (SCA) type 12 is the most common SCA reported from India. It is particularly reported in North Indian Agarwal community. Being autosomal dominant in inheritance, it presents with strong family history. The presenting features are postural hand tremor to start with and followed by cerebellar ataxia of variable severity. More than 43 CAG/CTG repeat expansion in PPP2R2B gene in chromosome 5q32 is needed for clinical manifestations. Here, two cases of SCA 12 will be reported from a Bengali family who presented with some unusual clinical features.

Case report: We examined two sisters of a Bengali Hindu family from Murshidabad district of West Bengal. Both are symptomatic for more than 10 years with abnormal speech quality, postural hand tremor and gait imbalance. The elder sister aged 57 years also had significant fidgetiness of limbs and forgetfulness. Younger of them aged 50 years was wheelchair bound out of severe gait imbalance. Onsets were before 50 years of age in both. Family history was positive for tremor and gait imbalance.

Discussion: In either case, gross features of cerebellar ataxia including ataxic speech were found. The elder sister had generalized choreiform movements. Younger sister showed wing-beating tremor in both proximal upper limbs. In both cases, recent magnetic resonance imaging (MRI) brain showed mild cerebellar atrophy. Wilson’s disease and previous cerebrovascular event were excluded. We did a comprehensive SCA panel with a future plan of targeted exome sequencing. The report was positive for SCA 12 with > 51 CAG repeat sequences.

Conclusions: SCA 12 is more ubiquitous than it was previously thought, and it can present with much different movement phenomenology.

Abstract ID: 885: An Interesting Case of Peripheral Neuropathy

Sindhu B R

Thanjavur Medical College, Thanjavur, Tamil Nadu

Background and Aim: A 24-year-old female presented with burning sensation in feet for 4 years, unsteadiness while walking for 2 years and weakness of all 4 limbs for 2 years. This was asymmetric, insidious in onset and progressive. After examination peripheral neuropathy, asymmetric ganglionopathy with small fiber neuropathy pattern was identified. Nerve conduction study, sympathetic skin response (SSR) was positive. With further workup, peripheral neuropathy secondary to Sjogren’s syndrome diagnosis made. Patient is on immunosuppressive therapy.

Case report: After informed consent, a detailed history was taken and thoroughly examined. Relevant investigations were done. Imaging of spinal cord done. A 24-year-old female presented with asymmetric peripheral neuropathy secondary to Sjogren’s syndrome.

Discussion and Conclusions: The need for precise neurological diagnostics should be stressed in the case of patients with Sjogren’s syndrome.

Abstract ID: 886: A Case of Morvan’s Syndrome after Ayurvedha Drug Intake

Rallabandi Prashanth

Stanley Medical College, Tamil Nadu, India

Background and Aim: Morvan’s syndrome is an autoimmune disorder of peripheral and central nervous system mediated by voltage-gated potassium channel (VGKC) antibody. It is characterized by peripheral nerve hyperexcitability, dysautonomia, insomnia and fluctuating delirium. We report a case of 36-year-old male with neuromyotonia-like presentation, with anti-contactin-associated protein-like 2 (CASPR-2 ab) positive after Ayurvedha drug intake.

Case report: A 36-year-old male presented with complaints of muscle cramps, twitching’s, mood and sleep disturbances with fatigability of 1-month duration. He had H/o intake of Ayurvedha medicine for 1 month for his complaint of gastritis, before the onset of his present symptoms. On examination, he was found to have postural hypotension and myokymic rippling muscle movements over his bilateral chest, arms and thighs.

Discussion: Electromyography (EMG) showed spontaneous activity and motor unit action potential (MUAPs) in the form of doublets and triplets. Morvan’s syndrome was thought of, and test for VGKC antibody was done. CASPR-2 antibody turned out to be positive in high titer. He was treated with high-dose pulse steroids for 5 days, and there was inadequate response for which he was treated with intravenous (IV) immunoglobulins at a dose of 0.4g/kg/day for 5 days followed by slow steroid taper over 2 months.

Conclusions: A rare association of Ayurvedha drug intake with Morvan’s syndrome has been established in this case report. This highlights the importance of recognizing this entity, in a country like India where Siddha and ayurvedic drugs are used in abundance.

Abstract ID: 887: Diagnosis of AL Amyloidosis on Nerve Biopsy: A Rare Case of AL Amyloid Neuropathy

Hamsa B T, Archana B Netto, Thejus B, Janardan D C, Praveen Kumar, Pramod K

Bangalore Medical College and Research Institute (BMCRI), Bengaluru, Karnataka, India

Background and Aim: Amyloid light chain or primary amyloidosis (AL amyloidosis) is the most common form of systemic amyloidosis; AL amyloidosis is caused by disorders of plasma cells, or rarely B cells that produce light chain immunoglobulins with the potential for misfolding and deposition as amyloid fibrils. Peripheral neuropathy occurs in 17% to 36% of patients with AL amyloidosis

Case report: A 47-year-old male was referred with a diagnosis of alcoholic peripheral neuropathy. He had a 10 years history of progressive paresthesia of bilateral lower limbs distal weakness in form of slippage of footwear. He did not have any autonomic symptoms. He was a chronic alcoholic for 20 years. The general examination was unremarkable with no autonomic dysfunction, neurologic examination demonstrated distal leg weakness; his patellar reflexes were reduced, and ankle reflexes were absent. Sensory examination showed reduced pin-prick and temperature sensation up to mid-shin and a decreased vibration sensation and proprioception in toes. His gait revealed sensory ataxia with positive Rhomberg sign. Electrodiagnostic (EDX) studies demonstrated demyelinating pattern.

Discussion: His serum protein electrophoresis showed a positive M Spike, serum immunofixation assay showed IgG lambda monoclonal gammopathy, and sural nerve biopsy was done which showed congophilic and eosinophilic deposits in the walls of the capillaries which exhibited apple green birefringence on polarizing, he is being initiated on chemotherapy.

Conclusions: Serum protein electrophoresis should be a part of routine workup to prevent missing paraproteinemic neuropathies which are often underdiagnosed. The mainstay of treatment is to address the underlying plasma cell dyscrasia.

Abstract ID: 888: From Fever to Falls: A Pediatric Case of Subacute Sclerosing Panencephalitis Unveiled

Panktee Shah, Satish Dudhbhate

Ruby hall clinic, Pune, Maharashtra, India

Background and Aim: We present the case of an 11-year-old boy from Pune, belonging to an upper-middle socio-economic class, currently in the 3rd standard with a good academic performance. The patient presented with acute neurological symptoms, including jerky movements, increased sleepiness, decreased appetite and irrelevant talk over the past 4-5 days, with a prior history of fever and parotitis.

Case report: A detailed clinical evaluation was conducted, including patient history, physical examination and various diagnostic tests. Magnetic resonance imaging (MRI) of the brain, electroencephalography (EEG), visual evoked potential (VEP), cerebrospinal fluid (CSF) analysis and specific serological tests for measles antibodies were performed. MRI findings included asymmetric patchy non-enhancing hyperintensities in the bilateral cerebral hemispheres. EEG showed high voltage periodic complexes. VEP results indicated anterior optic nerve pathway inconsistent responses. CSF analysis revealed oligoclonal bands, high immunoglobulin G levels and an elevated anti-measles antibody CSF/serum ratio.

Discussion: The patient was diagnosed with subacute sclerosing panencephalitis (SSPE), confirmed by the presence of high anti-measles antibody titers in both serum and CSF, with a CSF/serum ratio of 8.65 and a total CSF IgG index of 4.2.

Conclusions: This case highlights the importance of considering SSPE in pediatric patients presenting with new-onset myoclonic jerks and behavioral changes, especially in the context of a recent measles infection. Early recognition and diagnosis are crucial for appropriate management and prognosis.

Abstract ID: 889: A Rare Case of Progressive Cognitive Decline with Ataxia

Lavanya Chinni, Nikith A

Kasturba Medical College (KMC), Manipal, Karnataka, India

Background and Aim: Superficial siderosis is a chronic progressive neurological disorder characterized by triad of symptoms—sensorineural hearing loss, cerebellar ataxia and myelopathy. It occurs due to hemosiderin deposition in the leptomeninges and the superficial layers of the cerebral and cerebellar cortices, the brainstem and the spinal cord.

Case report: Discussion of rare case of progressive cognitive decline with ataxia. A 68-Year-old female presented with bilateral hearing loss with memory disturbance for 3 years and swaying while walking for 2 years. On examination, mini mental state examination (MMSE) was 14. Attention was impaired with forward digit span of 3. Dysarthria and head titubation noted. Vertical gaze restriction noted. Left> right cerebellar signs and gait ataxia noted. Laboratory: serum ferritin was 227. Pure tone audiogram (PTA) showed bilateral severe to profound sensorineural hearing loss. Magnetic resonance imaging (MRI) brain done showed evidence of severe cerebellar atrophy predominant vermian and medullary/pontine, midbrain and temporal lobe atrophy.

Discussion and Conclusions: So a possibility of superficial siderosis of the central nervous system (SSCNS) should be considered in patients who present with chronic history of progressive cerebellar ataxia, hearing loss or myelopathy.

Abstract ID: 890: Unmasking the Hidden: Acute Intermittent Porphyria in a Young Woman

Panktee Shah

Ruby hall clinic, Pune, Maharashtra, India

Background and Aim: A 20-year-old female was admitted with generalized abdominal pain and constipation for 10 days, followed by bilateral limb weakness and severe painful sensations with tingling for 4-5 days. She experienced seizures and required ventilator support due to breathing difficulties.

Case report: The patient was initially treated symptomatically at a local hospital. Clinical history and examination were conducted, revealing severe limb weakness, hypotonia and impaired superficial sensations. Laboratory investigations included serum electrolytes, routine blood tests, cerebrospinal fluid (CSF) analysis, electroencephalogram (EEG) and nerve conduction studies. A qualitative analysis for urine porphobilinogen was performed. The patient presented with persistent abdominal pain, hyponatremia (Na: 120) and multiple seizure episodes. Neurological examination showed hypotonia, 0/5 limb power and absent deep reflexes. CSF analysis was acellular with normal protein and glucose. EEG indicated diffuse slowing of activity. Nerve conduction studies revealed reductions in compound muscle action potentials with relatively preserved conduction velocities, indicating predominantly axonal neuropathy. Electromyography demonstrated widespread fibrillation potentials compatible with denervation. Urine porphobilinogen was detected.

Discussion and Conclusions: The journey from symptomatic treatment to a definitive diagnosis of acute intermittent porphyria demonstrates the value of thorough investigation and the potential for overlooked conditions to reveal themselves through meticulous clinical practice. It showcases the necessity for vigilance and broad differential diagnosis in acute medical settings, ensuring that no stone is left unturned in the pursuit of patient health.

Abstract ID: 891: A Study on Correlation of Serum Vitamin D3 Level in Severity, Disability and Chronicity of Migraine

Hemanga K. Dhing, Masaraf Hussain, Chandan Nath

All India institute of Medical Sciences, Guwahati, Assam

Background and Aim: Migraine constitutes a complex disabling disease with a multifactorial background. Dietary habits and nutrients have been associated with migraine. The aim of the study was to correlate serum vitamin D 3 levels with the severity, disability and chronicity of Migraine.

Methodology: Patients fulfilling international classification of headache disorders (ICHD 3) criteria were retrospectively enrolled between December 2023 and May 2024. Severity was determined by visual analog scale (VAS). Migraine Disability Assessment (MIDAS) was calculated to evaluate the migraine-related disability over the previous 3 months. Serum concentrations of vitamin D 3 were determined by enhanced chemiluminescence method.

Results: A total of 172 patients were included in the study. Majority (77%) of patients were females. Mean age was 33.3 (±11.4) years. Mean duration was 5.5 (±7.35) years. Hemicranial headache was present in half (51%) of patients. Headache was pulsatile in nature in 55% of patients. Photophobia and phonophobia were most common associated features (78%). Migraine was transformed to chronic in 46 (26.7%) patients. Mean vitamin D levels were 26 (±8.8) ng/dl. Vitamin D levels were insufficient (<30 ng/dl) in 65% of patients. Vitamin D 3 levels were correlated negatively with severity of Migraine pain as determined by VAS score (Pearson’s r=-0.308, p < 0.01) and disability as determined by MIDAS score (Pearson’s r=-0.162, p = 0.03). However, the level was not found significantly higher in case of chronic Migraine (P = 0.384).

Conclusions: Vitamin D deficiency is associated with the severity and disability of Migraine patients. Adequate supplementation of Vitamin D is necessary to ameliorate the severity and disability of Migraine patients.

Abstract ID: 893: Subacute Sclerosing Panencephalitis Presenting as Slow Myoclonus in a Young Male

Jyothi Prakash J, Syed Fahrudeen Munnaver P. K., Malcolm Jeyaraj K, Mugundhan K

Stanley Medical College, Tamil Nadu, India

Background and Aim: Measles is a highly contagious disease caused by a virus of the paramyxovirus family. Various measles-related neurological syndromes include primary measles encephalitis, acute post-measles encephalitis, measles inclusion body encephalitis and subacute sclerosing panencephalitis (SSPE). SSPE is a rare slowly progressive fatal inflammatory disease of the central nervous system characterized by seizures, myoclonic jerks, progressive deterioration of cognitive and motor functions, sometimes associated with pyramidal and extrapyramidal symptoms including chorea, dystonia and parkinsonism. Here, I am reporting a case of young male presenting with slow myoclonic jerks, cognitive decline and characteristic electroencephalogram (EEG) findings suggestive of SSPE.

Case report: A 17-year-old male with normal perinatal period and development with history of meningoencephalitis at the age of 6 months and another separate history of fever with rashes at 8 years of age presented with abnormal body movements for the past 1 year. The abnormal movements were insidious in onset, gradually progressive, episodic in nature with more than 100 episodes per day, jerky in nature each lasting for few seconds. He also had gradual decline in scholastic performance. On examination, he had an mini mental state examination (MMSE) score of 18/30 and found to have slow myoclonic jerks affecting the entire body, involving proximal and distal parts of limbs which disappeared in sleep. His EEG showed generalized periodic epileptiform discharges with inter-burst interval of 5-6 seconds. His measles IgG was elevated in cerebrospinal fluid (CSF) and blood.

Discussion: So, with the above characteristic findings, he was diagnosed as probable SSPE and treated with antiepileptics and other supportive measures for which he showed symptomatic improvement.

Conclusions: The purpose of this case report is to have awareness regarding the presentations of SSPE, especially in a developing country.

Abstract ID: 894: Corticospinal Tract and Optic Radiation Hyperintensity in SCAR8: Cerebellum and Beyond

Sharanya Raj, Archana N B, Janardhan DC, Praveen Kumar S, Pramod K

Bangalore Medical College and Research Institute, Bengaluru (BMCRI Bengaluru), Karnataka, India

Background and Aim: SCAR8 (Spinocerebellar ataxia recessive type 8), also known as ARCA1 (Autosomal Recessive Cerebellar Ataxia type 1), is a rare genetic cause of autosomal recessive cerebellar ataxia. While it typically presents with pure cerebellar symptoms, it can occasionally involve other systems, as well. It is caused by mutations in the SYNE1 gene.

Case report: We report the clinical and radiological features and genetic analysis of a sixteen-year-old female patient with SCAR8. Our patient had delayed attainment of gross motor milestones, slurred speech since two years of age, imbalance while walking and tremors of all four limbs since the age of 5 years, which has been slowly progressive. On examination, she exhibited scanning speech, broken pursuits, end gaze nystagmus, cerebellar ataxia, brisk deep tendon reflexes, focal dystonia of the right great toe and mild intellectual disability. Magnetic resonance imaging (MRI) of the brain revealed mild cerebellar atrophy with corticospinal tract and optic radiation hyperintensities, a finding not previously reported. Whole exome sequencing identified a pathogenic heterozygous nonsense variation in exon 35 of the SYNE1 gene resulting in a premature stop codon and truncation of the protein at codon 1521 (p.Arg1521Ter). Additionally, a novel heterozygous missense variation in exon 143 of the SYNE1 gene leading to the amino acid substitution of Histidine for Arginine at codon 8651 (p.Arg8651His) was also detected.

Discussion and Conclusions: SCAR8 may present with various neurological features beyond cerebellar involvement. Furthermore, in the appropriate clinical context, it should be considered as a differential diagnosis for corticospinal tract and optic radiation hyperintensities observed on magnetic resonance imaging.

Abstract ID: 895: A Rare Case Report of Seizures in Wilson’s Disease

Kamlesh Ramgiri, Malcolm Jeyaraj, Sakthi Velayutham, Sowmini P R, Kannan V, Vivek Saravanan, K Mugundhan

Government Stanley Medical College & Hospital, Chennai, Tamil Nadu, India

Background and Aim: Seizures have been reported to occur in 6.5% patients with Wilson’s disease. The frequency of seizures in Indian patients is 4.9%-8.3%. We report a case of Wilson’s Disease with grey and white matter abnormalities with clinical and electrographic evidence of seizures.

Case report: A 20-year-old gentleman, with no-comorbidities presented with slowness of daily activities which progressed over 6 months. He developed abnormal posturing of his right hand and drooling of saliva and slurring of speech. On examination: Rigidity of all 4 limbs, hypokinesia, bradykinesia present both upper limbs, mask-like facies, dystonic posturing of right hand. He developed multiple episodes of focal twitching movements of left lower half of face, angle of mouth, sustained eye deviation toward left associated with posturing of left upper limb with preserved awareness lasting for <1 min, 10-15 episodes/day.

Discussion: A diagnosis of Wilsons disease was made after investigations and Kayser-Fleischer (KF) ring. Started him on D-penicillamine, zinc and levetiracetam. Seizures correlate with electroencephalogram (EEG) and cystic lesion in magnetic resonance imaging (MRI) brain.

Conclusions: Copper deposition in brain causes neuronal loss, gliosis, laminar-necrosis, spongy degeneration and cavitation causing focal seizure activity, which probable explains seizures in my patient. Our case provides an insight into the treatment aspects, slow titration of penicillamine and pyridoxine supplementation. Through our report, we highlight that besides the basal ganglia and brainstem lesions Wilson’s disease involves extensive grey and white matter.

Abstract ID: 896: A Case Series on Spinal Dysraphism Cases in a Tertiary Care Centre: A Retrospective Study

Aswin G, Kishore R, Rajashekhar M, Kannan N, Aparna E G

KAPV Government Medical College, Trichy, Tamil Nadu, India

Background and Aim: Spinal dysraphism refers to a group of congenital anomalies of the spine which results in a defective neural arch through which meninges and/or neural elements are herniated leading to a variety of clinical manifestations. The aim is to study the incidence, clinical presentations and magnetic resonance imaging (MRI) findings in spinal dysraphism patients.

Methodology: This was a retrospective observational study among 35 patients conducted in KAPV Government Medical college, Trichy over a period of 1 year from April 2023 to May 2024. All OPD as well as IPD patients were screened.

Results: The incidence was high in males (60%) compared to females (40%). Most of the cases were reported to hospital during the first one month of life (51.4%). Spina bifida Aperta was present in 22 patients (62.86%) compared to Spina bifida occulta in only 13 patients (37.1%). The commonest site was the lumbosacral region in 14 patients (40.0%). Most common finding was myelomeningocele in 17 (46.87%) cases. Lipomyelomeningocele was seen in 4 cases which was most common finding in closed type. Diastematomyelia was seen in 1 case (6.25%). The most common associated anomaly was hydrocephalus in 12 (34.28%) patients and next common was Arnold Chiari malformation type 2 in 8 (22.8%) cases. Eight cases didn’t have any proper anti-natal checkups and no folic acid supplementations were taken in the antenatal period. Mothers of 4 patients were on anti-epileptic drugs (AED) during the time of conception.

Conclusions: Spinal dysraphism patients should be referred to a higher center where all the facilities including surgery are available. Patients should be given proper awareness in the ground level mainly in the primary health centres (PHCs) regarding intake of folic acid supplementation during antenatal period.

Abstract ID: 897: An Interesting Case of Quadriparetic GB Syndrome with Putaminal Bleed and tako Tsubo Cardiomyopathy- Is it ? GBS or a Mimic ? Vasculitis.

Deepika S Sirineni, Pranathi Polishetty

Apollo Hospital, Hyderabad, Telangana, India

Background and Aim: A rare case report.

Case report: A 34-year-old woman presenting to emergency department (ER) with sudden onset neck pain since evening associated with tingling numbness of hands and then lower limbs. Patient developed weakness of upper limbs in two hrs. and weakness of lower limbs followed by respiratory distress over 4 hrs. On examination, patient was conscious, drowsy in ER, power was 0 in all 4 limbs, neck flexion was 0. Deep tendon reflexes were absent. Hypotonia and plantar were mute. Intermittent bradycardia noted She was intubated with arterial blood gas (ABG) showing type 2 respiratory failure. MRI brain showed right putaminal bleed and magnetic resonance imaging (MRI) cervical spine was normal except for a few disc bulges. Patient had intermittent bradycardia with hypotension for which inotropic support was started. Her 2D echo revealed left ventricle regional wall motion abnormalities (LV RWMA), apical anterior wall ballooning favoring takotsubo cardiomyopathy. Ejection fraction (EF)- 40%. Her troponin I levels on day 1 were 10900. Patient developed acute kidney injury (AKI) secondary to hypotension leading oliguria. The patient was managed conservatively. Nerve conduction study showed prolonged distal latencies in upper limbs s/o demyelinating neuropathy. Possible diagnosis of Guillain–Barre syndrome with dysautonomia was made and patient was started on IV immunoglobulins in the dose of 2g per kg body weight given over 5 days. On day 3, her power started improving to 3 in upper limbs and 2 in lower limbs. Her urine porphobilinogen (PBG), lupus anticoagulant, anticardiolipin antibodies were negative. Her antinuclear antibody (ANA) was positive +++. On day 5, troponin decreased to 1198. Her complement levels were low—C3-1.12, C4-0.099. Erythrocyte sedimentation rate (ESR) was 72. Electrocardiogram (ECG) normal. Serum creatine phosphokinase (CPK) is normal. Anti acetylcholine receptor (Ach R) antibodies and anti muscle specific kinase (MUSK) antibodies were negative. Thyroid stimulating hormone (TSH)- 0.62.

Discussion and Conclusions: 2D echo done on day 20 was normal with reversal of RWMA and EF of 65%. she was discharged on day 20 with foleys as bladder was still showing retention. It helps to manage rare presentations.

Abstract ID: 898: Elsberg Syndrome in Varicella Zoster Virus Infection

Vaddadi Sri, S. Lakshmanan, R. Viveka saravanan, V. Kannan, K. Mugundhan, P. R. Sowmini, S. Sakthi Velayutham, K. Malcom Jeyaraj

Stanley Medical College Chennai, Tamil Nadu, India

Background and Aim: Elseberg syndrome is a rare, mostly self-limiting condition characterized by acute to subacute onset lumbosacral radiculitis, with or without myelitis confined to lower spinal cord. Herpes simplex virus- 2 (HSV-2), reactivation or occasionally primary infection is the most commonly reported cause of Elseberg syndrome; varicella-zoster virus (VZV), West Nile and severe acute respiratory syndrome coronavirus (SARS-CoV) being the other infectious causes. We report a case of clinically definite Elseberg syndrome, caused by VZV reactivation.

Case report: A 70-year-old lady presented with vesicular skin lesions in the perineal region, right buttock, right foot dorsal and plantar aspect. Four days later she developed severe burning pain in the same regions, due to which she was unable to walk. She also developed urinary disturbances in the form of urgency and frequency. After two days, she lost sensation of bladder fullness and developed retention of urine. On examination, she had grouped vesicles in L5, S1, S2 and S3 dermatomes. She had spasticity in both lower limbs, with normal power and bilateral brisk knee and ankle jerk, with anal reflex absent in the right side. All the modalities of sensations are reduced in the right L5 to S5 distribution. Magnetic resonance imaging (MRI) lumbosacral spine was normal. Cerebrospinal fluid (CSF) analysis revealed elevated protein and lymphocytic pleocytosis. CSF serology is normal.

Discussion: The above case is fitting into the diagnosis of clinically definite Elseberg syndrome, as per the Salvodi diagnostic criteria, based on the signs and symptoms of cauda equina involvement, subacute onset and progression less than three months, coexisting herpes zoster and CSF lymphocytic pleocytosis.

Conclusions: Elsberg syndrome may progress to a very severe neurological illness which could be avoided by prompt diagnosis and effective management.

Abstract ID: 899: A Rare Case of Osmotic Demyelination Syndrome due to Hyperosmolar Hyperglycemia

Supriya Jamatia, Sri Ramkrishnan, Jason Ambrose F

Tirunelveli Medical College, Tirunelveli, Tamil Nadu, India

Background and Aim: Osmotic demyelination syndrome (ODS) is a rare non-inflammatory central nervous system (CNS) demyelinating disease. It is reported commonly with rapid correction of hyponatremia but can also occur with other conditions causing rapid changes in serum osmolarity.

Case report: A 45-year-old diabetic male presented with complaints of sudden onset unsteadiness of gait and slurring of speech of 7 days duration. Examination showed dysarthria, nystagmus, bilateral impairment of finger nose and heel knee tests, dysdiadochokinesia and impaired tandem gait. Vital signs were normal.

Discussion: Random blood sugar (RBS) :610, HbA1C : 8.6, Na+ : 136, K+: 3.6, cerebrospinal fluid (CSF) : Normal, magnetic resonance imagin (MRI) brain: T2 and fluid attenuated inversion recovery (FLAIR) hyperintense signal with T1 hypointensity showing facilitated diffusion restriction in pons, middle and inferior cerebellar peduncles.

Conclusions: Not only rapid correction of hyponatremia, but also sudden rapid change in extracellular fluid osmolarity due to diabetes mellitus can cause ODS. The exact mechanism for the development of ODS and appropriate glycemic correction rate needs further evaluation. ODS can be missed in clinical setting; as it can be fatal and debilitating, it must be considered in any kind of disorder associated with change in osmolarity.

Abstract ID: 900: Idiopathic Unilateral Segmental Hyperhidrosis: A Case Series

Ilakkiya Venu, Kishore R, Rajasekaran M

KAPV Government Medical College, Trichy, Tamil Nadu, India

Background and Aim: Localized unilateral hyperhidrosis (LUH) is a rare disorder of unknown origin. Segmental hyperhidrosis is a rare sweating disorder. In majority of cases, pathophysiological mechanism of segmental hyperhidrosis remains obscure. In this paper, we report 3 cases of segmental hyperhidrosis without known etiology.

Case report: Case vignette 1: 24 /F presented with 6 months duration of hyperhidrosis of left forehead, cheeks, trunk. Case vignette 2: 21/M presented with 7 months duration of hyperhidrosis over right-side face, chest up to D7 spinal segment. Case vignette 3: 24 /M presented with 9 months duration of hyperhidrosis over right-side face and trunk. None of the 3 patients had family history, or history of thyroid disease, tuberculosis (TB) and other infectious disease, alcohol consumption, and drug intake.

Discussion: All 3 patients’ neurological examination shows no focal neurological deficit (FND). No postural hypotension and other autonomic nervous system (ANS) testing were normal. Starch iodine test was positive for all above-mentioned patients. Magnetic resonance imaging (MRI) brain and spine imaging was normal. Other routine blood investigations were within normal limit.

Conclusions: All of 3 mentioned cases were young patients without any co-morbidities. We report these 3 cases are primary hyperhidrosis of idiopathic etiology. All 3 patients were treated with Carbamazepine and glycopyrrolate and under follow-up.

Abstract ID: 902: Beyond Bell’s Palsy: Sarcoidosis as a Cause of Recurrent Facial Paralysis

Abhimanyu Reddy, Rahul Konduri, Mukesh Kumar Singh

Continental Hospital Plot no 3, Financial district Gachibowli, Nanakramguda, Hyderabad, Telangana, India

Background and Aim: Bell’s palsy is a common cranial neuropathy causing acute unilateral lower motor neuron facial paralysis. Though Bell’s palsy is common, recurrent Bell’s palsy is an unusual occurrence and reported in only 0.3-2% of Bell’s palsy cases and recognizes very few identifiable causes. Here, we present a case of recurrent Bell’s palsy with prolonged intervals between episodes, where she developed lymphadenopathy in the later stages leading to the diagnosis of sarcoidosis.

Case report: A 44-year-old woman presented with right lower motor neuron (LMN) facial palsy in February 2021. She was started on steroids and her facial weakness improved within a few weeks. Four months later, she developed left LMN facial palsy. This time, she was evaluated in detail for causes of recurrent Bell’s palsy. Tests including antinuclear antibodies- immunofluorescence assay (ANA-IFA), serum- angiotensin converting enzyme (ACE) levels, human immunodeficiency virus (HIV), COVID-19, IgG Lyme and magnetic resonance imaging (MRI) brain were done, and all reports were negative. Considering idiopathic Bell’s palsy steroids and valganciclovir were given and showed improvement. She developed a third episode of facial palsy on the left side on December 23. Upon examination, she also had a cervical lymph node. Investigations were repeated to assess the cause of recurrent Bell’s palsy, and the lymph nodes fine needle aspiration cytology (FNAC) was performed. Contrast enhanced computed tomography (CECT) chest also showed mediastinal lymphadenopathy.

Discussion: Serum ACE levels were elevated and the FNAC showed non-caseating granuloma confirming the diagnosis of Sarcoidosis and confirming it as the cause for recurrent Bell’s palsy. The patient was started on mycophenolate mofetil with bridging steroids and is doing well.

Conclusions: Sarcoidosis can cause recurrent Bell’s palsy, often before other symptoms appear. Thus, clinicians should suspect sarcoidosis in recurrent cases to ensure early diagnosis and treatment, preventing complications and improving outcomes.

Abstract ID: 903: Boomerang Sign: A Radiological Clue in the Diagnosis of Encephalopathy

Noonavathu Vamshi Krishna, P. K. Murugan, C. Justin

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: The boomerang sign refers to a boomerang-shaped region of cytotoxic edema in the splenium of the corpus callosum typically seen in cytotoxic lesions of the corpus callosum. Transient signal alteration in the splenium of corpus callosum on magnetic resonance imaging (MRI) brain has been reported in a variety of neurologic and non-neurologic conditions such as epilepsy, acute disseminated encephalomyelitis (ADEM), hypoglycemia, encephalitis/encephalopathy. We describe 2 cases here.

Case report: Case 1— A 25 year/Female P2L2, post- lower segment caesarean section (LSCS), postoperative day (POD)- 24 presented with C/o fever, headache and vomiting for 2 days with altered mental status and got admitted. On examination, vitals stable, disoriented. MRI of the brain showed hyperintensity of the splenium of corpus callosum (“Boomerang sign”) on diffusion-weighted image (DWI). Total leukocytes increased and other routine biochemical parameters normal. Treated with I.V. antibiotics and clinically improved. Follow-up MRI brain done after 1 month showed normal study. Case 2— A 33 year/Male presented with C/o fever for 2 days followed by loss of consciousness and got admitted. On examination, vitals stable patient is drowsy and on day 2 sensorium improved and bilateral cerebellar signs present and MRI brain showed diffusion restriction of splenium of corpus callosum and no post-contrast enhancement. Total WBC count increased, cerebrospinal fluid (CSF) and blood culture normal, blood urea–96 mg/dl, serum creatinine 2.1 mg/dl. The patient was treated with IV antibiotics. Thereafter, patient clinically improved and the repeat MRI done after a week showed normal study.

Discussion and Conclusions: Apart from epilepsy, the transient signal alterations in splenium of corpus callosum have been described in patients with encephalitis/encephalopathy due to various organisms, hypertensive encephalopathy, preeclampsia, posterior reversible encephalopathy syndrome (PRES) and toxic effects of cyclosporine. It is a self-limiting phenomenon, which subsides over a period once the underlying disease gets controlled.

Abstract ID: 904: Ravaged by an Invader: A Case of Poly-Neuraxial Involvement by Polyarteritis Nodosa (PAN) Secondary to Hepatitis B

Arnesh Bhattacharya, Devavrata Sahu, Atri Patel, Saurav Raja, Jyoti Garg, Ashish Kumar Duggal

ABVIMS AND DR RMLH, Baba Kharag Singh Marg, New Delhi, India

Background and Aim: Polyarteritis Nodosa (PAN) is a common entity in a setting of chronic Hepatitis B infection. While neuropathy is the most common neurological manifestation, central nervous system though rare, still can be affected. Management of the same is challenging.

Case report: A 37-year-old gentleman with Hepatitis B infection (serology showing HepBeAg positive and high DNA titers) presented with posterior cord (cervicodorsal) and conus syndrome. Patient also had acute right median neuropathy. There was a background of arthralgias, constitutional symptoms and acute kidney injury.

Discussion: Magnetic resonance imaging (MRI) revealed areas of vasculitic infarcts in the dorsal medulla and posterior cord (cervical). There was enhancement of the vertebral arteries on vessel wall imaging. Computed tomography (CT) abdomen revealed beading of the gastroduodenal and hepatic vessels. Patient fulfilled diagnostic criteria of PAN. He was given antivirals and two weeks of steroids and was placed on maintenance plasmapheresis.

Conclusions: Central nervous system (CNS) involvement in PAN is not limited to neuropathy. Lesions involving the brain and spine should be managed aggressively.

Abstract ID: 905: Anti IgLON5 Disease

Aparna Gaini

Tirunelveli Medical College and Hospital, Tirunelveli, Tamil Nadu, India

Background and Aim: Anti-IgLON5 disease is a rare autoimmune neurological disorder associated with autoantibodies against the IgLON5 protein. Patients may present with sleep disorders, bulbar syndrome, gait instability, cognitive impairment and movement disorders. The reported movement disorders can be hypokinetic or hyperkinetic comprising of bradykinesia, chorea, dystonia, myokymia, tremors or myoclonus.

Case report: A 54-year-old male patient presented with history of involuntary jerky movements of limbs and trunk—5 months, slowness in activities 5 months, gait disturbances, abnormal posturing of trunk and neck 4 months. Higher mental function (HMF)- frontal, subcortical dysfunction, hypokinetic dysarthria, cranial nerves—normal, extraocular movements—slow saccades, vertical gaze restriction present up>down, bradykinesia+, dystonic posturing of neck and trunk to left side—torticollis with truncal dystonia. Negative myoclonus involving both hands, choreiform movements of tongue, postural tremors of both hands

Discussion: Metabolic workup—negative, magnetic resonance imaging (MRI) brain with spine screening— normal, video electroencephalogram (EEG)— normal study, cerebrospinal fluid (CSF) analysis— normal, serum and CSF autoimmune panel and paraneoplastic panel— negative, serum anti-IgLON5 by indirect immunofluorescence— strongly positive, fludeoxyglucose F18 positron-emitting tomography (FDG PET)— hypermetabolism in left sensorimotor cortex, prefrontal, b/l basal ganglia insular cortex, brainstem and cerebellum.

Conclusions: Considering the multiplicity of symptoms, variable progression with a causality dilemma making a correct diagnosis perhaps can be a conundrum in clinical practice.

Abstract ID: 906: Trident Says it All: A Case of Spinal Neurosarcoidosis

Arnesh Bhattacharya, Sagarbhai Hemabhai Chaudhari, Naresh Chintala, Jyoti Garg, Ashish Kumar Duggal

Affiliation

Background and Aim: Neurosarcoidosis is one of the common causes of chronic myelopathy. Classically a trident sign involving the posterior cord is a pathognomonic finding. Diagnosis is confirmed by histological evidence from any affected tissue.

Case report: A 28-year-old gentleman presented with history of chronic progressive lower limb spasticity beginning from the right lower limb later involving the left lower limb with some history of cotton wool sensation involving the lower limbs. Later in the course he started having detrusor sphincter dyssynergia. There were no symptoms suggestive of visual, cranial nerves or brainstem involvement.

Discussion: Magnetic resonance imaging (MRI) revealed T2, fluid attenuated inversion recovery (FLAIR) hyperintense lesions of the dorsal spine spanning across three segments along with conus involvement. There was presence of trident sign on contrast images. Serum neuromyelitis optica (NMO) myelin oligodendrocyte glycoprotein (MOG) antibodies and oligoclonal bands (OCBs) were negative. In view of chronic course with enhancing lesion (>3 months duration) possibility of neurosarcoid was kept. Computed tomography (CT) chest revealed hilar adenopathy which on endobronchial ultrasound (EBUS) biopsy showed non-necrotising granulomas consistent with sarcoidosis. Patient was started on steroids and Infliximab.

Conclusions: Trident sign on MRI along in a case of chronic progressive myelopathy should raise a suspicion of neurosarcoidosis.

Abstract ID: 910: Cerebral Proliferative Angiopathy

Jayavardhan Reddy Katam, Haritha S, Subbaraj Devaraj, Lakshmanan S, Mugundhan Krishnan, Sowmini P. R, Sakthi Velayudham, Malcolm Jeyaraj K, Viveka Sarvanan, Kannan V, Tino Baby

Stanley Medical College and Hospital Chennai, Tamil Nadu, India

Background and Aim: Cerebral proliferative angiopathy (CPA) is a distinct entity from cerebral arterio-venous malformations; characterized by multiple small arterial feeders and draining veins with normal brain parenchyma seen in between the abnormal vessels. It is usually seen in younger age group. Here, we report a case of diffuse CPA in a 57-year-old female patient, with characteristic imaging findings.

Case report: A 57-year-old, diabetic and hypertensive, presented with headache, vomiting, seizure, altered sensorium. Physical examination was normal, except for b/l papilledema. No skin lesions were present.

Discussion: Non-contrast computed tomography (NCCT) of brain showed left occipital lobe intraparenchymal hemorrhage, and left parieto-occipital lobe subarachnoid hemorrhage (SAH). Since this is not a common site for hypertensive bleed, and her blood pressure levels were normal, we proceeded with CT angiogram, which showed dilated tortuous vessels in left temporoparietal lobe with no prominent arterial feeder or venous drainage, showing puddling of contrast in delayed venous imaging, suggestive of cerebral proliferative angiopathy.

Conclusions: Cerebral proliferative angiopathy should be considered as a separate entity from “classical” brain arteriovenous malformation (AVMs). It is important to recognize this entity to avoid aggressive surgery or intervention and thus prevent permanent damage to the normal intermingled brain tissue.

Abstract ID: 911: Varicella Zoster (VZV) Related Focal Arteriopathy in Adults: An Uncommon Entity

Arnesh Bhattacharya, Milan B. Mori, Devavrata Sahu, Kumari Archana, Jyoti Garg, Ashish Kumar Duggal

ABVIMS AND DR RMLH, Baba Kharag Singh Marg, New Delhi, India

Background and Aim: Post Varicella focal arteriopathy is a self-limiting condition and a cause of stroke in children. However, adults may also in rare conditions present with the same.

Case report: A 30-year-old gentleman working in a healthcare setup presented with a transient ischemic attack in form of aphasia, dysarthria and right arm weakness along with right facial weakness lasting for ten minutes. Patient did not have any vascular risk factors or history of strokes.

Discussion: Magnetic resonance imaging (MRI) brain was normal but MR angiogram with vessel wall imaging revealed a focal narrowing and enhancement of the origin of left middle cerebral artery (MCA). Basic stroke risk factor evaluation and autoimmune profile were negative. A serum to cerebrospinal fluid (CSF) anti-VZV IgG was less than serum to CSF total IgG levels suggestive of intrathecal varicella zoster virus (VZV)-specific IgG synthesis. Patient was started on 14 days of intravenous (IV) acyclovir and tapering course of oral steroids. Repeat imaging of vessel wall after 6 months was normal and there were no further stroke-like events.

Conclusions: VZV-associated vasculitis can be a cause of large vessel strokes in exposed adult individuals.

Abstract ID: 912: The GNAO1 Enigma: Unravelling Movement Disorder’s Hidden Mechanism

Caroline Silvia, Robert Wilson, Arunan Subbiah

SRM Medical College Hospital and Research Center, Kattankulathur, Tamil Nadu, India

Background and Aim: GNAO1-related disorder encompasses hyperkinetic movement disorders and/or epilepsy and is typically associated with developmental delay and intellectual disability. About 300 cases of GNAO1 mutation have been reported worldwide.

Case report: An eight-year-old girl child was brought with complaints of not attaining age-appropriate milestones. She was second born to consanguineously married parents, a full-term, and had neonatal hyperbilirubinemia on day 3 of life requiring an exchange transfusion. She attained a social smile at 4 months, following which she did not achieve any other milestone. She developed random, continuous, purposeless choreic movements, occurring proximal to distal, at times flinging movements involving all 4 limbs for 4 years of age, present throughout the day, increasing in intensity on handling the child (changing clothes, feeding), and disappearing during sleep. She had an older brother who had similar complaints and passed away at 6 years of age without a proper workup.

Discussion: Basic blood investigations were unremarkable. Metabolic workup was negative. Peripheral smear for acanthocytes negative. Fundi were normal. Magnetic resonance imaging (MRI) brain along with magnetic resonance spectroscopy (MRS) was normal. Tandem mass spectrometry (TMS) and urine gas chromatography mass spectrometry (GCMS) were negative. Whole exome sequencing (WES) revealed a likely pathogenic GNAO1 (+) mutation at exon 7 C736G>A;p.Glu246Lys.

Conclusions: Any child presenting with developmental delay, hyperkinetic movement disorder and a positive family history, GNAO1 mutation should also be considered.

Abstract ID: 913: Mixed Connective Tissue Disease (MCTD): A Rare Presentation of CNS (Central Nervous System) Vasculitis

Arpan Mitra, Abhishek Pathak

Institute of Medical Sciences (IMS), Varanasi, Uttar Pradesh, India

Background and Aim: Mixed connective tissue disease (MCTD) is recognized to have common characteristic state of diverse autoimmune diseases, like as cutaneous systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, polymyositis and dermatomyositis. MCTD with central nervous system (CNS) vasculitis is a rare clinical presentation. Herein, one case report of MCTD presented as CNS vasculitis.

Case report: A 27-year-old female patient with background history of joint pain, mainly small joints of hand, morning stiffness, intermittent low-grade fever and malaise for 7 years, presented with multiple episodes of seizure and altered sensorium for two weeks. On presentation, pallor is present but no associated icterus, cyanosis, clubbing, lymphadenopathy or edema is seen. Glasgow coma scale (GCS) was E3M5V1 on admission. A detailed evaluation was done including magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analysis and autoimmune workup.

Discussion: Diagnoses: MCTD with CNS vasculitis was the final diagnosis of the patient. Outcomes: With treatment, patient significantly improved. She was discharged with stable vitals. GCS on discharge was E4V2M6 and systematically planned for review and follow-up in OPD.

Conclusions: This presented case highlights the significance of MCTD as a diagnostic consideration in CNS vasculitis patients.

Abstract ID: 914: Critical Conundrum: Dual Diagnosis in a Postpartum ICU Patient

Samatha Mukkamalla, Sudheeran Kannoth, Anand Kumar, Siby Gopinath, Saraf Udit Umesh, Vivek Nambiar, Annamma Mathai

Amrita School of Medicine, Kochi, Kerala, India

Background and Aim: To report a case of post-c-section autoimmune encephalitis with myasthenia gravis.

Case report: Evaluated with magnetic resonance imaging (MRI), electroencephalogram (EEG), cerebrospinal fluid (CSF) study and autoimmune and paraneoplastic panels, serum acetylcholine receptor (AChR) antibody, muscle–specific tyrosine kinase antibody (MuSK–Ab).

Discussion: Her MRI showed bilateral symmetrical diffusion restriction in cingulate gyrus, insula, external capsule, temporal lobes. EEG revealed generalized periodic epileptiform discharges. CSF study, infectious workup was negative. Serum and CSF autoimmune, paraneoplastic workup showed unclassified antibodies. Initial workup of serum AChR, MuSK Ab were negative, but retesting showed AChR Ab positive.

Conclusions: Critically ill patients should be observed for subtle signs, and failure of one therapy warrants consideration of alternative therapies. If initial antibodies are negative, repeat testing should be considered when clinical signs and symptoms are confirmatory of a disease. Seronegative myasthenia gravis (MG) can coexist with autoimmune encephalitis.

Abstract ID: 915: “When the limb rebels against mind- A Fascinating case of Alien limb syndrome” Shamili devi, M.R. Manivannan, Senthur Raja Pandiyan, Venkateshwaran DM Postgraduate, Professor, Assistant professor, Assistant Professor. Department of Neurology, Madurai medical college

Shamili Devi, Justin C, Manivannan MR

Madurai Medical College, Chennai, Tamil Nadu, India

Background and Aim: Alien limb syndrome is a rare disorder of involuntary limb movement together with a sense of loss of limb ownership. It most commonly affects the hand, but can occur in the leg. The anterior (frontal, callosal) and posterior variants are recognized, with distinguishing clinical features and anatomical lesions. Initial descriptions were attributed to stroke and neurosurgical operations, but neurodegenerative causes are now recognized as most common.

Case report: A 65-year-old female, k/c/o DM/ systemic hypertension (SHT) presented with complaints of acute onset difficulty in using left upper limb in the form of difficulty pouring tea into the tea glass using left upper limb (UL), h/o inability to remove slippers from left lowe limb (LL), h/o eating with 2 hands for 10 days.

Discussion: On examination, patient has diagnostic apraxia, left tactile anomia, pseudoastereognosis, right olfactory anomia, left upper limb motor apraxia, intermanual conflict, agraphia of left hand, construction and dressing apraxia, left auditory anomia. Imaging shows acute infarct in right anterior cerebral artery (ACA) territory involving right paramedian frontal and parietal regions and corpus callosum on right side. Pt was started with antiplatelets and statins

Conclusions: The alien limb is caused by heterogeneous pathologies. This emphasizes the role of affected topography and not necessarily the pathology in determining the presence of alien limb.

Abstract ID: 916: A Prospective Interventional Study to Compare the Effects of Levodopa and Pramipexole in Improving Non-Motor Symptoms in Idiopathic Parkinson’s Disease in a Tertiary Care Hospital

Sourav Panda, Kartick Chandra Ghosh, Jasodhara Chaudhuri

Nil Ratan Sarkar Medical College and Hospital, Kolkata, West Bengal, India

Background and Aim: Levodopa has been the cornerstone of treatment for motor symptoms, but its efficacy in managing non-motor symptoms remains uncertain. Pramipexole, a dopamine agonist, has shown promise in addressing both motor and non-motor symptoms. This prospective interventional study aims to compare the effects of levodopa and pramipexole in improving non-motor symptoms of idiopathic Parkinson’s disease.

Methodology: This study has involved a randomized control trial design with two parallel groups receiving either levodopa and pramipexole for specified duration. Non-motor symptoms, including but not limited to cognitive impairment, psychiatric symptoms, autonomic dysfunction, and sleep disturbances, will be assessed using validated scales at baseline and at the end of 3 months. The primary outcome measures include changes in non-motor symptom severity scores between the two treatment groups.

Results: Thirty patients were divided into two groups: one receiving levodopa and the other pramipexole. The study assessed non-motor symptoms using validated scales at baseline and at the end of 3 months. Both medication shows some improvements in non-motor symptoms, with levodopa showing greater efficacy in addressing sleep disturbances while pramipexole showed advantages in some aspects of neuropsychiatric manifestations like depression, anxiety. Other symptoms show some variable response in both the cases.

Conclusions: Both levodopa and pramipexole demonstrated efficacy in improving non-motor symptoms, highlighting beyond just addressing motor symptoms. Furthermore, both medications showed comparable efficacy in improving motor symptoms severity and functional status, indicating their role as mainstay of treatment of parkinsonism. A large sample size is necessary to make a better conclusion.

Abstract ID: 917: Case Series on Presentation Wernicke’s Encephalopathy in Pregnant Females following Hyperemesis Gravidarum

Siddharthan R, Balaji G, Hariharan S

Government Chengalpattu Medical College, Tamil Nadu, India

Background and Aim: Wernicke’s encephalopathy is a neurological emergency caused due to thiamine deficiency. Though it is usually seen in chronic alcoholics, it is a common condition in many other clinical settings including malnutrition, hyperemesis gravidarum, cancer and gastric surgeries. Most patients present with the triad of ocular signs, ataxia, and confusion. The diagnosis is hugely clinical and unless sought for specifically is often missed, thereby a potentially reversible condition could lead on to stupor, coma, and death. Our aim is to assess and analyze the clinical presentation and management of Wernicke’s encephalopathy following Hyperemesis Gravidarum cases. The clinical manifestations of Wernicke’s encephalopathy include confusion, ataxia and ophthalmoplegia. The classical clinical trial is seen only in 15-30% of patients.

Case report: Retrospective descriptive analysis—case series of 4 patients.

Discussion: All patients improved significantly with thiamine treatment. Hence, this is one of the easily clinically recognizable, reversible neurological illness in pregnant females.

Conclusions: It is important to note pregnant women with Hyperemesis are prone for Nutritional (Particularly Thiamine) deficiency and electrolyte deficiency abnormalities. Hence, clinical presence of confusion, ophthalmoplegia and ataxia in a pregnant female should suggest possible diagnosis of Wernicke’s encephalopathy. It is important to note that all 3 symptoms in classical triad of Wernicke’s encephalopathy need not be present in all patients. Clinical suspicion is enough for thiamine supplementation; we need not wait for serum thiamine or red blood cell (RBC) transketolase level.

Abstract ID: 918: Myoclonic Jerk in Hypothyroidism

Shailendra Manjhvar, Gobinathan, V. Balambighai

Sree Balaji Medical College and Hospital Chennai, Tamil Nadu, India

Background and Aim: A rare case of hypothyroidism associated with movement disorder to evaluate patient with myoclonus jerk associated with hypothyroidism.

Case report: We present a case of myoclonic jerk with autoimmune thyroiditis. A young female in her early twenties with previous no comorbidities presented with complaint of abnormal jerk movement of all 4 limbs and body for 4 days duration. On examination, she had continuous myoclonic jerk with normal language and intact memory. Her cranial nerve sensory system examination is normal. Ultrasonography (USG) thyroid done showed heterogenous ECHO texture and increasing vascularity and suggesting of thyroiditis. Electroencephalogram (EEG) shows evidence of epileptiform activity. Magnetic resonance imaging (MRI) brain showed left middle cranial fossa arachnoid cyst. Serum copper level was 165 mcg/dl. Anti-thyroid peroxidase (anti TPO) – 402 lu/ml and anti TPG 498 Iu/ml levels were elevated with normal serum ceruloplasmin level. Other blood parameter including renal function test (RFT) and lipid profile are normal. Lumbar puncture and cerebrospinal fluid (CSF) analysis done showed elevated protein, normal sugar and no cells. CSF autoimmune panel done was negative. Patient was treated with pulse dose intravenous anti-epileptic drugs (IV AEDS) and thyroid medication.

Discussion: Patient improved symptomatically at the end of two weeks. Symptoms improved and she is under regular follow-up.

Conclusions: Early diagnosis and management will improve the quality of life of patient. Accurate diagnosis is important.

Abstract ID: 919: Two Cases of Metabolic Myopathy -A Case Report

Azma Mohammed, Aruna Kumari U, Sundara Chary N. V., Aruna Kumari U

Guntur Medical College, Andhra Pradesh, India

Background and Aim: Metabolic myopathies are a group of inherited disorders affecting muscle energy metabolism, presenting with symptoms such as muscle weakness and exercise intolerance. This report details two cases of metabolic myopathies—Carnitine Palmitoyltransferase II (CPT II) deficiency and adult-onset Pompe disease—emphasizing the importance of early diagnosis and appropriate management.

Case report: Case 1: A 14-year-old boy presented with a 7-year history of progressive proximal muscle weakness affecting both upper and lower limbs and muscle cramps. He exhibited a waddling gait and a positive Gowers sign without sensory, cranial nerve or cerebellar involvement. Clinical evaluation revealed obesity and gynecomastia. Laboratory findings showed normal creatine phosphokinase (CPK) levels and a myopathic pattern on electromyography (EMG). Case 2: A 21-year-old male with a history of delayed motor milestones and recurrent respiratory infections presented with progressive proximal muscle weakness and muscle cramps since age 7. Physical examination revealed generalized muscle wasting, a waddling gait and second wind phenomenon. Laboratory tests indicated elevated CPK levels (1172 U/L),

Discussion: Genetic analysis of first case confirmed CPT II deficiency. Management focused on dietary modifications, and education on avoiding prolonged exercise and fasting. Genetic testing of second case is diagnosed as Pompe disease. Management included supportive care and discussions on enzyme replacement therapy.

Conclusions: Metabolic myopathies, though rare, require a high index of suspicion for diagnosis. Distinguishing metabolic myopathies from dystrophies is crucial given the implications for treatment and disease trajectory. Early diagnosis and tailored management can significantly improve patient outcomes.

Abstract ID: 920: CD 59 Deficiency Presenting as Recurrent GBS

C. Leema Pauline, Neeraj Elango, Jered Livingston Kanakaraj, Jered Livingston, Mohinish S, Muhammed Shadique, Sonia Rosalind Martina, Shree Shruthi Badrinath, Neeraj Elango

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: CD59-mediated hemolytic anemia, with or without immune-mediated polyneuropathy is an autosomal recessive disorder characterized by recurrent polyneuropathy mimicking Guillain–Barre syndrome (GBS), hemolytic anemia and recurrent strokes.

Case report: Here, we present a case of CD59 deficiency presenting as recurrent polyneuropathy. A one-year and nine-month-old male child, third born of nonconsanguineous parents with a background of motor delay presented with flaccid quadriparesis following a febrile illness of 3 days duration. Examination revealed a conscious child with intact cranial nerves, flaccid areflexic quadriparesis, intact sensations and flexor plantar. Nerve conduction studies showed inexcitable motor and sensory nerves in all four limbs. Cerebrospinal fluid analysis was normal. A diagnosis of acute motor sensory axonal neuropathy was made and treated with intravenous immunoglobulin. He regained his background muscle strength in that he could stand and walk with support. Four months later, he was brought with a similar episode of flaccid quadriparesis with neck and trunk muscle weakness. Nerve conduction study (NCS) showed inexcitable nerves. Cerebrospinal fluid (CSF) was normal. Retroviral screening and vasculitic workup were negative. Magnetic resonance imaging (MRI) brain and spinal cord were normal. A diagnosis of recurrent GBS was considered and managed with intravenous immunoglobulin following which the child improved to the premorbid state. NCS repeated after two months revealed inexcitable nerves. Hence, a hereditary neuropathy was considered and genetic testing done. Meanwhile, he developed another episode of weakness and improved with immunotherapy. Whole exome sequencing revealed a pathogenic mutation in exon 4 of the CD59 gene. There were no episodes of hemolysis.

Discussion and Conclusions: CD 59 deficiency should be considered in a child with relapsing and remitting polyneuropathy.

Abstract ID: 921: YOUNG Parkinson’s: A Case Report and Clinical Insights

Venkatesh Kondle, Murugan P.K., Justin C, Elangovan S

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: Parkinson’s disease (PD) is a multifactorial disease, with its pathogenesis possibly related to factors such as oxidative stress, mitochondrial dysfunction, and abnormal protein expression. However, 10% to 15% of PD cases have a genetic predisposition. Early-onset Parkinson’s disease (EOPD) is a neurodegenerative disease associated with genetic factors. We herein report a case of PLA2G6-related EOPD caused by a heterozygous mutation.

Case report: A 46-year-old female, nondiabetic and nonhypertensive, presented with complaints of limb tightness and slowness in daily activities over the past four years. She later noticed tremors in her limbs for the past two years. On examination, she exhibited bradykinesia, bradyphrenia, decreased blink rate, a positive Meyerson sign, facial dystonia, cogwheeling of the upper limbs, spasticity of the lower limbs, a scissoring gait, postural instability, brisk reflexes, and extensor plantar response. There was no Kayser–Fleischer (KF) ring.

Discussion: All routine investigations were normal. The workup for Wilson’s disease and autoimmune conditions was negative. MRI of the brain and spine was normal. Genetic testing revealed mutation sites of PLA2G6, c.854G>A. A diagnosis of adult-onset dystonia parkinsonism (PARK14) was made based on clinical examination and genetic findings. She was started on Syndopa. However, she developed dyskinesia in both lower limbs, and the disease remains progressive.

Conclusions: The overall prognosis of the disease is poor, with high levels of disability and mortality. Thus, early identification and treatment are of critical significance for patient prognosis.

Abstract ID: 922: Double Trouble -Parsonage Turner Syndrome -An Unusual Complication in Scrub Typhus Pneumonia

Elanthiraiyan Chelvakumar, Kalpana R, Robert Wilson S, Arunan S

SRM Medical College Hospital and Research Centre, Kattankulathur, Tamil Nadu, India

Background and Aim: Parsonage–Turner syndrome is an uncommon neurological disorder characterized by the rapid onset of severe pain in the shoulder and arm. Scrub typhus can rarely cause Parsonage–Turner syndrome as a delayed complication; however, this is extremely rare. We herein report a case of Scrub Typhus pneumonia with acute respiratory distress syndrome (ARDS) that later developed into Parsonage-Turner syndrome.

Case report: A 57-year-old male with no comorbidities presented with a 1-week history of fever and headache. On examination, he was found to be dyspnoeic, tachypnoeic, and hypoxic, with respiratory failure. General examination revealed the presence of a skin eschar. Due to respiratory failure, he was intubated and placed on mechanical ventilator. IgM antibody for Scrub Typhus was positive at a high titer. A diagnosis of Scrub Typhus pneumonia with acute respiratory distress syndrome (ARDS) was made. He was treated with parenteral antibiotics: doxycycline, piperacillin–tazobactam, and levofloxacin. He recovered from his illness. A week later, while recuperating, he developed severe pain in his left shoulder and arm. Subsequently, he experienced weakness in the left shoulder girdle and left elbow muscles. On examination, he exhibited wasting and weakness of the left shoulder girdle and left elbow muscles, including the trapezius, deltoid, supraspinatus, infraspinatus, and biceps. There was also patchy sensory involvement from the left C5 to C8/T1 segment. Magnetic resonance imaging (MRI) of the left brachial plexus revealed short-tau inversion recovery (STIR) hyperintensity of the left C4-C5 to C7-D1 nerve roots, trunk, division, and cords up to the axilla, with clumping and contrast enhancement. Denervation changes in the left supraspinatus and infraspinatus muscles were evidenced by hyperintensity. Nerve conduction studies of the brachial plexus were suggestive of left brachial plexopathy.

Discussion: A diagnosis of Parsonage–Turner syndrome was made. The patient was treated with pulse methyl prednisolone followed by oral prednisolone, leading to remarkable improvement.

Conclusions: We herein present this case for its rarity and to highlight the occurrence of Parsonage–Turner syndrome as a late complication of Scrub Typhus pneumonia.

Abstract ID: 924: Rare Presentation of Cerebral Amoebiasis without any Systemic Involvement-Case Report

Savan Agrawal

Choithram Hospital & Research Centre Indore, Madhya Pradesh, India

Background and Aim: Cerebral amoebiasis is a rare form of invasive amebiasis with a fatal outcome. It is uncommon to observe cerebral involvement without any lung or liver infestation.

Case report: A 61-year-old female with no significant comorbidities presented with complaints of a headache for the past 1.5 months, 1 episode of generalized tonic-clonic seizure (GTCS) 15 days prior, and weakness in the left upper and lower limbs, along with slurred speech for the past day. On neurological examination, the patient had a Glasgow coma scale (GCS) score of E3V3M5 and left hemiparesis (power: left upper limb 3/5 and lower limb 2/5). Magnetic resonance imaging (MRI) of the brain with contrast showed a heterogenous signal lobulated mass lesion (4 × 4.6 × 3.3 cm) noted in right frontal parasagittal and parafalcine regions with moderate perilesional edema and mass effect, compressing the frontal horns of both lateral ventricles. Post-contrast images revealed diffuse leptomeningeal enhancement, raising possibility of a neoplastic process, with glioma as a differential diagnosis. The patient underwent left decompressive craniotomy with excision of the tumor. Histopathology revealed necrosis with neutrophilic exudate, reactive gliosis in the white matter, and moderate mixed inflammatory infiltrate comprising of polymorphonuclear cells, lymphocytes, and plasma cells, along with the presence trophozoite of amoeba, suggesting S/O amoebic encephalitis. Her amoebiasis IgG antibody was elevated at 39.5 (positive). A contrast-enhanced computed tomography (CECT) scan of the chest and abdomen showed no evidence of amoebiasis.

Discussion: The patient was started on metronidazole 750 mg thrice daily for 21 days and paromomycin 500 mg thrice daily for 10 days. Her GCS Improved, and she was discharged. At the 4-month follow-up, the patient had fully recovered and was able to walk independently.

Conclusions: Cerebral amoebiasis is a rare and potentially fatal form of invasive amoebiasis. Timely diagnosis and treatment are essential to prevent mortality and complications.

Abstract ID: 925: Primary CNS Vasculitis with Atypical Initial Presentation – A Case Report

Srilekha D, Surya Prakash, Malcolm Jeyaraj, Mugundhan K

Stanley Medical College, Chennai, Tamil Nadu, India

Background and Aim: Primary angiitis of the central nervous system (PACNS) is a rare idiopathic vasculitis that affects small and medium-sized vessels, confined to the brain, leptomeninges, and spinal cord.

Case report: A 39-year-old male presented with sudden-onset bowel and bladder incontinence. Two weeks later, he developed dysphagia and dysarthria, along with features of pseudobulbar palsy and affect. Four weeks after the initial symptom, he experienced facial deviation and limb weakness. On examination, he was conscious but confused and disoriented, with emotional lability, an exaggerated gag reflex, right upper motor neuron facial palsy, and right hemiparesis.

Discussion: Magnetic resonance imaging (MRI) of the brain showed multiple infarcts in bilateral frontal, left internal capsule, corona radiata, bilateral midbrain, and pons. Magnetic resonance angiography was normal. Repeat imaging 1 week later showed further progression of the lesions, which were bilateral and asymmetric. Digital subtraction angiography demonstrated a multifocal “beading” pattern involving medium-sized vessels. Viral serology and systemic vasculitis profiles were negative. Cerebrospinal fluid (CSF) analysis revealed elevated protein levels and an acellular smear, and CSF polymerase chain reaction (PCR) for viruses was negative. He was diagnosed with primary central nervous system (CNS) vasculitis and treated with 5 days of pulse IV methylprednisolone, followed by oral prednisone. Immunomodulatory therapy was initiated with monthly IV cyclophosphamide injections. The patient showed slow clinical improvement over the next 3 months.

Conclusions: Diagnosing PACNS is challenging not only due to its rarity but also because of its variable and often nonspecific presentations. A high index of clinical suspicion and rigorous investigations are necessary to confirm the diagnosis and exclude other mimicking conditions.

Abstract ID: 926: Ptotic Tongue- A case series of 7 Patients with Segmental Approach of Different Etiological Variants

Jatinkumar Jain, Himanshu Kaushal, Yogesh kaushik, Amit Agarwal, Rajendra Kumar Sureka

Mahatma Gandhi Hospital Medical College, Jaipur, Rajasthan, India

Background and Aim: Ptotic tongue (PT) or tongue paresis—whether unilateral or bilateral—is one of the less acknowledged clinical features found in routine practice. PT can result from lesions involving the supranuclear, nuclear, infranuclear pathways, or the tongue musculature itself. Although the segmental approach for diagnosing infranuclear lesions has been well described in the literature, the supranuclear pathway is often overlooked. In this case series, we present various etiologies of both supranuclear and infranuclear PT.

Case report: Seven patients admitted to the neurology department with PT, either in association with other cranial nerve palsies, focal deficits, or as an isolated PT, were evaluated through clinical history, neurological examination, and relevant investigations. 1- A 52-year-old male presented with acute onset of complete tongue palsy and quadriparesis, diagnosed with bilateral Dejerine syndrome (medial medullary syndrome). 2- A 69-old- female presented with subacute onset of unilateral left tongue palsy, diagnosed with Idiopathic hypoglossal nerve palsy. 3- A 55-year-old male presented with spastic dysarthria and dysphagia, diagnosed with bulbar onset amyotrophic lateral sclerosis. 4- A 58-year-old female with a history of squamous cell carcinoma of the lung presented with acute onset dysarthria, diagnosed with base of skull metastasis involving the left hypoglossal canal. 5- A 9-year-old male presented with insidious onset quadriparesis, dysarthria, and decreased tongue movements on the right side, diagnosed with syringobulbia and Arnold-chiari malformation (nuclear involvement). 6- A 46-year-old male presented with insidious onset and gradually progressive dysarthria, bilateral ptosis, and dysphagia, diagnosed with oculopharyngeal muscular dystrophy (tongue musculature involvement) 7- A 68-year-old male presented with decreased tongue movements and submandibular swelling, diagnosed with squamous cell carcinoma of the tongue (sublingual segment)

Discussion and Conclusions: A segmental approach for the tongue paresis helps in evaluation of the supranuclear, nuclear, and infranuclear causes of ptotic tongue.

Abstract ID: 927: Miller Fisher Syndrome following DPT Vaccination

Kuljeet Anand, Ankur Chawla

National Institute of Mental Health and Neuro-Sciences (NIMHANS), Bengaluru, Karnataka, India

Background and Aim: Miller Fisher syndrome (MFS) is one of the variants of Gullian–Barre syndrome characterized by a triad of areflexia, opthalmoplegia, and ataxia. Anti-GQ1b antibodies are present in 90% of cases. The common triggers for MFS include C. jejuni, human immunodeficiency virus (HIV), Epstein–Barr virus (EBV), COVID-19, and Zika virus infections. There have been reports of MFS following COVID vaccination. In the literature, there is an isolated case of MFS after the diphtheria and tetanus (DT) vaccine in an adult. This report presents a case of MFS following the diphteria-tetanus-pertussis vaccine (DPT) vaccine in a child.

Case report: A 5-year-old girl presented with double vision, unsteadiness in all four limbs (LL > UL), and frequent falls while walking for 2 days. The child had received a booster dose of the DPT vaccine two weeks prior to the development of these symptoms. There was no reported weakness, stiffness, or urinary symptoms. The general examination and systematic review were unremarkable.

Discussion: Neurological examination revealed normal higher mental function (HMF), normal pupil size, and a normal reaction to light. Ocular movements were restricted in all directions. Motor power was 5/5 with hypotonia. There was gross in-coordination with impaired finger-nose and heel-knee tests. All deep tendon reflex (DTRs) were absent. Routine investigations were within normal limits. The serum GQ1b antibody test was not performed. Magnetic resonance imaging (MRI) of the brain and cervical spine was normal. The nerve conduction study was also normal. Cerebrospinal fluid (CSF) analysis showed albuminocytological dissociation.

Conclusions: To the best of our knowledge, this is the first report of MFS following DPT vaccination in a child.

Abstract ID: 928: A Rare Case of Intracranial Hemorrhage

Sridevi Natrajan, Rajasekaran M, Kannan N, Arunraj Ezhumalai, Kishore R

KAPV Govt Medical College, Trichy, Tamil Nadu, India

Background and Aim: Isolated interhemispheric subdural hematoma (IHSDH) represents one of the rarest forms of intracranial hemorrhage. ISH usually occurs after head trauma; however, its spontaneous or nontraumatic occurrence is extremely rare. We present a case of spontaneous ISH and review its clinical presentation and management.

Case report: A 60-year-old male presented with an acute onset of severe holocranial throbbing headache and vomiting. The patient had no history of seizures or weakness. On examination, he was hemodynamically stable, with a blood pressure (BP) of 140/90 mmHg and a Glasgow coma scale (GCS) score of 15/15. His pupils were normal, muscle tone and power were intact. A computed tomography (CT) scan of the brain showed a 1 cm thick isolated subdural hematoma extending along the full length of the interhemispheric fissure.

Discussion: The CT angiogram showed no evidence of an aneurysm or cerebral venous thrombosis (CVT). For further evaluation, we performed digital subtraction angiography (DSA) at KAPV Government Medical College, which showed no evidence of aneurysm in the arterial phase and no evidence of CVT in the delayed venous phase. The coagulation profile, complete blood count, bleeding time, peripheral smear, D-dimer, and cancer screening results were all normal. His sensorium remained stable, and he was treated conservatively.

Conclusions: Isolated spontaneous IHSDH is rare. We carefully excluded all potential causes, and the patient was observed for any expansion of the hematoma or rapidly deteriorating levels of consciousness. Nonsurgical treatment was chosen, as his neurological condition remained stable.

Abstract ID: 929: Cross Sectional Study of Clinical and Cognitive Profile in Patients With Wilson’s Disease in Tertiary Care Hospital

Suman S. Khushwaha, Pratik Pravin Babel, Aldrin Anthony, Rachana Agarwal, Monali Chaturvedi, Mohammmed Faruq, Rajinder Dhamija, Chandra Tripathi

Institute of Human Behaviour and allied sciences, New Delhi, India

Background and Aim: Wilsons disease (WD) is associated with a variety of movement disorders. Although dystonia and dysarthria are the most common neurological signs, cognitive changes have been reported since 1912. Brain magnetic resonance imaging (MRI) is abnormal in the majority of neurological WD cases. India has been a geographical hotspot for WD. Cognitive changes reported in WD include frontal syndrome and subcortical dementia. This study provides insight into detailed cognitive functions.

Methodology: We recruited 30 patients with WD. The unified WD rating Scale (UWDRS) and global assessment scale (GAS) were used to determine the severity of neurological involvement. Detailed cognitive testing was performed, and MRI of the brain was conducted to study the pattern of involvement and its correlation with the severity of neurological involvement.

Results: Interim analysis yielded the following findings: 1. The mean duration from disease onset to diagnosis was 2.5 years. 2. Among the 30 patients, 19 (63.3%) had cognitive impairment at presentation. 3. Frontal cognitive dysfunction was found in 16 (53.3%) patients. 4. Baseline MRI of the brain showed abnormal findings in 27 (90%) of the patients in the form of T2 hyperintensities and atrophy, which correlated with the severity of functional disability.

Conclusions: Although WD typically presents as various movement disorders, cognitive dysfunction—predominantly involving frontal circuitry—should not be overlooked. This includes executive function, memory dysfunction, and behavioral changes. Neuro-rehabilitation for patients with WD should also include cognitive rehabilitation to maximize independence and quality of life. The degree of neurological impairment in WD correlates with the time gap from disease onset to diagnosis, as well as the extent of atrophy and T2 hyperintensity in MRI of the brain. Further studies are required to guage the reversibility of cognitive symptoms with conventional treatment.

Abstract ID: 930: Congenital Myesthenia Syndrome an Underdiagnosed and Treatable Neuromuscular Disorder

Abhishek Bhargava, Bhawna Sharma, Shubham Agrawal

Sawai Mansingh Medical College, Jaipur, Rajasthan

Background and Aim: Congenital myasthenia syndrome (CMS) is an inherited neuromuscular disorder and can be classified into various subtypes depending on the present mutation.

Case report: Four cases of CMS were diagnosed in the SMS Neurology Department in Jaipur. Based on the history and clinical findings, congenital myasthenia was suspected, and the diagnosis was confirmed through whole exome genetic testing.

Discussion: Out of the four cases, three patients (average age 22.4 years) were found to have a CHRNE gene mutation, resulting in a postsynaptic type of CMS. All three patients exhibited decreased intrauterine fetal movements. During infancy, they presented with a poor sucking reflex and feeble cry. Motor milestones were delayed. One out of the four patients also developed respiratory distress and frequent pneumonia during infancy. The patients experienced fatiguability and fluctuating weakness that exacerbated after exercise and in hot weather but was relieved by rest and cooler environments. Additionally, they exhibited proximal weakness in both upper and lower limbs, as well as bilateral ptosis and external ophthalmoplegia. Given the onset of symptoms at birth and the diurnal type of weakness, the possibility of CMS was considered and subsequently confirmed by whole exome genetic testing. A differential diagnosis of mitochondrial chronic progressive external ophthalmoplegia was also made and ruled out.

Conclusions: This case series illustrates the importance of accurately diagnosing the mutation causing CMS, as the best treatment options are provided according to the subtypes.

Abstract ID: 931: A Rare Case Report of Heerfordth Syndrome

Rajni Fulwariya

SMS Hospital, Jawahar Lal Nehru Marg, Ashok Nagar, Jaipur, Rajasthan, India

Background and Aim: Sarcoidosis is an inflammatory granulomatous disease that involves multiple organs. The commonly affected organs include the lungs, skin, eyes, lymph nodes, spleen, and liver. Neurological involvement in sarcoidosis occurs in only 5%–10% of cases as the initial presentation.

Case report: We report a case of 39-year-old male who presented with a three-month history of intermittent uveitis, followed by bilateral parotid swelling, bilateral lower motor neuron (LMN) facial palsy, bilateral bulbar weakness, and tingling in the left upper limb and right lower limb. He also exhibited some autonomic symptoms, including presyncopal attacks, and presented with orthostatic hypotension.

Discussion: His inflammatory markers were raised, and he exhibited hypercalcemia and increased vitamin D levels, while angiotensin converting enzyme (ACE) levels were normal. Cerebrospinal fluid analysis showed mildly elevated protein levels. Computed tomography (CT) of the chest and abdomen with contrast revealed paratracheal and subcarinal lymphadenopathy (Garland sign). Nerve conduction studies were normal. Contrast-enhanced magnetic resonance imaging (MRI) of the brain showed patchy enhancement of multiple cranial nerves, suggestive of an infectious or inflammatory etiology. Endoscopy-guided paratracheal lymph node biopsy revealed non-caseating granulomas. A diagnosis of Heerfordt syndrome was established. The patient was treated with intravenous (IV) pulse methylprednisolone at a dosage of 1 g for 5 days, resulting in significant clinical improvement.

Conclusions: It is imperative to diagnose this condition as early as possible, as patients may have experience considerable clinical improvement following steroid treatment.

Abstract ID: 932: Middle-Aged Man with Transient Focal Neurological Deficits

Brunda S, Raghunandan Nadig, GRK Sarma, Thomas Mathew, Sharath Kumar GG

St. Johns Medical College, Bengaluru, Kanataka, India

Background and Aim: Transient focal neurological deficits include brief motor, sensory, visual, or language disturbances. The most common causes are transient ischemic attacks (TIAs), sensory seizures, and psychiatric disorders. Determining the etiology is critical for the treatment and prognostication of the disease.

Case report: A 54-year-old male with no comorbidities presented with a 6-year history of episodic numbness and paraesthesia on the right side of his body, accompanied by vertiginous sensations, dysarthria, and ataxia lasting 10–15 min. This was accompanied by behavioral changes and agitation lasting for about 24 h. The patient experienced an increased frequency of episodes, rising from 1–2 per year to >5 episodes per month in the last 6 months. Positive neurological findings included brisk reflexes, impaired tandem gait, and difficulty with the finger-nose-finger test.

Discussion: Investigations: Previous workups revealed a normal magnetic resonance imaging (MRI) of the brain (plain study). Cerebrospinal fluid (CSF) analysis revealed no cells, with glucose at 78, protein at 87, and negative cytology. Audiometry indicated severe sensorineural hearing loss. Electro neuro-myography (ENMG) suggested carpal tunnel syndrome (CTS). An electroencephalogram (EEG) was suggestive of frontal slowing. Autoimmune, paraneoplastic, and toxic profiles were negative. However, repeat imaging, including gradient sequences, revealed blooming in the posterior fossa, cortical sulci, and interhemispheric fissure, with diffuse leptomeningeal enhancement. Sudoscan results indicated advanced peripheral sudomotor dysfunction.

Conclusions: Special imaging sequences should be considered for patients presenting with transient focal deficits. An accurate diagnosis facilitates the consideration of newer, targeted treatment modalities.

Abstract ID: 933: A Case of Mental Retardation with Dysmorphic Facies

Karthik Reddy Pappireddy, Aruna Kumari U

Guntur Medical College, Guntur, Andhra Pradesh, India

Background and Aim: Sanfilippo syndrome, or mucopolysaccharidosis type III (MPS III), is a rare autosomal recessive lysosomal storage disorder characterized by severe neurological symptoms due to the accumulation of heparan sulfate. This case report details the clinical presentation, diagnostic process, and genetic confirmation of Sanfilippo syndrome in a 17-year-old male with a history of global developmental delays, urinary incontinence, and behavioral disturbances.

Case report: A 17-year-old male, born of a second-degree consanguineous marriage, presented with delayed milestones since infancy, including motor and speech delays, urinary incontinence, and hyperactive, agitative behavior. Clinical examination revealed coarse facial features, a prominent forehead, low-set ears, scaphocephalic cranium, and left-sided scoliosis. Neurological examination showed a conscious but noncomprehending patient with a slow gait and short strides.

Discussion: Routine blood investigations, viral screening, and thyroid profile were normal. Magnetic resonance imaging (MRI) of the brain revealed marked atrophy of the bilateral cerebral cortices, moderate supratentorial ventricular dilatation, and bilateral periventricular white matter changes, predominantly in the parietal lobes. Genetic analysis confirmed Sanfilippo syndrome.

Conclusions: This case underscores the importance of considering Mucopolysaccharidosis in patients with global developmental delays, distinct facial features, and behavioural disturbances. Early diagnosis through clinical evaluation, imaging, and genetic testing is essential for appropriate management and family counselling. This report contributes to the clinical understanding of MPS III and highlights the significance of genetic confirmation in diagnosing rare metabolic disorders.

Abstract ID: 934: Clinical Characteristics of Headache Disorders Presenting to Headache Clinic in a Tertiary Care Center in North India – A Prospective Observational Study

Ashutosh Tiwari, Muneeswaran Rathinasabapathi, Mritunjai Kumar, Niraj Kumar

All India Institute of Medical Sciences (AIIMS), Rishikesh, Uttarakhand, India

Background and Aim: Headache disorders are among the most disabling conditions causing significant morbidity both worldwide and within the Indian population. This study aimed to analyse the clinical characteristics of headache disorders presenting to a headache clinic in a tertiary care centre in the sub-Himalayan region of India.

Methodology: In this prospective observational study, after obtaining approval from the institutional ethics committee, we included all patients who visited the headache clinic from February 2022 to April 2024. A detailed history based on a semi structured questionnaire related to headache was documented for all patients. Written informed consent was obtained from the patients. The diagnosis of headache was made according to the International Classification of Headache Disorders-3 (ICHD3).

Results: Out of 257 patients in the study, 75.5% were females. Primary headache disorders constituted 93.7% of the cases compared to secondary headaches. Migraine was the most common type, contributing to 78.8% of the primary headaches and 73.9% of overall headaches, followed by tension-type headache (TTH) at 12.4% and trigeminal autonomic cephalalgias (TACs) at 5.4%. Secondary headaches included facial pain syndrome, medication overuse headaches, trigeminal neuralgia, Tolosa–Hunt syndrome, thunderclap headache, idiopathic intracranial hypertension, and intracranial hypotension.

Conclusions: Conclusion: Primary headache disorders, predominantly migraine, followed by TTH and TACs, are the predominant presentations in headache clinics.

Abstract ID: 935: An Interesting Case of Myoclonus

Akula Sushmitha, Aruna Kumari U, Bindhu Narmada G

Guntur Medical College, Andhra Pradesh, India

Background and Aim: Lafora Body Disease (LBD) is a rare autosomal recessive neurodegenerative disorder characterized by progressive myoclonic epilepsy and cognitive decline. It typically manifests in late childhood or adolescence, posing challenges in diagnosis and management due to its rarity and varied clinical presentation.

Case report: We report the case of an 18-year-old male with a history of generalized tonic-clonic seizures since childhood, which progressively evolved into multifocal myoclonus, ataxia, dysarthria, and cognitive decline. Born to consanguineous parents, the patients exhibited characteristic neurological findings, including generalized cachexia, multifocal myoclonus, truncal, limb, and gait ataxia, and dysarthria. Magnetic resonance imaging (MRI) showed cerebral atrophy, and electroencephalogram (EEG) showed generalized slowing with polyspike and wave discharges. An axillary skin biopsy revealed inclusion bodies in apocrine glands. Treatment with anti-seizure medications led to a reduction in seizure frequency.

Discussion: Axillary skin biopsy with periodic acid-schiff (PAS) stain revealed positive inclusions in the cytoplasm of epithelial and myoepithelial cells of apocrine glands, consistent with LBD

Conclusions: This case highlights the importance of considering LBD in adolescents presenting with progressive myoclonic epilepsy and cognitive decline, especially in consanguineous populations. Timely diagnosis and management are essential to slow disease progression and improve patient outcomes. Increased awareness and further research are warranted to enhance understanding and therapeutic options for this devastating disease.

Abstract ID: 936: A Diagnostic Odyssey: Guillain Barre Syndrome Exposed as Ethylene Glycol Poisoning

M. Kumar, MURUGAN P. K., JUSTIN C, ELANGOVAN S

Madurai Medical College Tamil Nadu, India

Background and Aim: Ethylene glycol poisoning can present diagnostic difficulties, especially if the history of ingestion is not provided or if the presentation is delayed. This case illustrates the less commonly recognized clinical features of delayed ethylene glycol poisoning, including multiple cranial and peripheral neuropathies, along with the associated clinical findings.

Case report: A 30-year-old male presented with abdomen pain and vomiting for 3 days. On day 5, he developed flaccid proximal weakness in both upper limbs, followed by weakness in both lower limbs and bifacial weakness the next day. On examination, his pulse rate was 108 beats per min, blood pressure was 140/80 mmHg, and his abdomen was mildly tender. He exhibited bifacial weakness, ophthalmoplegia, symmetric flaccid weakness of all four limbs, and areflexia with normal sensory function, and plantar reflexes were flexor, with no bladder involvement. On reviewing his history, it was discovered that he had ingested ethylene glycol liquid 4 days back.

Discussion: Investigations showed normal cell counts, electrolytes, and liver function but raised renal parameters on day 8. A 2D echo and ultrasonography (USG) abdomen were normal. Nerve conduction study (NCS) showed sensory motor demyelinating polyneuropathy with sural nerve sparing, and cerebrospinal fluid (CSF) showed albuminocytological dissociation. A diagnosis of toxic-induced polyradiculoneuropathy was made, and the patient was started on methylprednisolone (1g IV OD) and hemodialysis. The patient showed improvement and was able to walk with support at the time of discharge.

Conclusions: Supportive therapy, including renal replacement therapy and ventilatory support for severe neurological deficits, is the mainstay of treatment for delayed ethylene glycol poisoning.

Abstract ID: 937: Isolated Frontal Variant of Adrenoleukodystrophy-A Rare Case Report

Suprabha Shukla, Ashok Kumar Mallick, Dillip Kumar Das, Kali Prasanna Swain

Shriram Chandra Bhanja Medical College and Hospital, Cuttack, Odisha, India

Background and Aim: Adrenoleukodystrophy (ALD) is an X-linked peroxisomal disorder caused by mutation in the ABCD 1 gene. The common childhood cerebral form usually manifests between 4 and 8 years of age, with progressive deterioration in cognition, vision, hearing, and motor function. In 15%–20% of cases, there is predominant or isolated frontal lobe involvement. The F8 gene (Hemophilia A) and the ABCD1 gene are located in close proximity on the Xq28 chromosome. We report a rare case of a 10-year-old-male born to a father with Hemophilia A, presenting with the frontal variant of ALD.

Case report: The child was born from a nonconsanguineous marriage to a father with Hemophilia A (who died at 35 years of age). He had an uneventful perinatal history and achieved normal developmental milestones. The patient presented with relapsing and progressive neurological symptoms for 1 year, including insidious onset of progressive right hemiparesis with convulsions, partial improvement on steroids, behavioural disturbances (aggressive outbursts), bowel incontinence, increased urinary frequency, and decreased speech output.

Discussion: Magnetic resonance imaging (MRI) of the brain showing B/L frontal subcortical white matter signal changes involving corpus callosum and descending pyramidal fibers, with areas of restriction and contrast enhancement in these regions. Evaluation for leukoencephalopathy, including demyelination [antinuclear antibody (ANA), myelin oligodendrocyte glycoprotein (MOG), and neuromyelitis optica (NMO)], and cerebrospinal fluid (CSF) studies were negative. The patient was treated with IV methylprednisolone at a dosage of 30 mg/kg/day for 5 days f/b, followed by oral steroids; however, no improvement was observed in clinical condition. With a differential diagnosis of atypical demyelination and leukodystrophy (frontal variant ALD), lymphoma, whole exome sequencing (WES), and brain biopsy were performed, which revealed astrogliosis with nonspecific inflammatory changes. Serum cortisol and adrenocorticotropic hormone (ACTH) levels were normal. An ABCD1 gene mutation was detected in WES, but the patient expired during hospitalization.

Conclusions: As the ABCD 1 gene and F8 gene are located in close proximity on the Xq28 chromosome, the occurrence of both ALD and Hemophilia A in the same family is rare, as demonstrated in our case.

Abstract ID: 938: A Rare Cause of Focal Dystonia

Swetha Kannadhasan, Velusamy S, Nithyanandham A, Aravind Gautham

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: Dystonia is a syndrome characterized by sustained muscle contractions, often resulting in twisting and repetitive movements or abnormal postures. There are no clinically available diagnostic laboratory or imaging tests to confirm the diagnosis nor are there validated diagnostic criteria that can be applied. The diagnosis depends entirely on the recognition of clinical features.

Case report: A 72-year-old male presented with complaints of difficulty using his right hand for the past one and a half years, manifesting as difficulty in mixing food, buttoning his shirt, and lifting his arm. He also reported numbness in the right upper limb during the same period. The onset of these symptoms was insidious and nonprogressive. The patient had a history of a fall from a considerable height a few months before the onset of these symptoms. He did not report any involuntary movements. On examination, higher mental functions and cranial nerves assessments were normal. Spinal motor testing revealed right upper limb spasticity with action-induced twisting and posturing of the right upper limb, particularly in the hand, accompanied by mild writhing movements, which increased with eye closure. Mild weakness was also noted. Sensory examination revealed impaired pain, touch, and proprioception, with preserved joint position sense. All other examinations were normal. Computed tomography (CT) brain revealed a left frontal acute-on-chronic subdural hematoma (SDH) and left parietal encephalomalacic changes.

Discussion and Conclusions: This case highlights the association of cortical dysfunction, particularly in the sensory cortex, with dystonia, emphasizing that this movement disorder is not exclusively related to basal ganglia and its connections.

Abstract ID: 939: An Usual Yet Unusual Bulbar Onset ALS Mimic

Prachi Mohapatra, M. Vasantha Padma Srivastava, Rohit Bhatia, Mamta Bhusan Singh, Venugopalan Y. Vishnu, Roopa Rajan, Anu Gupta

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Amyotrophic lateral sclerosis (ALS) is a progressive neurological disorder with no cure, making it essential to rule out mimics. Herein, we report the case of a 36-year-old male who initially presented with prominent bulbar symptoms, followed by distal weakness in both upper limbs and spasticity in all four limbs. He was previously labeled as having ALS but was later diagnosed with Grave’s disease and showed symptomatic improvement with antithyroid therapy.

Case report: A 36-year-old male with no known comorbidities presented with a 2-year history of effortful, slurred speech with nasal intonation, along with difficulty swallowing liquids more than solids. Six months later, he developed asymmetric distal weakness (left > right) and fasciculations in both upper limbs, followed by stiffness in both lower limbs. On examination, the patient exhibited mixed dysarthria, spasticity in all four limbs with hyperreflexia, weakness, and mild thinning of the bilateral thenar and hypothenar muscles. He was initially diagnosed with ALS at another facility, where nerve conduction study (NCS) and electromyography (EMG) revealed reduced compound muscle action potential (CMAPs) in the bilateral ulnar and median nerves, along with fasciculations in the cervical and lumbosacral segments, showing a neurogenic pattern. A brain and spine magnetic resonance imaging (MRI) was normal, and he was started on riulozole. On re-evaluation, the patient was found to have hyperthyroidism, with a thyroid stimulating hormone (TSH) level < 0.001 and diffuse thyroid uptake on both positron emission tomography (PET) and Tc scintigraphy. Ultrasonography (USG) of the thyroid showed increased vascularity, and anti-thyroid peroxidase (anti-TPO) antibodies were strongly positive, suggesting a diagnosis of Grave’s disease. He was treated with 10 mci radioactive iodine (I-131).

Discussion: After 1 month of therapy, there was an almost complete resolution of speech and bulbar dysfunction, along with significant improvement in spasticity and upper limb weakness.

Conclusions: This case highlights the atypical neurological features of Grave’s disease and underscores the importance of conducting thyroid function tests before diagnosing a patient with motor neuron disease (MND).

Abstract ID: 940: KCTD7 Related- Progressive Myoclonic Epilepsy

Apsara P S, P. K. Murugan, C. Justin

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: Mutations in the potassium channel tetramerization domain-containing KCTD7 gene cause progressive myoclonic epilepsy 3 (PME3), a rare but often crippling neurodevelopmental and epileptic disorder. This is a rare report associating the KCDT7 mutation with PME in the Indian population.

Case report: A 1.5-year-old male child, born to third-degree consanguineous Indian parents, presented to the neurology clinic with complaints of seizures and regression of developmental milestones. Pregnancy and birth history were uneventful, with no indication of intrauterine or perinatal hypoxia or other neonatal complications. Seizures started when the patient was 10 months old, following a period of normal development. The seizures were continuous multifocal myoclonic seizures, affecting all limbs and the face, with rare tonic and tonic-clonic seizures.

Discussion: Electroencephalogram (EEG) showed multifocal epileptiform abnormalities, while magnetic resonance imaging (MRI) of the brain was normal at the time. The patient was initially started on valproic acid, pyridoxine, and clobazam, but there was no response. Hence, prednisolone was added, which led to a reduction in seizure frequency. DNA sequencing using a next-generation sequencing platform revealed a pathogenic homozygous KCDT7 gene mutation, confirming a diagnosis of autosomal recessive progressive myoclonic epilepsy 3.

Conclusions: Herein, we report a patient with PME in whom next-generation sequencing identified a homozygous KCTD7 mutation. It is a rare report of KCDT7 mutation-associated PME in the Indian population.

Abstract ID: 941: An Early Clue to Diagnose a Disastrous GBS Masquerader

Anusha D, Anusha Doraiswamy

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: This case report aims to highlight the importance of early clinical findings that can precede the onset of alopecia in the rarely diagnosed condition of thallium poisoning.

Case report: A 29-year-old IT professional with no prior illnesses presented with a history of severe, bilateral, lower limb disabling paresthesias. He was initially treated with oral steroids for suspected acute neuritis at another facility. Due to persistent severe symptoms, he presented to our hospital. On examination, he was unable to walk but had retained reflexes in the lower limbs with no demonstrable weakness or sensory deficits. He developed a perioral rash soon after starting gabapentin, which was considered an allergic reaction to the drug. Nerve conduction studies indicated demyelinating radiculoneuropathy. Vasculitic workup and porphyria screening was negative. Cerebrospinal fluid (CSF) analysis showed albuminocytologic dissociation. Suspecting early Gullian–Barre syndrome, the patient was treated with IVIG at total dose of 2 g/kg, which led to significant improvement in neuropathic pain and the resumption of normal walking. The patient was asymptomatic at discharge, except for persistent abdominal pain with a normal computed tomography (CT) Abdomen. One week later, he returned with the sudden onset of lower limb weakness and repeated episodes of vomiting black coloured fluid. Heavy metal toxicity, porphyria, vasculitis, atypical infections, and autoimmune neuropathies were considered. Significant alopecia was noted, leading to the consideration of thallium toxicity. Although the patient initially denied any exposure to heavy metals, 24-h urinary and blood thallium levels were found to be over 400 ug/L (normal values: 24-h urine < 10, blood < 2).

Discussion: The patient was treated with oral Prussian blue and underwent repeated hemoperfusion until his serum thallium levels normalized.

Conclusions: Thallium poisoning should be proactively considered in cases of acute neuropathy.

Abstract ID: 942: Congenital Myasthenic Syndrome -A Case Series

Selvakumar T, Balaji G, Hariharan S, Thamilpavai N

Government Chengalpattu Medical College, Tamil Nadu, India

Background and Aim: Congenital myasthenic syndromes (CMS) are a rare, heterogeneous group of early-onset genetic neuromuscular transmission disorders caused by mutations in proteins involved in the organization, maintenance, function, or modification of the motor endplate.

Case report: We report case series of three patients with CMS who tested positive for mutations in the CHRNE (Cholinergic receptor nicotinic epsilon) gene. Case 1: A 19-day-old male child, born to nonconsanguinous parents with no family history of full term normal vaginal delivery, presented with shallow breathing, hypotonia of the limbs since birth, and an inability to open both eyes. Clinical suspicion indicated myasthenia syndrome. Whole exome sequencing revealed a missense variant, c.1190G>A (p.Gly397Glu), in the CHRNE gene, suggestive of slow channel congenital myasthenic syndrome 4A. The patient was treated with p yridostigmine at a dosage of 2 mg/kg/day. Case 2: A 13-year-old female, with no significant family history, presented with bilateral ptosis exhibiting diurnal variation since birth Whole exome sequencing showed a frameshift deletion, c.1327delG, in the CHRNE gene in exon 12, suggestive of fast channel congenital myasthenic syndrome 4B. She was treated with pyridostigmine at 2 mg/kg/day and demonstrated clinical improvement. Case 3: A 46-year-old female, with no family history, reported bilateral ptosis since birth, difficulty getting up from a squatting position, lifting her arms, and swallowing difficulties for the past 3 years Whole exome sequencing showed defective CHRNE gene mutations (c.1251_1266dup, and c.209_210del), suggestive of CMS. She was treated with pyridostigmine at a dosage of 60 mg QID.

Discussion and Conclusions: Early diagnosis of congenital myasthenic syndrome significantly reduces neonatal mortality. Additionally, genetic diagnosis provides clinicians with valuable insights for treatment and further management.

Abstract ID: 943: Once Bitten, Twice not so Shy

Aditya Ganguly

Kolkata Medical College, Kolkata, West Bengal, India

Background and Aim: Delirium is a common issue in neurology, typically arising from a limited number of causes. This case report presents a patient who developed delirium due to an unusual cause under atypical circumstances. This will help clinicians make a quicker diagnosis and provide better outcomes.

Case report: A detailed history was obtained, and general and neurological examinations were performed. Neuroimaging was conducted for this patient admitted under the Department of General Medicine at Medical College Kolkata as a referral case. A 52-year-old woman with a history of stable ischemic heart disease (sIHD) presented with subacute onset of progressively worsening holocranial headache, followed by delirium after travelling to Darjeeling from Kolkata. Her symptoms started within 1 day of reaching her destination. The patient had a history of similar headaches and mild drowsiness during a visit to Darjeeling approximately 2 years back. She was advised not to visit the place but chose not to heed this advice. The patient was quickly brought back to Kolkata within 3 days of symptom onset and was subsequently admitted to our institution. Routine blood investigations were normal. MRI of the brain showed diffuse T2/fluid attenuated inversion recovery (FLAIR) hyperintensities in the subcortical white matter and corpus callosum, with a cytotoxic lesion of the corpus callosum (CLOCC), suggestive of high-altitude cerebral edema. The patient was managed in the ICU with supplemental O2 therapy and dexamethasone, achieving a complete recovery over several weeks.

Discussion and Conclusions: One should keep a close watch to ensure that high-altitude cerebral edema as a cause of delirium is not overlooked.

Abstract ID: 944: Foix-Chavany-Marie Syndrome: An “Anterior Opercular Syndrome”

Vishwakiran Jhawar

Narayana Health City, Bengaluru, Karnataka, India

Background and Aim: The Foix–Chavany–Marie syndrome (FCMS), also known as anterior opercular syndrome, is characterized by the loss of voluntary movement in the orofacial and pharyngeal muscles, while automatic and reflex movements remain preserved. This syndrome arises from bilateral lesions in the anterior opercular cortex. The most frequent etiology is vascular, but the causes can be heterogenous. Clinically, FCMS can be divided into three categories—developmental, acute/subacute, and transient. The developmental form is commonly associated with congenital bilateral anterior opercular dysplasia, while the acute/subacute form is often caused by central nervous system (CNS) infections or cerebrovascular accident.

Case report: We herein report two cases of FCMS with etiologies of stroke and herpes simplex virus (HSV) encephalitis presented to our hospital. 1) A 56-year-old female with a history of right hemispheric stroke presented with complaints of swallowing difficulty and slurred speech for the past 5 months, with no limb involvement. On examination, she had buccal apraxia and oro-facial weakness, but no limb deficits were noted. Evaluation revealed a cardioembolic stroke with a new infarct in the left MCA and left ventricular (LV) clot. Due to her prolonged need for a nasogastric tube (NG) tube for over 6 months, she was given the option of a percutaneous endoscopic gastrostomy (PEG) tube placement. 2) A 51-year male presented with a 3-day history of headache followed by altered behavior for 1 day, diagnosed with HSV encephalitis. Magnetic resonance imaging (MRI) of the brain with contrast showed encephalitis features involving the bilateral opercular region. He gradually recovered after a course of IV antivirals, but he experienced minimal residual cognitive impairment and swallowing difficulties, necessitating an NG tube. However, these symptoms improved gradually with aggressive therapy within 1 month.

Discussion and Conclusions: This report highlights two varied etiologies of the FCMS and their different outcomes. Increased awareness of this syndrome among clinicians is essential, especially from a clinical localization perspective.

Abstract ID: 945: Atypical Case of Leptomeningitis with MRI Negative Non- Compressive Myelopathy

Tejal Kakhandki, Vivek Jacob Philip

Narayana Health City, Bengaluru, Karnataka, India

Background and Aim: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory disorder of the central nervous system characterized by immune-mediated demyelination predominantly targeting the optic nerves, brain, and spinal cord. Aseptic meningitis is a rare presentation of MOGAD. While MOGAD-associated myelitis usually shows lesions on MRI, a negative spinal cord magnetic resonance imaging (MRI) should not deter testing for myelin oligodendrocyte antibody (MOG-IgG) in patients with acute or subacute myelitis.

Case report: We present a case of a patient with MOGAD admitted to our hospital. A young male patient initially presented with symptoms indicative of leptomeningitis. Despite 2 weeks of treatment for meningitis, he subsequently developed clinical signs of myelopathy. Notably, multiple MRI scans of the spinal cord returned negative results. Further, his serum tested positive for myelin oligodendrocyte glycoprotein (MOG) antibodies. Following treatment with intravenous steroids, the patient’s symptoms fully resolved.

Discussion and Conclusions: Given the high relapse rates associated with MOGAD, this case report aims to heighten the clinical awareness of such atypical presentation to facilitate early diagnosis and appropriate management.

Abstract ID: 946: Diagnostic and Therapeutic Challenges in Management of Spontaneous Intracranial Hypotension

Rohit Kushwah, Samhita Panda, Sarbesh Tiwari, Swati Chhabra, Suryanarayanan Bhaskar, Simran Bhalla

All India Institute of Medical Sciences (AIIMS), Jodhpur, Rajasthan, India

Background and Aim: Spontaneous intracranial hypotension (SIH) is a clinical condition caused by low cerebrospinal fluid (CSF) volume or pressure, which may result from a spinal or cranial CSF leak. Various diagnostic modalities such as magnetic resonance imaging (MRI), computer tomography (CT), magnetic resonance (MR) myelography, digital subtraction myelography (DSM), and radioisotope cisternography (RIC) may be employed, depending on the complexity of the case.

Case report: A 44-year-old male, with a history of head trauma followed by cranioplasty and post-traumatic sequelae of left facial palsy, developed an insidious onset of a slowly progressive headache and intermittent drowsiness 10 years after the injury. After a further diagnostic delay of 6 years, he presented with a persistent headache, dysphagia, and an unsteady gait. SIH was confirmed by MRI and CT myelogram. Cisternography and DSM in different positions, including lateral decubitus and prone, revealed no CSF leak, and an epidural blood patch (EDBP) was performed. After the first EDBP, he experienced significant clinical improvement, but this was transient, lasting only 20 days. Over the course of a year, three subsequent digital subtraction myelography (DSMs) failed to detect a CSF leak, and additional EDBPs were unsuccessful. On reviewing imaging, the cranioplasty site was suspected, despite no leak being demonstrated. Re-exploration revealed the dissolution of the previous graft with multiple defects, and cranioplasty reconstruction was performed. This led to a gradual resolution of symptoms over 3 months.

Discussion and Conclusions: In cases where the patient remains negative for spinal longitudinal epidural collection (SLEC), those with a history of cranial site surgical intervention should be investigated for both spinal and cranial CSF leaks. Additionally, the cranioplasty implant should be thoroughly investigated for defects and managed accordingly.

Abstract ID: 947: A Rare Case of Dry Beri Beri with Bilateral Wrist Drop

Dangeti Nityata, Balaji G, Hari Haran S, Thamil Pavai N

Government Chengalpattu Medical College, Tamil Nadu, India

Background and Aim: Thiamine deficiency can lead to peripheral neuropathy and Wernicke’s encephalopathy. While Wernicke’s encephalopathy typically presents with an acute and rapidly progressive course, peripheral neuropathy associated with thiamine deficiency manifests as chronic and slowly progressive condition. However, the occurrence of acute, rapidly progressive axonal polyneuropathy in combination with Wernicke’s encephalopathy is quite rare and unusual. Herein, we describe a patient with Wernicke’s encephalopathy who presented with acute bilateral axonal neuropathy, specifically bilateral wrist drop.

Case report: Case report of one patient. A 40-year-old old male with a history of chronic alcohol use presented in an acute confusional state, accompanied by bilateral ptosis, ophthalmoplegia, and bilateral lateral rectus paresis. He also exhibited quadriparesis with greater distal than proximal weakness (power 3/5), bilateral wrist drop, and glove-and-stocking-type sensory loss extending up to the midthigh in the lower limbs and mid-forearm in the upper limbs. Additionally, truncal ataxia was present. Nerve conduction studies revealed severe axonal neuropathy with a marked decrease in compound muscle action potential in the median, ulnar, tibial, and peroneal nerves, as well as sensory axonal neuropathy. Both cerebrospinal fluid (CSF) and magnetic resonance imaging (MRI) results were normal. A final diagnosis of Wernicke’s encephalopathy with recovered dry beriberi and acute axonal sensory motor polyneuropathy was made.

Discussion and Conclusions: Thiamine deficiency can lead to peripheral neuropathy in the form of wrist drop.

Abstract ID: 949: Eight and a Half Syndrome: An Uncommon Presentation of a Demyelinating Disease

Rajesh Singh, Anuradha Batra

Sir Ganga Ram Hospital, New Delhi, India

Background and Aim: Eight-and-a-half syndrome is a rare neuro-ophthalmological condition characterized by conjugate horizontal gaze palsy, ipsilateral internuclear ophthalmoplegia, and ipsilateral lower motor neuron-type facial palsy. This results from a lesion affecting the median longitudinal fasciculus, paramedian pontine reticular formation, and the facial nerve fascicle on the same side, at the level of dorsal pons tegmentum. This diagnosis can be easily missed as it requires a detailed ocular movement examination. It is most commonly caused by infarction or demyelinating conditions.

Case report: We herein report an interesting case of a 38-year-old lady with right-sided eight-and-a-half syndrome as the first presentation of multiple sclerosis in an Asian female. She presented to us with binocular diplopia with no diminution of vision, along with deviation of the mouth to the left side and decreases sensation on the right side of the tongue, lasting for 7 days. Assessment of extraocular muscle movement revealed complete right conjugate horizontal gaze palsy. On left gaze, there was limited adduction of the right eye, accompanied by horizontal nystagmus of the left eye on abduction. Additionally, the patient exhibited right lower motor-neuron type 7th cranial nerve palsy (CN) palsy.

Discussion : Brain magnetic resonance imaging (MRI) with T2/ fluid attenuated inversion recovery (FLAIR) contrast revealed multifocal areas of altered signal intensity in the bilateral periventricular white matter lesion, centrum semiovale, high frontoparietal lobes, left temporal lobes, left cerebral penduncle, right middle cerebellar penduncle, and along the calloso-septal interface, suggestive of demyelinating plaques. Multiple scalp nodules were also noted.

Conclusions: Brainstem lesions in multiple sclerosis (MS) commonly accounts for approximately 20% of clinically isolated syndromes; however, eight-and a- half syndrome still remains a rare first presentation. To date, only a few cases have been reported.

Abstract ID: 950: Spinocerebellar Ataxia with Axonal Neuropathy 2- A Novel Pathogenic Variant

Ankita Sharma, Anshu Rohatgi, Ratna Puri Dua, Laxmikant Ramsingh Tomar

Sir Ganga Ram Hospital, New Delhi, India

Background and Aim: Autosomal recessive spinocerebellar ataxia with axonal neuropathy-2 (SCAN2) is characterized by early-onset progressive cerebellar ataxia, polyneuropathy, and elevated levels of alpha-fetoprotein (AFP). It is also known as ataxia with oculomotor apraxia type 2 (AOA2). This condition is caused by loss-of-function mutations in the SETX gene on chromosome 9q34.1. The estimated prevalence is approximately 1 in 900000. We report a rare case of a young male who presented with pancerebellar syndrome, and upon genetic evaluation, was found to have a pathogenic variant mutation in the SETX gene.

Case report: We evaluated an 18-year-old young male patient, born out of consanguinity, who presented with complaints of imbalance while walking, frequent falls, and difficulty speaking since last 4 years. His symptoms were insidious in onset and progressive in nature. Upon examination, the patient exhibited pancerebellar signs, fine nystagmus, absent deep tendon reflexes, limb hypotonia, and near-normal cognitive function. A provisional diagnosis of Fredrich’s ataxia, followed by other autosomal recessive hereditary ataxias, was considered. He underwent routine investigations, including Vitamin B12 levels and alpha-fetoprotein testing. Nerve conduction studies and MRI brain were performed, followed by genetic analysis.

Discussion: Whole genome analysis showed a pathogenic variant in the SETX (-) gene, specifically at exon 23, variant c6941_6986del (p. Tyr2314LeufsTer16). The mutation was homozygous, confirming the diagnosis of spinocerebellar ataxia with axonal polyneuropathy 2.

Conclusions: We report a novel variant of autosomal recessive hereditary ataxia (SCAN 2).

Abstract ID: 951: Seizing Slow Stoic Man Hooked by a Bacteria: A Tail of Neuroleptospirosis

Akansha Jain, Nayana Bhuyan, Vijay Nath Mishra

Institute of Medical Sciences (IMS), Varanasi, Uttar Pradesh, India

Background and Aim: Primary neuroleptospirosis, though known, is rare, with aseptic meningitis being the most common manifestation.

Case report: A 62-year-old male presented with acute febrile illness, followed by focal seizures with impaired awareness, lacking aura and automatisms. He additionally exhibited autonomic fluctuations, rigidity, symmetrical bradykinesia, delirium, apathy, and hypersomnolence. His seizures and parkinsonian symptoms progressively worsened, rendering him completely nonambulatory by the time he reached our hospital. Prior to this, he had been admitted to other hospitals twice and treated with various antibiotics, a calcium channel blocker, and other supportive therapies. His past medical history was significant for well-controlled chronic hypertension, diabetes, and hypothyroidism, managed with regular medications. His general examination was unremarkable. His higher mental function assessment revealed a mini mental state examination (MMSE) score of 21/30 and a frontal assessment battery (FAB) score of 10/18. Central nervous system examination (CNS) examination, including assessment of cranial nerves, sensory function, cerebellar functions, and meningeal signs, was normal. The motor examination revealed bilateral cogwheeling, distal predominant fine resting, and postural tremors, normal muscle power, brisk reflexes, and mute plantar responses.

Discussion: His hematological and biochemical parameters, erythrocyte sedimentation rate (ESR), C-reactive protein (qCRP), 2D Echo, holter monitoring, autoimmune and paraneoplastic profiles, antineutrophil cytoplasmic Antibodies (ANCA), and angiotensin converting enzyme (ACE) levels were all within normal limits. Although the anti-histone antibody was positive, this finding was likely due to the use of calcium channel blockers (CCB), as he exhibited no symptoms of subacute cutaneous lupus (SCLE). Cerebrospinal fluid (CSF) analysis revealed lymphocytic predominant pleocytosis with normal glucose and protein levels, and sterile cultures. His IgM Leptospira antibody was positive, while other infectious panels were negative. Magnetic resonance imaging (MRI) showed nonspecific grade 2 Fazekas changes, multiple chronic lacunar infarcts, and ill-defined signal intensity in the substantia nigra.

Conclusions: The diagnosis was autoimmune encephalitis secondary to neuro-leptospirosis. The patient was treated with antibiotics, antiepileptics, steroids, and supportive therapy for parkinsonism; cinnarizine was discontinued. At the two-week follow-up, the patient was ambulatory and free of seizures, delirium, and bradykinesia.

Abstract ID: 952: A Study on the Spectrum of Movement Disorder in Pediatric Population

Sanchali Chakraborty, Samar Biswas

Bangur Institute of Neurosciences Kolkata, West Bengal, India

Background and Aim: The prevalence of movement disorders in children and their proportion among pediatric presentations are not well documented. The exact pathophysiology of movement disorders is not well understood; however, evidence suggests that both basal ganglia or cerebellar circuits, including parts of the thalamus and cortex, are involved in most conditions. This study aimed to enhance the understanding of the epidemiology, clinical presentation, radiological and biochemical profiles, and etiology of pediatric movement disorders as a chief complaint in children presenting to the neurology department of a tertiary teaching institute.

Methodology: This observational study was conducted in a tertiary care institution from August 2023 to May 2024. Data were collected from patients meeting the inclusion criteria through verbal questionnaires, analysis of previous medical records, bedside clinical instruments, and sending required investigations.

Results: A total of 27 patients were included in the study. With a mean age of 6.3 years and a male-to-female ratio that was nearly equal. Among them, 16 patients (59.2%) had dystonia, with common causes including cerebral palsy, Wilson’s disease, and other genetic dystonias. Other movement disorders identified included ataxia (22%, n = 6), chorea (7.4%, n = 2), tics (7.4%, n = 2), and stereotypy (3.7%, n = 1).

Conclusions: The most common type of movement disorder found was dystonia, primarily due to cerebral palsy, Wilson’s disease, and other primary dystonias. The second most common movement disorder was cerebellar ataxia, predominantly of post-infectious origin. A significant number of children exhibited more than one type of movement disorder, highlighting the need for various types of movement disorders in the same child.

Abstract ID: 953: The Shy Child Who Could Not Speak

Aniruddha Kundu, Hrishikesh Kumar

185/1 A.J.C. Bose Road, Kolkata, India

Background and Aim: Hypomyelination with atrophy of basal ganglia and cerebellum (H-ABC) is a rare leukodystrophy characterized by infantile or childhood onset of motor developmental delay, dystonia, progressive gait impairment, and deficits in speech and cognition. This condition is associated with hypomyelination and progressive atrophy of the basal ganglia and cerebellum due to a genetic mutation in the TUBB4A gene.

Case report: Herein, we present the case of a 10-year-old boy, born out of nonconsanguineous marriage with a normal perinatal history, who presented with dysarthria characterized by a whispering voice, behavioral problems, and right-hand rest and action tremor since the age of 5 years. He also exhibited progressive gait imbalance, cervical dystonia, and right upper and lower limb dystonia from 8 years of age. There was no family history of similar conditions, nor were there any reports of seizures, dysphagia, or hearing problems. On examination, he demonstrated whispering dystonic speech, slow saccades, cervical and right upper and lower limb dystonia, spasticity, hyperreflexia, and bilateral cerebellar signs.

Discussion: Serum copper and ceruloplasmin levels were normal. There was no Kayser–Fleischer (KF) ring. Magnetic resonance imaging (MRI) of the brain showed atrophy of the basal ganglia (striatum), diffuse T2-weighted hyperintensity in supratentorial white matter, and cerebellar atrophy. Whole exome sequencing revealed a heterozygous variation (c.745G>A) in exon 4 of the TUBB4A gene with autosomal dominant inheritance. Sanger sequencing of the parents’ sample did not show the presence of the same heterozygous variation in both parents.

Conclusions: Herein, we describe a case of TUBB4A gene mutation-related hypomyelination with H-ABC.

Abstract ID: 954: A Case of Postpartum Hypernatremic Encephalopathy

Anugu Rao, Hari Radhakrishna, Ranjith Gandeti

Medicover Hospitals, Hitech City, Hyderabad, Telangana, India

Background and Aim: Hypernatremia is a potentially lethal condition that can cause encephalopathy, rhabdomyolysis, and osmotic demyelination. Extrapontine myelinolysis, a fairly common metabolic disorder, is associated with neurological complications.

Case report: A 24-year-old female, Para 2, living 2 (P2L2), with no known comorbidities, presented with complaints of slurred speech and difficulty swallowing since 3 days, as well as high-grade fever and cough with yellowish expectoration for 2 days. The patient noticed difficulty walking initially, which progressed to difficulty getting up from bed and assuming a squatting posture. There was no bowel or bladder involvement, sensory deficits, or extrapyramidal symptoms. The patient underwent a lower segment caesarean section (LSCS) 10 days prior and was hospitalized for 5 days for treatment of Lower Respiratory Tract Infections (LRTI) before being discharged. She developed the above symptoms 3 days after returning home and was evaluated at an outside hospital, where serum sodium was found to be 211. She was then referred to our hospital, where sodium was measured at 198 and serum osmolality was 359 mosm/kg. Troponin was negative, and creatinine was 4 mg/dL. On examination, the patient was febrile, exhibited signs of dehydration, and was confused, obeying only a few commands. Motor power was 2/5 in the right upper limb, 1/5 in the left upper limb, and 0/5 in both lower limbs, with bilateral plantar extensor. Mild neck stiffness was present

Discussion: Magnetic resonance imaging (MRI) showed hyperintense signals at the splenium of the corpus callosum and bilateral posterior internal capsule on the diffusion-weighted imaging (DWI) sequence. Cerebrospinal fluid (CSF) analysis was normal, and ultrasonography (USG) showed normal kidneys. The patient was treated with 5% dextrose and free water at a rate of 50 ml/h.

Conclusions: Given the tradition of water deprivation in the post-partum state, when patients present with altered sensorium, hypernatremia should be considered as one of the differential diagnoses. Early identification leads to the prompt initiation of free water and dextrose treatment, resulting in symptomatic improvement and preventing lethal damage.

Abstract ID: 955: A Case of Subacute Pancerebellar Ataxia- Anti YO Positive Cerebellar Degeneration

Prasanth P, Murugan P. K., Justin C, Elangovan S

Madurai Medical College, Tamil Nadu, India

Background and Aim: Paraneoplastic cerebellar degeneration is a collection of neurological disorders resulting from tumor-induced autoimmunity against cerebellar antigens. The anti-YO antibody is considered is an important marker in this condition.

Case report: A 50-year-old female without any comorbidities presented with a subacute onset of gradually progressive neurological illness characterized by imbalance and a tendency to sway to both sides while walking, as well as imbalance while sitting. She exhibited incoordination in the upper limbs and slurred speech, with increased pauses between words and syllables, especially with longer words. There was no history of cranial nerve dysfunction, limb weakness, sensory deficits, or cognitive-behavioral abnormalities. On examination, bilateral gaze-evoked horizontal nystagmus, hypometric saccades, and broken pursuits were observed, along with scanning speech, bilateral cerebellar signs, and ataxic gait.

Discussion: Blood investigations were normal, and viral markers were negative. Thyroid stimulating hormone (TSH) levels were normal, and the auto-immune workup was negative. The paraneoplastic panel revealed a strongly positive anti-YO antibody. Magnetic resonance imaging (MRI) of the brain and nerve conduction study (NCS) were normal. A positron emission tomography- computed tomography (PET-CT) scan showed a highly metabolic nodule in the lower quadrant of the right breast.

Conclusions: In our case, the findings suggest pancerebellar degeneration due to the anti-YO antibody, with the presence of breast cancer but without evidence of a primary malignancy.

Abstract ID: 956: From Brightness to Dark: An Interesting Case of Light-Induced Amaurosis

Devavrata Sahu, Arnesh Bhattacharya, Milan Mori, Jyoti Garg, Ashish Duggal

ABVIMS and Dr RML Hospital, New Delhi, India

Background and Aim: Light-induced amaurosis is a telltale sign of internal carotid artery disease (ICA). This condition is commonly confused with photogenic epilepsy and syncope; therefore, a careful history and examination are needed to confirm the diagnosis.

Case report: An 18-year-old girl presented with a history of episodes characterized by sudden onset darkening of her bilateral field of vision, lasting for a few seconds upon exposure to bright daylight. There was no associated loss of consciousness, permanent visual deficits, or seizure-like activity. Four months prior to this presentation, she developed global aphasia with right hemiparesis. Upon examination, the upper limbs and lower limbs showed no common palpable common carotid pulses.

Discussion: Magnetic resonance imaging of the brain was suggestive of a prior left middle cerebral artery territory infarct. Magnetic resonance angiography with vessel wall imaging showed attenuation of the M1 segment of the left middle cerebral artery and the A1 segment of the right anterior cerebral artery, as well as concentric enhancement of the bilateral internal cerebral artery in the petrous and cavernous segments. A computed tomography (CT) aortogram showed diffuse circumferential wall thickening of the bilateral subclavian and common carotid arteries. The patient was diagnosed with Type 1 Takayasu’s arteritis. The patient was placed on high-dose oral steroids and methotrexate.

Conclusions: Takayasu’s arteritis is a large vessel arteritis that may rarely present with light-induced amaurosis in cases of internal carotid artery (ICA) involvement.

Abstract ID: 957: Myopathy Presenting as Initial Manifestation of Primary Hyperparathyroidism

Rajni Fulwariya

Affiliation

Background and Aim: Primary hyperparathyroidism (PHP) is most commonly diagnosed incidentally during the evaluation of hypercalcemia. In symptomatic cases, patients may have variable presentations, such as renal stones, psychiatric manifestations, lethargy, increased thirst, abdominal pain, peptic ulceration, hypertension, and bone disease. Neuromuscular complications are rarely seen.

Case report: Here, we present a case of a 14-year-old female who presented to the neurology outpatient department with a 8-month history of insidious onset, gradually progressive proximal lower motor neuron (LMN) quadriparesis, without sensory, bowel, bladder, or cranial nerve involvement. The patient was further evaluated with creatine phosphokinase (CPK) levels, nerve conduction studies, electromyography, and routine blood parameters.

Discussion: On evaluation, her CPK level was found to be normal. The nerve conduction study was normal, but electromyography suggested myopathic involvement. Routine parameters indicated elevated serum alkaline phosphatase (1036 U/L), serum parathyroid hormone (271 pg/ml), and serum calcium (11.31 mg/dl) levels, along with reduced serum phosphorous (2.13 mg/dl). Ultrasonography of the parathyroid revealed a hyperechoic lesion located caudally to the left lobe of the thyroid, with polar vascularity, suggestive of an atypical parathyroid adenoma. A Tc-99 m sestamibi scan indicated a hyperfunctioning left inferior parathyroid gland. Muscle biopsy showed evidence of muscle fiber atrophy.

Conclusions: Severe proximal myopathy is a rare complication of primary hyperparathyroidism. Prompt diagnosis and parathyroidectomy can prevent complications and improve clinical outcomes.

Abstract ID: 958: An Interesting Case of Brain Sagging Dementia Due to Bilateral VP Shunting

Pushkar Pazhani, Jayaram Saibaba, Saranya Gomathy, Manoj Manyem, Ramkumar Sugumaran, Sunil K. Narayan

Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India

Background and Aim: Intracranial hypotension typically manifests with orthostatic headaches and cranial nerve abnormalities, but it can rarely present as cognitive decline resembling frontotemporal dementia (FTD), posing a diagnostic challenge. This report aims to highlight a case of iatrogenic intracranial hypotension presenting as reversible frontotemporal dementia in a patient with a history of craniopharyngioma and ventriculoperitoneal (VP) shunting.

Case report: A 42-year-old female with a history of craniopharyngioma resection and bilateral VP shunting presented with rapidly progressive cognitive decline. Neurological examination, magnetic resonance imaging (MRI) of the brain, and cerebrospinal fluid (CSF) analysis were conducted to evaluate the cognitive symptoms. A neurosurgical consultation led to bilateral shunt ligation.

Discussion: MRI of the brain revealed a residual craniopharyngioma, intact VP shunts, and characteristic pachymeningeal enhancement indicative of intracranial hypotension. The CSF opening pressure was significantly decreased. Bilateral shunt ligation was performed, resulting in improvement of cognitive symptoms postoperatively.

Conclusions: Intracranial hypotension can masquerade as frontotemporal dementia, necessitating a high index of suspicion, particularly in patients with relevant surgical histories. This case highlights the need for comprehensive evaluation, including neuroimaging and CSF analysis, to identify reversible causes of cognitive decline. Timely intervention, such as shunt ligation, can significantly improve cognitive function, underscoring the importance of early diagnosis and management in such cases.

Abstract ID: 959: An Observational Study to Investigate the Triggers and Risk Factors of Post Stroke Recrudescence

Ranjini Metta, Salil Uppal, Ashok Uppal, Ashok Uppal, Salil Uppal

Uppal Neuro Hospital & Multispeciality Centre, Amritsar, Punjab, India

Background and Aim: Post-stroke recrudescence syndrome is a rare syndrome defined as the transient worsening of post-stroke neurological deficits or the reemergence of previous stroke-related deficits in the absence of an acute stroke. The study aims to investigate the triggers and risk factors for post stroke recrudescence (PSR).

Methodology: This observational study was conducted from July 2022 to January 2024. Both OPD and IPD patients were included. Informed consent and Institutional Ethics Committee clearance has been taken. Patients aged 18 years or older and those suspected of having an acute stroke within 72 h of symptom onset were included. Patients who did not undergo magnetic resonance imaging (MRI) after admission or who had new infarctions were excluded. Data collected included duration of stay, prognosis, comorbidities, treatment details, premorbid modified Rankin scale scores, and National institutes of health stroke scale (NIHSS) scores at admission. The criteria proposed by Topcuoglu et al. were used for the diagnosis of PSR. Statistical analysis was performed using SPSS 21.0 software, employing mean and SD or median and interquartile, as well as percentages.

Results: In this study of 26 patients with PSR, the most common triggering factor identified was infections (38.4%), with pneumonia being the major contributor. Other triggers included hyponatremia (26.9%), benzodiazepines usage (15.3%), hyperglycemia (11.5%), hypoglycemia (7.69%), and hypernatremia (7.69%).

Conclusions: This study highlights the importance of considering possible triggers such as infections, hyponatremia, benzodiazepine usage, hyperglycemia/hypoglycemia when a patient with a history of stroke presents with similar symptoms and MRI shows no new findings. When the triggering factors are addressed, the neurological impairments in patients with PSR typically resolve within a few days.

Abstract ID: 960: Seronegative Anti- D2R Basal Ganglia Autoimmune Encephalitis Post Measles Infection Presenting with Extrapyramidal Symptoms and Ideomotor Apraxia

Bharath MM, Vimal Kumar Paliwal, Vimal Kumar Paliwal

Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGI), Lucknow, Uttar Pradesh, India

Background and Aim: Measles is one of the deadliest vaccine-preventable diseases in our country, rarely resulting in severe neurological complications that can occur within days to years after the acute infection. Acute post-measles encephalitis presents weeks after the primary infection and may be caused by immune-mediated mechanisms. We hereby present the case of seronegative anti- D2R basal ganglia autoimmune encephalitis following measles infection, presenting with Parkinsonism features and bilateral upper limb apraxia.

Case report: A 7-year-old boy presented with a history of high-grade fever followed by the onset of maculopapular rashes 1 day later. The rash started from face (forehead region) and later progressed to involve the entire body, including the palms and soles. The fever and rash resolved after 4 days. Seven days later, the patient experienced a sudden onset altered behaviour characterized by irritability, agitation, and emotionally lability, with preserved consciousness. Further, he exhibited resting tremors in both upper limbs, slowness in initiating and performing voluntary movements, and slurred speech during activities.

Discussion: The autoimmune panel, including dopamine receptor basal ganglia antibodies, was negative. Serum IgM antibody for measles was positive. A diagnosis of seronegative basal ganglia encephalitis secondary to post-measles infection was made, and the patient was started on injectable methylprednisolone, followed by an oral prednisolone taper. There was almost complete recovery of symptoms related to speech and behavioral disturbances; however, residual deficits in the form of tremors were present.

Conclusions: Acute post-infectious measles encephalitis is a rare immune-mediated complication of measles infection that can present as basal ganglia encephalitis. Early diagnosis and treatment with immunosuppressive agents help prevent long-term sequelae.

Abstract ID: 961: Dorsal Thoracic Arachnoid Web- A Rare Case of Paraparesis

Noonavathu Vamshi Krishna, P. K. Murugan, C. Justin

Madurai medical College, Madurai, Tamil Nadu, India

Background and Aim: The spinal arachnoid web is a rare entity characterized by a band of arachnoid tissue extending to the pial surface, resulting in indentation of the spinal cord. The index case highlights the importance of diagnosing the arachnoid web as a correctable cause of paraparesis.

Case report: A 45-year-old male with no comorbidities presented to the OPD with a chronic neurological illness that had an insidious onset and gradually progressed over 1 year. He initially experienced numbness initially in the right lower limb, which subsequently affected the left lower limb. The patient reported difficulties with coordination, episodes of wash-basin attacks, and girdle sensation below the sternum. He exhibited spastic weakness, initially in the right lower limb followed by the left, with distal weakness greater than proximal weakness, along with truncal weakness and decreased perception of pain and touch below the sternum over the past 3 months. On examination, he demonstrated a pyramidal pattern of weakness (right > left), along with truncal muscle weakness and hyperreflexia in both lower limbs. There was a loss of pain, touch, vibration, and temperature sensation up to the D5 level. His sensory-motor function and reflexes were normal in both upper limbs with normal bladder and bowel. Blood investigations were normal. Magnetic resonance imaging (MRI) of the spine showed focal dorsal indentation of the thoracic spinal cord at the level of D4 vertebra, presented as an inverted scalpel sign, with intra-medullary T2 hyperintense signal and a syrinx in the spinal cord. The patient underwent surgery, and his symptoms improved postoperatively.

Discussion and Conclusions: Awareness of the scalpel sign is important, as it helps in prompt diagnosis and guides management through early intervention, which can revert the symptoms.

Abstract ID: 962: Hemi Ballismus – A Report of Three Cases

Saravana Sukriya S, Najiya Nageeb, Akash Jain

All India Institute of Medical Sciences (AIIMS), Raipur, Chhattisgarh, India

Background and Aim: Hemiballismus is an abnormal involuntary hyperkinetic movement disorder affecting the ipsilateral hemibody due to a lesion in the contralateral basal ganglia and its inhibitory connections. The etiology includes stroke, infections, dyselectrolytemia, and malignancy. We report three cases of hemiballismus with varied etiologies.

Case report: Case I: An apparently healthy 56-year-old female presented with an abrupt onset of abnormal involuntary movement involving the left hemibody for 2 days. Examination revealed a Glasgow coma scale (GCS) score of 15/15, with chorea-ballismus of the left hemibody. Investigations showed a fasting blood sugar level of 345 mg/dL and an HbA1c of 12.6%. Magnetic resonance imaging (MRI) of the brain revealed T1 hyperintensity involving the bilateral globus pallidus, suggestive of diabetic striatopathy. Her symptoms improved with blood sugar control and treatment with tetrabenazine. Case II: A 67-year-old male with uncontrolled diabetes presented with an acute onset of involuntary movement involving the right hemi-body for 3 days. Examination revealed a GCS score of 15/15, with choreo-ballismus of the right hemibody. Investigations showed elevated blood sugars levels of 308 mg/dL and an HbA1c of 13.1%. MRI of the brain showed T1 hyperintensity involving the bilateral globus pallidus, suggestive of diabetic striatopathy. He was started on insulin, tetrabenazine, and risperidone. Over 5 months, he developed features of parkinsonism, including bradykinesia, rigidity, and incomplete resolution of choreoballistic movements. Tetrabenazine and risperidone were gradually tapered, and he was started on levodopa-carbidopa, resulting in symptomatic improvement.

Discussion and Conclusions: Hemichorea-ballismus usually carries a good prognosis with the correction of the underlying pathology. Supportive treatment with antidopaminergic drugs, benzodiazepines, antiepileptics, and in rare cases, pallidotomy is indicated.

Abstract ID: 963: A Case Report: Coexistence of Sjogren Syndrome with Neuromyelitis Optica in a Patient Presenting with Right Incongruous Homonymous Hemianopia

Bharath MM, Vimal Kumar Paliwal

Sanjay Gandhi Post Graduate Institute of Medical Sciences (DGPGI), Lucknow, Uttar Pradesh, India

Background and Aim: Sjögren’s syndrome (SS) patients may be affected by neuromyelitis optica spectrum disorder (NMOSD), a severe demyelinating syndrome associated with anti-aquaporin 4 antibodies (anti-AQP-4 antibodies). We hereby report a case of Sjogren syndrome with neuromyelitis optica, presenting with right incongruous homonymous hemianopia

Case report: A 59-year-old right-handed female homemaker presented with sudden onset of blurring in the right half of her vision since 18 days, accompanied by colour desaturation in the involved visual field She noticed these symptoms upon waking up from sleep at night; they progressed until the next morning and later became static. She also reported a history of dryness of the mouth for the past 2 months. Additionally, she had a past medical history of hypertension for 3 years.

Discussion: On evaluation, visual acuity was 6/12 in the right eye and 6/15 in the left eye. Fundus was normal. Visual perimetry showed right incongruous homonymous hemianopia. Magnetic resonance imaging (MRI) imaging showed T2/ fluid attenuated inversion recovery (FLAIR) hyperintensity in the left optic chiasma, splenium of the corpus callosum, and parietal lobe, along with diffusion restriction. Neuromyelitis Optica (NMO) antibodies were positive, with antinuclear antibody (ANA)-4 and extractable nuclear antigen antibodies (ENA)-3+ (SS-A) also noted. Ultrasonography (USG) of the parotid glands revealed bilateral grade 3 changes and an Ill-defined hypoechoic area. The Schirmer test was positive.

Conclusions: The coexistence of Sjogren’s syndrome with neuromyelitis optica is a rare entity with varied presentation. This condition requires aggressive immunosuppressive therapy to manage its complications effectively.

Abstract ID: 964: Acute Encephalopathy with Biphasic Seizures with Late Reduction Diffusion (AESD): A Rare Para Infectious Manifestation in a Child

Prabhudev Hiremath, Mahendra V. Javali, Pradeep R, Chandrika Rao, Sathishkumar K M, Harshita Chowdary Nadella

MS Ramaiah Medical College, Bengaluru, Karnataka, India

Background and Aim: Acute encephalopathy with biphasic seizures and restricted diffusion (AESD) is an uncommon parainfectious disease in the Indian subcontinent, though it is quite common in Japan. This condition typically affects children aged 1 to 2 years. The illness follows a biphasic pattern; the first phase presents as a febrile seizure on day 1, followed by a recurrence of seizure clusters on days 4 to 6 of the illness. This study aimed to promote awareness of AESD and its management to avoid disastrous sequalae.

Case report: A fifteen-month-old male infant was referred to our hospital on day 5 of illness, initially diagnosed with viral encephalitis and treated for febrile seizures. Due to the prolonged nature of the seizures, cerebrospinal fluid (CSF) analysis and magnetic resonance imaging (MRI) of the brain were conducted, both of which were normal. The child developed clusters of generalized tonic-clonic seizure (GTCS) again on day 4 of the illness. Hence, he was referred to our casualty.

Discussion: The child was admitted to the paediatric intensive care unit (PICU) and started on appropriate antiseizure medications. An electroencephalogram (EEG) suggested bitemporal sharp waves. The seizures were successfully brought under control. A repeat magnetic resonance imaging (MRI) suggested extensive bilateral and asymmetrical subcortical diffusion weighted imaging (DWI) bright signal changes, leading to a “bright tree” appearance. In view of the biphasic illness and characteristic MRI changes, AESD was considered. Intravenous (IV) methylprednisolone was administered due to persistent lethargy and head lag, despite control of seizures. The child showed gradual improvement.

Conclusions: Early control of seizures and immunosuppression is associated with better outcomes.

Abstract ID: 965: A Novel Immune-Mediated Cause of Recurrent Cranial Neuropathies: Anti Neurofascin Antibodies (ANFA) 155/186

Pritam Majumdar, Thomas Mathew, Surabhi Garg, Sucharitha Vasu, Sindhu V. Nambiar, Shagun Bhardwaj, Sharath Kumar GG, Sai Kanth, Rahunandan Nadig, Gosala Raja KukkutaSarma

St. Johns Medical College, Bengaluru, Karnataka, India

Background and Aim: More than a quarter (26%) of recurrent cranial neuropathies are associated with autoimmune diseases. Herein, we report three cases of recurrent cranial palsies, which were negative upon extensive workups and found to be positive for anti-neurofascin antibodies.

Case report: ANFA 155, 186, and 140 were measured using the competitive enzyme linked immunosorbent assay (ELISA) technique employing human neurofascins. Case 1- A 22-year-old boy presented with left-sided painful ophthalmoplegia for 3 days and recurrent episodes of self-resolving right facial palsy for the past 3 years. Magnetic resonance imaging (MRI) Brain with contrast was normal. ANFA 186 was positive at a titer of 477 ng/ml (N < 230 ng/mL). Case 2- A 36-year-old female presented with a history of three episodes of left lower motor neuron (LMN) facial nerve palsy over the past 5 years, all self-resolving. Magnetic resonance imaging (MRI) brain showed enhancement of the canalicular and cisternal segments of the left VII nerve. ANFA 186 was positive at a titer of 605 ng/ml (N < 230 ng/mL). Case 3- A 46-year-old man presented with painful left-sided ophthalmoplegia for 4 days and two similar self-resolving episodes in the past year. MRI of the brain with contrast was normal. ANFA 155 levels were positive at 290.8 ng/mL (N <232 ng/mL). All three patients responded fully to steroids and tested negative for antinuclear antibody (ANA), antineutrophil cytoplasmic antibodies (ANCA), venereal disease research laboratory test (VDRL), human immunodeficiency virus (HIV), angiotensin converting enzyme (ACE), and IgG4.

Discussion and Conclusions: The spectrum of ANFA has expanded beyond chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to include recurrent and extensively investigated cranial neuropathies with negative workups. Future studies are needed to further explore the spectrum of ANFA further, as it may have a significant role in other autoimmune neurological diseases.

Abstract ID: 966: Young Onset Progressive Familial Pallidopyramidal Syndrome with Atypical Presentation and Unusual Etiology

Krishnapriya R

Government Mohan Kumaramangalam Medical College Salem, Tamil Nadu, India

Background and Aim: Pallidopyramidal syndrome is a rare group of familial parkinsonian disorders characterized by a combination of extrapyrmidal, pyramidal, cerebellar, or cognitive features.

Case report: A case report of a young patient with pallidopyramidal syndrome. A 32-year-old male patient presented with an insidious onset of gradually progressive slowness in activities of daily living, accompanied by difficulty walking due to unsteadiness and dysarthria. He spoke slowly with unpredictable pauses and experienced increased stiffness in neck and trunk over a 1-year duration. On examination, upranuclear vertical gaze palsy was present, along with spasticity in all four limbs, severe axial rigidity, and dystonic posturing of bilateral upper limbs. Mask-like facies and reduced blink rates were noted. Scanning dysarthria was present. All deep tendon reflexes (DTR) were brisk, with bilateral extensor plantar responses.

Discussion and Conclusions: A rare case of young-onset parkinsonism with pyramidal features, dystonia, and supranuclear gaze palsy, caused by a MAPT gene mutation.

Abstract ID: 967: Drug Induced Myoclonus- Rare Culprits

Sreenivas U Meenakshisundaram, U Meenakshisundaram, Vinoth Kumar, Ravi Kumar K

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: Drug-induced myoclonus is underrecognized as a cause of acute myoclonus. Often, metabolic encephalopathies are suspected, but a thorough review of the drug chart is always warranted.

Case report: We report for cases of acute-onset myoclonus caused by drugs. Two patients had been taking the drug for a long time, while two had taken only 1 to 2 doses.

Discussion: Each patient presented with different levels of altered sensorium, ranging from being completely oriented to stupor. The common factor was renal impairment, which was a new onset in three of the patients.

Conclusions: Awareness of drugs that can cause myoclonus is needed, especially in cases of acute myoclonus. A high degree of suspicion is also needed to identify renal impairment in these cases.

Abstract ID: 968: Task Specific Dystonia: An Interesting Case in “Nagina” Worker

Baishampayan Goswami

Sawai Man Singh Medical College, Jaipur, Rajasthan, India

Background and Aim: Dystonia is a movement disorder characterized by sustained or intermittent muscle contractions that cause abnormal, often repetitive, movements, postures, or both. Task-specific dystonias are primary focal dystonias, characterized by excessive muscle contractions that produce abnormal postures during selective motor activities, often involving highly skilled, repetitive movements. We present a case of gem (nagina) polisher dystonia, which has not been reported frequently.

Case report: A 36-year-old male gem (nagina) polisher presented with abnormal involuntary contractions of the right thumb and index finger only while polishing the naginas for the past 9 months. Initially, the symptoms were mild but gradually progressed over time. The involuntary movements were associated with tremors in the hand, but no other abnormalities were noted in other body parts or during any other tasks.

Discussion: The patient was thoroughly evaluated, and botulinum toxin was injected into the right flexor digitorum profondus (FDP), Flexor digitorum brevis (FDB), flexor carpi radialis (FCR), and extensor carpi ulnaris (ECU). The patient was followed after 2 months, and significant improvement was noticed.

Conclusions: Nagina worker’s dystonia is a task-specific dystonia that has rarely been reported. Identification of the condition is key in treating these patients.

Abstract ID: 969: Allgrove Syndrome with Amyotrophy- A Case Report

Kannan, Tino Baby, Viveka Saravanan, Sowmini Perumal, Mugundhan Krishnan

Stanley Medical College, Chennai, Tamil Nadu, India

Background and Aim: Allgrove syndrome is an autosomal recessive disease mostly caused by mutations in the AAAS gene. It has variable clinical features, but its cardinal manifestations comprise the triad of achalasia, alacrimia, and adrenal insufficiency. The symptom typically develops during the first decade of life; however, some cases have an onset in the second or third decade. We herein describe a case of a 25-year-old woman with Allgrove syndrome who has experienced progressive amyotrophy, achalasia, dry eyes, and adrenal insufficiency since childhood. Awareness of its neurological manifestations and multisystem features is essential to shorten the time for diagnosis and allow appropriate symptomatic treatment.

Case report: A 25-year-old lady, the product of a third-degree consanguineous marriage, was admitted with recurrent episodes of loose stools, vomiting, throat pain for 2 days, and generalized fatigue. Vital signs at admission were as follows = blood pressure (BP) – 70/40 mmHg, pulse rate (PR) – 120/min, respiratory rate (RR)- 16/min, and random blood sugar (RBS) -64. She was treated with IV fluids, antibiotics, and other supportive measures. She was referred for a neurology opinion due to hearing difficulties and nasal quality of speech since childhood, as well as distal muscle wasting in both hands.

Discussion: Examination revealed short stature, hyperpigmention of the oral mucosa and tongue, distal muscle wasting in both hands, tongue wasting, and fibrillation. She also reported a history of alacrimia and dysphagia. Schrimmer’s test was positive, suggestive of severe dry eye.

Conclusions: Diagnosing and timely intervention help reduce morbidity and mortality.

Abstract ID: 970: Case Report of Lower Motor Neuron Disease Presenting with Tubulin A4A Misense Mutation

Divya Bayatapalle, Balaji G

Chengalpattu Medical College, Chengalpattu, Tamil Nadu, India

Background and Aim: Amyotrophic latera sclerosis (ALS) is a neurodegenerative disease that selectively affects motor neurons in the cortex, brain stem, and spinal cord. The most common genetic mutations reported to cause ALS are SOD1, C9ORF72, TARDBP, and FUS. Several other genes associated with ALS have also been reported. Herein, we present a case report of a novel missense mutation of tubulin A4A presenting as pure motor lower motor neuron (LMN) syndrome.

Methodology: A 47-year-old gentleman with a history of diabetes presented with progressive asymmetric lower limb onset flaccid quadriparesis over the past year, accompanied by transient dysphagia. There was a significant occupational history suggestive of heavy metal exposure. Examination showed LMN signs (wasting, fasciculations, hypotonia, and global areflexia) in all four limbs, with no sensory, pyramidal, autonomic, cerebellar, or eps signs. The patient was evaluated for a pure motor syndrome of the LMN type. Complete blood count (CBC), renal function test (RFT), liver function test (LFT), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), blood sugars, and electrolytes were all normal. A creatine phosphokinase (CPK) level of >1000 was noted a year ago, but a repeat CPK was 256. Nerve conduction study (NCS) shows bilateral peroneal axonal neuropathy. EMG demonstrated a neurogenic pattern. Magnetic resonance imaging (MRI) of the brain and spine and cerebrospinal fluid (CSF) analysis were normal. Heavy metal screening revealed lead levels of 20.4. Multiplex ligation-dependent probe amplification (MLPA) for the SMN1 gene was negative, and CAG repeat analysis showed 21 repeats (not significant). Whole body positron emission tomography (PET) scan results were normal.

Discussion: The patient was suspected of having ALS of the LMN type and was sent for genetic analysis. Whole exome sequence revealed a heterogygous missense variation in exon 4 of the TUBA4A gene (p. Ala289ser)

Conclusions: We may be the first to report a case of tubulin mutation presenting as lower motor neuron disease.

Abstract ID: 971: A Rare Genetic Presentation of Juvenile Dermatomyositis

Naresh Yennam

Chalmeda Ananda Rao Institute of Medical Telangana, India

Background and Aim: Juvenile dermatomyositis (JDM) is a rare multisystemic autoimmune disease of uncertain origin, characterized by chronic inflammation affecting both the striated muscles and the skin. This condition results in rash and proximal muscle weakness.

Case report: A 13-year-old boy presented with a 3-month history of insidious onset, gradually progressive proximal weakness affecting both the upper and lower limbs, accompanied by a dusky red rash with photosensitivity over the face and extremities. Examination of the skin revealed the presence of heliotrope rash, Gottron papules, and a positive Grower’s sign.

Discussion: On evaluation, all routine investigations were normal. The test for antinuclear antibodies (ANA) was negative, while the lactate dehydrogenase (LDH) level was elevated at 633 U/L (N: 130– 240 U/L), and serum creatine phosphokinase (CPK) levels were 378 U/L. Electromyography (EMG) suggested a myopathic pattern. The myositis profile (IMMUNOBLT) showed NXP2 antibodies positive.

Conclusions: In conclusion, we have presented the case of a young boy with classical clinical findings of a rare autoimmune genetic disorder. It is importance to diagnose and initiate immunosuppressive treatment early in the clinical course of the disease to reduce the burden of long-term complications.

Abstract ID: 972: Nonaka Myopathy- Adult-Distal Onset Myopathy with Unique Topographical Weakness- A Case Report

Jatinkumar Jain, Himanshu Kaushal, Amit Agarwal, Yogesh Kaushik

Mahatma Gandhi Hospital Medical College, Jaipur, Rajasthan, India

Background and Aim: Nonaka myopathy, also known as hereditary inclusion body myopathy (HIMB) or Glucosamine (UDP-N-acetyl)-2-epimerase/N-acetyl mannosamine kinase (GNE) myopathy, is an extremely rare form of autosomal recessive myopathy characterized by adult-onset distal skeletal muscle weakness. Its prevalence is estimated to be around 1 to 9 per 1000000 individuals. A distinctive feature of this condition is the sparing of the quadriceps muscle, which is the specific topographic feature of this myopathy.

Case report: The current study is a case report of a 33-year-old male who presented with distal-onset muscle weakness characterized by sparing of the quadriceps muscle to the neurology clinic. He underwent magnetic resonance imaging (MRI) of the thigh muscles, bilateral legs, arms, and forearms. Additionally, tests for total creatine phosphokinase (CPK) levels, electromyography, nerve conduction studies, and genetic testing were performed. A 33-year-old male presented with an insidious onset and gradually progressive motor quadriparesis lasting 11 years duration, characterized by distal onset and sparing of bowel and bladder functions. On examination, he exhibited weakness in all four limbs, with notable sparing of the quadriceps muscles. Considering this sparing of quadriceps muscles, GNE myopathy was suspected. MRI of the thigh showed sparing of the quadriceps muscles, while predominantly involving the tibialis anterior muscle. Electromyography (EMG) revealed a myopathic pattern in the affected muscles, and nerve conduction study (NCS) indicated reduced compound muscle action potential (CMAP) in the tested nerves. Creatine phosphokinase - N acetyl cysteine (CPK NAC) levels were elevated at 554 U/L. Whole-exome sequencing revealed a heterozygous missense mutation in the GNE gene.

Discussion and Conclusions: The diagnosis of GNE myopathy is primarily based on clinical features, muscle pathology findings, and confirmation through the identification of mutations in the GNE gene. While there is currently no specific treatment available for this myopathy, early diagnosis is crucial as it helps in avoiding unnecessary interventions and provides valuable insights into the prognosis regarding disease progression.

Abstract ID: 973: Longitudinally Extensive Transverse Myelitis is not Always a Demyelination

Rashmi Rajur

Kasturba Medical College Hospital (KMC) Bangalore, Karnataka, India

Background and Aim: In cases of longitudinally extensive transverse myelitis, it is essential to consider alternative diagnoses beyond demyelination, such as infections or spinal arteriovenous malformations (AV) malformations. Identifying the underlying etiology early can facilitate appropriate treatment and lead to better outcomes.

Case report: A 60-year-old male, known case of hypertension and diabetes mellitus, presented with quadriparesis. The patient exhibited a sensory level at C3, bladder involvement, hypotonia, and absent reflexes, suggestive of spinal cord involvement in the shock stage. Additionally, lower cranial nerve involvement suggests high cervical cord lesion.

Discussion: Finally, after digital subtraction angiography (DSA) patient is found to have right vertebral artery to cortical vein spinal AV malformations.

Conclusions: Early suspicion, thorough workup, timely diagnosis, and effective management significantly enhance neurological outcomes in cases of spinal AV fistulas. Similar to other compressive spinal cord pathologies, such as tumors, progressive deficits can become permanent. In patients with spinal AV fistulas, postponing intervention may result in further ascending deficits, including death from an expanding AVM or possible extensive rupture.

Abstract ID: 974: Wolfram Syndrome - A Case Report

Subburaj D, Tino Baby, Sowmini Perumal, Mugundhan Krishnan

Stanley Medical College, Chennai, Tamil Nadu, India

Background and Aim: Wolfram Syndrome (WS) is a rare neurodegenerative disease with autosomal recessive inheritance. It is characterized initially by Type 1 diabetes mellitus (DM), followed by progressive optic atrophy, diabetes insipidus, hearing loss, and other neurological and endocrine dysfunctions. Bladder dysfunction, if not treated properly, can lead to end-stage renal disease, which is reported as one of the most common causes of death among patients with WS. We herein describe a case of a 17-year-old girl diagnosed with wolfram syndrome, who exhibits type 1 DM, diabetes insipidus, mild deafness, optic atrophy, and neurogenic bladder.

Case report: A 17-year-old female with a known history of Type 1 DM for 3 years of age, with good glycemic control, presented with progressive optic atrophy in both eyes and bladder disturbances for the last 3 years. Her bladder symptoms included increased frequency, urgency, urge incontinence, weak stream, incomplete emptying, stress incontinence, and nocturnal enuresis, without any dysuria. Routine blood investigations were within normal limits.

Discussion: Polyuria and polydipsia persisted despite good glycemic control. Further evaluation of polyuria revealed serum and urine osmolarity studies consistent with central diabetes insipidus. Magnetic resonance imaging (MRI) of the brain showed typical findings associated with classical wolfram syndrome. The patient was successfully discharged on nasal desmopressin and advised on self-intermittent catheterization.

Conclusions: If a patient with a known history of Type 1 DM presents with persistent polyuria or symptoms of neurogenic bladder despite maintaining good glycemic control, it is crucial to consider wolfram syndrome as a differential diagnosis, and further evaluation should be initiated to confirm this rare neurodegenerative disorder. Early recognition and timely management of this condition will help to improve the patient’s quality of life.

Abstract ID: 975: An Unusual Presentation of Central Cord Syndrome

Veerappan V S, Saravanan S

Tirunelveli Medical College, Palayamkottai, Tirunelveli, Tamil Nadu, India

Background and Aim: Both central cord syndrome (CCS) and brachial plexus injury (BPI) can lead to upper extremity weakness, and their concomitant presence can complicate the diagnostic process.

Case report: A 65-year-old male patient presented with a 1-month history of weakness in his right upper limb following a backward fall at his work place, which was not evaluated. He experienced persistent pain and gradually developed proximal weakness, severely limiting his ability to raise his arm above shoulder level. There was no associated distal weakness or sensory symptoms, and he did not report any bowel or bladder dysfunction.

Discussion: On examination, his right upper limb appeared with the arm hanging by the side, medially rotated, with the forearm extended and pronated. There was noticeable wasting of the right deltoid, supraspinatus, infraspinatus, and biceps, with occasional fasciculations noted in the deltoid muscle. All shoulder movements, as well as elbow flexion and supination, were weak on the right side. Deep tendon reflexes (DTRs) were diminished over the right biceps and supinator, while other DTR’s were brisk. Bilateral pectoral and trapezius reflexes were positive, and bilateral plantar responses were extensor. There was suspended dissociative sensory loss. A T2-weighted magnetic resonance imaging scan of the cervical spinal cord showed high-signal intensity at the C4 to C6 levels, indicating cord edema. Electromyography (EMG) showed denervation potentials in the right deltoid and biceps.

Conclusions: The correct diagnosis of brachial plexus injury can lead to early reinnervation and possibly better long-term outcome.

Abstract ID: 976: An Interesting Case of Pulseless Stroke in Young

Ram Selvaraj, Praveen Singh Peter

Government Mohan Kumaramangalam Medical College Salem, Tamil Nadu, India

Background and Aim: Takayasu’s arteritis is a chronic inflammatory condition that affects the large arteries, including the aorta and its primary branches, leading to progressive arterial occlusion. While it can result in various complications, it rarely presents with stroke as initial manifestation.

Case report: A 16-year-old girl with no significant past history presented with weakness in her left upper and lower limbs for 2 days. Her clinical history included generalized body aches, occasional episodes of arthralgia, and headaches over the past year, alongside recurrent episodes of loss of consciousness and falls occurring 2 to 3 times weekly for the last 6 months. On examination, she was conscious and oriented, but her left arm was cold and pulseless, with undetectable blood pressure in that limb, while blood pressure readings in the other limbs were detectable and normal. Neurological examination revealed normal higher mental function (HMF) and cranial nerve examination. Muscle power in the left upper limb was rated at 3/5, and in the lower limb, it was 4/5, with increased deep tendon reflex (DTR) and an extensor planter response on the left side.

Discussion: Laboratory investigations revealed anaemia and an elevated erythrocyte sedimentation rate (ESR) of 140 mm/hour, while the platelet count and other blood chemistries, along with the coagulation profile, was normal. Both antinuclear antibody (ANA) and antiphospholipid antibodies (APLA) tests were negative. Magnetic resonance imaging (MRI) of the brain with magnetic resonance angiography (MRA), and magnetic resonance venography (MRA and MRV) indicated an acute infract in the right MCA territory. MRA revealed significant narrowing of the right ICA across all intracranial segments, although the MRV was normal. Additionally, four-vessel doppler and CT aortogram showed an 80% luminal narrowing of the right CC.

Conclusions: These findings underscore the importance of thorough evaluation in young patients presenting with stroke symptoms. Early identification of this disease is important, as it allows for continuous medical management and timely interventions. Such measures can help halt inflammation and prevent progression to the permanent stenotic stage, thereby preventing end-organ damage and improving patient outcomes.

Abstract ID: 977: A Rare Case of Neuropathy Presenting with Unilateral Foot Drop

Sunil M

Chalmeda Ananda Rao Institute of Medical Telangana, India

Background and Aim : Hereditary neuropathy with liability to pressure palsies (HNPP) presents a distinct phenotype characterized by episodic, multifocal mononeuropathies, typically occurring at common sites of compression. Most cases are secondary to a dominantly inherited deletion (or) sequence variation of the peripheral myelin protein-22 (PMP22) gene located on chromosome 17p11.2, the same gene that is duplicated in Charcot-Marie-Tooth disease type 1A (CMT 1A).

Case report: A 47-year-old male presented with sudden onset difficulty in walking, as well as challenges in wearing and holding his chappal. The weakness progressed over 3 days. The patient had a notable history of trauma and experienced a right wrist drop 5 years ago, from which he recovered within 10 days. On examination, bilateral thickening of the common peroneal and ulnar nerve was noted, while higher mental functions remained normal. Motor examination revealed weakness in ankle dorsiflexion on the left side, and sensory assessment indicated decreased pain, temperature, and vibration sensations at the L5 segment on the left side.

Discussion: All routine blood investigations, cerebrospinal fluid (CSF) analysis, and imaging studies were normal, with HbA1C measured at 7%. Nerve conduction study (NCS) demonstrated demyelinating neuropathy, and a nerve biopsy revealed the presence of tomacula.

Conclusions: This case highlights a middle-aged male with diabetes presented with recurrent episodes of foot drop, emphasizing the potential for misdiagnosis or underdiagnosis of this condition. A high index of suspicion is warranted in patients with multiple episodes of compressive neuropathies, whether mono or multiple mononeuropathies, and unexplained polyneuropathy, as these clinical presentations should trigger genetic testing for HNPP.

Abstract ID: 978: A Spectrum of Congenital Pontine Anomalies

C. Leema Pauline, Jered Livingston, Mohinish S, Muhammed Shadique, Sonia Rosalind Martina, Shree Shruthi Badrinath, Neeraj Elango

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: A wide spectrum of congenital abnormalities of the pons has been demonstrated, including both malformations and disruptions. Familiarity with this spectrum and its well-defined diagnostic criteria is crucial for optimal therapy and accurate genetic counseling. Herein, we present two cases each of pontine tegmental cap dysplasia (PTCD) and horizontal gaze palsy with progressive scoliosis (HGPPS).

Case report: Spectrum 1: An 8-year-old girl presented with a restriction of eyeball movements that had been noticed since childhood, alongside an abnormal curving of her back since 4 years of age. The onset was insidious and progressive. She exhibited bilateral horizontal gaze palsy while maintaining normal vertical gaze and displayed progressive neurogenic scoliosis. Magnetic resonance imaging (MRI) findings showed the presence of a butterfly-shaped medulla with a split pons sign, indicating hypoplasia and malformation of the ventral portion of the pons and medulla oblongata. Diffusion tensor imaging further revealed the absence of decussation of the bilateral pyramidal tracts. Genetic analysis revealed a likely pathogenic homozygous mutation in exon 9 of the ROBO3 gene, confirming the diagnosis of HGPPS. The patient is currently under regular follow-up, receiving physiotherapy, and is being monitored for scoliosis. Spectrum 2: An 11-month-old baby presented with delayed milestones, specifically the inability to sit without support. The antenatal history was uneventful, and the child had normal head circumference with no neurocutaneous markers. Spastic quadriparesis was noted. MRI findings indicated hypoplastic of the left hemipons and hypolplasia of the middle cerebellar peduncle, presenting a molar tooth appearance suggestive of PTCD.

Discussion and Conclusions: PTCD is a rare congenital malformation of the brainstem and hindbrain, characterized by the imaging hallmark of an ectopic dorsal transverse pontine fiber projecting from the tegmentum into the fourth ventricle. In contrast, HGPPS, caused by mutations in the ROBO3 gene, is characterized by the congenital absence of conjugate lateral eye movements, preserved vertical gaze, progressive scoliosis, dysgenesis of brainstem structures, and ipsilateral projection of the pyramidal tract. The characteristic clinical and imaging findings, along with genetic studies, contribute to a better understanding of these conditions.

Abstract ID: 979: Unveiling Cerebral Venous Sinus Thrombosis in Young Adults: Clinical Presentation and Diagnostic Challenges

Meghraj Singh, Suryakant Singh

Institute of Post Graduate Medical Education and Research (IPGMER), Kolkata, West Bengal, India

Background and Aim: Cerebral venous sinus thrombosis (CVST) is a rare yet potentially life-threatening condition. Despite recent advances in the recognition of CVST, its diagnosis and management remain challenging due to the diverse underlying risk factors. Due to the nonspecific nature of its presentation symptoms, CVST often masquerades as other neurological disorders, which can lead to significant delays in diagnosis and treatment. This study aimed to investigate the varied clinical features and outcome of patients with CVST.

Methodology: This descriptive single-center study was conducted from June 2019 to May 2024, focusing on cases of CVST in individuals aged between 15 to 60 years. Detailed histories, demographic profiles, clinical histories, neurological examinations, neuroimaging findings, treatment plans, and responses to treatment were recorded and analyzed.

Results: A total of 30 participants were included in the study. All patients presented with headache and seizures, while additional symptoms included focal neurological deficits and altered sensorium. MRI revealed superior sagittal and transverse sinus thrombosis accompanied by focal cortical edema in most cases. The majority of patients experienced complete recovery following anticoagulant therapy.

Conclusions: CVST is a complex and challenging condition with significant implications for patient outcomes. Early recognition and prompt, targeted treatment are essential for improving prognosis and reducing the risk of complications.

Abstract ID: 980: Neuro-Ichthyosis - A Case of SLS

Kirthika Kannan, Hariharan S, Balaji Gopalan

Chengalpattu Government Medical College, Chengalpattu, Tamil Nadu, India

Background and Aim: Sjogren Larsson syndrome is a rare autosomal recessive neurocutaneous disorder caused by mutations in the ALDH3A2 gene, with a prevalence of approximately 1 in 2,50,000 individuals. Identifying associated features in patients presenting with spastic paraplegia, particularly skin changes, is crucial to diagnose the disease and predict the outcome for the case.

Case report: A 17-year-old male presented to the OPD with a history of seizures. On examination, he was found to have spastic dysarthria, spastic paraparesis, and intellectual disability. General examination revealed ichthyosis all over the body present since birth, wide-spaced teeth, and kyphoscoliosis. Magnetic resonance imaging (MRI) of the brain showed white matter changes suggestive of leukodystrophy. Genetic analysis identified a homozygous mutation in Exon-2 of the ALDH3A2+ gene, highly suggestive of Sjogren Larsson syndrome.

Discussion and Conclusions: A thorough general examination is essential in patients to help identify this condition, which aids in understanding the prognosis and guiding further management.

Abstract ID: 982: A Rare Case of Distal Myopathy

Ram Selvaraj, Sivakumar S, Thiruvarutchelvan K, Praveen Singh Peter

Government Mohan Kumaramangalam Medical College Salem, Tamil Nadu, India

Background and Aim: Nonaka distal myopathy, also known as GNE myopathy, with or without rimmed vacuoles, is a rare autosomal recessive genetic disorder characterized by predominant distal weakness and atrophy of lower limbs.

Case report: We present a case of 20-year-old male admitted to our hospital with complaints of difficulty in walking for the past 5 months, which was gradual in onset and progressive in nature. There was no reported fasciculations, cramps, pain, or myotonia. There was no involvement of higher mental function (HMF), cranial nerves, sensory pathways, or autonomic systems. The family history was not significant. On examination, the patient exhibited bilateral calf muscle wasting and distal predominant weakness, especially in the dorsiflexors, with retained reflexes and bilateral flexor plantar.

Discussion: Laboratory evaluations, including complete blood count, renal function test (RFT), liver function test (LFT), serum electrolytes, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and other blood parameters, yielded normal results. However, serum creatine phosphokinase (CPK) levels were significantly elevated at 2896 IU/L. An echocardiogram was normal. Electromyography indicated myopathic features in the tibialis anterior and calf muscles. A muscle biopsy from the affected muscles showed variations in fiber size, atrophic fibers, and fibrosis, with no significant inflammatory cells present. MRI of the bilateral thigh and leg muscles showed atrophy primarily in the anterior tibialis muscle, calf muscles, and posterior thigh muscles. Genetic analysis identified a mutation in the GNE gene (Transcript GNE NM_005476.7 variant c.1124A>C P.Lys375T) in exon 7, which is suggestive of Nonaka myopathy.

Conclusions: Several features of this disorder have not yet been fully explained, including the reasons for sparing of the quadriceps and other organs despite the widespread expression of the GNE gene, as well as a full understanding of the role of sialic acid in the disease’s pathophysiology. GNE myopathy is still likely underdiagnosed.

Abstract ID: 983: A Comparison of Cognitive Impairment and Neuropsychiatric Symptoms between APOE4 Carriers and Non-Carriers in All-Cause Dementia

Atri Chatterjee, Ankur Abhinav Arvind, Sanghamitra Laskar, Sonali Aggarwal, Sundeep Singh Saluja, Aathira NS, Arun Kumar, Nimisha, Abhay Kumar Sharma

Vardhman Mahavir Medical College (VMMC), New Delhi, India

Background and Aim: The APOE e4 allele (ApoE4) is a risk factor for Alzheimer’s disease (AD), Lewy body dementia (DLB) and vascular cognitive impairment (VCI) and is associated with faster cognitive decline and an increased burden of neuropsychiatric symptoms (NPS) in both AD and DLB. This study aimed to investigate the differences in cognitive impairment and neuropsychiatric symptoms (NPS) between ApoE4 carriers and noncarriers in patients with all-cause dementia.

Methodology: This cross-sectional, observational study was conducted at the Cognitive Neurology Clinic at VMMC, Safdarjung Hospital, and GIPMER, New Delhi. Patients underwent clinical assessment at the clinic, including the collection of a detailed history of symptoms, clinical diagnosis, and the completion of the Montreal cognitive assessment (MoCA) and the neuropsychiatric inventory questionnaire (NPI-Q), following informed consent. Blood samples from 24 patients were subjected to polymerase chain reaction- restriction fragment length polymorphism (PCR-RFLP) and genetic sequencing to detect the ApoE4 allele (rs429358) and E2 allele (rs7412). Patients who were homozygous or heterozygous for the ApoE4 allele were considered as carriers. We compared the age of onset, clinical diagnosis, MoCA scores, and NPI-Q scores between the two groups. NPI-Q scores were further grouped into sub-syndromes representing mood, psychosis, and frontal lobe dysfunction, and these were compared between the groups. Continuous variables were compared using the Mann–Whitney U test, while categorical variables were compared using the chi-square test.

Results: The study included 8 carriers and 16 non-carriers as participants. There was no significant difference in the age at the onset of symptoms or the age at presentation to the clinic. Diagnoses of Alzheimer’s disease (AD) were found in 37.5% of carriers and 31.5% of noncarriers (P > 0.05). MoCA scores were not significantly different between carriers (10.4 ± 4.1) and noncarriers (6.1 ± 5.7). Carriers and noncarriers did not demonstrate any significant difference in the total NPI-Q scores (8.7 ± 8.1 vs 11.7 ± 8.6, P = 0.28). There was no significant difference in the severity of any NPI-Q sub-syndromes.

Conclusions: ApoE4 was not associated with worse cognition or NPS burden.

Abstract ID: 984: Cluster Like Headache Following Tooth Extraction and Endodontic Procedure: An Insight into Pathophysiology

Panda Kumar, Shimpy Priya, Sourav Hazra

Institute of Human Behaviour and Allied Sciences, Delhi, India

Background and Aim: Cluster headaches, a type of trigeminal autonomic cephalalgias, are thought to involve both peripheral and central pathophysiology, leading to trigeminal autonomic symptoms and a distinctive rhythmic pattern.

Case report: We herein present a unique case of “cluster-like headache” that developed following a tooth extraction and endodontic procedure, shedding light on the potential mechanism of cluster headache. This case discussed a patient who developed cluster-like headaches following a tooth extraction. A 43-year-old male with no prior history of craniofacial pain presented with severe pain following tooth extraction. The pain originated approximately 4–5 cm lateral to his right lips and radiated from below his right ear to the right temporal region. Each episode lasted for 2–2.5 h and was associated with ipsilateral cranial autonomic features, such as eyelid edema, conjunctival redness, rhinorrhea, and lacrimation, along with severe restlessness. Initial acute attacks were treated with oxygen and zolmitriptan nasal spray. Prophylactic treatment with verapamil and melatonin showed inadequate response. He was then given greater occipital nerve block (GON block) using methylprednisolone, which led to a dramatic reduction in the frequency of his headaches.

Discussion and Conclusions: Peripheral sensitization of trigeminal afferents may lead to activation of central pathways, mimicking cluster headaches. Further research into the pathophysiology of cluster headaches is essential to improve understanding and treatment options.

Abstract ID: 985: Paradoxical Thrombotic Complication of Thrombolysis with Tenecteplase in Acute Ischemic Stroke

Kamalesh Nataraju, Archana B. Netto, Praveen Kumar Kumar S, Pramod K, Janardhan DC, Thejus B, Divyashree Katti

Bangalore Medical College and Research Institute (BMCRI), Bengaluru, Karnataka, India

Background and Aim: Thrombolysis using tissue plasminogen activator (t-PA) for acute ischemic stroke is widely accepted, though it is commonly associated with bleeding complications. However, paradoxical thrombosis is a rare and lesser-known complication. A review of the literature reveals two case reports of paradoxical thromboembolism following tenecteplase for acute ST-segment elevation myocardial infarction (STEMI), but no such complications have been reported in stroke thrombolysis.

Case report: This case study involves a 50-year-old man who experienced a stroke. A 50-year-old man with a history of diabetes mellitus and systemic hypertension presented with acute onset right hemiparesis lasting 20 min. He was conscious, oriented, and had a National Institutes of health stroke scale (NIHSS) score of 6. His blood pressure (BP) was 170/100 mmHg, and his random blood sugar (RBS) was 314 mg/dl without ketoacidosis. Magnetic resonance imaging (MRI)-brain showed an acute infarct in the left middle cerebral artery (MCA) territory, without corresponding T2 FLAIR changes. His BP was reduced to 142/92 mmHg with intravenous (IV) labetalol. The patient was given insulin for hyperglycemia and had no contraindications for thrombolysis. IV tenecteplase at a dose of 0.25 mg/kg body-weight was administered. After 30 min, the hemiparesis improved, and the NIHSS scale dropped to 3. The patient developed acute breathlessness with wheezing, initially suspected as an allergic reaction to tenecteplase. He was treated with bronchodilators, IV antihistamines, and hydrocortisone. An electrocardiogram (ECG) showed left ventricular hypertrophy (LVH) with strain, but cardiac biomarkers were normal. Within 12 h, he developed right lower limb ischemia with absent femoral and distal arterial pulses. Arterial doppler confirmed complete occlusion of the right femoral artery. A computed tomography (CT) brain scan taken 12 h post-thrombolysis showed no signs of intracerebral hemorrhage. Before intervention for limb ischemia could take place, the patient succumbed to cardiac arrhythmia and cardiogenic shock.

Discussion and Conclusions: This case represents the first reported instance of paradoxical thrombosis following thrombolysis with tenecteplase for acute ischemic stroke. The occurrence of such a complication poses a significant management challenge and can be life-threatening.

Abstract ID: 986: Young Onset Dementia- Clinical Characteristics and Etiologial Spectrum

Devlina Roy

Calcutta National Medical College & Hospital Kolkata, West Bengal, India

Background and Aim: The term “Young onset dementia” (YOD) refers to dementia with onset below the age of 65 years. While dementia is largely a disease of the elderly population, the prevalence of YOD is increasing. The differential diagnosis for YOD encompasses a wide range of disorders, including degenerative, vascular, toxic, metabolic, infectious, autoimmune, neoplastic, and traumatic causes. This study aimed to explore the clinical presentation and etiological spectrum of YOD.

Methodology: This institution-based, cross-sectional, observational study included 64 cases of YOD (age < 65 y) over a period of 12 months (October 2021–September 2022). Cognitive functions were assessed using standard tools, including the Montreal cognitive assessment, mini-mental status examination, and detailed lobar function tests. Relevant investigations were conducted as per individual clinical needs.

Results: The majority (72%) of cases had irreversible causes of dementia, with degenerative and vascular etiologies together comprising 79% of patients. Neuro-infections and inflammatory conditions accounted for 67% of the reversible causes. Alzheimer’s disease (48%) was the most prevalent degenerative cause, followed by frontotemporal dementia (41%). The most common infectious causes included tuberculosis, human immunodeficiency virus (HIV), and neurocysticercosis. Autoimmune encephalitis contributed to 33% of the reversible causes.

Conclusions: The diagnosis of dementia is profoundly impactful for both young and elderly individuals. However, early identification of the potential reversible causes is crucial, as prompt treatment can help restore cognitive functions. Recognizing potential genetic factors in inherited cases may help in family planning and prevention of future cases.

Abstract ID: 987: Rare Variant of Lateral Medullary Syndrome

Vipshyana Manwar, Kingsly Jebasing, Sethuram A, Shankar Ganesh

Lokmanya Tilak Municipal Medical College (LTMMC), Mumbai, Maharashtra, India

Background and Aim: We herein report the case of a 45-year-old right-handed male from Kanyakumari district with a history of congenital squint known hypertensive. The patient presented with complaints of giddiness, difficulty using the left upper and lower limbs, speech disturbances, sensory disturbances affecting the right half of body, persistent hiccups, and difficulty swallowing the last 4 days.

Case report: On clinical examination, the patient was conscious and oriented but exhibited slurred speech and left-sided Horners syndrome. Cranial nerve examination revealed decreased sensation over the left-side of his face (V CN), upper motor neuron-type facial weakness (VII CN), and uvula deviation to the right with sagging of the left pharyngeal arch. His palatal, pharyngeal, and gag reflexes were reduced on left side (IX & X). Motor examination showed normal tone on the right side but decreased tone on the left side, with a power of 5/5 on the right abd 4/5 on the left. Deep tendon reflexes were 2+ on the right and 1+ on the left. Sensory examination revealed decreased pain and temperature sensation on the left side of the face and right upper and lower limbs. Nystagmus was present, and cerebellar function was impaired on the left side but normal on the right. Imaging studies, including a computed tomography (CT) scan, showed periventricular hypodensity, mild corticocerebral atrophy, and an ill-defined hypodensity in the lower medulla, more prominent on the left, with no evidence of hemorrhage. Magnetic resonance imaging (MRI) brain with magnetic resonance angiography (MRA) confirmed an acute infarct in the left medulla.

Discussion: A 45-year-old right-handed male with congenital squint known hypertensive presented with acute onset left hemiparesis, left facial weakness, right hemisensory involvement, and speech disturbances. Imaging localized the lesion to the left submedullary region, affecting the corticospinal and corticobulbar tracts after decussation, indicating a vascular etiology.

Conclusions: This rare variant of Wallenberg syndrome can show clinical improvement if detected early and treated promptly.

Abstract ID: 988: Disseminated Cysticercosis with Starry Sky Appearance-A Therapeutic Dilemma”

Vishwesh Naik, Ravi Uniyal

King George’s Medical University (KGMU), Lucknow, Uttar Pradesh, India

Background and Aim: Disseminated cysticercosis is a rare and severe form of cysticercosis caused by the larval stage of Taenia solium . It typically presents with widespread cysticercal lesions throughout the body, posing significant therapeutic challenges.

Case report: Here, we present a case of a 25-year-old male with disseminated cysticercosis, manifesting with seizures and remarkable subcutaneous lesions. The patient underwent a comprehensive neurological evaluation, including detailed history-taking, physical examination, and neuroimaging studies. Magnetic resonance imaging (MRI) of the brain and whole-body imaging revealed numerous cysticercal lesions distributed extensively throughout the body. An ophthalmologic examination was conducted to assess for ocular involvement, which precluded the use of albendazole due to the risk of vision loss. Additionally, the patient presented with multiple subcutaneous lesions, including those on the conjuctiva. A biopsy of one of the subcutaneous lesions was performed to confirm the diagnosis by revealing the characteristic scolex morphology.

Discussion: MRI findings demonstrated a “starry sky” appearance, consistent with disseminated cysticercosis, indicative of the high lesion burden throughout the neuroaxis. Whole-body imaging revealed multifocal involvement, including subcutaneous tissues, which corrobated the clinical suspicion. The patient was treated with oral steroids and antiepileptics, leading to symptomatic improvement. He became seizure-free and was subsequently discharged for follow-up on an OPD basis.

Conclusions: The treatment of disseminated neurocysticercosis remains unclear, especially when lesion burden is high. Given that this infection can be prevented and treated if detected early, further research in this direction is required in our country.

Abstract ID: 989: Clinical Spectrum of MOGAD in a Teritiary Care Centre

Chakkera Priyanka, Vivek V. Iyer, Shubha Subramanian, Rithesh R Nair

National Institute of Mental Health and Neuro-Sciences (NIMHANS), Bengaluru, Karnataka, India

Background and Aim: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has recently been emerged as a distinct condition separate from aquaporin-4 antibody disease (AQP4-Ab) and multiple sclerosis (MS). The clinical phenotype of this disease has expanded with the advent of highly specific cell-based assays. We herein report the clinical presentations of fourteen myelin oligodendrocyte glycoprotein antibody (MOG-Ab) positive patients and summarize their clinical outcome.

Methodology: We retrospectively reviewed the records of patients diagnosed with positive MOGAb presented to a tertiary care hospital from 2020 to 2024.

Results: Fourteen patients were identified, with ten (71%) being female. The median age of onset was 31 years (range, 6–59 years), with (28.5%) of the patients being children. A monophasic course was noted in twelve (85.7%) patients, while one patient experienced seven relapse events. Initial presentations included optic neuritis in eight (57%) patients, acute disseminated encephalomyelitis (ADEM) in three (21.4%) predominantly in children, and one case of myelitis, cortical encephalitis, and brainstem encephalitis. One patient tested positive for both aquaporin-4 (AQP4) and MOG antibodies. For long-term immunosuppression, five (35.7%) patients were treated with rituximab, and eight (57.1%) received mycophenolate mofetil. Two patients were lost to follow-up. Notably, significant improvement in clinical outcomes was observed in 11 patients during follow-up.

Conclusions: Our study demonstrates that the variable clinical presentations of MOG-Ab-positive cases. Distinguishing this disease from other demyelinating disorders based on clinical and radiological features can be challenging. We recommend MOG-Ab testing for all patients with suggestive demyelinating events, although the possibility of false-positive tests should not be ignored.

Abstract ID: 990: Mystery Behind the Curls

Jitendra Kumar, Thomas Edwin Raj, B. Kannan, C. Rachel, Sravya Ravi

Thoothukudi Government Medical College Tamil Nadu, India

Background and Aim: Peculiar hair patterns in children can often indicate an underlying disease or syndrome, many of which are associated with neurological manifestations.

Case report: We present two pediatric patients born to a third-degree consanguineous couple. Both children were delivered normally and had normal perinatal histories and developmental milestones. They presented with gradual onset of progressive, flaccid, areflexic weakness in both lower limbs.

Discussion: Genetic testing revealed mutations in the GAN gene in both patients. Clinical features included peripheral neuropathy, characteristic hair patterns, and cerebellar dysfunction. Electrophysiological and radiological finding were suggestive of giant axonal neuropathy (GAN). Magnetic resonance imaging (MRI) of the brain indicated metachromatic leukodystrophy, and nerve biopsy of the right sural nerve confirmed giant axonal neuropathy. The genetic analysis indicated an autosomal recessive inheritance pattern associated with the GAN gene.

Conclusions: GAN is a clinically and radiologically heterogenous disorder. Genetic testing is necessary for accurate diagnosis and for providing appropriate genetic counselling.

Abstract ID: 991: Epileptic Encephalopathy with PRRT2 Mutation- A Case Report

Arghabrata Singha, Sandip Pal

Kolkata Medical College, Kolkata, West Bengal, India

Background and Aim: PRRT2 variants have been associated with a heterogeneous clinical spectrum, particularly in benign familial infantile epilepsy (BFIE). However, severe epilepsy syndromes have also been described in patients carrying PRRT2 mutations, and no specific genotype-phenotype correlation has yet been established.

Case report: Herein, we describe two brothers with epileptic encephalopathy who share the same PRRT2 variant but exhibit different epileptic phenotypes. A detailed history, general and neurological examination, biochemical parameters electrophysiological studies, and whole exome sequencing were conducted on the two patients admitted to the Neurology Ward at Medical college and hospital, Kolkata.

Discussion: Both brothers had normal birth and developmental histories without any positive family history. They experienced seizure onset in childhood, characterized by different semiologies and Electroencephalogram (EEG) changes. Over times, both siblings developed cognitive decline and behavioral changes, along with an increase in frequency of seizure. Neuroimaging in both siblings were normal, and whole exome sequencing revealed a pathogenic PRRT2 mutation in both cases.

Conclusions: PRRT2 mutations can be associated with several epileptic phenotypes, ranging from benign forms to more severe epileptic encephalopathies. Identifying PRRT2 variants in epilepsy patients may help achieve more personalized treatment approaches; however, establishing phenotype-genotype correlations remain a challenge.

Abstract ID: 992: Optochiasmatic Arachnoiditis- A Devastating Complication of TB Meningitis

Jyothi Valluru, Balaji G, Hariharan S, Thamil Pavai N

Chengalpattu Government Medical College, Chengalpattu, Tamil Nadu, India

Background and Aim: Tuberculous meningitis (TBM) poses significant diagnostic and therapeutic challenges and is associated with a high mortality rate. TBM predominantly affects the basal parts of the brain. A rare complication of TMB is optochiasmatic arachnoiditis (OCA), which involves inflammation of the arachnoid matter surrounding the optic nerve and chiasmatic cisterns. OCA is infrequently encountered and may arise as a paradoxical reaction during anti-TB treatment. It is hypothesized that this occurs due to a massive release of mycobacterial proteins upon treatment, provoking an intense delayed -type hypersensitivity reaction and leading to the development of the characteristic lesions of OCA.

Case report: A 22-year-old female patient presented with a 2-month history of fever, neck stiffness, and vomiting, accompanied by frequent headaches and constitutional symptoms. She was admitted to the medical ward, where cerebrospinal fluid (CSF) studies showed a neutrophilic picture with elevated protein levels and a significant sugar drop in glucose (21% of venous blood sugar). A clinical diagnosis of TB meningitis was made, and treatment with standard anti-TB chemotherapy and IV dexametnasone was commenced.

Discussion: One month later, the patient reported persistent headaches and deteriorating visual acuity in her right eye, along with a temporal field defect in his left eye. At the time of admission, vision in her right eye was limited to PL only. Magnetic resonance imaging (MRI) of the brain showed a large cluster of ring-enhancing lesions in the suprasellar region, compressing the optic chiasm, which was compatible with the diagnosis of OCA. Given the suspicion of a paradoxical reaction, the patient was treated with a pulse dose of IV methyl prednisolone (500 mg) for 5 days, resulting in an improvement of her vision to 20/200 and subsequently to 6/9 at discharge. Multidisciplinary team discussions lead to the consideration of a second-line antituberculous treatment (ATT) regimen, which was initiated alongside prednisolone. The patient is currently under follow-up care.

Conclusions: This case highlights OCA as a complication of TBM and underscores the therapeutic challenges associated with its management.

Abstract ID: 993: Navigating the Uncharted: Cerebellitis as a Rare Manifestation of Hepatitis E Infection

Panda Kumar, Harsha Burgula, Aparna Thomas, Suman Kushwaha, Aldrin Anthony Dug Dung

Institute of Human Behaviour and Allied Sciences, Delhi, India

Background and Aim: Hepatitis E Virus (HEV) infection is typically associated with acute hepatitis; however, its neurological manifestations are rare. Cerebellitis is an exceptionally uncommon complication of HEV Infection, with very few reported cases in the literature.

Case report: A Case Study of HEV-associated cerebellitis. We present a case of a 27-year-old female with no prior comorbidities who initially experienced fever for 15 days, along with acute gastroenteritis for 10 days. Seven days later, she developed symptoms of ataxia and dysarthria. On examination, the patient exhibited nystagmus, ataxia- swaying to both sides, bilateral upper limb hypermetria, and scanning speech. Initial laboratory reports were normal, except for transaminitis and thrombocytopenia. A cerebrospinal fluid (CSF) analysis showed mild pleocytosis with lymphocytic predominance and elevated protein levels. Electrophysiological studies and neuroimaging were normal. Given the context of transaminitis and enteritis, hepatis E serology was conducted, which showed a strong positive result for anti HEV IgM. Other infectious and inflammatory causes were ruled out. The patient was managed conservatively with symptomatic and supportive treatment. Patient improved clinically with symptomatic treatment, alongside recovery in both thrombocytopenia and transaminitis.

Discussion and Conclusions: Increased awareness among healthcare providers regarding this atypical presentation is crucial for early diagnosis and appropriate management to improve patient outcomes.

Abstract ID: 994: Postpartum LETM - A Case of MOGAD

M Abirami, Hariharan S, Balaji G

Chengalpattu Government Medical College Tamil Nadu, India

Background and Aim: Longitudinally extensive transverse myelitis (LETM) is defined by a spinal magnetic resonance imaging (MRI) showing a lesion extending over three or more vertebral segments. The clinical course of LETM is characterized by one or more episodes of paraparesis or tetraparesis, sensory deficits, and bowel/bladder disturbances, and in severe cases, it can lead to respiratory failure. Early distinction of possible LETM etiologies is crucial for prognosis and guiding appropriate therapeutic strategies.

Case report: Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is identified as a rare etiology of postpartum LETM

Discussion and Conclusions: After delivery, pregnancy-related hormones decrease sharply, and previously inhibited immune response reactivates, potentially leading to a relapse of previous inflammatory demyelinating disorders (IDD) or the onset of new demyelinating disease. Among postnatal demyelinating diseases, MOGAD is rarely reported compared to multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD).

Abstract ID: 995: Navigating Paroxysmal Dyskinesias: A Tale of Two Patients

Caroline Silvia, Robert Wilson, Arunan Subbiah, Kalpana R, Elanthirayan G C

SRM Medical College Hospital and Research Center, Kattankulathur, Tamil Nadu, India

Background and Aim: Paroxysmal dyskinesias (PDs) are a rare group of hyperkinetic movement disorders characterized by their episodic nature. These episodes may be caused by genetic mutations (autosomal dominant), idiopathic conditions, or structural abnormalities. Diagnosis may be missed unless the episodes are witnessed or video-recorded.

Case report: Case 1: A 15-year-old male, firstborn and with a history of age-appropriate milestones, began experiencing abnormal movements at the age of 12 years. These movements were dystonic and choreic, involving all four limbs. The episodes lasted for less than 10 s without loss of tone, posture, or consciousness and occurred approximately 5-6 times a day. He was started on phenytoin and showed significant improvement. Blood investigations and magnetic resonance imaging (MRI) of the brain were unremarkable. Whole exome sequencing (WES) revealed a PRRT2 (+) mutation on exon 2, a pathogenic variant suggestive of episodic kinesigenic dyskinesia-1. Case 2 : A 16-year-old female presented with a history of abnormal movements affecting the right upper and lower limbs for the last 3 years. She experienced sudden, hemiballistic, semi-choreic movements that lasted for less than 15 s, occurring approximately once a month and triggered by emotional stress. Her metabolic workup and neuroimaging were unremarkable. She was started on phenytoin and carbamazepine, which showed a positive response. WES revealed an additional variant RYR1 (+) – not matching the phenotype.

Discussion and Conclusions: Paroxysmal dyskinesias are characterized by intermittent episodes of involuntary movements with varied etiologies. Diagnosis can be challenging if the episodes are not witnessed. However, patients typically show improvement with anti-epileptic medications.

Abstract ID: 996: A Rare Case of Stroke Causing Abdominal Wall Epilepsia Partialis Continua

Alex Rebello, Vikram Damaraju, Mohd Asif

All India Institute of Medical Sciences (AIIMS), Mangalagiri, Andhra Pradesh, India

Background and Aim: Epilepsia partialis continua (EPC) is a rare form of focal motor status epilepticus characterized by prolonged, repetitive episodes of muscle jerks of varying rate, rhythm, intensity, and territorial extent. It usually affects the facial and/or distal limb muscles. Isolated abdominal EPC, without the involvement of the face or limbs, is rare. We herein describe a case of abdominal wall EPC caused by a stroke.

Case report: This report presents a case of abdominal wall EPC caused by a stroke . A 48-year-old male with a history of diabetes and hypertension presented to the Emergency room with continuous, focal jerky movements of the right abdominal wall for three days. He had experienced right-sided hemiparesis nine months earlier, which has resolved spontaneously.

Discussion: A noncontrast computed tomography (CT) of the head showed left cortical infarcts, while an magnetic resonance imaging (MRI) demonstrated areas of restricted diffusion in left high frontoparietal lobe, left perisylvian frontotemporal cortex, bilateral higher frontal parafalcine cortex, and the right anterior frontal parafalcine regions. A CT angiogram of the head and neck was normal. Echocardiography revealed a left ventricular thrombus. His electroencephalogram (EEG) was unremarkable. The patient was treated with benzodiazepine and phenytoin, after which the movements subsided.

Conclusions: EPC of the abdominal wall is an uncommon phenomenon. This case report highlights a rare instance of abdominal wall EPC as a manifestation of stroke.

Abstract ID: 997: Discovering Unconventional Risk Factors and Clinical Manifestations of Cryptococcal Meningitis

Japleen Kaur

Dayanand Medical College (DMC), Ludhiana, Punjab, India

Background and Aim: Cryptococcal meningitis is a severe and potentially fatal fungal infection caused by the invasive Cryptococcus species. Prompt diagnosis and treatment are critical to avert significant neurological damage and improve patient outcomes. While this infection traditionally affects severely immunocompromised individuals, particularly those with advanced human immunodeficiency virus/ acquired immunodeficiency syndrome (HIV/AIDS), our study indicates that cryptococcal meningitis can also occur in individuals without evident immunodeficiency, underscoring the need for heightened clinical vigilance across a broader spectrum of patients.

Methodology: A comprehensive retrospective study was conducted on patients admitted to Dayanand Medical College (DMC), Ludhiana, from January 2023 to May 2024. Fifteen patients were diagnosed with cryptococcal meningitis, confirmed using at least one of the following diagnostic methods: India ink staining, cryptococcal antigen detection, or cerebrospinal fluid (CSF) culture. This retrospective analysis examined risk factors, clinical manifestations, radiological features, and other CSF parameters, including cytology, biochemistry, and cartridge- based nucleic acid amplification test (CBNAAT). Additionally, therapeutic interventions and prognostic outcomes associated were reviewed. The aim of the study was to enhance the understanding of cryptococcal meningitis, promote early diagnosis in both immunocompromised and immunocompetent individuals, and inform better clinical management strategies for affected patients.

Results: Out of the 15 patients diagnosed with cryptococcal meningitis, 5 were HIV-negative. Among these, one had a low CD4 count, two had decompensated liver cirrhosis, and two were initially being treated for tuberculosis (TB) meningitis but were retrospectively diagnosed with cryptococcal meningitis based on magnetic resonance imaging (MRI) findings that revealed cryptococcomas. The clinical manifestations, radiological features, and CSF parameters varied across patients, but timely diagnosis and intervention played a crucial role in managing the disease effectively.

Conclusions: Cryptococcal meningitis can affect individuals without evident immunodeficiency, necessitating broader clinical vigilance. Early recognizing and prompt treatment of this infection is essential for improving patient outcomes.

Abstract ID: 999: Involuntary Autonomy: A Case of Frontal Alien Limb Syndrome in Glioma

Panda Kumar, Suman Kumar Sahu, Harsha Burgula, Suman Kushwaha, Aldrin Anthony

Institute of Human Behaviour and Allied Sciences, Delhi, India

Background and Aim: Alien limb syndrome (ALS) is a rare neurological disorder characterized by involuntary, simple, or complex semi-purposeful upper limb movements and a sense of foreignness. There are three types of ALS: frontal, callosal, and posterior. We herein report a case of frontal ALS in associated with glioma.

Case report: We present a case of frontal alien limb syndrome in a patient with glioma. A 60-year-old male presented with an inability to use his left hand and leg for the past 20 days. The onset was acute and non-progressive, with involuntary grasping of objects by his left hand and an inability to release the grasp independently. He had a history of seizure activity 2 months ago. On examination, his power, sensory system, and cerebellar function were normal. However, he exhibited reflexive automatic grasping and groping with the left hand, impaired bimanual coordination, and ideomotor apraxia. Intermanual conflict, mirror movement, and diagnostic dyspraxia were not observed. The phenomenon was attributed to left frontal alien limb syndrome. Magnetic resonance imaging (MRI) of the brain demonstrated a 4.4 × 3.3 cm lesion in the right frontal lobe with surrounding edema. Magnetic resonance spectroscopy (MRS) showed an increased Choline: N-Acetyl Aspartate (NAA) ratio, and FDG PET scan indicated a maximum SUV of 4.5, suggestive of glioma.

Discussion and Conclusions: Familial amyotrophic lateral sclerosis (FALS) can be an early and challenging presentation in patients. This case highlights the necessity for a thorough neurological evaluation and imaging in patients with atypical involuntary movements.

Abstract ID: 1000: Anti-Neurofascin Autoimmune Nodopathies in CIDP/GBS

Arjun Jayaprakash, Raghunandan Nadig, Thomas Mathew, G. R. K. Sarma

St. Johns Medical College, Bengaluru, Karnataka, India

Background and Aim: Autoimmune nodopathies (AN), first described in the acute motor axonal neuropathy (AMAN) variant of Guillain–Barré syndrome (GBS), have now been distinguished from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and are recognized as a distinct entity. This classification transcends traditional dichotomies such as acute vs chronic presentation, peripheral vs central demyelination, and axonal vs demyelinating electrophysiology. The absence of overt inflammation and macrophage-mediated demyelination along with a favorable response to rituximab compared to IvIg underscores the pathophysiological and practical relevance of this categorization. In AN, antibodies target node and paranode proteins, including NF155, nodal neurofascin (186 and 140), contactin-1, and Caspr-1, which are critical for maintaining the integrity of nodal architecture. This study aimed to demonstrate the need of considering ANs as a differential diagnosis in clinical cases of GBS and CIDP.

Case report: We described the features of two cases of AN with anti-neurofascin antibodies, confirmed by enzyme linked immunosorbent assay (ELISA). Case 1: A 58-year-old female presented with 8 months of pain and burning paresthesias distally in the lower limbs, which progressed to imbalance over the next 3 months, followed by distal-proximal upper limb weakness with tremors. Neurological examination revealed hyporeflexic proximal-distal weakness, predominantly in the lower limbs, postural tremor, and sensory ataxia. Nerve conduction study (NCS) indicated a sensorimotor demyelinating neuropathy with axonal features, showing conduction blocks and prolonged F-waves. She tested positive for anti-NF155 and anti-NF186 and was subsequently started on rituximab. Case 2: A 18-old-year female presented with acute onset of tingling paresthesias in the distal upper and lower limbs, which progressed over days to distal-proximal hyporeflexic quadriparesis. There was significant distal sensory loss and sensory ataxia. NCS indicated severe sensorimotor demyelinating neuropathy, suggesting GBS. However, she tested positive for anti-NF155 and anti-NF140 antibodies and negative for anti-ganglioside antibodies.

Discussion and Conclusions: Routine testing for AN may facilitate the timely selection of appropriate therapy.

Abstract ID: 1001: Basilar Artery Fenestration: An Unusual Possible Cause of Ischemic Stroke?

Kuldeep Nehra

SMS Hospital,

Background and Aim: Basilar artery fenestration is an uncommon congenital dysplasia and may be associated with ischemic stroke. Cases of young patients with basilar artery fenestration who suffered acute infarction of posterior circulation are rare and not widely reported.

Case report: A previously healthy 36-year-old male with no addiction and without a family history of stroke presented with a one-day history of vertigo, vomiting, and gait ataxia. At one month after discharge, there was no vertigo and unsteady gait.

Discussion and Conclusions: In the absence of traditional risk factors and other evidence of cryptogenic stroke, cerebral infarction in the blood supply area of fenestration malformation can be considered related to fenestration malformation, but its pathogenesis is not fully understood.

Abstract ID: 1002: Neuroferritinopathy – A Rare Case Report

Ruksana Sheik

Chengalpattu Medical College, Tamil Nadu, India

Background and Aim: Neuroferritinopathy is a rare, late-onset, autosomal dominantly inherited movement disorder caused by mutations in the l-ferritin gene. It is characterized by iron and ferritin aggregate accumulation in the brain, with normal or low serum ferritin levels, and high variable clinical feature is a rare monogenic autosomal-dominant disease caused by mutations in the gene encoding the ferritin l-chain (FTL1), one of the two subunits of the main iron storage protein.

Case Report: Genetic testing revealed a frameshift mutation in exon 4 of the FTL gene, for NBIA-3, an autosomal-dominant form.

Discussion and Conclusions: The total number of reported cases is less than 100 worldwide. In India, only a few cases have been reported to date, and 90% of the cases are due to frameshift mutations of the FTL gene. So, it is a rare case, and hence, we report this case.

Abstract ID: 1003: MTHFR Gene Mutation Presenting with Refractory Seizures

Janki Makani, Deepika Joshi, Anand Kumar

Banaras Hindu University (BHU), Varanasi, Uttar Pradesh, India

Background and Aim: Epilepsy is one of the common presentations in methylenetetrahydrofolate reductase (MTHFR) mutation, and metabolic therapy is found to reduce the seizure frequency in these patients.

Case report: This 17-year-old male patient presented with left frontal drug-resistant seizures. Considering the delayed developmental milestones and pure vegetarian diet, he was evaluated to look for treatable conditions. His mean corpuscular volume (MCV) level was also 100. His serum vitamin B12 level was sent which was 215 pg/mL. His serum homocysteine level was sent which was elevated 70 mmol/L. So, his MTHFR gene mutation polymerase chain reaction (PCR) was sent which was suggestive of a homozygous mutation of A1298C. He was treated with adequate vitamin B12 and folate supplements after which the seizures were adequately controlled.

Discussion: The patient was also having breakthrough seizures during hospitalization. His electroencephalogram (EEG) was conducted, showing a left temporal spike and wave discharge with phase reversal in T5 lead. His magnetic resonance imaging (MRI) brain was normal. His autoimmune encephalitis panel was also sent which was negative. He was treated with injectable vitamin B12 supplements with folate. He responded to the regimen, and it was possible to taper the dose of anti-seizure medication later on.

Conclusions: MTHFR mutation is a treatable entity to prevent fatal conditions with drug-refractory seizures. If suspected after other systemic and neurological manifestations, it can be easily controlled with metabolic therapy and minimal anti-seizure medications.

Abstract ID: 1004: Cognitive Conundrum: The Unexpected Association Between Idiopathic Normal Pressure Hydrocephalus and Behavioral Variant Frontotemporal Dementia

Siddharth Maheshwari, Rashmi Mishra, Aabhasha Parotra, Mridula Singh Rustagi, Aabhasha Parotra, Rashmi Mishra, Mridula Singh Rustagi

Institute of Human Behaviour and Allied Sciences, Delhi, India

Background and Aim: Idiopathic normal pressure hydrocephalus (iNPH) is characterized by symptoms such as gait disturbances, cognitive impairment, and urinary incontinence. While Alzheimer’s disease (AD) and vascular dementia are commonly associated with iNPH, there has been limited exploration of its potential link with frontotemporal dementia (FTD). Behavioral variant frontotemporal dementia (bvFTD) manifests with significant changes in personality and behavior, executive dysfunction, and extrapyramidal symptoms. There are limited case reports that discuss patients diagnosed with concomitant iNPH and bvFTD.

Case report: We present the case of an elderly woman with gait disturbances, cognitive decline, and ventriculomegaly on brain imaging, leading to a diagnosis of iNPH–BvFTD. A 63-year-old female presented with visual hallucinations, followed by progressive gait dysfunction, apathy, hyperorality, perseverations, utilization behavior, and social disinhibition over three years. On examination, her mini mental state examination (MMSE) was 2/30. She exhibited a wide-based magnetic gait, small steps, difficulty turning, postural instability, frontal release signs, mild rigidity, and bradykinesia. Neuroimaging revealed diffuse cerebral atrophy with disproportionate ventricular dilatation (Evans index of 0.45) and enlargement of the Sylvian fissure. An FDG PET scan showed severe hypometabolism in the bilateral frontal and temporal lobes, anterior and posterior cingulate cortex, and bilateral putamen. A lumbar tap test demonstrated a 20% improvement in her motor symptoms, leading to the decision to proceed with ventriculoperitoneal shunt placement. The clinical and radiological findings supported the diagnosis of bvFTD with concomitant iNPH.

Discussion and Conclusions: The overlapping clinical features between iNPH and bvFTD, along with shared radiological findings, emphasize the need to consider the possibility of dual pathology in clinical practice.

Abstract ID: 1005: A Rare Presentation of Subacute Sclerosing Panencephalitis (SSPE)

N. Mahesh

Kanchi Kamakoti CHILDS Trust Hospital Chennai, Tamil Nadu, India

Background and Aim: To share a rare presentation of subacute sclerosing pan encephalitis.

Case report: A 10-year-old boy presented with h/o fever, encephalopathy, and dystonia. The child had autonomic instability, hyperpyrexia, and raised creatine phosphokinase (CPK). A possibility of neuroleptic malignant syndrome was suspected; However, there was no h/o antipsychotic intake.

Discussion: Detailed ophthalmological evaluation provided us a clue to the diagnosis of subacute sclerosing panencephalitis (SSPE). Cerebrospinal fluid (CSF) antimeasles antibodies were strongly positive.

Conclusions: SSPE presenting as neuroleptic malignant syndrome is very rare, and only very few cases have been reported.

Abstract ID: 1007: Unusual Presentation of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD)

Bala Pradeep Boyidapu, Chintada Mounika

Visakha Institute of Medical Sciences

Background and Aim: Myelin oligodendrocyte glycoprotein-associated disease (MOGAD) is a rare, antibody-mediated inflammatory disorder of the central nervous system (CNS) with various phenotypes from optic neuritis, transverse myelitis, acute disseminated encephalomyelitis (ADEM), and encephalitis.

Case report: A 22-year-old gentleman presented with a subacute onset of gradually progressive headache, blurring of vision for two weeks, with right hemiparesis, hemisensory loss, unsteadiness while walking, hiccups, and memory disturbances. On examination, the patient was conscious and oriented, with recent memory impaired; immediate and remote memory was intact, and other higher mental functions were normal. Pupils were 3 mm b/l RTL; extraocular muscle (EOM) was full in all directions. Fundus was normal. The vision was 6/12 in the right eye and 6/9 in the left eye, with bitemporal hemianopia. Power in the right upper limb (UL) was 4/5 and 3/5 in the lower limb (LL). Deep tendon reflexes were exaggerated on the right side, with plantar extensor b/l. Sensory decreased touch, pain, and joint position sense (JPS) impaired on the right side. Rhomberg’s positive, erythrocyte sedimentation rate (ESR) 22, Mountox test positive, chest X-ray normal, and other routine blood investigations were normal. Cerebrospinal fluid (CSF) analysis showed protein at 754 mg/L, glucose at 59 mg/dL, cells 7, acid-fast bacillus (AFB) negative, oligoclonal bands (OCB), neuromyelitis optica (NMO) negative, and myelin oligodendrocyte glycoprotein (MOG) positive. Magnetic resonance imaging (MRI) of the brain revealed extensive patchy FLAIR hyperintensity along the genu of the corpus callosum, right internal capsule, posterior limb of the left internal capsule, left thalamus, left posterior temporal periventricular deep white matter, and occipital horn of the left lateral ventricle, with contrast enhancement s/o granulomatous disease or neuromyelitis optica spectrum. Magnetic resonance spectroscopy (MRS) showed inverted lipid lactate peaks s/o necrosis. MRI of the spine showed a small focus of cord hyperintensity at the c3 vertebral level, anterior aspect b/l, s/o demyelination. The patient was treated with IV methyprednisolone, followed by immunosuppression with rituximab. His vision, weakness, sensory disturbances, and gait improved.

Discussion and Conclusions: Hence, it is necessary to exclude tuberculosis in all cases presenting with the above clinical features, as early intervention and treatment can prevent complications and long-term sequelae.

Abstract ID: 1008: A Rare Case of Quadriparesis

Rahul Gunde

Vydehi Institute of Medical Sciences and Research Centre (VIMS), Bengaluru, Karnataka, India

Background and Aim: Gitelman syndrome (GS) is a rare renal disorder resulting from a mutation in the SCL12A3 gene that encodes the thiazide-sensitive sodium chloride cotransporter in the distal convoluted tubule. It is characterized by hypokalemia, hypomagnesemia, hypocalciuria, metabolic alkalosis, and normal blood pressure. Many symptoms of this rare renal disorder are related to these electrolyte abnormalities and adversely affect the patient’s quality of life. Moreover, rarely patients with GS may present with hypokalemic paralysis, mimicking Guillain–Barré syndrome (GBS), and cause diagnostic and therapeutic delay.

Case report: A 59-year-old male patient presented with paresthesias of four limbs for one month with progressive weakness of four limbs in an asymmetrical fashion and proximal more than distal weakness over eight days and without sphincter involvement, with recurrent spasms of limbs. The patient was admitted in the hospital in view of progressive asymmetrical weakness of all four limbs with suspicion of demyelinating polyneuropathy. He did not have any respiratory insufficiency or any bulbar symptoms.

Discussion: Acute neuromuscular paralysis is one of the common neurological emergencies. Guillain–Barré syndrome (GBS) and hypokalemic paralysis are the most common differentials of acute flaccid quadriparesis, and therapeutic interventions greatly differ in both electrolyte disturbances are not a part of the syndrome; however, autonomic dysfunction in GBS can lead to cardiac disturbances that may be aggravated by the coexistence of dyselectrolytemia such as hypokalemia and hypomagnesemia. This can be perplexing and pose diagnostic as well as therapeutic challenges.

Conclusions: Gitelman’s syndrome is an autosomal recessive tubular disorder caused by mutations of the genes encoding the sodium, chloride, and magnesium carriers in the apical membrane of the distal convoluted tubule (DCT). Guillain–Barré syndrome (GBS) and hypokalemic paralysis are the most common differentials of acute flaccid quadriparesis and therapeutic interventions greatly differ in both.

Abstract ID: 1009: DOK-7 – Demystified!

Raj Gokul Prabhakar, Velusamy S, Nithyanandam A, Aravind Visweswaran

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: Docking Protein 7 ( DOK-7) is a postsynaptic cytoplasmic protein that activates muscle-specific tyrosine kinase, leading to Rapsyn-associated endplate acetylcholine receptor (AChR) clustering and normal folding of the postsynaptic membrane. Key differentiating features are onset after infancy, normal walking milestones, limb-girdle phenotype, normal eye movements, stridor, tongue-wasting, associated nonspecific myopathy, and poor response to pyridostigmine and 3,4-diaminopyridine.

Case report: We present a case of a 35-year-old female with a DOK-7 mutation, consistent with congenital myasthenia. The patient had normal developmental milestones and experienced difficulty using proximal upper limbs and getting up from a squatting position for 13 years. She also noted bilateral eyelid drooping for 12 years, and, for the past three months, experienced breathing difficulties and worsening limb weakness. Examination revealed bilateral ptosis, restricted extraocular movements, no tongue-wasting, normal tone, and 3/5 power in all limbs. The patient was tachypneic, and required continuous positive airway pressure therapy (CPAP) support. Routine investigations and serum creatine phosphokinase (CPK) were normal. Whole exome sequencing identified a DOK-7 mutation. Treatment with oral salbutamol led to improvement.

Discussion: This case report describes a 35-year-old female with bilateral ptosis, limb-girdle weakness progressing to distal muscles, and respiratory involvement. Differential diagnoses included congenital myopathy, congenital myasthenia, and mitochondrial myopathy. Whole exome sequencing confirmed a DOK-7 mutation. Favorable features for DOK-7 congenital myasthenic syndrome include onset after infancy, normal walking milestones, limb-girdle phenotype, and good response to salbutamol. Contradictory features include complete ophthalmoparesis and the absence of tongue-wasting.

Conclusions: DOK-7 congenital myasthenic syndrome (CMS) should be suspected in patients with long-standing limb-girdle weakness, ptosis, and nonspecific myopathy. High clinical suspicion is essential for diagnosis and appropriate management.

Abstract ID: 1010: Neurotransmitter-Related Disorder Presenting with Developmental Delay and Oculogyric Crisis

Shree Badrinath, Mohinish S, Muhammed Shadique, Sonia Rosalind Martina, Jered Livingston, Neeraj Elango, Leema Pauline C

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: Neurotransmitters are pivotal in regulating basal muscle tone, movement, mood, attention, thermoregulation, and pain modulation. Neurotransmitter-related disorders are often under-recognized and misdiagnosed as cerebral palsy. These conditions are amenable to treatment, making early diagnosis paramount. This case report is being presented to emphasize the importance of early recognition and appropriate treatment of neurotransmitter-related disorders.

Case report: A five-year-old boy presented with paroxysms of involuntary upward conjugate deviation of eyes with preserved consciousness since three years of age. The events occurred twice a day, more commonly during the initiation of sleep, and lasted for 15 min. A significant history of flailness of all four limbs was noticed since birth on a background of global developmental delay. There was no history of drug intake or similar illness in the family. Examination revealed microcephaly, lumbar lordosis, genu recurvatum, axial and appendicular hypotonia, and absent deep tendon reflexes (DTRs) with intact cranial nerves, sensory, cerebellar, and autonomic systems. Upon evaluation, the child had an IQ of 65, normal creatine phosphokinase (CPK) levels, and normal nerve conduction study. Magnetic resonance imaging (MRI) of the brain and whole spine screening, as well as electroencephalogram (EEG), were normal.

Discussion: Whole exome sequencing (WES) unraveled a likely pathogenic homozygous non-sense mutation in Exon 2 of the SPR gene, which codes for sepiapterin reductase, causing dopa-sensitive dystonia.

Conclusions: Children presenting with unexplained developmental delay and red flag signs like oculogyric crisis, dystonia or mixed movement disorders, diurnal variation of symptoms, or autonomic dysfunction should prompt the suspicion of neurotransmitter-related disorders.

Abstract ID: 1011: A Case of Ocular Flutter, Generalized Myoclonus and Ataxia (OF-AMS) Following Mild COVID-19 Infection

V V N S Ravi Sankar V

Andhra Medical College Visakhapatnam, Andhra Pradesh, India

Background and Aim: Neurological complications following COVID-19 infection have been reported in over 35% of cases, affecting both the central and peripheral nervous systems. There is scare prevalence of movement disorders in COVID-19 patients. Very few cases of ocular flutter myoclonus and ataxia are reported.

Case report: A five-year-old female presented with a one-day history of mild fever and vomiting, with no respiratory symptoms, who developed involuntary eye movements, gait instability, and sudden jerks on the third day of infection. Examination revealed rapid, conjugate periodic oscillations of the eyes with a strict preponderance to the horizontal plane, truncal ataxia, gait ataxia, occasional stimulus-sensitive myoclonic jerks, most pronounced at the neck, upper limbs, brisk deep tendon reflex (DTR), and plantar extensor.

Discussion: Magnetic resonance imaging (MRI) of the brain was normal, and cerebrospinal fluid (CSF) analysis showed mild lymphocytic pleocytosis. Screening for neoplasms was negative. D-dimers were elevated, and SARS-CoV-2 antibody titers were elevated.

Conclusion: SARS-CoV-2 should be considered as a potential cause of of a altered mental status (AMS) in this COVID-19 era.

Abstract ID: 1012: Evaluation of Executive, Attentiveness, and Attentional Functions in Migraineurs

Tanaya Chatterjee, Harshdeep Singh, Debashish Chowdhury, Sonam Saxena

Affiliation

Background and Aim: Converging evidence suggests that migraine patients frequently experience difficulties in various executive functions such as attention, information processing speed, visuo-spatial attention, and memory. Additionally, migraine is often associated with psychiatric comorbidities, most commonly depression and anxiety. This clinic-based study investigated specific cognitive functions and psychiatric comorbidities among episodic and chronic migraine patients without aura, compared to a normal subject group.

Methodology: Among 59 adult migraine patients, migraine was assessed using the International Classification of Headache Disorders- 3 (ICHD-3) classification. Participants were classified into three groups: episodic migraine patients ( n = 30), chronic migraine patients ( n = 29), and control subjects ( n = 30). Migraine characteristics were determined using a proforma. Cognition assessment was conducted using mini mental state examination (MMSE), frontal assessment battery (FAB), and other standardized cognitive tests. Psychiatric comorbidities were assessed using patient health questionnaire (PHQ), mood disorder questionnaire (MDQ), and Yale-Brown obsessive-compulsive scale (Y-BOCS). Only patients detected with psychiatric abnormalities on the PHQ were further assessed for depression and anxiety. Data on cognitive performance and psychiatric comorbidities among the three groups were analyzed.

Results: The mean age of presentation in episodic migraine patients was 31.1 ± 11.2 years, and in chronic migraine patients, it was 30.5 ± 9.5 years. Chronic migraine patients experienced greater activity restriction, sleep disturbance, and premonitory and postdrome symptoms. Both migraine groups showed lower performance on tests assessing attention, lexical fluency, language, and color recognition compared to controls. Cognitive performance in chronic migraine patients was partially influenced by psychiatric comorbidities.

Conclusions: This study provides evidence of deficits in specific cognitive domains among migraine patients and highlights the partial influence of psychiatric comorbidities on these neuropsychological findings.

Abstract ID: 1013: Effectiveness of Non-Pharmacological Therapy in the Management of Patients with Cognitive Impairment: An Experience from Tertiary Care Center

S. Sandeep Kumar, Arijit Bhattachrya, Bidisha Bhattacharya, Faheem Arshad, Faheem Arshad, Subasree Ramakrishnan, Priya Treesa Thomas, Suvarna Alladi

Vasudha Hospital, Hyderabad, Telangana, India

Background and Aim: Neuropsychiatric symptoms such as depression and anxiety are associated with progressive cognitive impairment in dementia. Both pharmacological and non-pharmacological interventions are available for these symptoms. Cognitive stimulation therapy (CST) is a psychosocial intervention based on the concept of implicit learning and language stimulation and includes principles of mental stimulation and reality orientation. This study aims to assess the effectiveness of iCST in patients with cognitive impairment.

Methodology: Patients with cognitive impairment were recruited based on their clinical dementia rating (CDR) score.

Results: Among 28 patients recruited, six patients dropped out from the study, with a midterm dropout of two patients. Among the 20 patients who completed the study, 13 patients had a positive response. Pre- and post-assessment of the CDR and neuropsychiatric inventory (NPI) revealed significant improvement in NPI ( P < 0.05).

Conclusions: The iCST intervention program showed an overall positive impact on cognitive functioning and neuropsychiatric symptoms in patients with cognitive impairment, confirming it as an effective mode of intervention in people with cognitive impairment.

Abstract ID: 1014: Two Rare Presentations of Stroke: Acute Onset Movement Disorders

Vignesh NeethiArasu, Senthur Rajapandian, Justin Cornelius

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: Vascular strokes are common in the Indian population due to common risk factors like diabetes, hypertension, coronary artery disease, smoking, and alcohol. This poster aims to present two rare movement disorders that have reversible causes.

Case report: Case 1: A 49-year-old male, an alcoholic and smoker, presented with acute onset left hemiparesis with sudden onset involuntary movements of the left hand. Evaluation with a computed tomography (CT) brain showed a right capsuloganglionic bleed extending into the insular cortex, right thalamus, and parietal lobe. Case 2: A 45-year-old male presented with acute onset writhing and choreoathetotic movements of the left upper limb. On evaluation, the patient was found to have a left basal ganglionic bleed extending into the subthalamic region.

Discussion: These two cases highlight the importance of screening for vascular causes in case of acute onset movement disorders which are reversible with therapy.

Conclusions: It is always important to screen for a vascular cause in case of such atypical presentations.

Abstract ID: 1015: A Study of Treatment Outcomes and Quality of Life in Patients with Juvenile Myoclonic Epilepsy

Priya K, Pramod Krishnan

Manipal Hospital, Bengaluru, Karnataka, India

Background and Aim: Juvenile myoclonic epilepsy (JME) is one of the most common epilepsy types seen in neurology practice. It primarily affects adolescents and is classified as one of the genetic generalized epilepsies (GGE) under the latest ILAE classification of epilepsies. The goal of the management of JME is to render the individual seizure-free and at the same time provide a good quality of life. This study aims to evaluate the treatment outcome and quality of life in patients with JME. Primary treatment outcome: 1. To study the seizure control during the study period for the various ASMs used in the treatment of JME. 2. The probability of achieving one year of seizure freedom will be estimated for each anti-epileptic drugs (AED) regimen prescribed. 3. To compare the seizure-free group and those with incomplete seizure control and identify the factors that predict treatment outcomes. 4. To study the quality-of-life outcomes in JME for each anti-seizure medication (ASM) regimen by selecting ASMs with adequate efficacy and minimal adverse effects.

Methodology: A prospective cohort study was conducted for 18 months (January 2023 to June 2024) at the Department of Neurology, Manipal Hospital, Bangalore. Study population: All patients with JME attending the neurology outpatient and inpatient services of Manipal Hospital, Bangalore, from the period January 2023 to April 2023 will constitute the study population. The same population will be followed up for one year from the time of enrollment. Sample size: 65. Quality of life will be assessed by questionnaire QUOLIE-31. At the time of enrollment, the patients will be assessed for seizure semiology, frequency, electroencephalogram (EEG) findings, and baseline quality of life. The patients will be followed up for one year and compared for treatment outcomes and quality of life at the end of one year, including the effect of specific ASMs on these parameters.

Results: The study is still going on and will be completed by July 2024.

Conclusions: Conclusions will be drawn upon the completion of the study.

Abstract ID: 1016: A Report of Three Patients with NCL Type 5 in an Indian Family

Shiny Joy, Ayush Agarwal, Divyani Garg, Mohammad Faruq, Pooja Sharma, Roopa Rajan, Ajay Garg, Achal Kumar Srivastava

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Neuronal Ceroid Lipofuscinosis (NCL) comprises lysosomal storage disorders marked by progressive neurodegeneration due to ceroid lipopigment accumulation, leading to cognitive decline, vision loss, and refractory seizures. NCL manifests from birth to adulthood, showing genetic and phenotypic diversity based on the gene involved. The most common subtypes are CLN1, CLN2, CLN3, and CLN6. In India, the prevalent subtype is NCL2, caused by a tripeptidyl peptidase one mutation, presenting with seizures, ataxia, progressive dementia, and motor and visual deterioration. We describe three siblings from an Indian family with genetically confirmed juvenile NCL type 5 (CLN5).

Case Report: Case 1: An eight-year-old female, born to consanguineous parents, presented with progressive vision loss, seizures, and cognitive decline starting at age 5. Symptoms included drop attacks, myoclonic jerks, generalized tonic-clonic seizures, progressive vision loss, and increasing motor and cognitive impairment. Examination showed optic atrophy, pigmentary retinopathy, hypertonia, hyperreflexia, and cerebral and cerebellar atrophy on neuroimaging. Case 2: The proband’s 10-year-old brother experienced progressive vision loss, myoclonic seizures, and cognitive decline starting at age 5, leading to total dependence in three years. He exhibited optic atrophy, pigmentary retinopathy, exaggerated reflexes, and bilateral clonus. Neuroimaging revealed cerebral and cerebellar atrophy. Case 3: The proband’s six-year-old sister, symptomatic for one year, presented with progressive vision loss and myoclonic seizures. Examination revealed pigmentary retinopathy and normal motor strength.

Discussion and Conclusions: We described one of the first NCL5 families from India. NCL should be considered in children with cognitive impairment, epilepsy, and vision loss. Early diagnosis can facilitate enrollment in gene therapy trials, potentially providing a cure.

Abstract ID: 1017: A Rare Case Report of Childhood Onset Cerebellar Ataxia

Sonakshi Puntambekar, Sangeeta H. Ravat, Neeraj Jain, Mayur Thakkar, Rishikesh Joshi

Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

Background and Aim: Ataxia telangiectasia-like disorders (ATLDs) are an unusual group of autosomal recessive disorders with clinical features and pathophysiological mechanisms similar to ataxia telangiectasia (AT). It may be associated with mutations in the MRE11A (ATLD type 1) and PCNA (ATLD type 2) genes. In the absence of telangiectasia and normal alpha-fetoprotein levels, ATLD should be considered. This is the just second reported case of ATLD from the Indian sub-continent.

Case report: A 17-year-old boy, born of a non-consanguineous marriage, presented with a normal perinatal and developmental history with a history of imbalance while walking since two years of age and speech disturbance, along with mild intellectual disability; however, there was no history of tremulousness of the head, abnormal posturing of limbs, myoclonus, or seizures and family history. Systemic examination was unremarkable with no oculocutaneous telangiectasia, cataracts, tendon xanthomas, scoliosis, or high-arched feet. Examination showed gait and limb ataxia, ataxic speech, no external ophthalmoplegia, hypometric vertical and horizontal saccades, and horizontal and vertical saccadic pursuits. Rest examination was normal except for wide-based and ataxic gait with negative Romberg’s sign.

Discussion: Routine investigations, including thyroid functions, lipid profile, and total creatinine phosphokinase, were normal. Serum alpha-fetoprotein levels were also normal. Nerve conduction was normal, and an magnetic resonance imaging (MRI) of the brain showed pancerebellar atrophy. Whole exome sequencing showed a homozygous variant in the MRE11 gene.

Conclusions: We should look for ATLD in patients without classical oculocutaneous telangiectasia or raised serum alpha-fetoprotein levels in ataxia telangiectasia phenotype patients as it has a relatively better prognosis.

Abstract ID: 1018: TOAST Subtypes of Ischemic Stroke and Its Risk Factors – A Hospital-Based Study in North-East India

Nidhin George, Marami Das

Gauhati Medical College and Hospital, Guwahati, Assam, India

Background and Aim: Stroke is rapidly developing clinical signs of focal (or global) disturbance of cerebral function, with symptoms lasting 24 h or longer, or leading to death, with no apparent cause other than vascular origin. Ischemic stroke can be classified into five subtypes according to the Trial of Org 10172 in Acute Stroke Treatment (TOAST) classification. Numerous studies have revealed that risk factors have variable correlations with different stroke subtypes. Currently, there is no data regarding this in North-East India. The aim is to study the TOAST subtypes of ischemic stroke and its risk factors.

Methodology: An observational study was carried out in the Department of Neurology, from November 2022 to April 2024. All patients underwent thorough clinical examination, routine blood tests, electrocardiogram (ECG), 2D echo, and neuroimaging. The enrolled patients were diagnosed with acute ischemic stroke and classified according to TOAST criteria, and the risk factors and other relevant data were documented.

Results: The study had 579 cases of acute ischemic stroke. When classified according to TOAST criteria, there were 229 (39.5%) cases of large artery atherosclerosis (LAA), 205 (35.4%) cases of small artery occlusion (SAO), 62 (10.7%) cases of cardio-embolism, 12 (2.07%) cases of stroke of other determined etiology, and 71 (12.26%) cases of stroke of undetermined etiology.

Conclusions: Large artery atherosclerosis was the most common ischemic stroke subtype. The most common risk factor in large artery atherosclerosis and small vessel occlusion was systemic hypertension, which was more prevalent in small artery occlusion. Non-valvular atrial fibrillation was the most common cause of cardioembolic stroke.

Abstract ID: 1019: A study on Clinical, Electrophysiological Profile in Acute Axonal Polyradiculoneuropathy Patients, Not Responding to Intravenous Immunoglobulin in a Tertiary Care Institute

Dipankar Pal

Institute of Post Graduate Medical Education and Research (IPGMER), Kolkata, West Bengal, India

Background and Aim: Background: The prevalence of acute axonal polyradiculoneuropathy (AMAN, AMSAN) variants of Guillain–Barré syndrome (GBS) is not well documented in existing literature. These axonal variants are known to respond less favorably to IVIG therapy than typical acute inflammatory demyelinating polyneuropathy (AIDP) variants of GBS. Those who are not responding to IVIG have different electrophysiological and clinical profiles in comparison to AIDP. The aim of the study is to describe the clinical and electrophysiological profiles of acute axonal polyradiculoneuropathy patients, not responding to intravenous immunoglobulin (IVIG).

Methodology: This observational study was conducted in a tertiary care institution from August 2023 to May 2024. Clinical and electrophysiological data were collected from the patients, meeting the inclusion criteria. The following clinical parameters were assessed: age, gender, duration of illness, UL/LL onset, severity of weakness [assessed by Medical Research Council (MRC) sum score], bulbar or respiratory involvement, and autonomic involvement. Electrophysiological characteristics were assessed. Following patterns of involvement were seen: pure axonal involvement axonal involvement with conduction block primary axonal with secondary demyelinating changes. Inclusion criteria patients diagnosed as acute motor or sensory-motor axonal polyradiculoneuropathy. Patients will give consent for participation in the study. Response to IVIG therapy was assessed at two weeks, following therapy in terms of MRC sum score at baseline and after two weeks. Data was analyzed appropriately.

Results: A total of 30 patients were enrolled in the study. Among them, ten patients (33%) did not respond to intravenous immunoglobulin (IVIG) therapy.

Conclusions: A study of clinical and electrophysiological characteristics in axonal variants of GB syndrome cases is of great value in predicting the response to IVIG therapy.

Abstract ID: 1020: A Rare Cause of Ptosis

Vignesh NeethiArasu, J. Muthukumar, P. K. Murugan

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: A rare cause of ptosis— a 42-year-old female presenting with a subacute onset unilateral ptosis.

Case report: A 42-year-old female presented with complaints of cough and cold for two weeks followed by headache and ptosis of the left eye associated with periorbital pain and discomfort. Her pupils were spared, her eye movements were not restricted, and she had no proptosis.

Discussion: The magnetic resonance imaging (MRI) of the brain revealed a large left frontal sinus mucocele that had eroded into the left orbit. The patient underwent an endoscopic sinus surgery under the ENT Team, and later, her ptosis had completely resolved.

Conclusions: We should always look for local causes in case of unilateral ptosis with no restriction of eye movement or pupil-sparing.

Abstract ID: 1021: A Rare Case of Peripheral Neuropathy Associated with M Protein

Perumalla Lakshmi, Akkireddi Sita Kanthima, U Aruna Kumari

Guntur Medical College, Guntur, Andhra Pradesh, India

Background and Aim: Monoclonal gammopathies consist of a spectrum of clonal plasma cell disorders, including monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma (MM), and Waldenstrom macroglobulinemia (WM). Peripheral neuropathy is a well-recognized complication of monoclonal gammopathies and a difficult clinical problem in terms of diagnosis and treatment.

Case report: A 54-year-old male presented with symmetrical chronic progressive lower motor neuron (LMN) type quadriparesis (distal > proximal) lower limb predominant with wasting, fasciculations, hypotonia, and hyporeflexia, with both small and large fiber sensory involvement with skin changes, with cachexia, with no cranial nerve involvement with no cerebellar or autonomic involvement with no cognitive or extrapyramidal signs with no signs of raised intracranial tension. Investigations: complete blood count (CBC), renal function test (RFT), liver function test (LFT), and serum electrolytes were within normal limits; nerve conduction study (NCS) showed bilateral severe sensory – motor demyelinating polyradiculoneuropathy (sensory predominant) with secondary axonal changes; cerebrospinal fluid (CSF) analysis showed total Count (TC)-4 cells, differential count (DC)- mostly lymphocytes, no red blood cells (RBC), protein of 144 mg/dL, and sugar of 74 mg/dL; serum protein electrophoresis indicated a non-discrete protein band in the gamma globulin region, possibly monoclonal gammopathy. Advised immunotyping revealed a very faint, non-discrete protein band in the gamma globulin region of the IgG and kappa lane, corresponding to M spike.

Discussion: Final diagnosis: Paraproteinemic neuropathy – monoclonal gammopathy of undetermined significance (MGUS).

Conclusions: Approximately 10% of patients with idiopathic peripheral neuropathy have an associated monoclonal gammopathy. Patients undergoing evaluation for chronic peripheral neuropathy should be screened for the presence of a monoclonal protein (M protein).

Abstract ID: 1022: Gut Microbiota Composition in Hypertensive Intracerebral Hemorrhage (ICH) and its Association with Outcomes: A Systematic Review

Subhasmita Tripathy, Deepti Vibha, Apoorva Balodhi, Manabesh Nath

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Intracerebral hemorrhage (ICH), a devastating stroke subtype with high mortality, necessitates novel therapeutic approaches. The gut-brain axis, with its influence on stroke outcomes via gut microbiota and immune responses, presents a promising avenue. We conducted a systematic review to explore associations between cytokine responses and disease progression in ICH patients.

Methodology: We searched PubMed, Embase, Scopus, Google Scholar, Cochrane, Trip, and Web of Science for studies investigating these associations. Case reports, animal studies, and non-English publications were excluded.

Results: Our review examined post-ICH cytokine dynamics and their impact on outcomes. Significant changes in Eotaxin, granulocyte-macrophage colony-stimulating factor (GM-CSF), IL-8, IL-9, IL-10, IL-12p70, IL-15, IL-23, IL-1RA, IP-10, RANTES, and tumour necrosis factor alpha (TNF-α) levels correlated with poor 90-day outcomes. Interestingly, GM-CSF displayed potential benefits in experimental stroke models.

Conclusions: This review highlights the association between Enterococcus enrichment and Prevotella depletion in the gut microbiota with poor ICH outcomes. Additionally, altered cytokine levels suggest microbiota-mediated effects on host immune responses, which are critical for understanding ICH pathogenesis. These findings propose gut microbiota and serum cytokine profiles as potential biomarkers for ICH triggers and highlight the potential for gut microbiota modulation as a therapeutic strategy targeting dysbiosis in ICH patients.

Abstract ID: 1023: A Case of ATP1A3 Mutation Presenting as Rapid-Onset Dystonia-Parkinsonism in a 14-Year-Old Child

Pranav Gupta, Neeraj Bhutani

Santokba Durlabhji Memorial Hospital Jaipur, Rajasthan, India

Background and Aim: Mutations in the ATP1A3 gene cause the classical disorders of rapid-onset dystonia-parkinsonism (RDP), alternating hemiplegia of childhood (AHC) and cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS). However, intermediate phenotypes have also been described, making the range of clinical manifestations associated with mutations in the ATP1A3 gene wider.

Case report: We present the case of a 14-year-old male patient with genetically confirmed ATP1A3 young-onset parkinsonism who presented with subacute onset difficulty in performing skilled tasks from the right upper limb and a change in his handwriting.

Discussion: This case demonstrates many typical features, including loss of dexterity and rigidity in the dominant hand with change in handwriting, suggesting dystonic phenomenology at an early age with diurnal variation and lack of family history and response to low dose levodopa. However, case reports do show a wide variation in clinical syndromes associated with this condition.

Conclusions: RDP (DYT12) and alternating hemiplegia of childhood-2 are caused by heterozygous mutations in the ATP1A3 gene. Dystonia-12 (DYT12), also known as RDP, is an autosomal-dominant disorder characterized by abrupt onset of asymmetric dystonia and parkinsonism in young adulthood, often after a trigger such as physical overexertion, trauma, heat, or fever.

Abstract ID: 1024: Bilateral Recurrent Painful Ophthalmoplegic Neuropathy in an Adolescent Female: A Unique Clinical Challenge

Siddharth Maheshwari, Aabhasha Parotra, Rashmi Mishra, Mridula Singh Rustagi

Institute of Human Behaviour and Allied Sciences, Delhi, India

Background and Aim: Recurrent painful ophthalmoplegic neuropathy (RPON), known historically as ophthalmoplegic migraine, is characterized by recurrent episodes of unilateral headache associated with one or more ipsilateral cranial nerve palsies (most commonly being third nerve). It predominantly affects children and young adults. Diagnosis of RPON is primarily of exclusion, necessitating a thorough investigation to rule out orbital, parasellar, or posterior fossa lesions and vascular or other cerebral causes.

Case report: We present a unique case of RPON in an 18-year-old female with bilateral ophthalmoparesis. An 18-year-old female presented with throbbing, bifrontal headache, accompanied by nausea, vomiting, photophobia, and phonophobia, followed by double vision after 10–12 h. She had a history of two similar episodes in the past that resolved spontaneously over two to three weeks with a family history of migraine in her mother. On examination, she exhibited binocular diplopia and restricted abduction in both eyes, with no ptosis and normal pupil reactivity. Other cranial nerves, motor, sensory, speech, and cerebellar exams were normal. There were no meningeal signs. Investigations, including complete blood count, erythrocyte sedimentation rate (ESR), blood glucose, and other metabolic profiles were normal. Cerebrospinal fluid analysis was normal, with no oligoclonal bands or elevated IgG index. Serum angiotensin-converting enzyme (ACE) levels, anti-thyroid peroxidase (anti-TPO), antinuclear antibodies (ANA) profile, and Lyme serology were negative. Magnetic resonance imaging (MRI) brain, visual evoked potential (VEP), and electroencephalogram (EEG) were also normal. After exclusion, a diagnosis of bilateral RPON was made. She was treated with nonsteroidal anti-inflammatory drugs (NSAIDs) and tapering oral steroids, leading to headache improvement within 72 h and resolution of ocular symptoms within two weeks.

Discussion and Conclusions: Further research is warranted to explore the cause and optimal preventive strategies for RPON episodes.

Abstract ID: 1025: A Rare Treatable Genetic Movement Disorder

Muhammed Shadique, Mohinish Sundar, Sonia Rosalind Martina, Shree Shruthi Badrinath, Neeraj Elango, Jered Livingston, Leema Pauline

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: A four-year nine-month-old female child, born to consanguineous parents, was brought in with c/o difficulty in walking since the child started walking.

Case report: Antenatal, birth history: Uneventful; Term: normal vaginal delivery (NVD), cried immediately after birth (CIAB); Development: Gross motor – Neck control at three months, Roll over O at five months, sits at nine months, standing and walking at two years; language and social milestones were normal. H/o frequent falls on either side+ initially. H/o abnormal gait in the form of flexion of toes in both lower limbs and protruded abdomen+. H/o fever with jaundice and abdominal distension for two years of age. H/o pale stools for two years of age. No H/o seizures. No similar family history.

Discussion and Conclusions: Manganese (Mn) homeostasis is normally regulated by intestinal absorption and hepatobiliary metal secretion into the gastrointestinal tract (GI) tract. Mn excess has a predilection to basal banglia disrupting various neurotransmitter functions causing various manifestations and neurodegenerative extrapyramidal syndrome. SLC30A10 mutation causes hypermanganesemia with dystonia type 1, presenting as dystonia, cock-walk gait, spasticity, and pyramidal signs, with relatively preserved cognition. Liver diseases and polycythemia+ are also associated. Management includes chelation therapy, iron supplementation, and other supportive measures.

Abstract ID: 1026: Biotin Thiamine Responsive Basal Ganglia Disease – A Treatable Vitamin-Responsive Disorder

C. Leema Pauline, Jered Kanakaraj, Neeraj Elango

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: Biotin thiamine-responsive basal ganglia disease (BTBGD) is a rare autosomal recessive disorder due to a mutation in the SLC19A3 gene encoding thiamine transporter 2, which allows thiamine to cross the blood-brain barrier. It is a treatable disorder if identified and treated early.

Case report: We present a case of infantile BTBGD missed as hypoxic ischemic sequelae. A six-month-old female child first born of third-degree consanguineous parents was brought with recurrent seizures and regression. Her neonatal period was uneventful. She achieved a social smile at two months and partial head control by three months. At four months, she developed fever, recurrent seizures, and altered sensorium. Magnetic resonance imaging (MRI) brain showed bilateral symmetrical hyperintensities in the caudate, putamen, and cerebral cortex reported as hypoxic ischemic changes. Cerebrospinal fluid (CSF) analysis was normal. Examination revealed an awake child with microcephaly and spastic quadriparesis. She was neither recognizing her mother nor following the light. Fundi were normal. There were no dysmorphic features, neurocutaneous markers, or organomegaly. MRI findings were suggestive of BTBGD and was started on biotin 5 mg/kg/day and thiamine 15 mg/kg/day. Repeat MRI showed striatal and cerebral atrophy. Seizures were controlled, and she made developmental gains. Whole exome sequencing revealed a pathogenic mutation in the SLC19A3 gene confirming the diagnosis of BTBGD. Genetic counseling regarding the risk of recurrence and prenatal screening was advised.

Discussion and Conclusions: There should be a high index of suspicion to diagnose BTBGD, and thiamine and biotin should be started early to have a better outcome.

Abstract ID: 1027: A Case Report of Celiac Disease Presenting as Peripheral Neuropathy

Taniya Upadhyaya

Fortis Hospital Jaipur, Rajasthan, India

Background and Aim: Celiac disease is a chronic, heterogeneous, autoimmune disorder that occurs in children and adults who have a genetic predisposition to the development of antibody-mediated damage to small intestinal mucosa (and other organs) resulting in gluten sensitivity. The disease is based on the synthesis of immunoglobulin (Ig)A- and IgG-class antigliadin antibodies (AGAs), anti-endomysial, and anti-transglutaminase type 2 antibodies.

Case report: The patient, a 47-year-old right-handed perimenopausal female presented with c/o numbness in both lower limbs for three to four months, along with a sense of imbalance. She also complained of paresthesias and pain in both her lower limbs. Her imbalance worsened in the dark and improved as she walked while looking downwards. About one month into the illness, she also complained of difficulty in gripping slippers, and there was slippage of slippers with her awareness. Her symptoms were symmetrical and progressive in nature. About 10–15 days back her numbness and paresthesias progressed and now involved both her hands and upper limbs. She also found difficulty in gripping objects. She also started complaining of dryness of her eyes. Her neurological examination was significant for weakness in all her four limbs (MMRC grade 4/5), weak hand grip, absent ankle jerks bilaterally, and impaired touch pain and temperature in length-dependent pattern along with impaired vibration sense, positive Rhomberg’s test and positive Schirmer’s test, suggesting large and small fiber neuropathy.

Discussion and Conclusions: This was an unusual case of celiac neuropathy. The gradual development of symmetric hypoesthesia in the lower extremities corresponded to the most common chronic course of gluten neuropathies.

Abstract ID: 1028: Study of Resting Motor Threshold in Patients with Idiopathic Generalized Epilepsy and the Effect of Anti-Seizure Medications

Ajay Asranna, Deepak Chadha, Shubham Gupta, Sreeraj V S, Urvaksh M. Mehta, Vishwanathan L.G., Raghavendra K, Ravindranadh C. M., Sanjib Sinha

National Institute of Mental Health and Neuro-Sciences (NIMHANS), Bengaluru, Karnataka, India

Background and Aim: Resting motor threshold (RMT), measured using transcranial magnetic stimulation (TMS), is believed to reflect the trans-synaptic excitability of cortico-spinal neurons. The pathogenesis of idiopathic generalized epilepsy (IGE) is associated with neuronal hyperexcitability. Hence, studying RMT in patients with IGE and the effect of anti-seizure medications (ASM) can offer significant insights into epileptogenesis in IGE.

Methodology: In this study, 31 patients with IGE were analyzed along with 31 age- and sex-matched healthy controls. Among these, eight patients with IGE were drug-naïve. A 32-channel electroencephalogram (EEG) system was utilized to record EEG signals connected to a TMS-compatible EEG amplifier. Magnetic stimuli were delivered using a figure-of-eight coil connected to a Magstim 200 stimulator. The target areas of the motor cortex were localized through neuronavigational techniques. Surface electromyography (EMG) measurements were made on the abductor pollicis brevis muscle. The RMT was calculated in patients and controls.

Results: The mean age of the patients was 23.56 years (7.89), ranging from 18 to 50 years. An age- and sex-matched group of 31 healthy controls, comprising 15 women and 16 men, was also included. The mean age of the control group was 26.02 years (6.13), ranging from 18 to 47 years. The mean RMT in patients with IGE was higher compared to control subjects (50.06 ± 9.53 vs. 44.52 ± 12.69; P = 0.003). However, there was no difference in RMT between controls and drug-naïve patients with IGE (44.75 ± 12.96; P > 0.05). Patients with IGE on Levetiracetam had a lower RMT (48 ± 4.5) compared to patients on valproate (55.73 ± 8.2).

Conclusions: Levetiracetam had a lesser impact on RMT compared to valproate. We could not demonstrate significant differences in RMT between drug-naïve patients with IGE and those on ASM.

Abstract ID: 1029: Case Report on Three Unusual Cases of GBS Mimics Presenting as Paraparesis

Pranav Gupta, Dinesh Sharma, Apoorv Sharma

Santokba Durlabhji Memorial Hospital Jaipur, Rajasthan

Background and Aim: Paraparesis is a common neurological emergency, and Guillain-Barré syndrome (GBS) is a common differential. We present here two cases that were initially thought to be GBS but later found to have compressive vascular ethology as the cause of paraparesis.

Case report: A 12-year-old child, 8th class student, with normal birth, development, and milestones, attended a marriage on April 22, 2024, involved dancing, came back at 1 AM, and slept. The next morning severe back pain radiating to both lower limbs followed by weakness and decreased sensation in both her legs and was unable to move her legs at all for about 1 h. Nerve conduction velocity (NCV) showed the absence of an F wave and was planned for intravenous immunoglobulin (IVIG). Later on, finding bladder retention, a magnetic resonance imaging (MRI) dorsal spine showed epidural hematoma in the posterior part of the upper thoracic region (D2-3 to D 4-5) level with prominent enhancing vascular channel – s/o A-V malformation with compressive myelopathy The second case was of 45-year-old hypertensive male who had burning sensation of throat and abdomen while riding his scooter one morning. On reaching home, he was unable to move his legs at all. He underwent an MRI of the brain as well as the cervical, dorsal lumbosacral spine, NCV, electromyography (EMG), and repetitive nerve stimulation test (RNS), and no abnormality was found. An abdominal angiogram was conducted which revealed a huge dissection of the abdominal aorta.

Discussion: These cases highlight wide differential diagnosis of paraparesis and structured examination, and tests can lead to a diagnosis of treatable etiologies.

Conclusions: Parapresis has a wide differential diagnosis.

Abstract ID: 1030: Spinal Dural Arteriovenous Fistula with Longitudinally Extensive Myelitis – Report of Two Cases

Saravana Sukriya S, Nihar Kathrani, Richa Singh Chauhan

All India Institute of Medical Sciences (AIIMS), Raipur, Chhattisgarh, India

Background and Aim: Spinal dural arteriovenous fistula is a common vascular anomaly, affecting the spinal cord. The early clinical features are nonspecific with low back pain and paresthesia, and patients develop weakness of limbs, bladder, and bowel disturbances eventually. The diagnosis is often overlooked resulting in morbidity. We report two such cases diagnosed later into illness.

Case report: Case I: A 48-year-old male presented with a one-year duration of low back pain radiating to bilateral lower limbs. It was associated with progressive weakness and numbness of the right followed by the left lower limb. Subsequently, he developed bladder disturbances with urinary incontinence. Examination revealed hypotonia, MRC 1/5 bilateral lower limbs with brisk deep tendon reflex (biceps, supinator, triceps, knee), absent ankle jerk, mute plantar, sensory level around T6 suggestive of myeloradiculopathy. Magnetic resonance imaging (MRI) revealed longitudinally extensive myelitis from D6 to conus with dilated perimedullary vessels (flow voids). Digital subtraction angiography (DSA) showed fistulous communication between the left sacral artery and filum terminale vein and he underwent ethylene-venyl-alcohol embolization. Two months later, power had improved to MRC 2/5 bilateral lower limbs. Case II: A 50-year-old male presented with four months duration of low back pain followed by progressive weakness and paresthesia of the left followed by the right lower limb. Examination revealed wasting, hypotonia, power MRC 4/5 bilateral hip, right knee and ankle, 3/5 in left knee, 2/5 in the left ankle with sluggish knee and ankle jerk, and sensory level around L2.

Discussion and Conclusions: Spinal dural arteriovenous fistula is often an underdiagnosed cause of myelopathy. Spinal angiogram is the gold standard in the diagnosis. Early identification and timely intervention prevent disability and morbidity.

Abstract ID: 1031: Deciphering A Rare Case of Cerebral Arteriopathy in a Patient with ACTA Two Gene Mutation

Abhishek Bhargava, Bhawana Sharma, Trilochan Srivastava

Sawai Mansingh Medical College, Jaipur, Rajasthan, India

Background and Aim: We describe an interesting case of abnormal neurovascular vasculopathy.

Case report: A three-year-old male child presented to us with a history of delayed developmental milestones, multiple episodes of drug-refractory focal seizures with impaired awareness, and macrocephaly. The patient’s MRI brain whole was performed along with computed tomography (CT) angiography of brain and neck vessels and electroencephalogram (EEG). ACTA2 gene mutation was confirmed by whole exome genetic testing.

Discussion: The patient’s magnetic resonance imaging (MRI) brain showed bending and hypoplasia of the anterior corpus callosum with malformed and hypoplastic posterior corpus callosum manifesting as colpocephaly. On axial images, the anterior corpus callosum demonstrated a characteristic V-shaped appearance. There was abnormal radial gyration of the frontal, parietal, and temporal lobes. “Twin peaks sign” was seen in the pons. CT angiography of brain and neck vessels showed abnormally straight morphology of proximal branches of the circle of Willis (broomstick appearance), vascular ectasia of the proximal internal carotid artery, and stenotic features in the terminal part of the internal carotid artery.

Conclusions: Patients with these mutations are also predisposed to premature coronary artery disease, young-onset stroke, epilepsy, and aortic dissections in their adulthood.

Abstract ID: 1032: LAMC3-Associated Cortical Malformations – A Rare Clinical Entity

Vamsi Krishna Mudamanchu

Vydehi Institute of Medical Sciences and Research Centre (VIMS), Bengaluru, Karnataka, India

Background and Aim: LAMC3-associated cortical malformations: The biological basis for regional and inter-species differences in cerebral cortical morphology is poorly understood. Occipital cortical malformation (OCCM) is a disease caused by malformations of cortical development characterized by polymicrogyria and pachygyria of the occipital lobes and childhood-onset seizures. LAMC3 expression peaks between late gestation and late infancy, paralleling the expression of molecules that are important in dendritogenesis and synapse formation.

Case report: A 14-year-old male, born of non-consanguineous parentage, presented with bilateral visual disturbance and childhood onset of seizures with no family history of similar complaints. On examination, only the second cranial nerve is affected with visual acuity of 6/9 on both sides and no other focal deficits.

Discussion: Magnetic resonance imaging (MRI) and genetic testing show features of LAMC3 mutation-associated OCCM, which is autosomal recessive in nature. Visual evoked potentials showed bilateral prolonged P100 latency, mainly on the right. The patient has a homozygous variant, with both parents being heterozygous carriers. This highlights the importance of genetic testing and counseling.

Conclusions: The LAMC3 gene is located on chromosome 9 and encodes the α 3 chain of the extracellular laminin family proteins, associated with cell differentiation, migration, and adhesion (Hamill et al. , 2009), and it is involved in the organization of the cerebral cortex and its gyration (Radner et al. , 2013). Homozygous or compound heterozygous variants in LAMC3 have been described to cause occipital cortical malformations (OMIM #614115).

Abstract ID: 1033: An Interesting Case of Periodic Hypersomnolence and Behavioral Symptoms

Chaudhari Hemabhai, Arnesh Bhattacharya, Devavrata Sahu, Ashish Duggal, Jyoti Garg

ABVIMS & DR RML Hospital, New Delhi, India

Background and Aim: Kleine-Levin syndrome (KLS) is a rare disease characterized by recurrent episodes of hypersomnia associated with behavior disturbances, abnormal eating behavior, and hypersexuality.

Case report: A 12-year-old boy presented with episodic hypersomnolence, hallucinations, irritability, and behavioral abnormalities. Examination revealed the patient to be drowsy, and disoriented, with deficits of attention and memory on higher mental function examination. The rest of the neuraxial and systemic examinations were normal. The patient used to be completely lucid in between the episodes.

Discussion: Routine blood investigations were normal. In view of the apparent steroid responsiveness of the episodes, the patient had been evaluated on the line of autoimmune encephalitis, the testing for which was normal. Contrast-enhanced magnetic resonance imaging (CEMRI) brain was normal. PET CT was normal. Electroencephalogram (EEG) during the episodes was suggestive of intermittent diffuse delta bursts with background slowing.

Conclusions: Patients presenting with cyclical hypersomnolence and behavioral symptoms should be considered for KLS syndrome, which can mimic other common etiologies for hypersomnolence.

Abstract ID: 1034: Recurrent Strokes in HIV: Are We Missing Vasculitis?

Chaudhari Hemabhai, Arnesh Bhattacharya, Milan Mori, Kumari Archana, Ashish Duggal, Jyoti Garg

ABVIMS & DR RML Hospital, New Delhi, India

Background and Aim: Human immunodeficiency (HIV)-associated vasculitis, a subset of HIV vasculopathy, is a lesser-known complication in patients afflicted with HIV. It may present as recurrent strokes or in the form of a white matter disease. Management generally includes anti-retroviral therapy, and the role of immunomodulators is unclear.

Case report: A 37-year-old gentleman with known HIV-positive status came with a history of recurrent strokes over a month. He has developed left hemiparesis, dysarthria, and left facial weakness with a degree of motor aphasia. The patient was compliant with antiretroviral therapy (ART), and there was no evidence of immune reconstitution inflammatory syndrome (IRIS)-like phenomena.

Discussion: Magnetic resonance imaging (MRI) revealed T2 and fluid attenuated inversion recovery (FLAIR) hyperintense lesions with areas of diffusion restriction along the deep gray matter, deep white matter, and brainstem suggestive of multiple lacunar infarcts and features of white matter disease burden. Evaluation for active tuberculosis, cryptococcosis, varicella-zoster virus (VZV)-related illness, syphilis, and JC virus infection came to be negative. The autoimmune profile was negative. The vessel wall study revealed multiple areas of focal enhancement of the large and medium cerebral vessels, involving both anterior and posterior circulation. The patient was continued on ART, and steroids were added along with anti-tubercular coverage.

Conclusions: Strokes in HIV patients should be evaluated for vasculitis by vessel imaging once opportunists, and vascular risk factors have been ruled out.

Abstract ID: 1035: Identification of STUB1 as a Novel Cause of Adolescent-Onset Ataxia with Spasticity and Cognitive Decline in First Cousins

Madhav Bahadur, Anshumalini Panda, Ajai Kumar Singh

Dr. Ram Manohar Lohia Avadh University Faizabad, Uttar Pradesh, India

Background and Aim: A subset of hereditary cerebellar ataxias is inherited as autosomal recessive spinocerebellar ataxias (SCARs). Their classification is constantly evolving due to newly identified genes. Recently, reports have linked mutations in genes involved in ubiquitination (RNF216, OTUD4, STUB1) to SCARs. To our knowledge, no SCAR16 cases have been reported from the Indian sub-continent.

Case report: Retrospective collection of two patients with SCAR16 diagnosed. We report two male SCAR16 patients from two related Indian families with non-consanguineous parents. Both presented with adolescent-onset progressive cerebellar ataxia. They developed cognitive deterioration, dysarthria, and spasticity over the course of the illness. Brain magnetic resonance imaging (MRI) showed marked cerebellar atrophy in the older and early atrophy in the younger patients. The proband was earlier tested negative for autosomal-dominant spinocerebellar ataxia (SCA); subsequently, next gen sequencing (NGS) ataxia panel showed STUB1 mutation linked to SCAR16. His younger maternal cousin, although on follow-up for a year, was afterward identified with the same mutation. STUB1-associated phenotypic findings cover a broad spectrum, ranging from isolated slowly progressive ataxia to cognitive decline, spasticity, hypogonadism, and extrapyramidal features. The younger patient had a delayed onset of puberty with gynecomastia and cushingoid features. Both cases did not have any extrapyramidal features.

Discussion and Conclusions: SCAR16 is a rare condition, which should be taken into account in the ever-growing list of differentials of SCA, especially when suspecting an autosomal recessive inherited ataxia.

Abstract ID: 1036: Unraveling the Enigma of KIF1A-Associated Neurological Disorder: A Rare Presentation

Sidharth Sharma, Ajai kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Abdul Qavi

Affiliation

Background and Aim: Kinesin family member 1A (KIF1A) is a motor protein crucial for intracellular transport along microtubules. Mutations in the KIF1A gene have been implicated in a spectrum of neurological disorders, ranging from hereditary spastic paraplegia to sensory and autonomic neuropathy.

Case report: Here, we present a rare case of KIF1A-associated neurological disorder in a 17-year-old female, characterized by dystonia, sensory axonal neuropathy, and developmental delay. Our patient exhibited developmental delay, characterized by delays in achieving motor and language milestones. She started speaking at three years of age and toe walking at four years of age which improved over four to five years; following this, she used to keep her left hand on her left knee as a support while walking, and when she removed this hand from the knee, there would be abnormal jerky movement of left thigh and leg like a hobby horse gait accompanied with abnormal jerky movement of both hand suggestive of dystonic tremors.

Discussion: Based on the above findings, the possibility of pediatric dystonia was kept. Sensory axonal neuropathy was evident in our patient through a combination of sensory deficits and nerve conduction study (NCS) findings. Magnetic resonance imaging (MRI) brain was normal. A dystonia panel was sent which came positive for KIF-1A mutation.

Conclusions: This case highlights the diverse clinical spectrum of KIF1A-associated neurological disorders and underscores the importance of considering this diagnosis in patients presenting with dystonia, sensory neuropathy, and developmental delay. Further research is warranted to elucidate the underlying pathophysiological mechanisms and explore potential therapeutic interventions for this rare but debilitating condition.

Abstract ID: 1037: Deciphering the Diagnostic and Prognostic Role of MicroRNAs in Stroke: A Systematic Review and Meta-Analysis

Manabesh Nath, Pradeep Kumar, Deepti Vibha

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: MicroRNAs (miRNAs) are a class of small and non-coding RNA molecules that play a crucial role in gene regulation. Studies have shown that miRNAs can be involved in the pathogenesis of many diseases, including stroke. Stroke is a major public health problem and a leading cause of death and disability worldwide. In this systematic review and meta-analysis, we aimed to investigate the role of miRNAs in stroke.

Methodology: We conducted a comprehensive search of several databases, including PubMed, Embase, and Cochrane Library, from inception to April 2024. We included studies that reported miRNA expression in stroke patients compared to healthy controls or in stroke patients with different stroke subtypes. We performed a meta-analysis using a random-effects model to estimate the pooled effect size.

Results: A total of 34 studies were included in this systematic review and meta-analysis. The meta-analysis showed that the expression of miR-21 was significantly upregulated in stroke patients compared to healthy controls (standardized mean difference [SMD] = 1.35, 95% confidence interval [CI]: 0.99–1.71, P < 0.001). In addition, the expression of miR-145 was significantly downregulated in stroke patients compared to healthy controls (SMD = −1.09, 95% CI: −1.47 to −0.71, P < 0.001). Furthermore, the expression of miR-221 was significantly upregulated in ischemic stroke patients compared to hemorrhagic stroke patients (SMD = 1.13, 95% CI: 0.57–1.68, P < 0.001).

Conclusions: Future research should focus on the mechanisms of miRNA dysregulation in stroke and the development of miRNA-based diagnostic and therapeutic approaches for stroke management.

Abstract ID: 1039: Questionnaire-Based Neuropsychological Assessment in Patients with Idiopathic Intracranial Hypertension – A Cross-Sectional Study with a Comparison Group

Vaishali Sharma, Kamalesh Chakravarty

Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

Background and Aim: Patients with idiopathic intracranial hypertension (IIH) frequently have co-existent psychiatric abnormalities and poor quality of life. Both these factors might be a contributing factor for increased disease burden.

Methodology: A total of 46 IIH patients and 34 controls were enrolled. Baseline characteristics were noted for both groups. Diagnosis of IIH was based on Friedman’s criteria. Age – (±5) and sex-matched normal healthy controls were used for comparison. Patient Health Questionnaire-9 (PHQ-9), Patient Health Questionnaire- 15 (PHQ-15), Generalised Anxiety Disorder Questionnaire-7 (GAD-7), World Health Organization Disability Assessment Schedule (WHODAS), and World Health Organization Quality of Life Questionnaire (WHOQOL) were used for the assessment of depression, somatization, anxiety, disability, and quality of life, respectively.

Results: The mean age of patients was 37.02 ± 10.89 and controls 32.85 ± 7.90 having male to female ratio of 5:41 and 4:30, respectively. IIH patients had a higher body mass index (BMI) 35 of .69 ± 4.09 and Migraine Disability Assessment (MIDAS) scores of 18.84 ± 15.21 and 51.48 ± 12.24 headache impact test (HIT-6) score. We have seen significant differences in PHQ-9 (<0.0001), PHQ-15 (<0.0001), GAD-7 (<0.0001), and WHODAS (0.0001). In WHO-QOL BREF, only component 3 has shown a significant difference (0.0014).

Conclusions: Patients with IIH tend to have co-existent depression, anxiety, somatization, mild disability along with poor quality of life. All these factors might be contributing to the increased disease burden. Hence, all IIH patients must undergo psychiatric evaluation along with routine medical management.

Abstract ID: 1040: Study the Correlation of Clinical Electrophysiological and Radiological Features of Hirayama Disease in a Tertiary Care Hospital in Eastern India

Subrata Halder, Ayan Ghorai

Calcutta National Medical College & Hospital, Kolkata, West Bengal, India

Background and Aim: To study discriminative clinical electrophysiological radiological criteria and their correlation in patients of Hirayama disease (HD).

Methodology: This is an observational retrospective study of 42 cases attending neurology from 2021 to 2023. After fulfilling the inclusion and exclusion criteria, 42 cases were included in my study. Data are collected and analyzed subsequently.

Results: In our study, all are male, aged 12 to 42, averaging 20.5 years mostly before 25 years. Most cases presented late on an average of 13.7 months. A significant number have a bilateral presentation (47%). It is right-hand predominant in both unilateral and bilateral cases. “Reverse split hand syndrome” was observed in 90% of cases and forearm weakness and atrophy significantly over medial aspect sparing brachioradialis (93%). Polyminimyoclonus is a significant finding noted in 88% of cases. Deep tendon reflex (DTR) is usually preserved except in 14% of cases where triceps jerk is reduced. All these are sensitive clinical markers of the disease. Nerve conduction study (NCS) shows >50% reduction of ulnar compound muscle action potential (CMAP) in the affected side where median CMAP is relatively preserved and ulnar/Median CMAP ratio was <0.5, but velocity and SNAP are preserved. These are good electrophysiological findings. Electromyography (EMG) revealed a neurogenic pattern in all cases in C8-T1, with 26 (81%) C7 and 4 (12.5%) C5-6. EMG also shows bilateral involvement. MRI cervical spine shows straightening with loss of lordosis, cord atrophy, and occasionally focal demyelination. Flexion MRI detects anterior buckling of the posterior cervical dura with flow voids that not only restrict to the cervical spine but may extend extensively.

Conclusions: HD can be diagnosed clinically with the help of electrophysiology which is a cost-effective way of diagnosis, and radiology only helps further confirmation.

Abstract ID: 1041: A Case of Hypoparathyroidism Presenting as Mixed Movement Disorder

Anshumalini Panda, Abdul Qavi, Ajai Kumar Singh, Dinkar Kulshrestha, Pradeep Kumar Maurya, Rohit Keshav, Shrikrishna

Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background and Aim: “Fahr syndrome” is a disorder of basal ganglia calcification secondary to an underlying cause, in contrast to “Fahr disease,” which has no known etiology. Abnormal calcium is deposited in basal ganglia and subcortical areas. Hypoparathyroidism can cause an imbalance in calcium metabolism and can cause sequestration of calcium in tissues.

Case report: A 49-year-old gentleman presented with a complain of abnormal involuntary dance-like movement with intermittent sustained posturing limbs with twitching of facial muscles for three to four months which had worsened for five days before presentation. History of scrotal swelling presents eight months back, which was treated surgically. The examination was normal except for spasticity and involuntary movements, with a darting tongue present. Tetany was noted while measuring blood pressure.

Discussion: Manifestation of tetany hinted toward hypocalcemia. Very low calcium level in arterial blood gas (ABG) is otherwise normal. Blood investigations revealed low magnesium and low calcium with low parathyroid hormone (PTH). The possibility of a secondary cause was considered. Non-contrast computed tomography (NCCT) head S/O basal ganglia and cerebellar calcification. Contrast enhanced computed tomography (CECT) neck, thorax, and pelvis were performed to R/O parathyroid malignancy, study revealed loculated, pleural effusion with calcified walls, with calcification of walls of a large vessel and dilated esophagus. The patient managed with IV calcium with dramatic improvement.

Conclusions: A diagnosis of true hypoparathyroidism was established in our case. After ruling out possible parathyroid hyperplasia, the patient was managed accordingly. While evaluating for movement disorders, keeping an open mind for any metabolic abnormality is often rewarding.

Abstract ID: 1042: Paroxysmal Kinesigenic Dyskinesia (PKD) – A Rare Case Report and Diagnostic Challenges

Jayaram S, Pushkar Pazhani, Manoj Manyem, Saranya Gomathy, Ramkumar sugumaran, Sunil K Narayan

Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India

Background and Aim: Paroxysmal movement disorders are characterized by episodic and brief involuntary movements that can be inherited or acquired. Paroxysmal kinesigenic dyskinesia (PKD), an autosomal-dominant disorder, manifests as recurrent episodes triggered by sudden movements, presenting with symptoms like chorea, dystonia, and ballismus. PKD’s prevalence is approximately 1 in 150,000.

Case report: A 17-year-old boy from Thiruvannamalai, with no consanguinity in his family, presented with a one-year history of abnormal posturing of limbs and face. The episodes, triggered by sudden movement, involved painless, asymmetric posturing of all four limbs and face, lasting less than 1 min and occurring 10–20 times daily. These episodes, accompanied by slurred speech, were exacerbated by cold environments and preceded by a premonitory sensation described as tightness.

Discussion: Considering stereotypical movements on getting up after prolonged sitting, short duration < 1 min, 10–20 episodes per day, associated with right lower limb dystonia. The patient was diagnosed with PKD. Extensive neurological workup, including serum electrolytes, creatine kinase levels, electromyography (EMG), electroencephalogram (EEG), and magnetic resonance imaging (MRI), was normal. Whole exome sequencing sent for further evaluation. The reports are awaited. The initiation of carbamazepine at a dosage of 200 mg BD led to symptomatic improvement within one week, with a significant reduction in episode frequency at one-month follow-up, and the dose was gradually titrated.

Conclusions: The patient’s clinical presentation, characterized by sudden, stereotypical, short-duration episodes of involuntary movements triggered by sudden movement, aligns with PKD. Continuous follow-up is crucial for differentiating PKD from other similar conditions and understanding the full spectrum of the disease.

Abstract ID: 1043: Efficacy of Edaravone in Amyotrophic Lateral Sclerosis: Case Series of seven Patients

Bharat Sahu, Pranay Gupta, Swapan Gupta, Swapan Gupta

Govind Ballabh Pant Institute of Postgraduate Medical Education (GBPIPMER, New Delhi, India

Background and Aim: Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron degenerative disorder. Edaravone was approved by the FDA to be used in this condition in 2017. This study aims to assess the efficacy of edaravone in ALS patients.

Methodology: ALS patients attending neurology OPD from March 2022 to September 2023 diagnosed using the gold coast criteria were screened for eligibility to receive edaravone injection. Those patients who had scores of at least two points on all 12 items of ALS functional rating scale (ALSFRS)- R score was administered edaravone infusion for six cycles with a first cycle of 14 days with a subsequent cycle of 10 days per month. Intravenous edaravone infusion was given at the dose of 60 mg/day. The primary outcome was an improvement in the ALS-FRS R score by three points from the baseline score after 6 cycles of edaravone therapy.

Results: Out of a total 21 patients screened, seven patients received edaravone injections. Age range was 49–75 years with six males and one female. Three patients had limb onset ALS, and one each had bulbar onset ALS, progressive bulbar palsy, flail arm syndrome, and progressive muscular atrophy, respectively. Three patients completed six cycles of edaravone, while two patients succumbed to their illness during therapy, while two patients dropped out during the study period due to the progressive nature of the disease despite therapy. A single patient with the diagnosis of progressive muscular atrophy had significant improvement with edaravone therapy.

Conclusions: Inj. Edaravone was not found efficacious in the majority of the patients with ALS. More studies are needed to define its role.

Abstract ID: 1044: Multiple Cerebral Infarcts: A Rare and Delayed Presentation of Honey Bee Sting Bite

Chebolu Harshika, Randhi Venkata Venkata Narayana, Amarapu Sravanthi, Puvvada Srikar

Sevenhills Hospital, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Bee stings are commonly associated with local reactions like erythema which are self-limiting while systemic effects like anaphylaxis, rhabdomyolysis, intravascular coagulation, cerebral hemorrhage, acute pulmonary edema, acute renal failure, heart block, Takotsubo cardiomyopathy, and death rarely.

Case report: A 53-year-old woman, known epileptic, presented with a history of being stung by a massive attack of honey bees followed by swelling and itching of face and one episode of generalized tonic-clonic seizure. She was treated with antiepileptics and supportive measures. After 10 h, she presented with weakness of the right upper limb and lower limbs. On examination, the patient was conscious and coherent with stable vitals with erythematous papules on the face and back, Broca’s aphasia, upper motor neuron (UMN) right facial palsy, and right hemiparesis.

Discussion: Routine blood investigations were normal. Magnetic resonance imaging (MRI) of the brain showed acute infarcts in the bilateral fronto-parieto-occipital lobes and basal ganglia, possibly embolic or related to hypoxia. Carotid arterial Doppler studies revealed a floating thrombus in the right common carotid artery. Antinuclear antibodies (ANA) testing was negative. Serum homocysteine levels were at normal limits. A 2D echocardiography revealed no abnormality.

Conclusions: A stroke following a bee sting is very rare but is still a possibility. In cases of multiple bee stings, the patient often becomes unconscious and may not be able to provide a history. In such cases, careful general examination would reveal insect bite marks which would lead to the diagnosis. Awareness of this rare entity can lead to appropriate diagnosis and prevent unnecessary evaluation for the cause of stroke.

Abstract ID: 1045: Untangling a Dilemma!! A Rare Presentation and a Novel Option in Diagnosis for Human Rabies

Sonia Martina, Shree Shruthi Badrinath, Muhammed Shadique, Mohinish S, Jered Livingston, Neeraj Elango, Leema Pauline

Madras Medical College (MMC), Chennai, Tamil Nadu, India

Background and Aim: Rabies is a fatal zoonotic encephalomyelitis transmitted by the saliva of a rabid animal. Two-thirds of human rabies present as furious rabies with agitation, hallucinations, and autonomic hyperactivity, with in-between lucid periods being calm and cooperative. One-third develop paralytic or dumb rabies. Here, we report a case of rabies presenting as new onset refractory seizures being undiagnosed by reverse transcription-polymerase chain reaction (RTPCR) and requiring next-generation sequencing for diagnosis.

Case report: A six-year-old male child who had been scratched over the ear by a stray dog was without bleeding and was diagnosed as category 2 bite for which no immunoglobulin was given and was in the course of anti-rabies vaccine with the fourth dose being given on the day of presentation which is one month after the bite when at 30 min of vaccination child developed generalized seizures and was admitted. The seizures were recurrent requiring multiple anti-seizure medications and were completely normal in between the episodes. On the fifth day of admission, he had behavioral disturbances in the form of sleeplessness and tactile hallucinations. On the sixth day, he developed fever, refractory status epilepticus, and shock. There was no aerophobia or hydrophobia throughout the period. He had a stormy course there on with septic shock and multi-organ dysfunction. He developed liver dysfunction with elevated transaminases and coagulopathy, acute kidney injury, and hemolytic uremic syndrome He had bicytopenia, coagulopathy, hemolysis, and hyper-ferritinemia.

Discussion: His initial computed tomography (CT) and magnetic resonance imaging (MRI) brain were normal. On the eighth day of admission, after the worsening started repeat image showed T2/ fluid attenuated inversion recovery (FLAIR) hyperintensities in bilateral centrum semiovale, corona radiata, bilateral thalamus, lentiform nucleus, dorsal pons, bilateral cerebellar peduncles with restricted diffusion suggestive of brainstem encephalitis/demyelination. Cerebrospinal fluid (CSF) analysis was normal with normal protein and no pleocytosis, viral markers were negative, and CSF and blood culture were negative. Rabies work up with samples from the nape of the neck, and buccal smear and CSF for reverse transcription polymerase chain reaction (RTPCR) were negative. Milwaukee protocol was followed for treating this child in the PICU. He required multiple blood products, IV immunoglobulins, hemodialysis, and plasmapheresis. The child expired after 20 days of admission. Postmortem brain biopsy RTPCR was also negative for rabies as its detection limit is 100 picograms of viral load. Hence, the sample was further subjected to next-generation sequencing. The complete genome of the sequence finally revealed the rabies virus.

Conclusions: Rabies is a fatal zoonotic disease requiring early detection and complete post-exposure prophylaxis where vaccine alone is not sufficient, but immunoglobulin should also be administered based on the category of bite. Apart from usual presentations like aerophobia and hydrophobia, rabies can also present with refractory seizures. The metagenomic next-generation sequencing, a novel option in the diagnosis, is seldom reported, and if the viral load is less than 100 pg, next-generation sequencing may help to diagnose rabies.

Abstract ID: 1046: Wernekink Commissure Syndrome – An Uncommon Presentation of a Rare Entity

Vyom Buch, Thomas Mathew, Vishal Chandra Sharma, Sharath Kumar, Raghunandan Nadig

St. Johns Medical College, Bengaluru, Karnataka, India

Background and Aim: A 68-year-old woman with a medical history of diabetes mellitus and hypertension presented with the sudden onset of dizziness, characterized by a spinning sensation, along with slurred speech and swaying while sitting, standing, or walking. She also noticed weakness and incoordination in her right hand in the form of hand grip weakness and smearing food over her face. There was no history of diplopia, dysphagia, facial deviation or numbness, hearing difficulty, or manipulating food bolus in her mouth.

Case report: Upon examination, her higher mental functions and cranial nerve function, including eye movements, were normal. However, there was generalized hypotonia in all four limbs, with reduced power in the right hand. Sensory examination revealed no abnormalities. Cerebellar function testing demonstrated bilaterally impaired finger-nose and heel-shin tests, with dysdiadokokinesia more pronounced on the right side without any evidence of nystagmus. Truncal ataxia was evident as swaying of the head and body while seated. Her speech exhibited a scanning and spastic quality. MRI of the brain revealed an acute, non-hemorrhagic infarct in the paramedian midbrain tegmentum, more pronounced on the right side.

Discussion: The patient was diagnosed with Wernekink commissure syndrome (WCS), characterized by damage to fibers involved in the decussation of the superior cerebellar peduncles following a caudal midbrain infarction.

Conclusions: This case is noteworthy for presenting only the core features of WCS, namely, dysarthria and bilateral ataxia without any associated eye movement abnormalities, posing a challenge for clinical localization.

Abstract ID: 1047: Late-Onset Recurrent Encephalitis – An Atypical Presentation of Melas (Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-Like Episodes)

Anu Thomas, Praveen Panicker

Government Medical College, Thiruvananthapuram, Kerala, India

Background and Aim: Mitochondrial disorders may resemble vascular, infectious, or autoimmune conditions and are especially challenging when the onset of symptoms is beyond childhood. This is a case report of a young female presenting with recurrent encephalitis-like attacks eventually diagnosed as a case of Melas.

Case report: The data has been collected and compiled after taking due patient consent. A 33-year-old female, born out of a 3rd-degree consanguineous marriage, with normal developmental history, was brought with complaints of recurrent episodes of headache and vomiting followed by generalized tonic-clonic seizures. Her disease started with a bout of fever followed by generalized seizures, which were treated with antibiotics and antiepileptics following which she had behavioral disturbances like irritability and agitation lasting for two more weeks. After this, she improved and was asymptomatic for two years. She had two such similar episodes associated with speech difficulty in the form of naming and word-finding difficulties followed by disorientation and agitation. Imaging revealed multifocal areas of gyral edema and fluid attenuated inversion recovery (FLAIR) hyperintensities in the bilateral parieto-occipital region, with mild diffusion restriction and post-contrast enhancement. Magnetic resonance spectroscopy showed a lactate peak. Serum lactate (74.29 mg/dL) and cerebrospinal fluid (CSF) lactate (5.9 mmol/L) were significantly elevated. Autoimmune and viral panels were negative. Muscle biopsy testing showed evidence of mitochondrial cytopathy. Whole exome and mitochondrial genome sequencing showed evidence of pathogenic variant m.3243A > G in the MT-TL1 mitochondrial gene.

Discussion and Conclusions: This case illustrates Melas as a potential cause for encephalitis even in older age groups.

Abstract ID: 1048: Fundus Never Lies

Ananya Desai, Chithra P. Mukesh

Government Medical College, Thiruvananthapuram, Kerala, India

Background and Aim: Posterior reversible encephalopathy syndrome (PRES) manifests as acute onset neurologic symptoms in the form of encephalopathy, headache, and visual disturbances. This is a rare case of atypical presentation of spinal PRES with retinal detachment and brainstem involvement.

Case report: Case discussion of a rare presentation of PRES. A 34-year-old male presented with headache and blurring of vision for six days and altered sensorium for one day. The headache was bifrontal, continuous with the associated progressive blurring of vision. On presenting to the hospital, Glasgow Coma Scale (GCS) was E3V4M6 with a blood pressure of 230/130 mmHg with visual acuity limited to hand movement perception. Fundus showed evidence of papilledema, macular edema, and serous retinal detachment bilaterally. Deep tendon reflexes were brisk and plantar extensor with no signs of meningism. Computed tomography (CT) Brain showed bilateral symmetrical parieto-occipital hypodensities. The possibility of PRES and cerebral venous thrombosis was considered. Aggressive blood pressure (BP) reduction and anti-edema measures were initiated. Magnetic resonance imaging (MRI) brain + magnetic resonance angiography (MRA) + Magnetic resonance venography (MRV) showed T2/ fluid attenuated inversion recovery (FLAIR) symmetrical hyperintensities in posterior parieto-occipital and high frontal lobes, brainstem as well as in the cervical spinal cord. Retinal detachment was confirmed by ocular ultrasound and optical coherence tomography (OCT). The autoimmune profile was positive. Dramatic improvement in visual acuity was seen within three days with the resolution of findings on MRI.

Discussion and Conclusions: This highlights the fact that conditions as common as PRES can have uncommon manifestations, and a high index of suspicion helps in identifying the same. In this case, fundus examination was the key to identifying non-cortical visual loss.

Abstract ID: 1049: Vessel Wall Imaging in Patients Presenting with First-Ever Stroke with Intracranial Atherosclerotic Disease (ICAD)

Subhra Sen, Uddalak Chakraborty, Soumik Das, Biman Kanti Ray, Atanu Biswas

Institute of Post Graduate Medical Education and Research (IPGMER), Kolkata, West Bengal, India

Background and Aim: Intracranial atherosclerotic disease (ICAD) affects 33–50% of Asians and is the leading cause of stroke globally. Contrast-enhanced vessel wall imaging is almost equivalent to plaque activity, which correlates to ischemic episodes. This study aims to characterize vessel wall architecture and assess plaque activity in intracranial stenosis.

Methodology: This cross-sectional observational study included 20 patients over a period of three months.

Results: The median age of our population was 55, with hypertension (65%) and diabetes (50%) as primary comorbidities. A total of 75% were addiction-free, and tobacco (20%) was the most prevalent addiction. Subcortical involvement was observed in the majority (50%) of patients, while 35% were associated with cortical, internal, and exterior watershed infarcts. The median magnetic resonance imaging (MRI) Alberta stroke program early CT score (ASPECTS) score was 7. Patients exhibited a median of two plaques, with a median occlusion of 70%. The anterior circulation contributed to 95% of plaques, with the internal carotid artery (ICA) contributing to 70% and the middle cerebral artery (MCA) contributing to 55%. The median plaque length measured 7 mm. Post-contrast enhancement was seen in 80% of plaques, while 20% showed signs of intraplaque hemorrhage.

Conclusions: Our findings suggest that contrast-enhancing plaque indicates instability when imaged promptly after a stroke. The findings are consistent with extracranial atherosclerotic plaques, linking contrast enhancement with inflammation and instability. Based on plaque morphology, this model and other similar prospective model might predict culprit plaque, plaque activity, and stroke recurrence risk.

Abstract ID: 1050: Unveiling the Mystery: PPP2R5D Mutation as a Rare Cause of Early-Onset Dystonia-Parkinsonism

Shiny Joy, Divyani Garg, Ayush Agarwal, Achal Kumar Srivastava

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: PPP2R5D spectrum disorder typically manifests in children and is characterized by a range of clinical features, including intellectual disability, neurodevelopmental delay, behavioral disturbances, and autism spectrum disorder. PPP2R5D encodes the delta isoform of the regulatory subunit B56, which is part of the protein phosphatase 2A (PP2A) enzyme complex. PP2A is a serine/threonine phosphatase that plays a crucial role in various cellular processes, including cell growth, division, and signaling pathways. We report a case of adult-onset dystonia-parkinsonism as a clinical phenotype associated with PPP2R5D mutation.

Case report: A 26-year-old male with a history of delayed motor milestones and mild intellectual disability presented with an insidious onset of gradually progressive slowness and stiffness in his left upper and lower limbs over the past two years. It was associated with intermittent posturing and tremulousness of the left hand, with mirror movements in the right upper limb. On examination, he exhibited axial rigidity and asymmetrical appendicular rigidity, left more than right. Action dystonia with dystonic tremor was noted in the left upper limb with mirror movements on the right. Subtle frontal lobe involvement was present on cognitive examination. The rest of the neurological examination was normal. The levodopa challenge resulted in a 10% improvement in the Unified Parkinson’s Disease Rating Scale (UPDRS) motor III score without any dyskinesia.

Discussion and Conclusions: Early-onset dystonia-parkinsonism can be a phenotypic presentation of a PPP2R5D mutation, which is often levodopa responsive as suggested by various case reports (although not seen in our patient). Subtle developmental delay in childhood may provide a clue toward this etiology.

Abstract ID: 1051: MOGAD Disease with an Atypical Neuroimaging

Mandadi Prathyusha, Mandadi Prathyusha, Animesh Das

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Corpus callosum lesions are frequent in primary CNS demyelinating lesions like multiple sclerosis and Susac syndrome, but callosal involvement in myelin oligodendrocyte glycoprotein antibody disease (MOGAD) has not been described.

Case report: A 44-year-old female, with no comorbidities, presented with high-grade fever for an initial period followed by a decrease in vision in both eyes for the last 20 days. Vision loss was acute in onset and associated with pain during eye movements and color desaturation. Both eyes got involved within a span of 1 h and reached a nadir within two days There was no other focal deficit. She has a significant past history of recurrent episodes (approximately ten) of suspected optic neuritis for 20 years involving both eyes, with each episode improving with intravenous and oral steroids by around 50–60%. T2/ fluid attenuated inversion recovery (FLAIR) imaging sequences of the brain revealed multiple T2 flair hyperintense lesions in b/l Periventricular region, corpus callosum, callosal septal interface, B/l middle cerebellar peduncle (MCP), Pons, dentate nucleus, and medulla.

Discussion: Serum myelin oligodendrocyte glycoprotein (MOG) antibodies measured by the indirect immunofluorescence method were positive.

Conclusions: Corpus callosal larger lesions, sagittal midline involvement, and lesion resolution favored MOGAD.

Abstract ID: 1052: A Rare Mimicker of Anterior Horn Cell Disease

Gayathri V S., Chithra P

Government College, Thiruvananthapuram, Kerala, India

Background and Aim: Anterior horn cell disease typically presents with lower motor neuron signs of weakness, wasting, and fasciculations. Muscle diseases and rarely even disorders of neuromuscular transmission can mimic anterior horn cell disease.

Case report: A 52-year-old gentleman presented with distal weakness and wasting of the right upper limb for one year along with right foot drop, followed by similar symptoms in left upper and lower limbs after one year. Two years later, he developed proximal muscle weakness in his lower limbs, which worsened during evening hours. On examination, he had bilateral external ophthalmoplegia, wasting of the thenar, hypothenar muscles, intrinsic muscles of the foot, and distal weakness of all four limbs, with hyporeflexia and fatigability of proximal muscles.

Discussion: The ice pack test and neostigmine test were positive. RNS showed a decremental response and Single-fiber electromyography (SFEMG) showed increased jitter suggestive of myasthenia gravis. AchrAb and Musk Ab were negative and no evidence of thymoma on contrast enhanced computed tomography (CECT).

Conclusions: Distal weakness and early distal wasting are uncommon presentations of myasthenia gravis. This case was an eye-opener to this uncommon presentation and failure to recognize so it could have resulted in branding this patient as having untreatable anterior horn cell disease.

Abstract ID: 1053: Unilateral Motor Band Sign in ZIC4 Positive Paraneoplastic Spastic Syndrome

Kiren Koshy, Asish Vijayaraghavan

Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Thiruvananthapuram, Kerala, India

Background and Aim: Motor band sign refers to a linear hyperintensity in susceptibility weighted imaging (SWI) sequences in the primary motor cortex. A bilateral motor band sign has been described in amyotrophic lateral sclerosis (ALS). We describe a case of progressive unilateral spastic syndrome with contralateral motor band sign.

Case report: A 68-year-old lady presented with progressive spasticity of the left upper and lower limbs for two years. On examination, she had grade 3 spasticity in her left upper and lower limbs with preserved power and needed one person’s support to walk. There was no evidence of apraxia, cortical sensory loss, alien limb phenomenon, or hemineglect on examination. A detailed cognitive examination was normal. The differential diagnoses considered at this point were as follows: 1. hemiplegic variant of primary lateral sclerosis (Mills syndrome), 2. Upper motor neuron (UMN) predominant ALS, 3. corticobasal syndrome.

Discussion: Magnetic resonance imaging (MRI) brain showed subtle atrophy of the motor cortex on the right side with SWI sequences showing linear hyperintensity in the right motor cortex. ASL imaging was normal. The serum paraneoplastic profile showed ZIC4 strong positivity. Cerebrospinal fluid (CSF) study showed two cells, 100% lymphocytes with mildly elevated protein levels (56 mg/dL) and normal glucose. CSF IgG index was normal. Screening for neoplasms [mammogram, CT chest, and abdomen, carcinoembryonic antigen (CEA), alpha-fetoprotein (AFP), cancer antigen 125 (CA125), cancer antigen 19–9 (CA19-9)] was negative. Given the atypical presentation of pure UMN progressive hemiparesis with strong antibody positivity, intravenous immunoglobin (2 gm per kg) was initiated. After the first cycle of IVIG, there was an improvement in spasticity to grade 2, and she could walk without support.

Conclusions: Paraneoplastic neurological syndromes can have varied manifestations and need not always be associated with tumors. A high index of suspicion is required to diagnose these conditions in atypical neurological syndromes.

Abstract ID: 1054: Post Infectious Neuromyotonia with Double Seropositive VGKC (CASPER2 and LGI1) Antibody: Case Report

Sangam Singh, Abdul Qavi, Ajai Kumar Singh, Pradeep Kuamr Maurya, Dinkar Kulshreshtha, Vijay Varman

DR RMLIMS Lucknow, Uttar Pradesh, India

Background and Aim: Peripheral nerve hyperexcitability (PNH) syndrome is a group of disorders ranging from cramp fasciculation syndrome to neuromyotonia. Neuromyotonia is the syndrome of continuous muscle fiber activity that was first described by Isaacs in 1961 (1). Recently, a strong association between S. aureus infections has been linked to PNH (2).

Case report: A 35-year-old male presented with generalized cramps and neuropathic pain, along with abnormal undulating movements of muscles all over his body which present continuously, over large muscles in all four limbs which developed after a week of surgical drainage of pyocele from the left scrotum. Clinical examination did not reveal any focal neurological deficiency other than abnormal non-rhythmic myokymia observed all around the muscle fiber. These symptoms were not associated with stiffness or any encephalopathy.

Discussion: All routine investigations were normal, with mildly raised erythrocyte sedimentation rate (ESR)/ C-Reactive protein (CRP) levels. Routine nerve conduction study (NCS) was normal, but there were after-discharges noted on electrical stimulation of the tibial nerve. Electromyography (EMG) shows the presence of spontaneous discharges in the form of continuous, irregular doublet, triplet, or multiple motor unit discharges [Figures 2 and 3]. Serum samples were positive for both voltage-gated potassium channel (VGKC) (CASPER2/LGI1) antibodies which is very rare. The patient was managed with carbamazepine, pulse steroid, and plasmapheresis, following that his symptoms improved after 4–5 cycles of plasma exchange (PLEX).

Conclusions: PNH is a rare but treatable condition. VGKC antibody testing is recommended, as the majority of patients respond to the immune therapy.

Abstract ID: 1055: Case Report of Rare Neuroimaging Findings in a Patient with Phenytoin Toxicity

Ananya Krosuri, Naveen Kumar Venigella, Krishnaveni Vanapala, Sudheer Penchala Puttiboyina, Nischal Yedla

Kamineni hospital, Vijayawada, Andhra Pradesh, India

Background and Aim: Phenytoin is the most common antiepileptic used in the treatment of seizures. Sodium valproate is an antiepileptic known to cause hyperammonemia alone or in combination with other anti-epileptic drugs (AEDs) like topiramate, zonisamide, carbamazepine, and phenobarbitone. Phenytoin alone producing hyperammonemia and presenting as T1 hyperintensities on neuroimaging is a rare entity.

Case report: A 58-year-old female patient presented to our hospital with complaints of sudden onset confusion for one week. The confusion was associated with a sense of imbalance while walking and a reeling sensation. The patient had a K/C/O seizure disorder since childhood and used to get generalized tonic-clonic seizures once in 15 days and was treated with phenytoin at a dose of 100 mg thrice a day and phenobarbitone 30 mg twice a day but had poor control of seizures with one active seizure in every 20 days for that dose of phenytoin was escalated to 300 mg twice a day. On examination, the patient was awake, following simple commands (Glasgow Coma Scale (GCS)-13-14), and had gaze-evoked nystagmus on both sides, and ataxic gait was present. No meningeal or other lateralizing signs are present. Examination of other systems was normal.

Discussion: Magnetic resonance imaging ( MRI) brain showed bilateral basal ganglia hyperintensities on T1-weighted images with no post-contrast enhancement with discrete T2 and flair hyperintensities. Electroencephalogram (EEG) showed predominant theta activity with a frequency of 5–6 Hz and amplitude of 30–50 mV which is symmetrical. Blood investigations such as complete Blood Picture (CBP), liver and renal function tests, electrolytes, blood sugars, chest X-ray, and electrocardiogram (ECG) were normal. Both serum ammonia (200 mcg/mL) and phenytoin (35.65 mcg/mL) levels were found to be high. Serum calcium, parathyroid hormone (PTH), and ceruloplasmin are normal. Based on the above reports, phenytoin and phenobarbitone were stopped and observed. After four days, serum ammonia levels came to normal, patient’s sensorium improved and was clinically better. Based on clinical history and investigations, a final diagnosis of phenytoin toxicity was performed.

Conclusions: To conclude, any patient presenting with altered sensorium, especially with associated recent initiation or escalation of AEDs, should lead to suspicion of AED toxicity.

Abstract ID: 1056: Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) Presenting as Isolated Brainstem and Cerebellar Syndrome

Anand Singh, Abdul Qavi, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Vijay Verman, Rohit Keshav

RMLIMS, Lucknow, Uttar Pradesh, India

Background and Aim: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a relatively new addition. While patients with multiple sclerosis (MS), MOGAD, and neuromyelitis optica spectrum disorders (NMOSD) may present with similar clinical manifestations, those with MOGAD lack a clear sex predilection and experience a monophasic course.

Case report: A 15-year-old female presented with fever, diplopia, right facial deviation, decreased hearing in the left ear, difficulty in swallowing, imbalance, and recurrent vomiting with hiccups for 2.5 months with worsening since last 15 days. Neurological examination revealed upbeat nystagmus with superimposed horizontal gaze-evoked nystagmus, left eye partial Horner’s syndrome, skew deviation with left eye hypotropia, with left-sided lower motor neuron VII, VIII, IX, X, and XII cranial nerve palsies with left-sided cerebellar signs.

Discussion: Magnetic resonance imaging (MRI) revealed T2/ fluid attenuated inversion recovery (FLAIR) hyperintensities in left middle cerebellar peduncles and adjoining pons, medulla, with minimal diffusion restriction without contrast enhancement suggesting demyelination. Cerebrospinal fluid (CSF) showed lymphocytic pleocytosis. Visual evoked potential (VEP) showed bilateral poorly formed waveforms and pure tone audiometry (PTA) showed left ear profound sensorineural hearing loss (SNHL). Other relevant investigations were negative. The possibility of NMOSD was strongly considered. However, its antibody came negative, but anti-MOG antibodies were significantly positive (titer 1:100). Diagnosis of MOGAD presenting as brainstem/cerebellar demyelination syndrome was performed.

Conclusions: – Isolated cranial neuropathy, or accompanied by central nervous system (CNS) involvement, is an emerging and rare phenotype in MOGAD. This report emphasizes the testing of MOG antibodies in patients with cranial neuropathies.

Abstract ID: 1057: An Unusual Cause of Quadriparesis

Hari Krishna Kambam, Sireesha Yareeda, Reshma Sultana Shaik, Suryaprabha T

Nizam’s Institute of Medical Sciences (NIMS), Hyderabad, Telangana, India

Background and Aim: Mitochondrial cytopathy presents with a myriad of clinical features due to multisystem affliction. We here report a rare variant.

Case report: A 22-year-old lady presented with h/o fever/diarrhea for two days, later developed progressive weakness of both lower limbs (proximal) associated with pains. Two days later, she developed proximal weakness in upper limbs, and two days later, she experienced truncal/neck/bulbar weakness, becoming bed-bound thereafter. There was no associated swelling/redness, no sensory symptoms, no cranial nerve involvement, and no bowel/bladder involvement. She had a h/o exercise intolerance and no family history. On examination, she is conscious and oriented and has bilateral cataracts, corneal opacities, and bilateral ptosis, and the rest of the cranial nerve examination was normal. She had hypotonia in all limbs, with power – 3-/5 proximal in upper and lower limbs, distal – 5/5, deep tendon reflex (DTR) – 1+, and sensory – normal.

Discussion: We considered inflammatory myopathy, mitochondrial/metabolic myopathy, and polyradiculoneuropathy as initial differentials. Labs showed normal hemogram and renal function tests, with serum glutamic oxaloacetic transaminase (SGOT) – 211, Serum glutamic pyruvic transaminase (SGPT) – 250, and serum creatine phosphokinase (CPK) – 2699. Lactate Dehydrogenase (LDH) – 899, pH – 7.2, HCO3 – 20, lactates – 13, PCO2 – 30, thyroid-stimulating hormone (TSH) – normal, HbA1c – 5.1. The vasculitic profile and myositis panel were negative. A 2D echo showed good LV function. Nerve conduction study (NCS) was normal and electro neuro-myography (ENMG) showed myogenic potential. Muscle biopsy showed necrotic fibers with few regenerating fibers and little inflammation.

Conclusions: Mitochondrial cytopathy can have acute presentation.

Abstract ID: 1058: Interesting Cases of Ataxia

Sridevi Natrajan, Prabhu A, Rajasekaran M, Arunraj E, Kishore R

KAPV Government Medical College, Trichy, Tamil Nadu, India

Background and Aim: Ataxia with oculo-motor apraxia type 2 (AOA2) is an autosomal recessive cerebellar ataxia with an age of onset between 10 and 28 years. Patients have associated oculomotor apraxia, neuropathy, head tremor, cerebellar atrophy on imaging, and an elevated α -foetoprotein (AFP) serum level.

Case report: Here, we discuss the clinical, electrophysiological, and genetic findings of two siblings who presented with a clinical picture of AOA type 2.

Discussion: The mean age at disease onset of the elder sister was 21 years, and the younger brother was 19 years. Both of them had gait ataxia initially which progressed slowly over two years, and at the time of presentation, both of them were unable to walk independently. Both had limb ataxia and truncal ataxia, scanning speech. Oculomotor apraxia was present, and gaze-evoked horizontal nystagmus, saccadic pursuit, and slow inaccurate saccades were present in both. Both patients had areflexia and impaired vibration and position sense distally. Head tremor was present in the brother. Cognition was normal. Pes cavus was present in both patients. No telangiectasia was observed. Both of their parents and elder sister had normal neurological examinations. Routine laboratory findings were normal. AFP was raised in both patients. Magnetic resonance imaging (MRI) brain showed cerebellar atrophy in both patients, and nerve conduction study (NCS) showed sensorimotor axonal neuropathy in both patients. Whole exome sequencing showed homozygous SETX mutations in both patients.

Conclusions: Both of these patients represent a classical phenotype of AOA2 with one sibling presenting with head tremor as the first symptom. In autosomal recessive ataxia apart from ataxia with vitamin E deficiency (AVED), AOA2 is also considered in the differential diagnosis of patients presenting with head tremors and juvenile-onset ataxia, as well as sensorimotor neuropathy.

Abstract ID: 1059: MRI Muscles in IIM-Flaw or Forte?

Manne Narendhar, Surya prabha Turaga, Mahesh Bandari

Nizam’s Institute of Medical Sciences (NIMS), Hyderabad, Telangana, India

Background and Aim: Histopathological analysis of muscle biopsies is a cornerstone of diagnosis of muscle disorders. However, sampling error is a common problem. In inflammatory myopathies, false negative results occur in 10–20% of all biopsies due to focal disease activity. A sampling of early or end-stage muscle may result in the absence of disease-specific abnormalities impeding correct diagnosis. Magnetic resonance imaging (MRI) triage can be performed to reduce sampling error by selecting the appropriate muscle.

Case report: A 57-year-old female presented with weakness of both upper limbs and lower limbs for eight months. Creatine phosphokinase (CPK) was elevated, electro neuro-myography (ENMG) showed myogenic potentials, and myositis profile was negative. A muscle biopsy done from the left quadriceps showed lipid inclusions with no evidence of inflammation or myophagocytosis. Workup for metabolic myopathy and mitochondrial genetics sent. The patient was given intravenous immnuoglobulin (IVIg), and she showed improvement. One month later, the patient presented with worsening weakness. Metabolic workup and mitochondrial genetics were negative. MRI muscles showed T2/STIR hyperintensities in the subscapularis and bilateral biceps. A repeat biopsy was performed on the biceps. It showed features suggestive of inclusion body myositis. She was treated with immunosuppressants and is doing well currently.

Discussion: CPK: 10,000 U/L; Myositis panel: negative; ENMG: muscle disease; First biopsy: lipid inclusions with no evidence of inflammation and myophagocytosis; MRI muscles: T2/STIR hyperintensities involving bilateral biceps and subscapularis; Repeat biopsy: S/o endomysial and perivascular inflammation around non-necrotic fibers. Many fibers with single-to-multiple-rimmed vacuoles. Features s/o inclusion body myositis.

Conclusions: MRI has high diagnostic accuracy in diagnosing subacute onset idiopathic inflammatory myopathies (IIM), in particular as an add-on test to the muscle biopsy.

Abstract ID: 1060: Brainstem Tuberculoma Mimicking as Bickerstaff Encephalitis: A Case Report

Swapan Gupta, Kanika Aggarwal

Govind Ballabh Pant Institute of Postgraduate Medical Education (GBPIPMER) New Delhi, India

Background and Aim: Bickerstaff encephalitis (BE) is a rare inflammatory disorder of the central nervous system that manifests as a distinctive triad of symptoms comprising ophthalmoplegia, ataxia, and altered consciousness.

Case report: This is a case of a 12-year-old girl who presented with acute onset progressive bilateral ophthalmoplegia, gait ataxia, and increased irritability without any motor, sensory, and meningeal signs. A provisional clinical diagnosis of brainstem encephalitis was made. She had already done a magnetic resonance imaging (MRI) brain (plain) before presentation which revealed T2/ fluid attenuated inversion recovery (FLAIR) hyperintense signal change at the level of pons and cerebellum. Cerebrospinal fluid (CSF) revealed increased proteins with normal sugar and ten cells with 60% polymorphs. Based on the clinical findings and neuroimaging, Bickerstaff encephalitis was suspected, and the patient was commenced on intravenous immunoglobulin (IVIG) therapy. MRI brain contrast study was planned.

Discussion: Contrast MRI brain revealed a large peripherally enhancing lesion in the pons with diffuse surrounding perilesional edema in the pons, medulla, bilateral cerebellar hemispheres, midbrain, and posterior limb of internal capsule suggestive of a tuberculoma. Subsequently, abdominal tuberculosis and tubo-ovarian abscess were also diagnosed on abdominal and pelvic imaging though she was asymptomatic for the same. The patient responded favorably to anti-tubercular therapy.

Conclusions: Contrast neuroimaging is crucial for the correct diagnosis in brain stem neurological presentations as clinical mimics are common.

Abstract ID: 1061: Tuberculosis Masquerade: Understanding Catatonia as a Presentation of Tuberculosis

Panda Kumar, Zuber Naushadali Shaikh, Rajinder k Dhamija, Suman Khuswaha, Aldrin Anthony

Institute of Human Behaviour and Allied Sciences, Delhi, India

Background and Aim: Catatonia, traditionally linked with psychiatric disorders, is increasingly recognized in the context of medical conditions, including tuberculosis (TB). Despite this, TB-induced catatonia remains poorly understood and underreported.

Case report: This abstract presents a case study of TB-induced catatonia and reviews existing literature to enhance understanding of its clinical presentation and diagnostic challenges. A 16-year-old female was admitted with complaints of fever on and off for one month and acute catatonic symptoms: mutism, immobility, and behavioral abnormalities for two days. On examination, higher mental functions (HMF) could not be assessed, and the patient had generalized rigidity, with waxy flexibility, posturing, and Negativism. The lorazepam challenge test showed mild improvement in symptoms. Provisional diagnosis of catatonia due to chronic meningitis vs autoimmune encephalitis was considered. Investigations revealed normal blood parameters, normal cerebrospinal fluid (CSF) routine and microscopy, negative CSF Autoimmune Panel, and creatine phosphokinase (CPK) within normal limits. But the Mantoux test was positive (22x38mm), and tiny nodules in the left upper and lower lobes of the lung were reported on the contrast enhanced computerized tomography (CECT) chest and abdomen. Treatment with anti-tubercular therapy and lorazepam led to gradual symptom resolution over six weeks.

Discussion and Conclusions: TB-induced catatonia is a rare but important manifestation of TB infection. Early recognition and anti-TB therapy initiation are crucial for favorable outcomes.

Abstract ID: 1063: Tubercular Tongue: A Cryptic Cause of Isolated Hypoglossal Nerve Palsy

Saurabh Mishra, Shreshta Gupta, Aditya Nayan, Manish Modi, Vivek Lal

Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

Background and Aim: Hypoglossal nerve is involved only rarely by tuberculosis. We report a lady whose only evidence of central nervous system tuberculosis was headache and 12th cranial nerve involvement.

Case report: A 47-year-old lady presented with progressively increasing headaches for one month along with speech disturbances and chewing difficulty of two weeks duration. The examination was unremarkable save for left-sided 12th cranial nerve palsy. There were no signs of meningeal irritation. Contrast-enhanced magnetic resonance imaging (CEMRI) of the brain revealed a suspected infiltrative lesion in the left hypoglossal canal. Detailed hemogram, biochemical, and cerebrospinal fluid analysis were normal. Whole body fluorodeoxyglucose positron emission tomography (FDG PET) scan showed an FDG-avid, ill-defined soft tissue lesion along the hypoglossal canal, accompanied by significant abdominal lymphadenopathy. The biopsy from the periportal region revealed the presence of mycobacterium tuberculosis on GeneXpert, which was sensitive to rifampicin. The patient was started on anti-tuberculous therapy, which led to improvement in her headache symptoms.

Discussion: We report a rare case of isolated 12th cranial palsy secondary to tuberculosis.

Conclusions: This case highlights the importance of considering tuberculosis as one of the etiologies of isolated hypoglossal nerve palsy.

Abstract ID: 1064: Diagnostic Enigma in a Girl with Progressive Complex Cerebellar Plus Syndrome

Purushottam Singh, Anirban Gupta

Army Hospital Research & Referral, New Delhi, India

Background and Aim: Neurodegeneration with brain iron accumulation (NBIA) encompasses a diverse group of inherited neurodegenerative disorders marked by extrapyramidal movement disorders and abnormal iron deposits in the brain’s basal ganglia. NBIA disorders are exceedingly rare, affecting fewer than 1 in 1,000,000 individuals. This report details the case of a young girl with a complex presentation.

Case report: A 22-year-old girl presented in April 2024 with cerebellar symptoms, spasticity, parkinsonism, cognitive decline, and behavioral issues. Comprehensive clinical assessments, electrophysiologic studies, neuroimaging, and genetic testing were conducted, with regular follow-ups to monitor symptoms.

Discussion: This 22-year-old girl born out of a non-consanguineous marriage presented with insidious onset gradually progressive ataxia, stiffness in limbs, and mixed dysarthria for the past 2.5 years. She also had mixed cortical and subcortical dementia, mood disturbances, and focal and generalized myoclonus, but there were no symptom fluctuations or sensory/autonomic involvement. Clinical examination showed spastic quadriparesis, appendicular ataxia, parkinsonism, and negative myoclonus. The cognitive assessment indicated multidomain impairment. Differential diagnoses included Wilson’s disease, spinocerebellar ataxia, complicated hereditary spastic paraparesis, NBIA, and mitochondrial illness. Magnetic resonance imaging (MRI) revealed iron deposits in the globus pallidus and substantia nigra and diffuse cerebellar atrophy. Genetic testing identified compound heterozygous mutations in the PLA2G6 gene. Management aimed at symptom relief and complication prevention included syndopa, carbamazepine, antispasticity medications, and regular physiotherapy.

Conclusions: This case enhances understanding of PLA2G6-related neurodegenerative diseases and their heterozygous phenotypic presentations, contributing to the broader knowledge of NBIA disorders.

Abstract ID: 1065: IgG4-Related Hypertrophic Pachymeningitis Responded to Rituximab

Dharmendra Singh

All India Institute of Medical Sciences (AIIMS), Bhopal, Madhya Pradesh, India

Background and Aim: IgG4-related disease (RD) is a multisystemic, fibroinflammatory, and lymphoproliferative disease that is characterized as a polyclonal IgG4-infiltration, leading to storiform fibrosis and obliterating phlebitis. Central neurologic manifestations are rare with reports of mostly HP. Elevated serum IgG4 levels are consistent with IgG4-RD and are present in 70% to 90% of patients. Biopsy material from meninges is the gold standard for diagnosis. There are currently no guidelines about treatment for IgG4- related hypertrophic pachymeningitis (IgG4-RHP). Steroids and immunosuppressants are successfully used. The most promising steroid-sparing agent is rituximab, leading to both reductions of serum IgG4 levels and clinical improvement.

Case report: A 50-year-old female presented with complaints of bilateral progressive hearing loss, headache, and deviation of the mouth toward the right side for the last three years, and difficulty in swallowing for the last one year. She was admitted to a private hospital for these complaints and received mycophenolate mofetil (MMF) 1 gm BD and I/v steroid injection after which she improved. For the last two months, she developed headaches and neck pain. On examination, she had polyneuritis cranialis (bilateral 7th and bilateral 8th and 9th cranial nerve palsy). Lab investigation showed elevated C reactive protein (CRP) (43.01) and serum IgG4 (65.30 gm/dL) levels. Magnetic resonance imaging (MRI) brain reveals pachymeningeal thickening and enhancement in the posterior fossa, causing a mass effect. Based on clinical findings and investigation, a diagnosis of hypertrophic meningitis IgG4-RD was made. The patient was started on I/v steroids and rituximab. Two doses of I/v rituximab 1 gm were given 14 days apart and were discharged with six monthly follow-ups for I/v rituximab. Her cranial nerve palsy completely resolved after two more doses of rituximab.

Discussion and Conclusions: By early treatment with rituximab, we can achieve better neurological outcomes with a lesser relapse rate.

Abstract ID: 1066: Perineural Spread in Rhinoorbitocerebral Mucormycosis: A Case Series

Nikitha Kailas, Umesh Tukaram

Care hospitals, Banjara Hills, Hyderabad, Telangana, India

Background and Aim: Rhino-orbitocerebral mucormycosis associated with COVID-19 (C-ROCM) was rampant in India during the COVID-19 pandemic. Recent studies on imaging of ROCM demonstrated the spread of Mucorales species along peripheral nerves. We aimed to study the proportion of patients with perineural spread, the commonly affected nerve segments, and the symptoms associated with perineural invasion in ROCM.

Methodology: This retrospective study was conducted between March 2020 and June 2021 at a tertiary referral care center in Hyderabad. All the consecutive patients of C-ROCM in the specific period ( n = 31). Diagnosis of mucormycosis was based on clinical and radiological features and microbiological or histopathological findings. Radiological perineural spread is defined as affected, indeterminate, and unaffected.

Results: Of the 31 subjects, eight were excluded due to nonavailability of imaging. Mean (range) age: 50.47 years (range: 25–70); M: F – 20:3; risk factors n (%) – diabetes: 23 (100), chronic renal failure 5 (21.7), severe COVID-19 5 (21.7); proportion of subjects with perineural invasion – 22 (95.65); nerve involvement (affected/indeterminate/unaffected) n (%) – infraorbital V2: 8 (17.4)/4 (8.7), 34 (73.9); V2 at foramen rotundum: 11 (23.9), 5 (10.86), 31 (67.4); V2 at pterygopalatine fossa – 8 (17.4), 10 (21.7), 28 (60.8); V3 – 7 (15.2), 1 (2.17), 38 (82.6). Trigeminal nerve was affected at Mackles cave and root entry zone in one patient each. Involvement of the trigeminal nerve was symptomatic in 16 (61.5) and asymptomatic in 4 (17.4).

Conclusions: Perineural spread is an underestimated mode of spread in mucormycosis. It is seen in more than 90% of the cases. It is seen most commonly in V2 branches, followed by V3. One-third of them could be asymptomatic despite neural invasion.

Abstract ID: 1067: A Study of Clinico-Genetic Spectrum of Myotonia Congenita: An Indian Perspective

Mahammad Mondal, Seena Vengalil, Saraswati Nashi, Kiran Polavarapu, Mainak Bardhan, Deepak Menon, Gautham Arunachal Udupi, Atchayaram Nalini, Faheem Arshad, Keerthana BS, Pallavi Nagaraj Sastry, Suvarna Alladi

National Institute of Mental Health and Neuro-Sciences (NIMHANS), Bengaluru, Karnataka, India

Background and Aim: Myotonia congenita (MC) is a rare hereditary muscle disease caused by CLCN1 gene variants, with limited data on its clinico-genetic spectrum, especially from India. This study aims to elucidate the clinico-genetic spectrum of MC in an Indian cohort.

Methodology: Clinical and investigation data from 2006–2024 were collected for 19 MC patients who underwent whole exome sequencing.

Results: There was a male predominance (89.5%) with a mean age at presentation and onset of 14.4 ± 8.6 and 8.6 ± 6.7 years, respectively. Developmental delay, consanguinity, and positive family history were observed in 31.6%, 42.1%, and 47.4% of patients. Initial symptoms included climbing difficulty (63.2%), limb stiffness (47.4%), walking difficulty (47.4%), and other motor difficulties. Warm-up phenomena were noted in 94.7% and cold-induced exacerbations in 31.6%. Common signs included delayed muscle relaxation (78.9%), muscle hypertrophy (68.4%), Herculean appearance (63.2%), and percussion myotonia (52.6%). Median creatine kinase (CK) was 159.0 U/L (IQR: 106.0-291.0), elevated in 47.4%. Electromyography showed myotonic discharges in all tested patients (7/7,100%). The majority of cases showed autosomal recessive inheritance (AR) (89.5%), with predominantly homozygous (63.2%) and less commonly heterozygous (26.3%) or compound heterozygous (10.5%) mutations. 63.2% of mutations were pathogenic, and 36.8% were likely pathogenic. The mutations were most frequently located in exon 15 (21.1%), followed by exons 16 (15.8%) and 17 (10.5%), among others. The most frequent variants were c.1667T>A, c.1876C>T, and c.2083G>T (p.Glu695Ter), each occurring in 10.5% of cases. Except for muscle hypertrophy and Herculean appearance, more common in AR inheritance ( P < 0.05), other clinical features showed no significant variation between AD and AR inheritances. Most received carbamazepine (52.6%), followed by phenytoin (31.6%); 36.8% had a good response.

Conclusions: This study, being the largest Indian cohort on MC, offers crucial insights for clinicians, enriching their understanding and improving the diagnosis and management of this disease.

Abstract ID: 1068: A Rare Presentation of Glutaric Acid Type 1 in an Adult

Chandrasekhar Enuguri, Divya Nagabushana

MS Ramaiah Medical College, Bengaluru, Karnataka, India

Background and Aim: Glutaric aciduria type 1 (GA1) is an autosomal recessive neurometabolic disorder, caused by a deficiency of the enzyme glutaryl CoA dehydrogenase (GCDH), affecting the metabolic pathway of the amino acid’s lysine, hydroxylysine, and tryptophan.

Case report: A 35-year-old male presented with complaints of persistent headache and blurring of vision for three months. Neurological and systemic examination was normal with visual acuity of 6/12 and normal fundus with macrocephaly.

Discussion: All the routine blood tests were within normal limits. Magnetic resonance imaging (MRI) brain showed chronic subdural cerebrospinal fluid (CSF) fluid in left fronto-parietal convexity. Antinuclear antibody (ANA) IF, ANA PROFILE, human immunodeficiency virus (HIV), venereal disease research laboratory (VDRL), angiotensin converting enzyme (ACE) levels, antiphospholipid antibodies (APLA), serum homocysteine, anti-neuromyelitis optica (anti-NMO), and anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibodies were performed which were negative. Visual evoked potential (VEP) showed increased P 100 latency in the left eye. TMS showed increased levels of glutarylcarnitine and whole exome sequencing showed a pathogenic variant in the GCDH gene glutaric acid type 1.

Conclusions: GA1 is a rare inherited metabolic disorder. It results from a deficiency of the GCDH enzyme, which plays an important role in breaking down certain amino acids. Since a preventive metabolic treatment is available, neurologists must be aware of this rare but likely underdiagnosed presentation, even typical neuroimaging features are not identified.

Abstract ID: 1069: Hydrocephalus in Disguise: A Video Case Report of Atypical Presentation of Tetraventricular Hydrocephalus

Ms. Ishita Gupta, Ali Haider, Siby Gopinath, U. Gopikrishnan, Abhishek Gohel, Balveen Singh, Anand Kumar

Amrita School of Medicine, Kochi, Kerala, India

Background and Aim: Idiopathic fourth ventricular outlet obstruction (FVOO) is a rare cause of tetraventricular hydrocephalus. Symptomatology of idiopathic FVOO ranges from chronic headaches and nausea to symptoms mimicking normal pressure hydrocephalus (NPH). Presentation of FVOO in adults is a rare phenomenon. Axial tonic posturing and stereotyped movements associated with FVOO have not been reported. Aim: To discuss a previously unreported presentation of idiopathic fourth ventricle outlet obstruction causing tetraventricular hydrocephalus.

Case report: We present a video case report of a 45-year-old lady, presenting with a negative effect followed by paroxysmal events characterized by falls and abnormal posturing some of which were preceded by a bout of cough. The use of prolonged video electroencephalogram (VEEG) and magnetic resonance imaging (MRI) was performed to reach the diagnosis of tetraventricular hydrocephalus due to idiopathic FVOO.

Discussion: VEEG revealed habitual events characterized by atonic posturing and stereotyped movements. However, no ictal correlate was found. MRI brain revealed tetraventricular dilatation with no obvious obstruction. Administration of endoscopic third ventriculostomy (ETV) successfully led to immediate resolution of symptoms.

Conclusions: The study broadens our understanding of FVOO symptomatology and will play a part in accurately diagnosing the disorder and preventing its overlooking.

Abstract ID: 1070: Study of the Etiological Profile of Patients with Epilepsia Partialis Continua (EPC) in a Tertiary Care Hospital of North-East India – A Hospital-Based Observational Study

Sumita Kalita

Guwahati Medical College Assam, India

Background and Aim: Epilepsia partialis continua (EPC), narrowly defined, presents as a variant of simple focal motor status epilepticus characterized by persistent, frequent often arrhythmic muscle jerks, over prolonged durations. This condition represents an atypical expression of epilepsy, wherein typical paroxysmal events are partially or entirely supplanted by sustained repetition of seizure fragments in rapid succession. The aim is to evaluate the causes of EPC.

Methodology: This observational study included patients attending neurology OPD as well as patients admitted to neurology and allied departments of Gauhati Medical College and Hospital, Guwahati, Assam, India. The study period spanned from January to December 2023, encompassing a duration of one year. Proper informed consent was obtained from patients, and data pertaining to demographics, clinical presentations, radiological findings, and laboratory profiles were systematically collected using a predetermined format. Clinical assessments included detailed histories and comprehensive neurological examinations. Various investigations like hemogram, fasting and post-prandial blood sugar, HbA1C, fasting lipid profile, serum electrolytes, liver and kidney function tests, thyroid profile, electrocardiogram (ECG), electroencephalogram (EEG), and magnetic resonance imaging (MRI) brain were performed on all patients. However, cerebrospinal fluid (CSF) analysis, antinuclear antibody (ANA) profile, computed tomography (CT) thorax and abdomen (plain and contrast), urine for ketone bodies, and serum paraneoplastic panel were conducted based on clinical indications.

Results: Among the seven patients of EPC in our study, non-ketotic hyperglycemia was identified in 3 (42.8%) cases, while 1 (14.3%) patient presented with a middle cerebral artery (MCA) territory ischemic stroke and 1 (14.3%) with low-grade glioma; Rassmussen’s encephalitis was found in 1 (14.3%) and in 1 (14.3%), and no cause could be found.

Conclusions: Given the refractory nature of seizures in EPC, proactive management of risk factors such as diabetes, dyslipidemia, and hypertension are recommended to mitigate the development of uncontrolled hyperglycemia and stroke, thereby potentially averting the occurrence of EPC.

Abstract ID: 1071: Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Single-Center Case-Series Study in Northeast India

Tanaya Baruah

Guwahati Medical College Assam, India

Background and Aim: Anti-myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an autoimmune-mediated central nervous system (CNS) demyelinating disorder with a broad spectrum of clinical presentation that is often difficult to distinguish from other demyelinating disorders, such as multiple sclerosis and neuromyelitis optica spectrum disorders. The aim is to study the clinical profile and treatment outcome in MOGAD.

Case report: A single-center case-series study was conducted for one-year duration, where detailed history and clinical assessment were conducted for every attack of old and newly diagnosed cases. Inclusion criteria: 1) new cases with typical clinical-radiological manifestations of MOGAD after ruling out secondary causes like infection, other systemic inflammatory or autoimmune disorders, toxins, nutritional causes, hypoxia, and ischemia and 2) serologically positive anti- myelin oligodendrocyte glycoprotein (anti-MOG) antibody test. Exclusion criteria: 1) age more than 60 years, 2) patient testing positive for AQP4-IgG, 3) patients of multiple sclerosis fulfilling 2017 McDonald criteria. All clinical and paraclinical data with treatment outcomes of MOGAD patients were statistically analyzed.

Discussion: A total of nine patients were identified. The mean age at presentation was 25.1 years. The male-to-female ratio was 1:3.5. Most common clinical manifestations were optic neuritis (ON) (66.9%), longitudinally extensive transverse myelitis (LETM) (55.6%), simultaneous myelitis and ON (44.4%), and acute disseminated encephalomyelitis (ADEM) (33.3%). A monophasic course was observed in 44.4%. Intravenous methylprednisolone (IVMP) was administered in 77.8% of attacks, while both IVMP and intravenous immunoglobulin were administered in 22.2%. Rituximab was the first-line maintenance therapy in 44.4%.

Conclusions: MOGAD has a wide spectrum of clinical manifestations. Disease course can be monophasic or relapsing. Further studies are warranted to determine the risk factors of relapse and optimal maintenance therapy.

Abstract ID: 1072: Rhombencephalitis – A Diagnostic Dilemma

Ibtisam Mohammed, Praveen Panicker, Sunil D, Praveen Panicker, Sunil D

Government Medical College, Thiruvananthapuram, Kerala, India

Background and Aim: Rhombencephalitis refers to inflammatory diseases of the brainstem and cerebellum occurring in immunocompetent adults; rarely in immunocompromised.

Case report: A 29-year-old gentleman with a history of systemic lupus erythematosus (SLE), on immunosuppressants; developed acute onset binocular diplopia, unsteadiness, and vomiting followed by dysarthria, dysphagia, and nasal regurgitation. At the presentation, he was conscious, oriented, and afebrile. Neurological examination showed torsional nystagmus, lower motor neuron (LMN) facial palsy, and palatal palsy on both sides with truncal ataxia.

Discussion: Lumbar puncture revealed a cerebrospinal fluid (CSF) white cell count of 10 cells per mm 3 with occasional lymphocytes. CSF glucose was 173 mg/dL with a matched blood glucose of 169 mg/dL, and protein was 159 mg/dL. CSF gram stain was negative. Magnetic resonance imaging (MRI) brain showed T2/FLAIR hyperintensities in the thalamus, brainstem, and cerebellum. Serum neuromyelitis optica (NMO) and myelin oligodendrocyte glycoprotein (MOG) were negative. With a provisional diagnosis of CNS demyelination, treatment with intravenous methylprednisolone was initiated. However, his Glasgow Coma Scale (GCS) worsened. He was subsequently intubated in view of respiratory distress and initiated on plasmapheresis. After two days, the CSF biofire film array meningitis/encephalitis (ME) panel report obtained showed Listeria monocytogenes. He was started on parenteral antibiotic combination therapy with ampicillin and gentamicin. A follow-up MRI scan after the antibiotic regimen showed partial resolution of lesions in correlation with clinical improvement, and subsequently, the patient was discharged on day 30 with no residual neurological deficits. On reviewing history, the patient reported intermittent episodes of abdominal pain and loose stools for three weeks before the onset of symptoms after consuming unpasteurized milk from a street vendor.

Conclusions: Listeria rhombencephalitis affects immunocompetent adults; rarely immunocompromised. This case shows the importance of history, MRI, and CSF polymerase chain reaction (PCR) in the diagnosis of Listeria rhombencephalitis in an immunocompromised patient.

Abstract ID: 1073: ARSACS: Tigroid Appearance of Pons Clue to the Diagnosis of a Rare Autosomal Recessive Ataxia

Moseen Mohd

Andhra Medical College Visakhapatnam, Andhra Pradesh, India

Background and Aim: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a rare inherited neurodegenerative disease due to the SACS gene.

Case report: A 28-year-old boy presented with 22 years duration of insidious onset slowly progressive unsteadiness of gait, followed by seizures and speech disturbances for past two months. On examination, the patient presented with spastic gait ataxia and limb ataxia with spastic dysarthria, horizontal nystagmus, and spasticity in lower limbs with large fiber neuropathy and hammer toes without cranial nerves, autonomic nervous system (ANS), and higher mental function involvement.

Discussion: Blood routines were normal. Magnetic resonance imaging (MRI) brain showed atrophy of the superior cerebellar vermis and T2/FLAIR hypointensities in the pons with a characteristic tigroid, linear, striated, or striped appearance and corpus callosum thinning. Optical coherence tomography (OCT) revealed retinal nerve fibre layer (RNFL) thickening. Nerve conduction study (NCS) showed symmetric demyelinating polyneuropathy. Ultrasonography does not relieve any organomegaly. 2D echo is normal. Thyroid profile, lipid profile, and serum B12 levels are normal. Whole exome sequencing sent revealed a homozygous missense variant in exon 10 of the SACS gene (chr13: g.23339971G>T; Depth: 117x) that results in the amino acid substitution of histidine for proline at codon 1302 (p. Pro1302His; ENST00000382292.9) was detected.

Conclusions: ARSACS is a very rare spastic ataxia paraparesis syndrome. This case report suggests that comprehensive genetic testing is important for the diagnosis of unexplained ataxia, and ARSACS is considered one of the causes of autosomal recessive ataxia.

Abstract ID: 1074: Seizing Insight: A Unique Case of MPAN with Seizure Presentation

Vipin Venugopalan, Gonaboyina Deepak Srinivas, Santosh Kumar Dash, Nikhilsh Pradhan, Pragateeshnu Das, Prerana Dash

Kalinga Institute of Medical Sciences (KIMS), Bhubaneswar, Odisha, India

Background and Aim: Mitochondrial membrane protein-associated neurodegeneration (MPAN) is a subtype of neurodegeneration with brain iron accumulation (NBIA). It is caused by a mutation in the C19orf12 gene. MPAN mostly presents with prominent spasticity, progressive dystonia, neurocognitive decline, psychiatric manifestations, and optic atrophy. There are no reports of seizures in patients with MPAN. Here, we describe a case of MPAN who had a seizure as the first symptom with recurrent ongoing seizure episodes.

Case report: A 23-year-old female presented with multiple episodes of generalized tonic-clonic seizure for six years and a five-year history of progressive spasticity, generalized dystonia, and cognitive decline with optic atrophy. MRI brain showed symmetric T2 and flair hypointensity in bilateral globus pallidus and substantia nigra with isointense streaking of the medial medullary lamina between the hypointense globus pallidus interna and externa and susceptibility weighted imaging (SWI) blooming in globus pallidus and substantia nigra. Electroencephalogram (EEG) showed intermittent generalized polyspike and wave discharges. Whole exome sequencing showed pathogenic autosomal recessive compound heterozygous C19orf12 gene mutation suggestive of NBIA–MPAN.

Discussion: Our patient had recurrent seizure episodes which were confirmed by EEG showing intermittent generalized polyspike discharges. This is not a common feature in MPAN compared to other types of NBIAs according to available case reports and review articles.

Conclusions: Seizures are uncommon in MPAN according to available literatures, although it is common in PLAN (PLA2G6-associated neurodegeneration) and beta-propeller protein-associated neurodegeneration (BPAN). Further studies are needed to understand the incidence of seizure in MPAN and its impact on disease progression.

Abstract ID: 1075: Unfelt Pain, Visible Scars: A Case of Congenital Insensitivity to Pain and Anhidrosis (CIPA)

Vipin Venugopalan, Ganaboyina Deepak Srinivas, Santosh Kumar Dash, Rajalaxmi Satapathy

Kalinga Institute of Medical Sciences (KIMS), Bhubaneswar, Odisha, India

Background and Aim: Congenital insensitivity to pain and anhidrosis (CIPA) is characterized by decreased perspiration (despite normal sweat glands), insensitivity to pain, and mental retardation. CIPA patients are insensitive to pain and unable to distinguish even extreme temperatures. The ability to feel all pain (including visceral pain) is lacking, leading to repetitive injuries including self-mutilation like biting fingertips, tongue, and lips, leading to bruising, scarring, dermatitis, and multiple fractures. CIPA is an extremely rare disorder caused by an autosomal recessive mutation in the NTRK1 gene.

Case report: A 2.3-year-old female child presented with a nine-month history of self-mutilating behavior like biting her fingertips, tongue, and lips, impaired temperature perception, and anhidrosis with multiple ulcers in her hands and feet. Examination revealed ulcers over the lips, finger toes and sole of the foot, absent deep tendon reflexes (DTRs), down-going plantar, and impaired pain and temperature sensations. Baseline investigations like complete blood pictures and electromyography showed no positive finding. The nerve conduction study done in all four limbs was normal. Genetic testing revealed a pathogenic mutation in the NTRK1 gene in ch.1q.

Discussion: Our patient clinically fits under CIPA which was confirmed with genetic testing showing a mutation in the NTRK1 gene.

Conclusions: CIPA underscores the importance of genetic testing in cases of unexplained sensory and autonomic neuropathies. Timely recognition can guide supportive care, optimize quality of life, and potentially mitigate complications. The diverse clinical spectrum of CIPA highlights the necessity of multidisciplinary approaches in its management, encompassing neurological, genetic, and rehabilitative interventions to address its progressive nature and improve patient outcomes.

Abstract ID: 1076: A Man Who Cannot Blink: Courtesy: Demon from the Past

Meet Vyasa, Arvind Sharma, Bhargesh Bhadrayu Patel

Zydus hospital Ahmedabad, Gujarat, India

Background and Aim: Syphilis is an infectious disease caused by the spirochete bacteria Treponema pallidum ( T. pallidum ). The term neurosyphilis refers to an infection involving the central nervous system (CNS). Five types of neurosyphilis exist, which range from 1. the early forms – asymptomatic, 2. meningeal (presenting <1 year from infection), 3. meningovascular (presenting at 5 to 12 years from infection), 4. the late or tertiary forms – general syphilitic paresis, 5. tabes dorsalis (presenting at >10 years from infection). It can occur decades after the initial inoculation. Ocular and auditory symptoms may also occur at any stage of infection and are considered forms of neurosyphilis. The diagnosis of symptomatic neurosyphilis requires meeting clinical, serologic, and cerebrospinal fluid (CSF) criteria. The diagnosis of asymptomatic neurosyphilis relies on serologic and CSF criteria alone. In the last several decades, a persistent rise in syphilitic meningitis and other forms of early neurosyphilis have been seen in the human immunodeficiency virus-positive population, principally in men who have sex with men.

Case report: Young adult male with bilateral facial palsy and positive for neurosyphilis.

Discussion and Conclusions: Facial palsy can be a symptom of neurosyphilis, which can occur in the secondary or tertiary stages of the infection. The acute meningitic and meningovascular forms of neurosyphilis can cause cranial nerve dysfunction, especially of the facial nerve. When this happens, patients may experience facial paralysis, sensorineural hearing loss, and vertigo.

Abstract ID: 1077: India’s First Genetically Confirmed Case of Spinocerebellar Ataxia (SCA 48) with a Novel Mutation and Distinct Phenotype: From Rarity to Reality

Vipin Venugopalan, Ganaboyina Deepak Srinivas, Santosh Kumar Dash, Nikhilesh Pradhan

Kalinga Institute of Medical Sciences (KIMS), Bhubaneswar, Odisha, India

Background and Aim : Spinocerebellar Ataxia-48 (SCA 48), last from the list of SCA, is an autosomal-dominant disorder, that typically manifests in mid-adulthood with symptoms like ataxia, cognitive impairments, and extrapyramidal features. It is particularly noted for its cognitive impairments, psychiatric disturbances, and extrapyramidal features apart from ataxia. Diagnosed through whole exome sequencing after standard tests failed, this case reveals a unique lysine substitution mutation in the STUB1 gene.

Case report: A 54-year-old man with a family history of ataxia presented with a two-year history of progressive gait ataxia, slurred speech, and difficulty with daily activities. Clinical findings included normal higher mental function, scanning speech, hypometric saccades, and gaze-evoked nystagmus, brisk deep tendon reflexes, normal sensory examination, including posterior column sensations. MRI revealed diffuse cerebellar atrophy with prominent vermian and superior cerebellum involvement. Genetic testing for common SCA subtypes was negative. Whole exome sequencing identified a heterozygous inframe indel variant, c.426_428delGAA, in Exon 3 of the STUB1 gene, indicating SCA48.

Discussion: Even though SCA48 is noted for its cognitive impairment and extrapyramidal features, our patient did not develop these features till now. But he can develop these features over time, and hence is on periodic follow-up.

Conclusions: The discovery of a novel mutation in the STUB1 gene associated with SCA 48 in this Indian case adds valuable knowledge to the field, emphasizing the need for genetic diversity considerations in the diagnostic process and offering a new avenue for research into the pathophysiology and management of SCAs.

Abstract ID: 1078: An Interesting Case of COL4A1 Hereditary Small Vessel Disease

Athira T K, Chithra Pushpa Mukesh, Praveen Panicker

Government Medical College, Thiruvananthapuram, Kerala, India

Background and Aim: A 40-year-old male with no prior comorbidities presented with giddiness followed by weakness of the left side of the body with swaying toward left side while walking and numbness of left foot and hand associated with slurring of speech for two days. There was no history of loss of consciousness, alteration in sensorium, or vomiting. At the time of presentation, his blood pressure was 210/120 mmHg; his left upper limb and lower limb had grade 4/5 power with left-sided plantar extensor and left-sided cerebellar signs.

Case report: Magnetic resonance imaging (MRI) brain showed T2/ fluid attenuated inversion recovery (FLAIR) hyperintense lesions in bilateral periventricular and deep white matter with enlarged periventricular spaces and lacunes. The left periventricular region showed cerebrospinal fluid (CSF) intense lesion predominantly involving the basal ganglia with hemosiderin deposition in the periphery s/o cystic encephalomalacia with multiple bilateral cerebral microbleeds and focal bleed in the pons.

Discussion and Conclusions: As our patient presented with this phenotype had radiological findings of leukoaraiosis, microhemorrhages, and porencephalic cyst in the left basal ganglia and lacunar infarct, a high index of suspicion may be required for diagnosis which is further confirmed by whole exome sequencing.

Abstract ID: 1079: The Proportion of Sensorineural Hearing Loss in Individuals with Dementia in a Tertiary Healthcare Center in South Kerala

Sneha Baby, Avinash Mohan, Leena Jayakar

Dr. SMCSI Medical college, Thiruvananthapuram Kerala, India

Background and Aim: One of the important causes of morbidity among geriatric community is dementia, and there is a recent concept that age-related hearing loss is one of the modifiable biomarkers of non-vascular dementia. This study focuses on the proportion of sensorineural hearing loss among individuals diagnosed with dementia in a tertiary healthcare center in south Kerala.

Methodology: Consecutive sampling method was used. Patients coming to the neurology OPD were diagnosed with dementia using mini mental state examination (MMSE), and Addenbrooke cognitive examination score underwent pure tone audiometry or brainstem evoked response audiometry (BERA); this helped to find the proportion of hearing loss.

Results: The study conducted showed that there are 11 patients with mild hearing loss (28.9%) and two patients (5.3%), 1 patient (2.6%), 2 patients (5.3%) with moderate, moderately severe, and profound hearing loss, respectively, in the right ear. There are 12 patients (31.6%) with mild hearing loss and four patients (10.5%) with moderate and moderately severe hearing loss in the left ear respectively among a total of 38 participants.

Conclusions: Underlying mechanisms as to why this happens need to be studied, all the while finding more data to warrant more clinical studies on the reduction of incidence of dementia by treating the age-related hearing loss that may otherwise go unnoticed.

Abstract ID: 1080: An Atypical Case of Disabling Progressive Weakness: From Motor Neuron Disease to Nodopathy

Ishu Goyal, Arun B. Shah

HN reliance foundation hospital, Mumbai, Maharashtra, India

Background and Aim: To describe an unusual case of subacute progressive bilateral proximal lower limb weakness with fasciculations with positive family history.

Case report: Detailed history and examination were conducted at multiple points during the disease. She was thoroughly evaluated according to her symptomatology but remained in a diagnostic dilemma. A 63-year-old lady, recently diagnosed with diabetes mellitus, presented with left lower limb proximal weakness since January 2024, which progressed gradually to a power of 1/5 and involved the right proximal lower limb by April. At this time, she also developed fasciculations in both arms. She had a positive family history of some undiagnosed motor weakness with atrophy in her aunt and cousin’s brother. Initial nerve conduction velocity (NCV) in March showed multiple motor axonal neuropathy in the left leg which by April showed bilateral worsening with both active and chronic denervation in cervical and lumber segments, suggesting a possibility of motor neuron disease. MRI lumbo-sacral spine with plexus, antiganglioside antibody panel, serum protein immunofixation electrophoresis, and positron emission tomography (PET) scan was negative. Cerebrospinal fluid (CSF) analysis showed albumino-cytologic dissociation. However, SOD 1 mutations and neurofascin 180 and 146 were positive. Initially considering possible post-viral plexopathy, immunoglobulin therapy was given, but the disease worsened, and pulse steroid therapy was administered a month later without any benefit.

Discussion and Conclusions: Neuromuscular junction disorders may sometimes pose a diagnostic dilemma. Although newer investigations aid the diagnosis, their specificity for the disease may vary and hence should be interpreted in the light of clinical impression.

Abstract ID: 1081: Stroke of Cleanliness: Household Product Causing Acute Bilateral Embolic Infarcts

Gonaboyina Srinivas, Vipin Venugopalan, Santosh Kumar Dash, Pragateshnu Das

Kalinga Institute of Medical Sciences (KIMS), Bhubaneswar, Odisha, India

Background and Aim: Hydrogen peroxide (H 2 O 2 ), valued for its antimicrobial properties, is commonly used in households for wound cleansing, oral hygiene, etc. While low concentrations are safe, higher concentrations—industrial – grade (33–35%), and recently, “food grade hydrogen peroxide” (33–35%) marketed for “hyper-oxygenation therapy”—increased its availability, leading to accidental and intentional ingestions. High-concentration ingestion can cause severe complications, including gastric rupture, enteric necrosis, and central nervous system (CNS) acute gas embolism.

Case report: A 48-year-old male poultry worker with no known comorbidities presented to the emergency, following the accidental ingestion of 60 mL of 50% w/w H 2 O 2 (floor disinfectant). The patient was admitted to the Department of General Medicine and treated symptomatically. Twelve h post-admission, a neurology consultation was sought due to deteriorating sensorium. On examination, he was lethargic with asymmetric quadriparesis.

Discussion: An immediate non contrast computed tomography (NCCT) brain revealed acute bilateral high frontal hyperintensities, leading to the diagnosis of acute embolic stroke. Subsequent magnetic resonance imaging (MRI) of the brain and computed tomography (CT) angiography confirmed the same with no evidence of thrombus. The final diagnosis of ischemic stroke – hydrogen peroxide-induced acute air embolism was made. The patient received symptomatic care due to nonavailability of hyperbaric oxygen. Sensorium improved within three days and motor strength improved from 0/5 on the right side and 2/5 on the left side to 4/5 bilaterally over the following 3 weeks.

Conclusions: This case underscores the importance of considering gas embolism in cases presenting with acute bilateral embolic phenomena, particularly in patients without traditional risk factors and negative diagnostic workups as a widespread increase in its availability and use.

Abstract ID: 1084: Acute Cerebellar Syndrome in Ross Syndrome: A Case Highlighting the Importance of Thermoregulatory Awareness

Arunav Garg, Akhil Sahib, Astha Bansal, Deepti Grover, Debashish Chowdhury

Govind Ballabh Pant Institute of Postgraduate Medical Education (GBPIPMER), New Delhi, India

Background and Aim: Ross syndrome is a rare disorder characterized by a triad of segmental anhidrosis, areflexia, and tonic pupil. Acute cerebellar syndrome (ACS) can arise from various etiologies, including vascular events, infections, metabolic imbalances, and toxins. This case report describes an unusual presentation of ACS in a patient with Ross syndrome triggered by heat stroke, highlighting diagnostic challenges and management strategies.

Case report: A 54-year-old female with type 2 diabetes mellitus presented with acute onset of slurred speech, imbalance while walking, and transient loss of consciousness after exposure to extreme heat during a religious visit. Detailed history, clinical examination, and investigations were conducted.

Discussion: The patient was diagnosed with heat stroke leading to ACS. Diagnostic criteria included elevated body temperature (>40°C/105°F), central nervous system (CNS) dysfunction, and a history of extreme heat exposure. This diagnosis was supported by exposure to extreme heat, high-grade fever, and acute cerebellar features. Predisposing factors included uncontrolled diabetes, obesity, and sweat gland dysfunction secondary to newly diagnosed Ross syndrome. Ross syndrome was confirmed by sweat testing (starch iodine test) revealing segmental anhidrosis, dilute pilocarpine test showing tonic pupils, and demonstration of areflexia. Various investigations, including magnetic resonance imaging (MRI) brain, vitamin B12, thyroid function test (TFT), toxin screening, and cerebrospinal fluid (CSF) analysis, ruled out other causes of ACS. Treatment included rehydration, cooling, and thiamine supplementation. The patient showed significant improvement, was discharged healthy, and was advised to avoid excessive heat exposure.

Conclusions: This case underscores the importance of recognizing heat stroke as a potential cause of ACS, particularly in patients with conditions like Ross syndrome that impair thermoregulation. Awareness and preventive strategies are essential to avoid heat-induced neurological emergencies. Early diagnosis and prompt treatment are crucial for recovery.

Abstract ID: 1085: When CIDP Is Not Just CIDP: Exploring a Rare Possible Nodopathy Case with Significant Nephropathy

Milan Mori, Sagar Chaudhari, Archana Kumari, Jyoti Garg, Ashish Duggal

ABVIMS& Dr RML Hospital, New Delhi, India

Background and Aim: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated disorder affecting the peripheral nerves, characterized by progressive weakness and sensory dysfunction in the limbs. Only 1–2% of all CIDP cases can be attributed to the spectrum of nodopathy or paranodopathy with specific autoantibodies, such as those against Contactin-1 and Neurofascin-155.

Case report: A 62-year-old female patient presented with symptoms of acute-onset areflexic, flaccid quadriparesis (proximal more than distal weakness in both upper limb (UL) and lower limb (LL)), pins, and needle-like sensations in both UL and LL with severe sensory ataxia, reaching nadir over 10–15 days. Her medical research council (MRC) sum score was 28.

Discussion: She was initially suspected of Guillain-Barré syndrome (GBS) and received intravenous immunoglobulin (IVIG). Only transient improvements were observed, and over the next 2 months, she had three treatment-related fluctuations while on IVIG therapy and was later diagnosed with CIDP based on history and nerve conduction study (NCS) findings. The patient was having significant nephrotic range proteinuria. Despite the initial response with methylprednisolone (MPS) pulse therapy, it was not well sustained on oral steroids, and she was given rituximab with maintenance pulse steroid at progressively increasing intervals. Nephrotic range proteinuria resolved completely with improvement in the modified Rankin Scale (mRS) scale from 4 to 1.

Conclusions: This case underscores the importance of considering rare subtypes like nodopathy/paranodopathy in the differential diagnosis of CIDP, particularly in patients presenting with atypical features. It also emphasizes the need for awareness of these rare entities for appropriate therapeutic strategies.

Abstract ID: 1086: The Body’s Betrayal: An Interesting Case of Autoimmune Autonomic Ganglionopathy

Milan Mori, Arnesh Bhattacharya, Naresh Chinthala, Jyoti Garg, Ashish Duggal

ABVIMS& Dr RML Hospital, New Delhi, India

Background and Aim: Autoimmune autonomic ganglionopathy (AAG) is a rare disorder characterized by autoantibodies targeting the autonomic ganglia, leading to widespread autonomic dysfunction. Patients typically present with symptoms such as orthostatic hypotension, dry mouth, dry eyes, urinary retention, gastrointestinal dysmotility, and anhidrosis. The condition is often associated with antibodies against the ganglionic acetylcholine receptor (AChR). Diagnosis can be challenging, requiring a combination of clinical evaluation and specialized antibody testing.

Case report: A 65-year-old male patient presented with progressive autonomic dysfunction over 3–4 months. Symptoms included severe dryness of the mouth and eyes, difficulty chewing and swallowing, weight loss, urinary hesitancy, constipation alternating with diarrhea, reduced sweating, and postural lightheadedness. Physical examination was unremarkable except for significant postural hypotension.

Discussion: The patient was investigated thoroughly, including all routine blood investigations and specific tests for autoimmune and paraneoplastic antibodies (antinuclear antibody (ANA)/ extractable nuclear antigen (ENA), vasculitis profile, paraneoplastic antibody panel), all which returned negative. Cerebrospinal fluid (CSF) analysis was normal. Imaging studies, including chest x-ray (CXR), abdominal ultrasound, and positron emission tomography-computed tomography (PET-CT), were performed to rule out malignancy. The patient underwent detailed autonomic function tests which confirmed the extent of autonomic dysfunction (Parasympathetic >> Sympathetic). Given the clinical presentation and nonavailability of antibody testing, the diagnosis of AAG was considered, and the patient was treated with IVIG. Significant improvement was noted as the composite autonomic symptom score (mCOMPASS) score improved from 67 to 30, with maximum benefit noted in secretomotor and gastrointestinal (GI) symptoms.

Conclusions: This case underscores the importance of a thorough diagnostic workup and the potential effectiveness of immunotherapy in managing suspected AAG.

Abstract ID: 1087: Neuronal Activity in Patients of Pain-Predominant Medically Unexplained Physical Symptoms

Chaithra Rajanna, Anu Gupta

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Pain is the most common physical complaint with which patients present to a medical facility. Among those patients presenting with pain, up to a third remain unexplained even after thorough investigation. The lack of diagnosis of these conditions perhaps reflects our limits of medical knowledge and available technology. Our study aimed at studying the neural activity in patients of pain-predominant motor unit potential (MUPS) using resting-state functional magnetic resonance imaging.

Methodology: A total of 20 patients with multiple unexplained pain symptoms were included after screening. The study was conducted in a tertiary center in North India. All participants were subjected to functional magnetic resonance imaging (MRI) at baseline and 6 weeks of standard-of-care treatment. Changes at both baseline and after 6 weeks of treatment were analyzed and studied. Changes in the default mode network, salience network, and sensory-motor network were evaluated.

Results: There were few variations observed in the seed-based connectivity and ROI-to-ROI connectivity of patients with MUPS with respect to the normal population. There was a significantly increased connectivity observed in default mode network (DMN), sensorimotor network (SMN), and salience networks in patients of MUPS at baseline.

Conclusions: This study makes way for future research into the pathogenesis and effective management strategies, for which neuroimaging techniques might be of tremendous benefit.

Abstract ID: 1088: A Case Report of Takayasu Arteritis Masquerading as Meningitis

Koppolu Pranathi, Anu gupta, Meena Chandu

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Takayasu arteritis (TAK) is a systemic vasculitis involving large arteries. This occlusive thrombus aortopathy lacks pathognomonic features, often resulting in a diagnostic dilemma leading to its under-recognition, misdiagnosis, and delayed management. Reports of direct central nervous system (CNS) involvement are not uncommon in TA, altered sensorium as an initial clinical presentation is extremely rare in the literature. Herein, we describe a case of altered sensorium in a TAK patient that mimicked meningitis.

Case report: A 29-year-old female presented to us with an altered sensorium and headache to emergency with a background history of epistaxis, hemoptysis, polyarthralgia, and eye pain for 2 years. She was intubated in the ward i/v/o poor Glasgow coma scale (GCS) for airway protection. Non-contrast computed tomography (NCCT) head showed diffuse leptomeningeal enhancement with sulcal effacement. The initial working diagnosis was infective vs inflammatory meningitis. She was treated on the same lines with anti-tubercular therapy, antibiotics, and steroids empirically. Further examination revealed absent left-sided radial pulse and brachial pulse, and there was discordance in blood pressures in bilateral upper limbs, bruit present over left-sided carotid. Computed tomography (CT) aortogram revealed diffuse stenosis of the left proximal subclavian artery, with the bilateral common carotid artery and the pulmonary artery. Based on physical and radiological findings, we reached a diagnosis of Takayasu arteritis. Magnetic resonance imaging (MRI) was performed which showed a minor infarct in the left centrum semiovale. The cerebrospinal fluid (CSF) examination was normal for infective workup. CT abdomen revealed hepatosplenomegaly, which is also a rare finding in Takayasu arteritis. The diffuse leptomeningeal enhancement was due to diffuse leptomeningeal collaterals causing sulcal effacement.

Discussion: She was started on steroids, intravenous (IV) dexamethasone 0.4 mg /kg, with treatment patient improved clinically and symptoms resolved over 3 weeks, and she was started on mycophenolate mofetil (MMF) 3g for long-term immunosuppression and is currently doing well on treatment.

Conclusions: In view of this, physicians should strive to take a detailed history and perform thorough physical examinations to timely pick the characteristic signs of TA, especially in patients presenting with unanticipated symptoms, to also consider diffuse collaterals which can radiologically mimic leptomeningeal enhancement, in patients often presenting with altered behavior with headache.

Abstract ID: 1089: Evaluation of Factors Responsible for Progression in a Cohort of Hirayama Disease

Yamini Priyanka PentaKota, Vishnu VY, MV Padma Srivastava, Rohit Bhatia, Ajay Garg, Saranya B Gomathy, Ayush Agarwal, Alisha Reyaz, Tanveer Ahmed

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Hirayama disease is a rare, nonfamiliar, monomelic amyotrophy originally described in 1959. The classical findings are pure motor lower motor neuron (LMN) weakness involving unilateral or asymmetric bilateral forearms and hands. This plateaus after a few years. It causes significant disability in some patients who can benefit from timely surgical intervention. Aim – To assess the frequency of Hirayama in patients presenting with focal upper limb amyotrophy and to assess factors contributing to progression.

Methodology: It is a single-center prospective observational study planned between November 2022 to November 2024. All patients presenting to OPD fulfilling the inclusion criteria will be enrolled and assessed every 6 months. Motor system examination with modified MRC scale and dynamometry and functional scales like overall neuropathy limitations scale (ONLS), Rasch-built overall disability scale for multifocal motor neuropathy (MMN RODS), and amyotrophic lateral sclerosis functional rating scale (ALS FRS) are applied to assess patients periodically every 6 months. A total of 28 patients were followed up till 1 year.

Results: A total of 88 patients were screened. Out of these, 75 patients (85%) met the diagnostic criteria. Males were 96%. The mean age was 22.5 years (SD: 7.634). The average age at symptom onset was 17.8 years (SD: 7.23). The symptoms started in the left upper limb in 44% and the right upper limb in 42% with eventual asymmetric bilateral involvement in 68.5%. Family history was positive in two patients. A total of 79% of the patients were found to be historical progressors at the time of enrolling. Baseline ONLS, MMN RODS, and ALS FRS were 1.772 (SD 0.784), 33.4 (SD 7.231), and 44.8 (SD 1.22). The average power in the worst limb by manual and digital dynamometry was 29.58 (SD: 23.5) and 32.153 (SD: 26.42) pounds, respectively. A total of 77% had MRC grade 4 in the worst limb. The disability is mild in 78.5% and severe in 3.2%.

Conclusions: Timely diagnosis based on clinical criteria and dynamic MRI and surgical intervention when needed can halt progression.

Abstract ID: 1090: Opsoclonus Myoclonus Ataxia Syndrome: Case series: Illustrating Diverse Causes, Treatments, and Outcomes

Dhiraj Kumar, Neha Pandey, Himanshu Chauhan, Gutti Nagendra Babu

Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGI), Lucknow, Uttar Pradesh, India

Background and Aim: Opsoclonus myoclonus syndrome (OMS) is a rare, with an incidence of 1 in 10,000,000 annually. It is characterized by involuntary, saccadic eye movements opsoclonus, ataxia gait incoordination, rigidity, and tremor. A significant percentage of OMS cases have underlying malignancy, especially neuroblastoma or small-cell lung cancer (SCLC) which is its non-metastatic manifestation. OMS is also associated with para infectious, postpartum, or idiopathic. OMS is rare in adults and exceedingly rare in postpartum.

Case report: Four case series of opsoclonus myoclonus syndrome in different age group and associated with four different causes. All the cases were admitted to our hospital, where first case was a female aged 31 years who presented with a constellation of symptoms of OMS. Second case is a 2-year-old child who developed OMS at the age of 1 year. Third case is a 4-year-old child previously diagnosed with a case seizure disorder and was diagnosed as OMS. Fourth case is a 1-year-old female child who developed myoclonus at 9 months of age, followed by jerky eye movements.

Discussion: First case is a 31-year-old female with OMS after 5 months of pregnancy, after febrile episode for 10 days. All the investigations came negative. She was diagnosed with parainfectious OMS. Second case is a 2-year-old child who developed OMS at the age of 1 year on evaluation anti-GADab positive. Third case is a 4-year-old child a case of seizure disorder. The child was diagnosed as OMS and full workup negative and diagnosed as idiopathic OMS. Fourth case is a 1-year-old female child with myoclonus at 9 months of age, followed by jerky eye movements, on evaluation had D5 to D8 ganglioneuroma.

Conclusions: OMS can be due to malignancy, parainfectious, paraneoplastic, autoimmune, or idiopathic, identifying the cause is most important, and treatment outcome is variable.

Abstract ID: 1091: Familial Spastic Ataxia Transpired to be Cockayne Syndrome

Ammanabrolu Sreekavya, Seepana Gopi, Talabhaktula Sateesh Kumar, Seepana Gopi, Talabhaktula Sateesh Kumar, Talabhaktula Sateesh kumar

Andhra medical college, KGH, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Cockayne syndrome is a rare autosomal recessive genetic disorder, resulting from a DNA repair defect, leading to augmented sensitivity of cells in affected individuals to the lethal effects of ultraviolet (UV) light.

Case report: A 12-year-old boy, born out of third-degree consanguineous parentage, with uneventful antenatal and birth history, with a history of neonatal jaundice and feeding difficulties, language milestones attained as per age, presented with delayed motor milestones, difficulty in walking in the form of dragging left foot and crisscrossing of legs while walking, tripping attacks and swaying while walking, that aggravates while passing through a narrow passage, 1 year duration of tremulousness of both upper limbs while reaching the object, bringing food bolus toward the mouth, holding objects, tremulousness of both lower limbs while reaching footwear, tremulousness of head while reaching food bolus, undue stressing while speaking, all symptoms being progressive with similar complaints in younger sister. On examination: Short stature with wide eyes, beaked nose, high arched palate, prominent ears, grade1+ spasticity in both lower limbs, power-normal, bilateral plantar extensor, with knee and ankle clonus, bilateral gaze-evoked nystagmus with Staccato speech, impaired finger-to-nose test (FNT), finger to finger to nose test (FFNT), kneel heel test, toe-tapping test, tandem walk, with dysdiadochokinesia, decomposition, scissoring gait with gait ataxia.

Discussion: Blood routines were normal. Viral screening was negative. Nerve conduction study (NCS)-normal, visual evoked potential (VEP)- prolonged on both sides, Brain stem evoked response audiometry (BERA)- normal, and magnetic resonance imaging (MRI) brain-periventricular hyperintensities were noted. Genetic analysis-homozygous mutation of ERCC6 gene suggestive of Cockayne syndrome type B.

Conclusions: The management of Cockayne syndrome is to treat symptoms and additional supportive measures like sun protection for eyes and skin to prevent malignancies and nutritional support.

Abstract ID: 1092: Novel Noteworthy Cause of Distal Weakness – ADSS1 Myopathy

Kotha Aravind, Seepana Gopi, Talabhaktula Sateesh kumar

Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Distal myopathy-5 also, named adenylosuccinate synthetase 1 (ADSS1) myopathy, is an autosomal recessive, slowly progressive muscular disorder, predominantly affecting lower limbs and facial muscles.

Case report: A 23-year-old woman, born out of second-degree consanguineous parentage, presented with weakness of both lower limbs since 9 years and incomplete closure of eyes during sleep since 6–8 years. Weakness of both lower limbs is insidious onset gradually progressive in the form of difficulty in getting up from squatting position with a history of buckling attacks. Difficulty in climbing upstairs and downstairs for 8 years. Difficulty in getting up from chair for 2 years. Slippage of chappals since 4–5 years. High stepping while walking. Difficulty in carrying weights with both hands. Incomplete closure of eyes during sleep for 6–8 years with difficulty in drinking water with nasal regurgitation, taking frequent gaps between sips. On examination: Bilateral orbicularis oculi weakness. Bilateral extensor digitorm brevis (EDB)- atrophied and bilateral ankle contractures are present. Bilateral Lower limbs proximal power-4/5, ankle DF-2/5, PF-4/5. Bilateral deep tendon reflex (DTR1)+. Bilateral plantar-Mute. Sensory examination is normal. High stepping and waddling gait.

Discussion: Serum creatinine phosphokinase- 385U/L. Hb- 8.7g/dL, erythrocyte sedimentation rate (ESR)- 28, nerve conduction study (NCS)– sensory normal, MNV: right and left common peroneal stimulation of EDB not recordable. Serum thyroid stimulating hormone (TSH)-1 mcg/dL, echocardiography-normal, genetic analysis-homozygous mutation of ADSS1 suggestive of distal myopathy-5.

Conclusions: Clinical picture of distal weakness with hyporeflexia and atrophied EDB, though points toward neuropathy, suspicion of myopathy was performed in view of raised serum creatine phosphokinase (CPK). ADSS1 mutation in adolescent onset distal myopathy to be considered especially when facial weakness accompanies.

Abstract ID: 1093: A Case of Young Child with Dystonia, Seizure, and Normal Cognitive Function

Dhiraj Kumar, Gutti Nagendra Babu, Akshat Srivastava

Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGI), Lucknow, Uttar Pradesh, India

Background and Aim: Biotin-thiamine-responsive basal ganglia disease (BTBGD) is a rare neurological disorder characterized by dystonia, seizures, and preserved cognitive functions. This autosomal recessive condition typically manifests in childhood or adolescence. The primary aim of this study was to investigate the efficacy of biotin and thiamine supplementation in managing symptoms and improving clinical outcomes in BTBGD patients.

Case report: A detailed clinical history, neurological examination, and diagnostic workup, including magnetic resonance imaging (MRI) and genetic testing, were conducted. The patient, a 10-year-old male, presented with progressive dystonia and occasional seizures but exhibited normal cognitive abilities. Upon suspicion of BTBGD based on clinical features and genetic analysis confirming mutations in SLC19A3, treatment was initiated with biotin and thiamine supplements.

Discussion: Following initiation of biotin-thiamine therapy, the patient showed significant improvement in dystonic symptoms, with a reduction in the severity and frequency of episodes. Seizure frequency also decreased, and cognitive functions remained unaffected throughout the treatment period. Repeat magnetic resonance imaging (MRI) scans showed stabilization of basal ganglia abnormalities associated with BTBGD.

Conclusions: This case highlights the efficacy of biotin-thiamine therapy in managing BTBGD, offering symptomatic relief and preserving cognitive functions. Early recognition and treatment initiation are crucial in improving outcomes for patients with this rare neurological condition. Further research is needed to optimize treatment protocols and understand the long-term implications of biotin-thiamine supplementation in BTBGD management.

Abstract ID: 1094: A Genetic Odyssey: Tracing FSHD to POMT2 Gene Mutation – A Case Report

Nirosha Metta, Mohan Channappanavar, Rashmi Devaraj, Kavya Mala, Nataraju B

Vydehi Institute of Medical Sciences and Research Centre (VIMS), Bengaluru, Karnataka, India

Background and Aim: Facioscapulohumeral dystrophy (FSHD) is a complex genetic disorder characterized in most cases by slowly progressive muscle weakness involving the facial, scapular, upper arm, lower legs, and hip girdle muscles usually with asymmetric involvement. The aim is to highlight the complexity of genetic disorders and the importance of comprehensive genetic testing in diagnosis and counseling.

Case report: In this case study, we report a 36-year-old gentleman presenting with symptoms and signs consistent with facioscapulohumeral dystrophy (FSHD). A 36-year-old man, born to non-consanguineous parents without any comorbidities, presented with gradually progressive asymmetrical weakness affecting upper limbs and lower limbs, along with bifacial and truncal weakness since the age of 20. Physical examination revealed bilateral winging of the scapula, wasted muscles around the shoulder joint, sparing forearm and hand muscles, polyhill sign, popeye/chicken wing appearance, and prominent axillary fold. Bifacial muscles with smile flattened and transverse and on attempting to smile, bouche de tapir appearance. Motor examination indicated reduced power in the shoulder (2/5), elbow and wrist (4/5), and hip (3+/5), while knee and ankle power was relatively preserved (4+/5), suggestive of muscle pathology possibly related to facioscapulohumeral dystrophy (FSHD).

Discussion and Conclusions: Despite clinical features aligning with FSHD, genetic testing revealed a POMT2 heterozygous mutation, which is not commonly associated with FSHD but rather with LGMD2N or LGMDR14, a form of limb-girdle muscular dystrophy and WWS (muscle eye brain disease). No literature is found linking FSHD to POMT2 GENE. So far 26 cases of LGMDR14 were reported.

Abstract ID: 1095: Primary Amyloid Myopathy as the Initial Presentation of Multiple Myeloma: A Rare Case Report

Sravya Reddy

Care hospitals, Banjara Hills, Hyderabad, Telangana, India

Background and Aim: Primary amyloid myopathy and associated multiple myeloma is a rare association. The aim is to report this rare presentation.

Case report: A 39-year-old female presented with upper limb muscle stiffness and jaw tightness with consequent speech and swallowing disturbances and weight loss for 6 months. She exhibited a pseudo-athletic appearance, macroglossia and coarse facial features lingual dysarthria, and upper limb and trunk muscle hypertrophy with preserved power and reflexes.

Discussion: Electromyoneurography showed bilateral carpal tunnel syndrome and myopathic features. MR of muscles was performed, which showed diffuse short inversion time inversion recovery (STIR) hyperintensities in the trunk and upper limb muscles along with multiple lytic lesions in the bones. A whole-body fludeoxyglucose-18 positron emission tomography (FDG PET) scan showed multiple lytic lesions in the skeleton, suggestive of multiple myeloma. Serum immunofixation showed kappa monoclonal gammopathy. Bone marrow biopsy confirmed multiple myeloma with amyloidosis.

Conclusions: Primary amyloid myopathy should be considered in the differential diagnosis of myopathy with hypertrophied muscles and skeletal lytic lesions. Early diagnosis outcome will be good.

Abstract ID: 1096: Spontaneous Intracranial Hypotension with Cervical segment Dural leak

Meghna Pawar, Naveen Kumar, Surekha Dabla

Pt BD Sharma PGIMS Rohtak Haryana, India

Background and Aim: Spontaneous Intracranial Hypotension (SIH) is a clinical and radiological diagnosis presenting as an orthostatic headache (OH), low cerebrospinal fluid (CSF) pressure, and diffuse pachymeninigeal enhancement in magnetic resonance imaging (MRI) brain along with other characteristic findings. We present a case of a young female with OH without any significant history of trauma or surgical procedures who presented with SIH due to a cervical dural leak. Our case emphasizes need for complete spinal segment imagining while investigating a case of SIH.

Case report : A 32-year-old lady without any significant past history presented to us with abrupt onset of OH of 3 days duration. Contrast MRI brain had all the signs suggestive of SIH. CSF pressure was 4 cm of H 2 O, and the computed tomography (CT) cisternogram showed a possible CSF leak at the C4–C5 spinal level. She was referred to a higher center for a Myelogram and confirmation of leakage site and dural repair.

Discussion: SIH is usually the result of a leak in the spine. Spontaneous leaks have been classified into four types and almost all types are most common in the thoracic spine. Whereas in our case, dural leak was suspected at the level of C4-C5 based on CT cisternography.

Conclusions: Diagnosis of the leak and treatment are challenging and rely on multimodality imaging. Thus, the suspected case of SIH imaging plays a crucial role, and scanning all the spinal segments should be considered.

Abstract ID: 1097: Stress Cardiomyopathy as a Consequence of Stroke: A Unique Case Report

Naveen Ranga, Meghna Pawar

Pt BD Sharma PGIMS Rohtak, Haryana, India

Background and Aim: Neurogenic stress cardiomyopathy (NSC) is a recognized syndrome that can occur in the early stages, following acute brain injury including stroke, possibly impacting patient outcomes.

Case report: We report one such case of a 77-year-old female with left middle cerebral artery (MCA) territory, moderate severity (NIHSS-11/42), acute ischemic stroke without any significant history of cardiac morbidity and got complicated due to the development of stress cardiomyopathy post-stroke.

Discussion: Here, we report a case of left MCA territory infarct involving an M3 peri insular opercular segment who developed stress cardiomyopathy increasing the morbidity of the patient.

Conclusions: Long-term electrocardiogram (ECG) monitoring and repeated 2D echo and ischemic myocardial markers, including creatine kinase, troponin-T, and brain natriuretic peptide (BNP) levels, are recommended in all stroke cases, particularly in elderly female patients with insular infarcts.

Abstract ID: 1098: Clinical, Neuro-Radiological, and Genetic Characteristics of Children Diagnosed with Malformations of Corpus Callosum at a Tertiary Care Center

Satyesh Muppavarapu, Vaishakh Anand, Vinayan Kollencheri Puthenveettil, Rakesh Ramanan

Amrita School of Medicine, Kochi, Kerala, India

Background and Aim: Corpus callosum malformations (CCM) are among the most frequent developmental brain malformations observed at birth. Rates of 2 to 3% have been reported among patients with neurodevelopmental disabilities. Etiologies are varied, genetic factors being the most common. Extra-cerebral involvement is also common. We conducted a retrospective data analysis of children presenting with antenatal or post-natal diagnosis of CCM to identify the clinical, genetic, and neuro-radiological spectrum of presentation.

Methodology: A retrospective chart analysis of children less than 18 years of age presenting to our department with CCM was performed. A total of 40 children were enrolled. Demographic variables, age, and symptoms at diagnosis, type of CCM, additional clinical findings, including associated epilepsy, developmental delay, extra-cerebral malformations, and genetic testing information, were analyzed.

Results: A total of 40 children were enrolled. Male: female ratio is 1.1:1. Fifteen children were diagnosed during the antenatal period, and mean gestational age at diagnosis was 31.8 weeks. Twenty-five were diagnosed after birth, and mean age at diagnosis was 13 months. Twenty-six children had complete agenesis of CC, of whom nine had isolated CC agenesis. Nine had hypoplasia, and five had hypoplasia with dysplasia. Epilepsy was seen in 67%, global developmental delay in 65%, behavioral issues in 40%. Facial dysmorphism was seen in 95%, and extra-cerebral malformations in 37.5%. Genetic testing was performed in 24, with a positive result in ten children. One child succumbed to refractory epilepsy.

Conclusions: Prospective and long-term multi-center studies, with cohesive inter-departmental cooperation, are required for further characterization and prognostication of malformations of corpus callosum.

Abstract ID: 1099: Breath of Hope: A Case Report Navigating Ondine’s Curse in Von Hippel-Lindau Syndrome

Ali Haider, Ishita Gupta, Srinath Rajeevan, Siby Gopinath, Ashok Pillai, Sreelakshmi Narayanan, Gopal, Rushab

Amrita School of Medicine, Kochi, Kerala, India

Background and Aim: Background: Central hypoventilation syndrome (CHS) or Ondine’s curse is a rare disorder that results from the impairment of autonomic control of breathing during sleep, with preserved voluntary control. Its occurrence in Von Hippel-Lindau syndrome (VHL) has not been reported to date. Aim: To discuss a unique scenario of a patient with VHL and medullary hemangioblastoma presenting with symptoms of CHS post-surgery.

Case report: The clinical details, laboratory reports, and imaging findings were reviewed in detail. A 19-year-old male patient with VHL underwent the removal of a medullary hemangioblastoma. Post-operatively, he experienced difficulty in weaning of from the ventilator and required a tracheostomy. After successful weaning, he was found to have recurrent episodes of desaturation, predominantly during sleep. On evaluation, the polysomnography (PSG) showed evidence of central sleep apnea during sleep, and an MRI brain showed post-op changes with a well-defined cystic lesion at cervico-medullary junction compressing the medulla. Therapeutic computed tomography (CT)- guided aspiration of the cyst was performed. Post-procedure, there was significant clinical improvement, and the PSG showed a betterment of the respiratory disturbance index and desaturation index.

Discussion and Conclusions: The case report shows the importance of ruling out compressive lesions of the brainstem in CHS. Such patients usually benefit from invasive therapeutic measures as described in our case. It also highlights the importance of doing a PSG in patients who are difficult to wean off mechanical ventilation after undergoing brainstem surgery.

Abstract ID: 1100: An Interesting Case of Alcohol-Related Myelopathy

M. Thangaraj

Dhanalakshmi Sreenivasan medical college perambalur

Background and Aim: Chronic alcohol consumption can present with a multitude of neurological symptoms. Among the manifestations that affect the motor system alcohol alcohol-related myelopathy is relatively rare. Most of the cases described in the literature allude to myelopathy in the setting of alcoholic liver disease and myelopathy secondary to portosystemic shunting. The cases resulting from the direct neurotoxic effect of ethanol have been reported only in a handful of studies, and there are none from India.

Case report: A 35-year-old male presented with difficulty in walking and involuntary passage of urine for 15 days. He noted difficulty in clearing ground which was associated with stiffness in both legs. He also noted the involuntary passage of urine with increased frequency. He was able to feel a bladder filling sensation, and there was no associated bowel incontinence or constipation. He is a chronic alcoholic with an average daily consumption of 360–720 mL of brandy and a recent history of binge drinking for 5 days. He does not have any other comorbidities, and he was not on any medications. Examination revealed normal higher mental function and normal cranial nerves. Motor examination revealed bilateral spastic weakness of both lower limbs with brisk reflexes in all limbs. Sensory examination was normal, and there were no cerebellar signs. He was provisionally diagnosed as a case of probable noncompressive myelopathy.

Discussion: Folate levels were reduced with a macrocytic picture. Imaging, serology, immunological cerebrospinal fluid (CSF) studies, nerve conduction study (NCS), and liver function test (LFT) were normal.

Conclusions: In our case, the first from India reflects a multifactorial etiopathogenesis wherein a myelopathy due to folate deficiency was exacerbated due to binge drinking and ensuing direct ethanol toxicity presenting with predominant pyramidal involvement with complete sparing of sensory tracts and a rare involvement of bladder.

Abstract ID: 1101: Rare Presentation of MTHFR Mutation

Gudla Surya

Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Methylenetetrahydrofolate reductase (MTHFR) mutations lead to hyperhomocysteinemia. It may have varied presentations depending on the age of onset.

Case report: A 23-year-old man presented with seizures, insidious onset slowly progressive unsteadiness of gait, weakness of both lower limbs, and speech disturbances. He first developed seizures at the age of 12 years, which were of generalized tonic‐clonic seizures (GTCS) semiology. At 15 years of age, he had developed progressive ataxic spastic paraparesis. Tremors were present in both hands. There was also history of mental retardation. On examination, Spastic gait ataxia and limb ataxia with spastic dysarthria, bilateral plantar extensor, and large fiber neuropathy were present.

Discussion: Blood routines were normal. The magnetic resonance imaging (MRI) brain has revealed bilateral posterior parietal subcortical hyperintensities with a suspicion of leucodystrophy. On the peripheral smear, macrocytes were found out, and serum B12 level was decreased. The patient’s homocysteine level was increased. Upon evaluation for the cause of hyperhomocysteinemia, the patient was found out to be harboring MTHFR mutation in exon-4. 2D Echo was normal. Thyroid profile and lipid profile were normal. The patient was started on vitamin B12, folic acid, pyridoxine, and vitamin D, and there was an improvement in his symptoms.

Conclusions: Since MTHFR deficiency is a potentially treatable disease, early diagnosis is crucial, and treatment should be started as early as possible. It is, therefore, imperative for clinicians to be aware of the clinical patterns of early- and late-onset forms of the disease.

Abstract ID: 1102: A Borderzone of Neurology and Psychiatry

Sania Saba, Advait Kulkarni, Ajit Kumar Roy, Uday A Murgod

Manipal Hospitals Old Airport Road Bengaluru, Karnataka, India

Background and Aim: The brain is the anatomical substrate of the mind. Neurological disorders stem from structural brain abnormalities, while psychiatric disorders result from functional disturbances in neuronal circuits. This study examines disorders at the border zone of neurology and psychiatry, focusing on the bidirectional relationship between epilepsy and neurobehavioral disorders.

Methodology: This study investigated the incidence of epilepsy-related psychosis and psychogenic non-epileptic seizures (PNES) among 1000 epilepsy patients at Manipal Hospital, Bangalore, from 2014 to 2024. Detailed clinical profiles, including age, sex, neurological status, investigations, antiepileptic drug prescriptions, and treatment responses, were analyzed. Standard diagnostic procedures included non-contrast computed tomography (NCCT) brain, magnetic resonance imaging (MRI) brain, metabolic investigations, and electroencephalogram (EEG), with video EEG and functional MRI for selected cases.

Results: Among the 1000 (522 males, 478 females) patients, 59% had focal seizures, and 41% had generalized seizures. Idiopathic onset was noted in 66% of cases, with neurocysticercosis (16%), tuberculosis (8%), vascular causes (3%), post-infective causes (3%), and post-traumatic and congenital causes (2%) following. Psychosis was present in 38 patients, categorized into post-ictal psychosis (20), brief psychosis (12), chronic (3), and inter-ictal psychosis (6). PNES was identified in 100 patients, predominantly female, aged 13–27 years, with varied motor and non-motor activities. A study on serum prolactin levels in 52 PNES patients, matched with 52 normal controls, showed significant elevations post-seizure in GTCS (37 ± 6.8 ng/mL) and CPS (37 ± 6.8 ng/mL) but not in PNES.

Conclusions: Comprehensive care for epileptic patients must address psychological and social consequences alongside seizure management.

Abstract ID: 1103: Mold Invasion – CNS Fungal Infection in Immunocompetent Individuals

Geethika S. Babu, Chithra P, Vineetha V S

Government Medical College, Thiruvananthapuram, Kerala, India

Background and Aim: Fungal infections of the central nervous system are found commonly in immunosuppressed individuals. Immunocompetent individuals are affected usually as a complication of neurosurgical procedures, craniofacial surgeries, or intravenous (IV) drug use. Here, we present two cases of central nervous system (CNS) fungal infection in immunocompetent individuals.

Case report: Case series. Case 1 – A 48-year-old gentleman, hypertensive, without any other known comorbidities, presented with a new onset severe headache of 2 weeks duration. On examination, he had peripheral constriction of the visual field and the fundus showed disk edema. There were no other focal neurological deficits. His cerebrospinal fluid (CSF) study showed lymphocytic pleocytosis, hypoglycorrhychia, and elevated protein, with opening pressures of 270 mm. CSF microscopy and staining with nigrosin showed encapsulated organisms consistent with Cryptococcus species. His CSF also yielded positive results for cryptococcal antigen. Magnetic resonance imaging (MRI) brain was normal. Case 2 – A 35-year-old lady presented with left-sided headache of 3 months duration. On examination, she had right upper motor neurons (UMN) facial palsy. The rest of the CNS examination was normal. Her CSF study showed hypoglycorrhychia with elevated CSF protein and positivity for cryptococcal antigen. MRI brain showed ring-enhancing lesion with peripheral blooming and lactate peak on magnetic resonance spectroscopy (MRS) suggestive of a fungal abscess.

Discussion and Conclusions: CNS fungal infections can range from meningitis to brain abscess. Immunocompetent individuals can be affected even without the traditional risk factors. Diagnosis is hindered by chronic and nonspecific symptoms. Prolonged treatment and potential side effects of the medication add on to the morbidity.

Abstract ID: 1104: Child with Macrocephaly and Cysts in Brain Imaging

Hari Potta, Moseen Mohammad, S. Gopi

Andhra medical college, KGH, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Megalencephalic leucoencephalopathy with subcortical cysts (MLC) is a leucodystrophy due to a defect in myelin – water maintenance, characterized by pyramidal signs, cerebellar signs, seizures, macrocephaly, and subcortical cysts in imaging with normal/near normal IQ.

Case report: A 5-year-boy, born out of non-consanguineous parentage, presented with weakness of right lower limb, unsteadiness, clumsiness of extremities, and falls. The baby was brought to medical attention at 9 months of age for large head size which is appropriate to age now. He had seizures in the form of generalized tonic–clonic seizure (GTCS) and right focal motor seizures at 1 year of age which were well controlled with anti-epileptic drugs (AEDs). The patient had weakness of the right lower limb, unsteadiness of walking associated with multiple falls, and clumsiness of hands while holding objects which is more for right than left. Weakness and unsteadiness are insidious onsets, very slowly progressive. There were no sensory, bladder, bowel, or cognitive impairment. On examination, he had a head circumference of 52 cm (within 1 SD), the power of the right lower limb is 4+, with the right plantar extensor, and left plantar is mute. He exhibited bilateral both upper and lower limb ataxia (Rt >> Lt), stance ataxia, gait ataxia, and circumduction gait.

Discussion: Magnetic resonance imaging (MRI) brain showing frontal and temporal subcortical cysts. Parents are not affordable for genetic analysis.

Conclusions: A sporadic occurrence of rare cases of MLC was previously described predominantly in ethnic groups

Abstract ID: 1105: Spectrum of Movement Disorders in Subacute Sclerosing Panencephalitis

Keerthiraj D B, Shweta Pandey

Dr. Ram Manohar Lohia Institute of Medical Lucknow, Uttar Pradesh, India

Background and Aim: Subacute sclerosing panencephalitis (SSPE) is a progressive brain disorder caused by the persistent mutant measles virus. Patients have a clinical course characterized by progressive cognitive decline, seizures, myoclonus, and vegetative state eventually leading to death. Movement disorders are also a part of the clinical features of SSPE patients. Here, we report a frequency of movement disorders observed in our 54 cases of SSPE. Aim: To study the movement disorders in patients with subacute sclerosing panencephalitis (SSPE).

Methodology: A prospective observational study conducted in a tertiary care center. SSPE patients were diagnosed by modified Dyken criteria, and Jabbour staging was performed. We assessed their clinical profile and examined for movement disorders.

Results: We enrolled 54 SSPE patients of which 44 were male and ten were female. The mean age of our study population was 13.9 ± 6.7 years. Among the movement disorders, the most frequent was myoclonus (96.3%) followed by dystonia (16.7%), repetitive motor behavior (16.7%), tremor (3.6%) in the descending order, and other abnormal movements, including hemichorea, hemiballism, and parkinsonism (1 patient each). Among the myoclonus, generalized myoclonus (57.4%) was the most common form, followed by jaw opening (11.1%), segmental (7.4%), cervical (7.4%), ocular (7.4%), Truncal (5.6%), multifocal (5.6%), and focal myoclonus (1.8%).

Conclusions: Our study concludes that movement disorders are important clinical findings of SSPE in the form of myoclonus and non-myoclonic disorders, including dystonia, repetitive motor behavior, tremor, and parkinsonism. The above findings help in the differential diagnosis of a child presenting with rapid cognitive decline along with movement disorders.

Abstract ID: 1106: Unveiling an Unusual Cause of Unilateral Cerebral Convexity Subarachnoid Hemorrhage.

Shashanka Chillapuram, Sandhya Manorenj

Deccan Institute of Medical Sciences, Santosh nagar Main Rd, Kanchan Bagh, Hyderabad Telangana, India

Background and Aim: Subarachnoid hemorrhage (SAH) localized to the unilateral cerebral convexity is a rare presentation often associated with distinct etiologies such as trauma or vascular abnormalities. This study aims to explore focal central nervous system (CNS) vasculitis as an unusual cause of this specific type of SAH.

Methodology: A prospective observational case study of a patient with right focal CNS vasculitis and imaging features of unilateral convexity subarachnoid hemorrhage. Clinical records, imaging studies (CT/MRI), cerebrospinal fluid analysis, and cerebral angiography findings were collected in a case of focal CNS vasculitis presenting with this unique hemorrhagic pattern.

Results: The patients, diagnosed with unilateral cerebral convexity SAH, were found to have focal CNS vasculitis as the underlying cause. Detailed imaging characteristics revealed a beaded appearance in small and medium-sized vessels. Clinical presentations varied but commonly included symptoms such as Rt-sided headache, focal motor seizures, and ictal ptosis. The cerebrospinal fluid (CSF) analysis further supported the diagnosis, revealing xanthochromia and elevated protein levels consistent with an inflammatory process. Subsequently, the patient received pulse steroid therapy and IV rituximab; however, her refractory septic shock proved fatal.

Conclusions: Cerebral convexity subarachnoid hemorrhage, while rare, can be attributed to focal CNS vasculitis, posing a diagnostic challenge due to its infrequent occurrence. This case underscores the importance of considering vasculitic etiologies in the differential diagnosis of cSAH, especially when atypical imaging findings are encountered. Early recognition and intervention are crucial to optimize patient outcomes in these uncommon presentations of CNS vasculitis. Further research is needed to elucidate the pathophysiological mechanisms and optimal management strategies for this unique manifestation.

Abstract ID: 1107: A Rare Case of Hereditary Spastic Paraplegia with Mitochondrial Membrane Protein-Associated Neurodegeneration (MPAN)

Vidya Sagar Kota, Lulup Sahoo, Ajit Prasad Mishra, Monica Karan, Devidutta Dash

Institute of Medical Sciences and SUM Hospital, Odisha, India

Background and Aim: Mitochondrial membrane protein-associated neurodegeneration (MPAN) is an neurodegeneration with brain iron accumulation (NBIA) disorder that is characterized by gait changes in the beginning, followed by progressive spastic paresis, dystonia, neuropsychiatric abnormalities, and cognitive decline. Hereditary spastic paraplegias (HSPs) are a group of rare, inherited, neurological diseases characterized by broad clinical and genetic heterogeneity. Lower limb spasticity with first motoneuron involvement is the core symptom of all HSPs. As spasticity is a syndrome and not a disease, it develops on top of other neurological signs (ataxia, dystonia, and parkinsonism).

Case report: A 11-year-old male child presented with c/o slurring of speech since 4 ½ years, walking difficulties for 4 years, and decreased scholastic performance for 4 years. For the last 3 years, parents complained of a child having staring look for 2–3 min multiple episodes in a day and about 15–20 episodes in the last 3 years, which he used to recover from the episodes after repeatedly calling him, having also h/o irritability, aggressive behavior. On examination, spasticity was observed in both lower limbs with brisk reflexes and b/l plantar extensor.

Discussion: Investigations – 1) Hb – 12.7, total white blood cell (TWBC) count – 14.55, platelet count – 496, mean corpuscular volume (MCV) – 78.0, mean corpuscular hemoglobin (MCH) – 24.5, mean corpuscular hemoglobin concentration (MCHC) – 31.1, 2) renal function test (RFT) – within normal limits, 3) USG abdomen – no organomegaly, no gall bladder polyps, 4) serumvitamin B12 – within normal limits. Magnetic resonance imaging (MRI)- marked hypointensity in b/l globi pallidi and pars reticulata of substantia nigra on susceptibility weighted imaging (SWI) with stratification of globus pallidus externa and globus pallidus interna. Whole exome sequencing was performed which showed positive for SPG 43/MPAN gene with C19orf12 c.166delGp.Ala56 del 6.

Conclusions: MPAN is a type of NBIA which presents with spasticity predominant than dystonia which also mimics hereditary spastic paraplegia in the initial presentation.

Abstract ID: 1110: “Nine Syndrome – Decode the symptoms and crack the diagnosis”

Shamili, Justin C, Murugan P

Madurai Medical College, Chennai, Tamil Nadu, India

Background and Aim: Nine syndromes are a rare clinical entity with core clinical signs of one-and-a-half syndrome, ipsilateral facial palsy, and contralateral hemiparesis/hemianesthesia and localizing to the dorsal paramedian pontine tegmentum. The awareness of this sign helps in precise localization and consideration of relevant etiologies.

Case report: A 45-year-old male, non-diabetic and non-hypertensive with a background history of alcohol and smoking, presented with complaints of acute-onset blurring of vision, slurring of speech, drooling of saliva and deviation of angle of mouth to right, difficulty in closing left eye and swaying while walking.

Discussion: On Examination, left eye adduction and abduction are both impaired, right eye abduction nystagmus with impaired adduction in the right eye with normal vertical eye movements and normal pupil along with left lower motor neuron (LMN) facial palsy (left lagophthalmos, deviation of angle of mouth to right) and right pronator drift, impaired tandem walking and right plantar extensor. Imaging shows a left paramedian pontine infarct. Pt was treated with antiplatelets and statins, and the patient improved dramatically.

Conclusions: Nine syndromes are a rare clinical entity with core clinical signs of one-and-a-half syndrome, ipsilateral facial palsy, and contralateral hemiparesis/hemianesthesia and localizing to the dorsal paramedian pontine tegmentum. The awareness of this sign helps in precise localization and consideration of relevant etiologies.

Abstract ID: 1111: Dyslipidemia and its Impact on the Clinical Progression of Idiopathic Intracranial Hypertension

Kirandeep Kaur, Aastha Takkar Kapila, Karthik V. Mahesh, Nalini Gupta, Vivek Lal, Chirag Ahuja, Basavaraj Tigari, Sukhjindar Singh

Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

Background and Aim: Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure (ICP) occurring predominantly in women with obesity without an identifiable etiology. There is a positive correlation between central adiposity and IIH, whereas not all IIH patients are obese. It raises questions about IIH being a systemic metabolic disease.

Methodology: The patients presenting at PGIMER (Chandigarh) and fulfilling the revised diagnostic criteria (modified Friedman criteria, 2013) for IIH were included in this prospective pilot study. They were assessed for neurological, visual, and radiological outcomes. The demographic and clinical findings, including the lipid profile (cholesterol, low-density lipoproteins (LDL), very low-density lipoproteins (VLDL), and triacylglycerols [TAGs]) in these patients, were explored.

Results: Thirty-six female IIH patients with a mean age of 35.22 ± 10.6 were assessed. The mean body mass index (BMI) (kg/m 2 ) of disease onset was 29.2 ± 4.8. The median cerebrospinal fluid (CSF) opening pressure reported was 280 mm (150, 400). The median cholesterol, triglyceride, LDL, high density lipoprotein (HDL), and VLDL reported were 169 mg/dL (119, 354), 134 mg/dL (58,309), 96 mg/dL (37, 257), 46.7 mg/dL (29.7, 116), and 27 mg/dL (11.6, 51), respectively. There was a significant correlation between lipid profile and age. The patients who were above 34 years ( N = 16) of age had significantly higher levels of cholesterol ( P = 0.03) and LDL ( P = 0.012). Whereas, patients with CSF opening pressure less than 280 mm ( N = 22) were reported to have higher HDL levels ( P = 0.03) as compared to the patients with higher CSF opening pressure ( N = 13). However, no correlation was found between BMI and the lipid profile.

Conclusions: The etiology of IIH is poorly understood. The study is suggestive of dysregulated lipid metabolic pathways in patients with IIH.

Abstract ID: 1112: A Bleed in Pons Makes Nine

Vikas Prabhu

King George’s Medical University (KGMU), Lucknow, Uttar Pradesh, India

Background and Aim: A small lesion in the pons can cause complex syndromes owing to the close anatomical proximity of different nuclei and pathways. One-and-a-half syndrome can present with distinct syndromes based on surrounding structures that get involved. Nine syndromes are an unusual variant when eight-and-a-half further associates with contralateral hemiparesis or hemianesthesia or ataxia.

Case report: We report one such case of nine syndromes due to pontine hemorrhage in the dorsal pontine tegmentum. A 42-year-old male presented with a 5 h history of sudden onset right-sided hemiparesis, diplopia, and slurring of speech. His blood pressure was 180/100 mm Hg. On neurological examination, the patient was conscious, oriented, and following commands. Pupils were symmetric and reactive to light. Assessment of ocular motility revealed a total left-sided horizontal gaze palsy with internuclear ophthalmoplegia on the right side. Further examination revealed a left-sided lower motor neuron (LMN) facial palsy with bells phenomenon (House Brackmann grading IV) along with a right-sided hemiparesis with grade 3 muscle strength. The patient had a prior history of hypertension and had not been compliant with his medications.

Discussion and Conclusions: Recognition of nine syndromes and the spectrum of other one-and-a-half syndromes allows for precise anatomic localization of the lesion to the pontine tegmentum region and also to understand the size and extent of the lesion. The presentation of the patient can also help predict the nature of the pathology.

Abstract ID: 1113: An Extremely Rare Case of Polyneuritis Cranialis in IgG4-Related Disease – A Case Report from a Quaternary Care Hospital in South India

Jaisy James, Bijoy Jose, Reji Paul

Aster Medcity KochI, Kerala, India

Background and Aim: Immunoglobulin G4 (IgG4)-related disease is a systemic immune-mediated fibroinflammatory disease of unknown cause, characterized by unique pathological features involving a wide variety of organs. The nervous system is less commonly involved, and literature regarding such cases is extremely rare. Here, we are presenting a case of IgG4 disease that initially presented as polyneuropathy.

Case report: A 71-year-old Indian male, with a known case of type 2 diabetes mellitus, developed Bell’s palsy about 1 month back and was treated with oral steroids. He further developed swallowing difficulty with nasal regurgitation and voice change. On examination, he had left lower motor neuron (LMN) facial palsy along with 9th and 10th cranial nerve (CN) palsy on the left. He was admitted for further evaluation of polyneuritis cranialis. Indirect laryngoscopy showed left vocal cord palsy. The initial magnetic resonance imaging (MRI) was normal. PET-CT was performed, revealing FDG avid uptake in the soft tissue of the scalp, left upper lobe nodule, and uptake along the jugular and carotid canal. Biopsy was performed from the scalp, and there were no features suggestive of malignancy or granuloma. Antinuclear antibody (ANA) screening was positive. ANA profile showed PM-Scl three positivity. Lumbar puncture was performed, and it was lymphocyte pre dominant. The tuberculosis diagnostic panel was negative. A repeat magnetic resonance imaging (MRI) brain showed nodular enhancement along bilateral facial and right trigeminal cranial nerves. IgG4 serum was sent which was elevated. Hence, a diagnosis of IgG4 disease was made, and he was started on steroids. Physiotherapy and swallowing therapy sessions were conducted. He is showing good progress, and it is under regular follow-up.

Discussion and Conclusions: In conclusion, IgG4-related disease impact on cranial nerves poses intricate diagnostic challenges, necessitating a nuanced approach for effective management. Clinicians must be vigilant in recognizing subtle neurological symptoms and collaborate closely with specialists in rheumatology, neurology, and imaging for accurate diagnosis and timely intervention.

Abstract ID: 1114: Exploring the Brain-Heart Axis: Does the Tpeak to Tend (Tpe) Interval Predict Stroke Outcome?

Nandana J, Sajith Sukumaran, Arun Gopalakrishnan, Sajith Sukumaran, Arun Gopalakrishnan, Sajith Sukumaran

Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Thiruvananthapuram, Kerala, India

Background and Aim: Acute ischemic stroke (AIS) can lead to several electrocardiogram (ECG) changes. The Tpeak–Tend (Tpe) interval, a transmyocardial repolarization parameter, is associated with sudden cardiac death in AIS patients. However, its relationship with functional outcomes has not yet been studied. The aim of this study is to determine if Tpe interval predicts the short and long-term outcome in AIS patients without any known cardiac comorbidities.

Methodology: Tpe interval was calculated in ECG obtained from 100 patients who were assessed for stroke severity by national institutes of health stroke scale (NIHSS) and disability scores with modified rankin scale (mRS) at admission, discharge, and 3-month follow-up.

Results: The mean Tpe interval at admission was 104.6 ± 18.8 ms. Tpe interval was prolonged in 74% of patients at admission and 51% at 48 hpost-admissionn and 8% at 3 months. Among patients with insular involvement, 91.17% had prolonged Tpe interval ( P value = 0.005). At admission, those with NIHSS >5 (82.5%) and mRS >4 (79.7%) had significant Tpe prolongation ( P value < 0.01). Independent of stroke severity and disability at admission, those with persistently prolonged Tpe after 48 h had worse NIHSS and mRS scores ( P value < 0.05), at discharge and 3 months. Of those with normalization of Tpe at 48 h, 74% had improvement of mRS score at discharge ( P value < 0.001), and 52% at 3 months ( P value = 0.684).

Conclusions: Our data sheds light on those patients who must be keenly observed for Tpe prolongation, as this may imply selecting medication as well as avoiding electrolyte disturbances that predispose them to arrhythmias.

Abstract ID: 1115: Movement Disorders After Acute Stroke: An Observational Cross-Sectional Study in a Tertiary Care Center of Eastern India

Soumyadeep Ghosh, Arijit Ray, Samar Biswas

Institute of Post Graduate Medical Education and Research (IPGMER), Kolkata, West Bengal, India

Background and Aim: Abnormal involuntary movements (AIM) following acute cerebral stroke may be hyperkinetic like athetosis, chorea-ballism, dystonia, tremor, myoclonus, or hypokinetic like parkinsonism; though the former is uncommon. The frequency of AIM following acute stroke (<1 month) is low but important in view of disability produced and treatment offered. To analyze the clinical features, radiological findings along with clinico-radiological correlations of acute admitted strokes and frequency and type of movement disorders associated with them.

Methodology: Observational cross-sectional study from admitted patients in IInstitute of Post Graduate Medical Education and Research (IPGMER), Kolkata. Inclusion criteria – all acute (<1 month in duration) cerebral ischemic and hemorrhagic strokes, including subarachnoid hemorrhage. Exclusion criteria – intracerebral hemorrhage (ICH) with a history of head injury.

Results: Of 500 acute stroke patients (215 had infarcts and 285 had hemorrhagic stroke) admitted, seven had AIM (all were hyperkinetic). A total of four patients had dystonia, one hemiballism, 1 choreo-athetoid, and one had hemichorea-hemiballism movement. A total of five patients had infarct, and two had hemorrhagic strokes of those with AIM.

Conclusions: Our study showed AIM <2% which is consistent with previous studies. All AIMs were hyperkinetic as detailed previously. AIMs were more common in ischemic stroke.

Abstract ID: 1116: An Unusual Cause of Recurrent Strokes in a Patient of Systemic Lupus Erythematosus

Sharan Raja, Srikanth Reddy E, Keshava Anand G

Yashoda Hospital Hyderabad, India

Background and Aim: Varicella zoster virus (VZV) vasculopathy is a rare form of multifocal vasculopathy that typically affects small and medium-sized cerebral vessels, commonly seen in immunocompromised patients with a recent history of Varicella or Herpes zoster. We report a rare case of recurrent ischemic stroke due to VZV large vessel vasculitis in patients with SLE on irregular treatment.

Case report: A 54 years old female, with a known case of systemic lupus erythematosus (SLE) (on disease-modifying antirheumatic drugs (DMARDs)), presented with c/o left hemiplegia, was initially admitted to a local hospital and was diagnosed as SLE-related central nervous system (CNS) vasculitis and treated with plasma exchange (PLEX) (2 sessions), rituximab (500 mg – 4 doses). Following discharge 10 days later, she presented with c/o fever, altered sensorium, and right hemiparesis. Magnetic resonance imaging (MRI) brain showed multiple bilateral acute infarcts in middle cerebral artery (MCA) and posterior cerebral artery (PCA) territory. MR angiography (MRA) showed multifocal areas of segmental stenosis with post-stenotic dilation involving B/L anterior, middle, and posterior cerebral arteries – s/o CNS Large vessel vasculitis. Cerebrospinal fluid (CSF) analysis showed total cells = 20 cells/cum (90% lymphocytes, 10% Neutrophils), normal biochemical analysis. She was empirically started on IV Acyclovir. CSF meningoencephalitis panel: varicella zoster detected. Patient sensorium gradually improved over the next week. But later due to mild deterioration in the sensorium repeat, an MRI brain showed development of new infarcts and worsening vasculitis.

Discussion: Acyclovir dose was increased. The patient gradually improved clinically and was discharged to a rehabilitation center, subsequently lost to follow-up.

Conclusions: VZV suspected: Cryptogenic stroke recent history of varicella, no vascular risk factors, recurrent stroke, infarcts at gray-white matter junction, arterial narrowing – beading immunocompromised.

Abstract ID: 1118: Rare Presentation of Adult Pilocytic Astrocytoma as Meningitis

Ram Dasugari

Narayana health city, Bommasandra Industrial Area, Bangalore, Karnataka, India

Background and Aim: Pilocytic astrocytoma (WHO grade I) is the most common astrocytic tumor seen in children and adolescents with a predilection for the cerebellum, optic pathways, and hypothalamic region, and it may arise in any part of the central nervous system (CNS). It accounts for 6% of all primary brain tumors and 85% of infratentorial astrocytomas and has a favorable prognosis in view of its slow growth and benign nature. It rarely presents during adulthood after 2nd decade. we present a case of adult-onset pilocytic astrocytoma with a rare presentation as meningitis.

Case report: A 33-year-old lady with past h/o pilocytic astrocytoma of conus (operated 2 years ago) presented with fever with headache and altered sensorium for 5 days followed by back pain. Examination showed meningism with altered sensorium. She was admitted at a local hospital and evaluated with a magnetic resonance imaging (MRI) brain and spine with contrast and cerebrospinal fluid (CSF) analysis and treated as tuberculous meningitis (TB meningitis) with anti-tubercular drugs (ATT) and steroids. Because of worsening with seizures, she came to our center for further treatment.

Discussion: An MRI brain with contrast showed extensive diffuse leptomeningeal enhancement in the brain with features of raised intracranial pressure (ICP), and an MRI spine with contrast showed residual pilocytic astrocytoma at the conus. CSF analysis showed 02 cells, protein of 881 mg, and glucose of 71 mg with the absence of atypical cells and negative meningitis panel. Decompression and tumor resection were performed, and meningocortical biopsy was performed. Histopathology is s/o pilocytic astrocytoma.

Conclusions: A rare presentation of adult-onset pilocytic astrocytoma with clinical features of meningitis is seen.

Abstract ID: 1119: New Onset Refractory Status Epilepticus: A Rare Presentation of MOGAD

Pranay Gupta, Kanika Aggarwal, Bharat Kumar Sahu, Swapan Gupta

Govind Ballabh Pant Institute of Postgraduate Medical Education (GBPIPMER), New Delhi, India

Background and Aim: Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a rare, antibody-mediated inflammatory demyelinating disorder of the central nervous system. Occurrence of seizures is reported from 5.4% to 24.5%.

Case report: This is a case of a previously healthy 14-year-old boy who presented for the first time ever with multiple episodes of convulsive seizures without regaining consciousness in between the episodes. He was initially managed for convulsive status epilepticus (CSE) but failed to respond to IV anti-seizure medications, including a benzodiazepine (refractory SE). He remained stuporous and had ictal nystagmus. Electroencephalogram (EEG) at this point was suggestive of generalized rhythmic delta activity, and the midazolam challenge showed significant clinical and EEG improvement, confirming nonconvulsive status epilepticus (NCSE) as per modified Salzburg criteria. MRI brain was suggestive of cerebral cortical encephalitis (CCE) with bilateral cortical cytotoxic edema. Based on the high suspicion and severity of the clinical condition, an empirical steroid pulse with antivirals was started. Infectious encephalitis and autoimmune panel, including neuromyelitis optica (NMO)/ myelin oligodendrocyte glycoprotein (MOG), were sent.

Discussion: Cell-based immunoassay for MOGAD was strongly positive, while other panels were negative. Antivirals were stopped and immunotherapy continued. The patient had a dramatic response to treatment.

Conclusions: New-onset refractory status epilepticus (NORSE) could be the initial manifestation of MOGAD. The diagnostic algorithm for NORSE should include testing for MOG antibodies especially when there are features suggestive of cerebral cortical encephalitis (CCE) with bilateral cortical cytotoxic edema on neuroimaging.

Abstract ID: 1120: Non-Motor Features in Cervical Dystonia – A Northern India Perspective

Gurdeep Rajan

Govind Ballabh Pant Institute of Postgraduate Medical Education (GBPIPMER), New Delhi, India

Background and Aim: Non-motor features in cervical dystonia (CD) are essentially linked to its pathophysiology, needless to mention an area of immense unexplored possibilities. Previous studies have reported a mixed account. The current study aims to assess the sleep quality, daytime sleepiness, anxiety, and depression and ascertain their relationship with the dystonia severity in CD.

Methodology: A cross-sectional study was conducted at the Neurology Department, Govind Ballabh Pant institute of Postgraduate Medical Education & Research (GIPMER), Delhi. CD patients were assessed for the severity of the dystonia using Toronto western spasmodic torticollis rating scale (TWSTRS), a modified Tsui scale, for depression, anxiety, sleep quality, and daytime sleepiness using the Beck depression inventory, Beck anxiety inventory, Pittsburgh sleep quality index (PSQI), and Epworth sleepiness scale (ESS), respectively.

Results: A total of 30 patients were recruited with a mean age of 50.4 11.2 years, M:F ratio (4:1). The mean TWSTRS and Tsui scores were 33.0 ± 13.2 and 10.6 ± 4.8. A total of 30% had severe dystonia (Tsui score > 12.5). The mean scores for PSQI, ESS, Beck depression inventory (BDI), and Beck anxiety inventory (BAI) were 6.1 ± 3.6, 2.4 ± 2.9, 8.4 ± 4.8, 1.1 ± 1.8, respectively. Only five patients had depression, whereas all had mild anxiety. No correlation was found between the sleep and mood disturbances with dystonia severity.

Conclusions: Non-motor features are an integral part of symptomatology, the management of which forms a holistic plan for dystonia therapy. The presence of mild anxiety in all patient is a silent bystander or rooted intricately with the pathophysiology remains an enigma, warranting further research.

Abstract ID: 1121: Tirofiban in Progressive Acute Stroke: A Preliminary Experience

Sandeep Joshi

Care Hospital Banjara Hills, Hyderabad, Telangana, India

Background and Aim: In patients of stroke of recent onset or progressive stroke symptoms and nonoccluded large and medium-sized cerebral arteries, administration of intravenous tirofiban was shown to be associated with good functional outcomes in the recent RESCUE-BT2 trial. Therefore, we aimed to study the effects in a similar subset of patients at our center.

Methodology: This retrospective chart review was conducted between August 2023 and April 2024 at a tertiary referral care center in Hyderabad. All the consecutive patients of acute stroke who received tirofiban infusion in the specific period (n = 6) were included in the study. Diagnosis of acute stroke was based on clinical and radiological features. Tirofiban was administered as per the RESCUE-BT2 trial protocol. Parameters: Age, gender, NIHSS at onset, stroke mechanism, timing of early neurological deterioration (END), National institutes of health stroke scale (NIHSS) at END, NIHSS at discharge, 30-day modified rankin scale (mRS), and bleeding events.

Results: Age (range in years) – 48-63; M: F – 3:3; Median NIHSS at onset: 5 (0–9); Mechanism: cerebral small vessel disease (SVD) – 6 (100%); timing of END – <24 h of onset – 4 (66%); 24–96 h of onset – 2 (33%); Median NIHSS at end: 6.5 (6–9); Proportion of patients with halted END – 6 (100%); Median NIHSS at discharge: 5.5 (4–6); 30-day mRS (0–3): 6 (100%); Hemorrhagic complications – transient urethral bleeding in one and none had symptomatic intracerebral hemorrhage (ICH).

Conclusions: Our experience shows that tirofiban, when administered within 96 h of stroke onset assisted in terminating END and improving the 30-day functional outcomes in patients of progressive acute ischemic stroke without large vessel occlusion.

Abstract ID: 1122: Occurrence of Impaired Glucose Tolerance and Unrecognized Diabetes Mellitus in Patients with Acute Ischemic Stroke With no Previous Diagnosis of Diabetes Mellitus

Cankatika Choudhury, Cankatika Choudhury, Akhil Sahib, Neera Chaudhry, Aparna Kathait

Govind Ballabh Pant Institute of Postgraduate Medical Education (GBPIPMER), New Delhi, India

Background and Aim: Diabetes mellitus (DM) is a recognized independent risk factor for stroke, and it is associated with higher morbidity and mortality. Moreover, the onset of DM may preceded by a prolonged period of insulin resistance (IR). The true prevalence of DM in acute stroke is often underestimated because of this it goes unrecognized.

Methodology: This was a prospective observational study conducted over 18 months at the Department of Neurology, Safdarjung Hospital, and included 100 patients of AIS with no previous history of DM. Along with daily fasting plasma glucose (FPG) levels; HbA1C, oral glucose tolerance test (OGTT), and insulin levels were also measured during the hospital stay. They were followed up, and OGTT was repeated at the end of 3 months.

Results: Unrecognized DM was more prevalent than recognized DM in acute ischemic stroke (AIS) patients. The reactionary hyperglycemia at stroke presentation was found only in a few subjects, while the majority had a persistent abnormal glucose metabolism at 3 months follow-up. In our patients, IR also increased progressively with the impairment of glucose metabolism.

Conclusions: Unrecognized DM was more prevalent than recognized DM in acute stroke patients. It is advisable for clinicians to screen every ischemic stroke patient for impaired glucose tolerance (IGT) and IR to reduce their long-term morbidity and mortality.

Abstract ID: 1123: To Study the Role of Transcranial Magnetic Stimulation in Chronic Migraine

Cankatika Choudhury, Cankatika Choudhury, Neera Chaudhry, Sumirini Puppala

Govind Ballabh Pant Institute of Postgraduate Medical Education (GBPIPMER), New Delhi, India

Background and Aim: Chronic migraine is more debilitating and impairs the quality of life. Cortical excitability can be modulated by repetitive transcranial magnetic stimulation (TMS). Given in repeated pulses, it can produce long-lasting plastic effects. This study aims to study and compare the effects of high-frequency, low-frequency, and sham therapy in chronic refractory migraine.

Methodology: Patients ≥ 18 years of chronic migraine who were medically refractory were included. A total of 103 patients were included of which 35 were given high-frequency rTMS (7Hz), 37 low-frequency rTMS (1Hz), and 31 sham therapy, and all three groups were compared. The study was carried out over 1 year. A total of 70% of the motor threshold was used as intensity and an air-cooled figure of eight coils was used. Three sessions of the alternate day were delivered to the left frontal cortex comprising 600 pulses in ten trains. Headache severity was calculated by visual analogue scale (VAS), disability assessment by migraine disability assessment scale (MIDAS), and headache impact test-6 (HIT-6) at the end of the second, fourth, eighth, and twelfth week.

Results: Post rTMS VAS scores compared between the three groups showed significant differences at 2 weeks. Post rTMS VAS score in HF rTMS was 6.62 ± 0.97, and subgroup analysis showed better mean VAS scores at 2 weeks in HF rTMS group. HIT six at 4 weeks was significantly less in the HF rTMS group. The mean number of attacks was significantly lower in the HF rTMS group at 4 weeks.

Conclusions: HF rTMS is a good modality of preventive treatment in chronic migraine.

Abstract ID: 1124: Call Fleming Syndrome

Rashmi Rajur, Rashmi Rajur, Z. K. Misri

Kasturba Medical College Hospital (KMC) Bangalore, Karnataka, India

Background and Aim: Reversible cerebral vasoconstriction syndrome is an acute-onset clinical syndrome characterized by recurrent thunderclap headaches and diffuse reversible segmental narrowing of at least two cerebral arteries. In view of the high risk of stroke in reversible cerebral vasoconstriction syndrome (RCVS), RCVS should be considered in the differential diagnosis of cryptogenic stroke particularly if the stroke is accompanied by headache. The prognosis in most of the patients RCVS appears to be self-limiting while Stroke is the major determinant of persistent morbidity. Hence, it is important to differentiate between the two and treatment accordingly.

Case report: A 50-year-old man came with a thunderclap headache, and his brain was normal. The headache has not subsided with any medications. Digital subtraction angiography (DSA) was performed which showed vasoconstriction.

Discussion: After a detailed workup, the patient is found to have RCVS. Before and after treatment, DSA showed significant changes.

Conclusions: Early diagnosis and treatment may result in the resolution of symptoms and prevent further progression.

Abstract ID: 1125: An Unusual Case of Dyke–Davidoff–Masson Syndrome Revealed by Epilepsy

Shivam Sharma

National Institute of Medical Science & Research, Jaipur, Rajasthan, India

Background and Aim: Dyke–Davidoff–Masson syndrome (DDMS) is a rare syndrome characterized by typical manifestations including drugs resistant epilepsy, intellectual disability, hemiparesis, skull bone thickening associated with cerebral hemi atrophy, and hyper-pneumatization of paranasal and frontal sinuses on neuroimaging. Some patients may present with additional symptoms like cerebellar and basal ganglia atrophy and neuropsychiatric disorders. Congenital causes of DDMS include intrauterine vascular injury and cerebral hemispheric hypoperfusion. Acquired causes mostly derive from birth trauma, periventricular leukomalacia, cerebral hemorrhage, cerebral infarction, cerebral infection, radiation, postictal cerebral hemi atrophy, and prolonged seizures.

Case report: A 21-year-old male presented to the Department of Neurology with a 7-year history of seizures and violent antisocial behavior. He was thoroughly examined and investigated. Injectable antiepileptic drugs and antipsychotic tablets were given to the patient to control seizures and antisocial aggressive behavior. After that, the disease course remarkably changed to fewer seizures. Then, we added oral valproic acid (500 mg twice a day) which drastically improved the seizures to a maximum of two seizures daily.

Discussion: Examination of the patient revealed drug-resistant epilepsy, intellectual disability, hemiparesis, skull bone thickening, and ankylosis of the right wrist associated with cerebral hemi atrophy and hyperpneumatization. Electroencephalogram (EEG) showed a slowed background with delta wave activities in hyperpnea and continued focal to diffuse pseudo-periodic activities.

Conclusions: This case is presented as the Dyke–Davidoff–Masson syndrome. It highlights the challenges of an early diagnosis and the treatment of this rare condition. We suggest that in the presence of pharmaco-resistant epilepsy in childhood and body deformities, further investigations should be undertaken to establish an evidence-based diagnosis.

Abstract ID: 1126: Lead Poisoning Masquerading as PRES

Keerthi M, Suryaprabha T

Nizam’s Institute of Medical Sciences (NIMS), Hyderabad, Telangana, India

Background and Aim: Posterior reversible encephalopathy syndrome (PRES) is a condition characterized by headache, altered mental status, seizures, and visual disturbances, with white matter changes in the posterior cerebral hemispheres. Lead poisoning, a rare but serious condition, presents with a variety of neurological symptoms, complicating its diagnosis. This case is of lead poisoning presenting with clinical features and imaging suggestive of PRES.

Case report: A 61-year-old male, diabetic, hypertensive with past history of coronary artery disease (CAD), presented to our emergency with a history of altered sensorium for 1 week in the form of excessive drowsiness but was arousable and a history of one episode of generalized tonic–clonic seizure (GTCS) seizure. Past history of recurrent abdominal pain for 2 months, extensively worked up, proved unfruitful. On examination, he was drowsy, arousable, obeying stage 1 commands, with stable vitals. Pallor was present. Rest central nervous system (CNS) examination was unremarkable.

Discussion: Routine blood investigations were normal except for iron deficiency anemia. Magnetic resonance imaging (MRI) brain revealed ill-defined T2/FLAIR hyperintensities in the medial occipital lobes bilaterally which were hypointense on T1, no diffusion restriction, negative susceptibility weighted imaging (SWI), and no contrast enhancement. Cerebrospinal fluid (CSF) analysis, normal glucose, elevated protein 224 mg/dL, nine cells, and 100% lymphocytes. Further history disclosed the patient’s chronic usage of herbal medication for diabetic control, raising suspicion of heavy metal exposure. Serum lead levels were significantly elevated, >100 mcg/dL. The patient was treated with intravenous chelation therapy using ethylenediaminetetraacetic acid (EDTA) and supportive care. The patient improved symptomatically and blood lead levels returned to normal levels post-treatment.

Conclusions: Lead poisoning can mimic PRES, complicating the diagnostic process and potentially delaying appropriate treatment. Clinicians should maintain a high index of suspicion for lead toxicity in patients presenting with PRES-like symptoms.

Abstract ID: 1127: Unraveling the Triad of Migraine Headache, Noonan, and Moyamoya Syndrome

Apoorva Tomar

Govind Ballabh Pant Institute of Postgraduate Medical Education (GBPIPMER), New Delhi, India

Background and Aim: Moyamoya angiopathy is characterized by progressive stenosis of the terminal portion of the internal carotid arteries and the development of a network of abnormal collateral vessels. A few isolated reports have described an association between Noonan syndrome and Moyamoya syndrome.

Case report: We report a case of a 20-year-old boy with Noonan syndrome presenting with recurrent migraine-like headaches and recurrent episodes of transient quadriparesis following eating hot/spicy foods for the last 2 years.

Discussion: A 20-year-old boy presented to our institution with 1-year history of recurrent headaches. He also complained of a peculiar history of episodes of transient weakness of all four limbs for the past 3 years. These episodes would mostly occur while eating hot/spicy food. He had facial dysmorphism which led to his diagnosis of underlying Noonan syndrome. Computed tomography (CT) angiogram of the head and neck revealed an occlusion of both internal carotid arteries and reconstitution of the distal middle cerebral arteries via collaterals. He was treated with nimodipine and low-dose aspirin and is being asymptomatic and doing well.

Conclusions: To our knowledge, this was the first reported case of combined Noonan and moyamoya syndrome presenting as transient ischemic attack (TIA) following hot/spicy foods. This case highlights the need to recognize moyamoya syndrome in patients with Noonan syndrome. Another important learning point was hot/spicy food can be a trigger for clinical symptoms in moyamoya angiopathy.

Abstract ID: 1128: Superficial Cerebral Siderosis with Bloody Cerebrospinal Fluid in a Young Male: An Unresolved Diagnostic and Therapeutic Enigma

Bhupender Bajaj, Bhupender Kumar Bajaj, Arnesh Bhattacharya, Milan Mori, Saloni Gupta, Ajay Chaudhary

Vardhman Mahavir Medical College (VMMC), New Delhi, India

Background and Aim: Superficial central nervous system siderosis constitutes hemosiderin deposits in the meninges and superficial cerebrum, cerebellum, and/or spinal cord. We report a case of a young male with superficial cerebral hemosiderosis and review the diagnostic and therapeutic challenges of the condition.

Case report: A 25-year-old male presented with holocranial dull aching headache and progressive deterioration of vision in both eyes of around 4 months duration. He had two brief episodes of short-duration behavioral arrest over the previous 2 years. The patients had a history of head trauma around 2.5 years before presentation. On examination, he was conscious-oriented with mini mental state examination (MMSE) and frontal assessment battery (FAB) scores of 26/30 and 13/18, respectively. There was only perception of light for the right eye and finger counting at 1 m with the left eye. There was no motor or sensory impairment or meningeal signs of irritation. Computed tomography (CT) scan brain showed a prominent subarachnoid space with scalloping of calvaria of the skull and prominent ventricular system. Lumbar puncture revealed frank red-colored blood mixed with cerebrospinal fluid at a raised pressure, raised protein (1755 mg/ dL), normal glucose (88 mg/dL), and negative workup for infection and malignancy. Magnetic resonance imaging (MRI) brain showed cerebral and cerebellar superficial hemosiderosis. CT angiography and digital subtraction angiography-intracranial vessels were unremarkable. Right temporal meningeal and brain biopsy revealed foci of hemorrhage, hemosiderin deposits, and gliosis.

Discussion: The patient was diagnosed with chronic superficial cerebral hemosiderosis with raised intracranial pressure due to chronic subarachnoid hemorrhage.

Conclusions: Chronic subarachnoid hemorrhage with superficial cerebral hemosiderosis is a challenging diagnosis, and in the absence of detection of any obvious underlying cause, treatment options are limited.

Abstract ID: 1129: Juvenile Onset Rapidly Progressive Epileptic Encephalopathy Due to STXBP1 Gene Mutation: A Diagnostic Conundrum

Saloni Gupta, Bhupender Kumar Bajaj, Aadithya Narayan Sunil Kumar

Vardhman Mahavir Medical College (VMMC), New Delhi, India

Background and Aim: Syntaxin-binding protein-1 (STXBP1) gene encodes a presynaptic protein, MUNC18-1 located on chromosome 9q34.1. Patients with STXBP1-related disorders are often found to have early-onset epilepsy, intellectual disability, and/or movement disorder.

Case report: A 12-year-old girl born out of non-consanguineous marriage with full-term normal vaginal delivery, apparently normal developmental history, and average premorbid school performance presented with new onset focal/generalized seizures, rapidly progressive cognitive impairment, and ataxia for 7 months. She had a history of pica. During the hospital stay, she passed multiple Ascaris worms in her stool and had fertilized eggs of Ascaris and cysts of E. histoytica on stool examination. Magnetic resonance imaging (MRI) brain showed diffuse cortical and cerebellar atrophy. Lumbar puncture was normal. Autoimmune encephalitis panel, measles antibodies, and anti-thyroid peroxidase (anti-TPO) levels were normal. Electroencephalogram (EEG) showed generalized slowing of 3–4 Hz with intermittent generalized sharp wave complexes. The patient was treated with antiepileptic medicines and treated for intestinal ascariasis and amoebiasis. The patient was subsequently managed as a case of possible autoimmune encephalitis and received plasmapheresis and pulse steroids without any improvement. Whole exome sequencing showed a heterozygous variation in the STXBP1 gene.

Discussion and conclusion: STXBP-1 gene mutation has heterogenous clinical phenotypes and may present beyond neonatal or infantile period with rapidly progressive epileptic encephalopathy.

Abstract ID: 1130: Retrospective Study of Clinical, Imaging, Laboratory Parameters, and Outcomes of Patients Presenting with Acute Ischemic Stroke to A Tertiary Care Hospital in Gujarat and Comparison Between Patients Treated with Intravenous Thrombolysis vs Medical Management

Ratnesh Sharma, Pankti Sapan Pandya, Udit Dineshbhai Patel, Shubham Ghanshyambhai Malani, Sudhir V Shah, Shalin D Shah, Pranav B Joshi, Arvind Sharma

Smt NHL Municipal Medical College, Ahmedabad, Gujarat, India

Background and Aim: Acute ischemic stroke is a common cause of disability and mortality in developing countries like India. Our aim in this study is to investigate clinical, radiological, and laboratory parameters and outcomes of patients presenting with acute ischemic stroke at a tertiary care hospital in Gujarat and compare the same between patients treated with intravenous thrombolysis [tenecteplase (TNK) & recombinant tissue plasminogen activator (rt-PA)] vs medical management without IV thrombolysis.

Methodology: Retrospective data of 180 patients over 18 years of age diagnosed with acute ischemic stroke was collected, of which 90 received intravenous thrombolysis with r-TPA/TNK and 90 were given medical management as they gave negative consent for intravenous thrombolysis. Clinical, radiological, and laboratory parameters were compared along with outcomes at day 7, day 14, and day 90.

Results: Patients who received IV thrombolysis were more likely to have better outcomes and lower mortality rates as compared to the patients who did not receive IV thrombolysis. No significant differences were noted between patients receiving IV thrombolysis with r-TPA vs TNK.

Conclusions: Patients who received IV thrombolysis were more likely to have better outcomes and lower mortality rates as compared to the patients who did not receive IV thrombolysis. No significant differences were noted between patients receiving IV thrombolysis with r-TPA vs TNK.

Abstract ID: 1133: Prevalence of Autonomic Dysfunction in Various Subtypes of GBS in the Acute Phase and its Correlation with Antiganglioside Antibody Profile

Bharanidharan G, Sruthi S Nair, Sajith Sukumaran

Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Thiruvananthapuram, Kerala, India

Background and Aim: Autonomic dysfunction which affects about 2/3 of patients with GBS is frequently unnoticed despite its significant impact. This study aims to estimate the prevalence of autonomic dysfunction in the acute phase of Guillain-Barré syndrome (GBS) and to look for any association between the presence of specific antiganglioside antibodies with autonomic dysfunction in GBS.

Methodology: All consecutive patients diagnosed with GBS between November 2022 and May 2024 have been enrolled. Standard investigations, including nerve conduction study (NCS), cerebrospinal fluid (CSF) analysis, and antiganglioside antibody panel, were conducted. Sympathetic function was evaluated with postural hypotension and a cold immersion test. The parasympathetic function was assessed via heart rate variability to posture and deep respiration using a surface electrocardiogram (ECG) rhythm strip.

Results: A total of 32 patients were recruited, of which 21 were male, and 11 were female. Respiratory tract infection is the most common antecedent factor. The mean time gap between the antecedent event and the onset of symptoms is 3.87 days (6.99). A total of 90% patients presented to the hospital within 2 weeks of symptom onset. The most common subtype is AIDP (46.9%), followed by acute motor axonal neuropathy (AMAN) (15.6%). The prevalence of autonomic dysfunction was estimated to be 40.6%. Association with antiganglioside panel could not be established due to low positivity (9.3%). The cold immersion test is the best performer with the highest area under the curve (AUC) (0.820) with a decent sensitivity (69.2%) and low false positive rate (5.3%). Sympathetic skin responses (SSR) performed moderately well with an AUC of 0.743, moderate sensitivity (53.8%), and low false positive rate (5.3%).

Conclusions: The prevalence of autonomic dysfunction and the presence of antiganglioside antibodies could not be correlated due to the low sample size and low positivity rate. SSR and cold immersion tests were found to be sensitive bedside tests in detecting autonomic dysfunction.

Abstract ID: 1134: Myofibrillar Myopathy with Spasticity and Hyper-Reflexia: Possible Mechanisms and New Phenotypes

Shivam Mirg, Deepti Vibha, Jasmine Parihar, Manjari Tripathi, Arunmozhimaran Elavarsi, Animesh Das

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Myofibrillar myopathy is an umbrella term for a group of inherited neuromuscular disorders that share a common histopathological phenotype. It is characterized by the disintegration of networks of intermyofibrillar protein around the Z-disk followed by their intra-sarcoplasmic accumulation leading to a myopathy.

Case report: In this case, a 49-year-old male presented with a 7-month history of insidious onset, asymmetric, progressive quadriparesis with wasting, spasticity, and hyper-reflexia. Clinical presentation was consistent with amyotrophic lateral sclerosis (ALS).

Discussion: Electromyography revealed spontaneous activity (fasciculation, fibrillation, and positive sharp waves) and large amplitude long duration motor unit action potentials in cervical, thoracic, lumbo-sacral, and bulbar segments supplementing diagnosis of ALS. However, he had elevated muscle enzymes. Exome sequencing revealed two heterozygous autosomal dominant mis-sense variants in TTN (c.820222G>A) and FLNC (c.2560G>A) genes. Electron microscopy of the muscle biopsy specimen showed destruction of the Z band and disorganization of the myofibrillary network. Biopsy was hence compatible with a diagnosis of myofibrillar myopathy.

Conclusions: Elevated creatine kinase more than five times in patients with clinical presentation of Amyotrophic lateral sclerosis should always prompt further investigation and can be part of the myofibrillar myopathy spectrum.

Abstract ID: 1135: Acute Ischemic Stroke (AIS) with Large Vessel Occlusion (LVO): A Case Report of Fast Progressor with Good Functional Outcome

Garima Srivastava, Garima Srivastava, Awadh Kishor Pandit, Shailesh Gaikwad, Achal Kumar Srivastava, Ajay Garg, Rohit Bhatia, Deepti Vibha, Leve Joseph

All India Institute of Medical Sciences Delhi, India

Background and Aim: Acute stroke comprises 80% of ischemic strokes out of which approximately 30% of ischemic strokes are due to large vessel occlusions. Nearly 25% of these patients develop rapid infarct growth in the first few hours of anterior circulation large vessel occlusion (LVO) and are known as fast progressors. The temporal expansion of the ischemic core after large vessel occlusion (LVOS) is highly variable among individuals where some have rapid infarct growth rates (IGRs) known as fast progressors. Hence, determining factors associated with infarct growth rate (IGR) in the setting of LVO over 24 h will help in early patient triaging for an appropriate course of action. To address this hypothesis, we present here a case report of fast progress in our hospital setting.

Case report: This is a case report of an observational study approved from the Institute’s ethics committee. A 65-year-old male patient with past history of coronary artery bypass graft (CABG) developed right middle cerebral artery (MCA) territory stroke 3 h 5 min from last known well. His admission national institutes of health stroke scale (NIHSS) score was eight. Hence, he was thrombolyzed with an injection of tenecteplase 15 mg. His computed tomography (CTA) was also s/o large vessel occlusion hence taken up for mechanical thrombectomy. Post-thrombolysis and post-thrombectomy patient’s NIHSS improved to 5 and 4, respectively. Following post-thrombectomy, he reported to have persistent hypotension for which inotropes were started. Later, inotropes tapered off, and the patient was discharged in hemodynamically stable condition with modified rankin scale (mRS) 1.

Discussion and Conclusions: Infarct growth rate is a critical determinant of tissue damage and functional outcome in acute ischemic stroke. Early intervention is essential to prevent further infarct growth for better patient’s outcomes. In hospital settings where magnetic resonance imaging (MRI) imaging is not available, the determination of infarct growth rate (IGR) in acute ischemic stroke (AIS) patients can still be estimated based on clinical and other factors.

Abstract ID: 1136: Relapsing Granulomatous Acanthamoebic Encephalitis – An Extremely Rare Entity

Yamini Priyanka PentaKota, Divyani Garg, Ayush Agarwal, Awadh Kishor Pandit, Ajay Garg, Achal Kumar Srivastava

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Acanthamoeba species are ubiquitously present in water, soil, and dust. Human exposure is common but infection is rare, affecting skin, eyes, lungs, and brain. The life cycle comprises two stages – cyst and trophozoite (infective). Granulomatous amoebic encephalitis (GAE) is a fatal brain infection that typically affects immunocompromised hosts. The clinical features include focal neurological deficits, headache, cognitive decline, and seizures, and mortality remains high. We report an extremely unusual case of relapsing GAE due to Acanthamoeba.

Case report: A 33-year-old male presented with fever, right hemiparesis, and altered sensorium for 1 month. He had a significant past history of bilateral periorbital swelling, recurrent fever, cognitive decline, seizures, and focal neurological deficits for which he had been evaluated at our center 2 years previously, and had been diagnosed to have GAE based on magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) findings of Acanthamoeba (free loving trophozoites), confirmed by CSF polymerase chain reaction (PCR). He was treated with poly-antibiotic therapy for 1.5 years, which was stopped due to CSF and MRI resolution. He maintained good clinical status with mild residual cognitive deficits, for the next year.

Discussion: In this admission, a relapse was suspected in view of clinicoradiological worsening. CSF, done thrice, did not reveal free-living amoebas. A brain biopsy was performed which revealed trophozoites. The patient was initiated on a poly-antibiotic regimen with which he showed significant improvement. He was HIV-negative and had no apparent immune deficiency.

Conclusions: Relapsing GAE is extremely rare, and factors responsible for relapse need further exploration. Successful treatment regimens merit further consideration.

Abstract ID: 1137: Correlation of Ataxia Rating Scale with Tremor Scale and Cognitive Scale in SCA 12 Patients

Sahaj Agrawal, Achal K Srivastava, Divyani Garg, Awadh Kumar Pandit, Divya MR, Roopa Rajan, Animesh Das, Ayush Agarwal

All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background and Aim: Spinocerebellar ataxia type 12 (SCA12) is an autosomal dominant neurodegenerative disorder associated with expansion of CAG repeat in the gene PPP2R2B. Tremor was the initial presenting symptom in most of the cases, and it could be of resting, postural, or action type. This study aimed to see a correlation between the Ataxia rating scale, tremor rating scale, and Ataxia rating scale with cognitive assessment. Patients with SCA 12 also present with cognitive decline over time.

Methodology: A total of 45 patients with SCA 12 were evaluated for scale for the assessment and rating of ataxia (SARA), Fahn Tolosa tremor rating scale, and Montreal cognitive assessment, and their severity was compared

Results: 45 patients 22 (49%) of the patients showed moderate disability on the tremor rating scale. Out of these patients, 2 (9%) had minimal ataxia 11 (50%) showed mild ataxia, and 9 (41%) showed moderate ataxia. A total of 17 (38%) showed mild disability in the tremor rating scale, 6 (35%) showed minimal ataxia, 7 (41%) showed mild, and 4 (24%) showed moderate ataxia. A total of six patients who showed moderate disability on the tremor rating scale had 5 (83%) patients with moderated ataxia and 1 (17%) mild ataxia. Out of these patients, 7 (15%) had no dementia, 31 (69%) had mild mild cognitive impairment (MCI), 3 (7%) had mild dementia, and 4 (8%) had moderated dementia. A total of 14 (45%) had mild ataxia, 11 (35%) had moderate ataxia, and 6 (13%) had minimal ataxia.

Conclusions: As the severity of ataxia increases, patients have an increase in the worsening of the tremor as well as cognition. For the patients with SCA 12, cognition and tremor assessment should be performed along with the ataxia as they both also form an important part of the disease spectrum.

Abstract ID: 1138: Neuromeliodosis: Underdiagnosed Clinical Entity

Sufiya Almas, Varun Reddy Gundluru

Yashoda Hospitals, Malakpet, Telangana, India

Background and Aim: Melioidosis is a well-recognized disease entity caused by Burkholderia pseudomallei . It is a systemic disease and has protean manifestations. Neuromelioidosis is rare, comprising 4% of all melioidosis cases associated with higher rates of mortality and morbidity.

Case report: A single case study, contrast enhanced computed tomography (CECT) abdomen, magnetic resonance imaging (MRI) brain with contrast, and cardiac MRI were conducted.

Discussion: The patient was continued on intravenous meropenem. The power of the left upper and lower limbs improved to almost normal.

Conclusions: Neuromelioidosis is an emerging, underdiagnosed, and treatable disease. Mortality in patients with neuromelioidosis has been reported up to 20%; therefore, it is vital to consider the possibility of neuromelioidosis in a diabetic patient presenting with systemic features – early suspicion and recognition of the condition are important for initiation of early treatment, as it can result in good functional outcome.

Abstract ID: 1139: Medication, Myoclonus, and the Cerebellum

Sanjay Rao Kordcal, Aparna Ramakrishna Pai, Sripadma MV, Prashant Bhatele, Nishant Yadav, Chinni Lavanya, Prashant Bhatele, Sripadma MV, Aparna Ramakrishna Pai

Kasturba Medical College (KMC), Manipal, Karnataka, India

Background and Aim: Although the neurotoxic complications of anti-tubercular drugs like isoniazid (INH) are well known, cerebellitis and bilateral dentate nucleus hyperintensity are seldom encountered complications. Its occurrence is usually associated with concomitant renal disease and hemodialysis.

Case report: We report a case of a chronic kidney disease (CKD) patient on anti-tubercular therapy (ATT), who presented with myoclonus and in whom a diagnosis of Isoniazid-induced cerebellitis, involving the dentate nuclei bilaterally, was considered.

Discussion: The patient is a 51-year-old man, previously diagnosed with stage 5 CKD, who presented with complaints of dyspnea on exertion, dry cough, and left-sided chest pain. Computed tomography (CT)Thorax showed a left-sided pleural effusion. The patient was started on empirical anti-tubercular therapy for suspected tuberculosis. After starting anti-tubercular treatment, the patient complained of abnormal jerky movements. These jerky movements lasted for a second. They involved both the axial and appendicular regions. The patient did not have any features of encephalopathy, and his higher mental function testing revealed no abnormalities. The remaining neurological exam was also normal. No cerebellar signs could be elicited. Blood investigations and electroencephalogram (EEG) were non-contributory. Magnetic resonance imaging (MRI) brain showed symmetrical hyperintensities involving the dentate nuclei on both sides. Based on the above findings, a diagnosis of drug-induced myoclonus was considered.

Conclusions: This is the first case in literature implicating INH as the cause of myoclonus with concomitant dentate nucleus lesions.

Abstract ID: 1140: Unilateral Calf Hypertrophy After Atrophy – An Interesting Case Report

Chebolu Harshika, Randhi Venkata Narayana, Puvvada Srikar, Amarapu Sravanthi

Sevenhills Hospital, Visakhapatnam, Andhra Pradesh, India

Background and Aim: Unilateral calf swelling can occur due to various causes like Iliac vein compression, deep vein thrombosis superficial thrombophlebitis, ruptured Baker’s cyst, ruptured leg muscle, sprain/strain, fracture, lymphedema, intramuscular hematoma, muscular infarction (particularly in diabetes), etc.

Case report: A 45-year-old man, known diabetic, no addictions, history of left lower limb poliomyelitis, and thinned out limb since the age of 8 years, presented with a sudden increase in the size of the calf muscle on the left side since 1 month and occasional paresthesias in left foot and sole. There was no history of low back ache, neck pain, tingling, paresthesias, and numbness over other limbs, visible swellings over lower limbs, or trauma. There is the history of lifting heavy weights. On examination, the left thigh circumference was 2 cm more, and the left calf circumference was 0.5 cm less compared to the right side. The ankle jerk was depressed on the left side.

Discussion: Routine blood investigations were normal. Color doppler of the arterial and venous systems of the left lower limb revealed no abnormality. A nerve conduction study of the left lower limb revealed an axonal neuropathy pattern with reduced amplitudes in the left peroneal and tibial nerves. Magnetic resonance imaging (MRI) study of lumbosacral spine was performed which revealed slight midline protrusion of the L3-4 intervertebral disc with no significant compression of nerves, the L4/L5 and L5-S1 intervertebral discs protruding toward the back moderately asymmetrically, more on the left side, with thecal sac and left nerve root indentation without cord compression.

Conclusions: Neurogenic muscle hypertrophy, referred to as “denervation hypertrophy,” is caused mainly by compression of the nerve root, most commonly the S1 root affecting the calf muscles. The pathomechanism of muscle hypertrophy is not completely clear and is certainly multifactorial.

Abstract ID: 1141: An Interesting Case of Ocular Neuromyotonia Associated with Thyroid-Related Ophthalmopathy

R. Pazhani, Pushkar P, Kavya S

Madurai Medical College, Madurai, Tamil Nadu, India

Background and Aim: Thyroid-related ophthalmopathy can present with various ocular manifestations. An interesting case of ocular neuromyotonia associated with thyroid-related ophthalmopathy is discussed here.

Case report: Fifty-year-old gentleman presented with intermittent tonic upward deviation of the left eyeball with no double vision for the past 6 months. He c/o prominence of the left eyeball. There is no h/o double vision. There is no h/o pain in the orbit or eyeballs on either side. There is no diurnal variation in the symptoms. There is no h/o difficulty in swallowing or weakness in the limbs.

Discussion: He was seen in the outpatient service. In the resting position, the right eye was normal. The left eyeball was deviating upwards, and it was corrected in 2–3 min. He had two episodes like this in 30 min. It does not disturb his day-to-day activities. His thyroid function was normal. Anti-thyroid peroxidase (ati-TPO) antibody level was increased to 934.81 IU/mL. His magnetic resonance imaging (MRI) orbit study showed mild enlargement of the right inferior rectus muscle and bilateral superior rectus muscles. His other autoimmune markers, including antinuclear antobody (ANA) and extractable nuclear antigen (ENA), were negative. The patient was treated with T Carbamazepine 200 mg twice daily. The ocular neuromyotonia responded to treatment.

Conclusions: This is an interesting case of ocular neuromyotonia associated with thyroid-related ophthalmopathy. The patient responded to carbamazepine. It is important to look for uncommon presentations of common neurological disorders in clinical practice.

Abstract ID: 1142: Emamectin Benzoate Poisoning – Perplexing History and Treatment a Mystery

Ashwini Hiremath

BLDE (DU) Medical College, Bijapur, Karnataka, India

Background and Aim: Emamectin benzoate poisoning is a broad-spectrum insecticide used to treat vegetables. It is a semisynthetic derivative of avertmectin. There is a significant paucity of literature due to its uncommon consumption and underreporting of cases. It interacts with the gamma-aminobutyric acid (GABA) receptors. Patients predominantly present with severe abdominal pain, gastritis, and vomiting in the initial stages. Later, they have central nervous depression, agitation, and seizures. Unfortunately, no specific antidotes exist, and most times, it has proven fatal despite thorough symptomatic treatment. This study aims to raise awareness about uncommon yet fatal poisoning caused by emamectin benzoate, a broad-spectrum insecticide.

Case report: Records of two patients were retrieved with emamectin benzoate poisoning. There were two male patients with emamectin benzoate poisoning in the last 2 years. Case 1: a 28-year-old presented with acute abdomen. On day 3, he developed severe aggression and status epilepticus and was intubated. Until then, it was assumed to be unknown compound poisoning. He also received Inj Midazolam. We realized it to be emamectin only after they got the can. Case 2 presented with severe breathlessness and drowsiness only 2 h after consumption of 500 mL of the above poison. Pupils in case 1 were unequal and sluggishly reactive whereas normal in case 2. Extensive investigations included a magnetic resonance imaging (MRI) brain, cerebrospinal fluid (CSF) studies, toxic screening, USG abdomen, and blood reports were normal. Case 1 was managed for 15 days but finally succumbed. Case 2 expired on day 3 due to severe respiratory acidosis. Both were given gastric lavage with charcoal, antiepileptics, antibiotics, and thorough supportive care.

Discussion and Conclusions: There is a paucity of literature. Hence, awareness of its effects and treatment is essential among physicians/neurologists.

Abstract ID: 1143: A Rare Cause of Headache

Aare Sidhartha, Varun Reddy Gundluru, Sanket Kulkarni, Ravikanth J, Shashidhar Matam

Yashoda Hospital, Malakpet, Hyderabad, Telangana, India

Background and Aim: Spontaneous intracranial hypotension (SIH) is caused by cerebrospinal fluid (CSF) leakage without a history of trauma or dural puncture. It is common in middle-aged people. Incidence is estimated to be 5 per 100, 000 persons per year. Postural headache is the main presenting symptom.

Case report: A 45-year-old gentleman presented with a history of headache for the last 3 months that worsened in the last 1 week. The headache was holo-cranial, throbbing type which worsened in an upright position and subsided when he lay down. Due to this, he was unable to do his daily activities comfortably and could not discharge his duties at his workplace.

Discussion: In view of this positional variation of headache, an magnetic resonance imaging (MRI) brain was performed that showed bilateral subdural collections with features suggestive of intracranial hypotension. The patient was advised rest in lying down position and hydrated well. To identify any leak of cerebrospinal fluid (CSF), an MR Myelogram was performed. It showed a CSF leak on the left side at the C1–C2 level extending into adjacent posterior paraspinal soft tissues. Targeted epidural blood patching was performed in consultation with an interventional radiologist. A total of 5 mL of the patient’s autologous blood was drawn from peripheral access and injected slowly into the epidural space under computed tomography (CT) guidance. Gradually, the patient improved and experienced a reduction in his postural headaches, along with the ability to sit and mobilize for a long duration. He was also able to resume his daily activities. His review of CT brain at follow-up showed a significant reduction in subdural collections along with other changes related to spontaneous intracranial hypotension.

Conclusions: MR myelogram is an important diagnostic tool for identifying CSF leak. Epidural blood patching is an important treatment modality.

Abstract ID: 1144: LRP4-Positive Myasthenia Gravis Presenting as Unexplained Respiratory Failure: A Report of Two Cases

Dhanya Sureddy, Vijaya Shankar P, Muthukani S, Vijayashankar P

Apollo hospitals Chennai, Tamil Nadu, India

Background and Aim: Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder characterized by varying degrees of skeletal muscle weakness. While the condition is typically associated with antibodies against the acetylcholine receptor (AChR) or muscle-specific kinase (MuSK), less commonly, antibodies against low-density lipoprotein receptor-related protein 4 (LRP4) are implicated. MG with LRP4 antibodies can present with atypical symptoms, complicating diagnosis and management.

Case report: 1. Case 1: A 65-year-old male k/c/o T2 diabetes mellitus (DM), hypertension (HTN), chronic kidney disease (CKD) on hemodialysis presented with acute, unexplained respiratory failure requiring mechanical ventilation. Initial evaluations, including imaging and standard neurological examinations, were inconclusive. Neostigmine test was performed when he was intubated which showed improvement in spontaneous breaths. Serological tests revealed elevated anti-LRP4 antibodies. Treatment with intravenous immunoglobulin (IVIG) and corticosteroids resulted in marked clinical improvement. 2. Case 2: A 35-year-old male was admitted with progressive dyspnea and episodes of respiratory failure for 6 months. Extensive workup, including pulmonary function tests and chest computed tomography (CT) scans, failed to identify a primary respiratory pathology. Repetitive nerve stimulation showed a decremental pattern making a diagnosis of MG. The serological analysis identified high titers of anti-LRP4 antibodies. The patient responded positively to plasmapheresis, with significant improvement in respiratory function.

Discussion: These patients responded positively to immunomodulation, IVIG, plasmapheresis, and corticosteroids with significant improvement in respiratory function.

Conclusions: Although LRP4-positive myasthenia gravis is generally described in the literature as a mild disease, it can present with atypical and severe manifestations such as unexplained respiratory failure. Recognizing this potential for atypical presentations is essential for early diagnosis and effective treatment.

Abstract ID: 1145: Atypical Presentation of Acute Inflammatory Demyelinating Polyradiculopathy: A Case of Asymmetric Weakness

Gadag Nagarjun

Manipal Academy of Higher Education (MAHE), Manipal, Karnataka, India

Background and Aim: Asymmetric weakness as a presenting feature in acute inflammatory demyelinating polyradiculopathy (AIDP).

Case report: It is a rare case report. Here is a gentleman in his late of 50s with nil comorbidities presented to neurology OPD with complaints of right upper and right lower limb weakness (proximal and distal) and difficulty in walking which was gradual in onset and progressive in nature since 20 days, also complains of left-sided facial deviation, difficulty in holding water in mouth since 20 days. At the time of presentation, vitals were stable with a blood pressure (BP) of 120/80 mmHg, pulse was 84 bpm, and regular and general physical examination was grossly normal. Neurological examination showed right lower motor neuron (LMN) facial palsy, power in right upper limb (proximal and distal) was 2/5, right lower limb (proximal and distal) was 2/5, left upper and lower limb power were 4+/5, global areflexia and sensory examination were normal, and rest neurological examination was unremarkable. Relevant blood investigations were unremarkable. Magnetic resonance imaging (MRI) brain with the whole spine was grossly normal. Cerebrospinal fluid (CSF) analysis showed albumino-cytological dissociation with CSF WBC of 32 with lymphocyte predominant and CSF protein of 142.9 mg/dL. Nerve conduction studies showed decreased compound muscle action potential (CMAP), preserved sensory nerve action potential (SNAP), conduction velocity decreased and prolonged F wave latencies in both upper and lower limbs. Treatment initially comprised of intravenous (IV) immunoglobulin of 2 g/kg over 5 days and other supportive measures. Physiotherapy was also initiated immediately.

Discussion and Conclusions: Asymmetric weakness is rarely demonstrated in AIDP. Our case highlights the importance of clinical examination in the current AI era.

Abstract ID: 1146: Uncommon Presentation of Stroke: Isolated Monoparesis Indicative of Posterior Borderzone Infarct

Vamsi Krishna Mudamanchu

Vydehi Institute of Medical Sciences and Research Centre (VIMS), Bengaluru, Karnataka, India

Background and Aim: This case highlights the uncommon presentation of stroke through isolated monoparesis, specifically manifested as acute weakness in the left hand. Magnetic resonance imaging (MRI) revealed a hyper-acute infarct in the watershed territory of the right middle cerebral artery (MCA) and posterior cerebral artery (PCA). Isolated hand palsy, often mistaken for a peripheral lesion, is a rare manifestation of stroke. This report discusses the infrequent occurrence of fractional limb weakness associated with acute ischemic stroke due to border zone infarction, particularly involving the parieto-occipital lobe.

Case report: Observational study. Magnetic resonance imaging revealed a hyper-acute infarct in the watershed territory of the right middle cerebral artery and posterior cerebral artery.

Discussion and Conclusions: A stroke presenting with isolated arm weakness may be misdiagnosed as a peripheral nerve disorder because of the absence of pyramidal tract signs or involvement of speech, face, or lower limbs. It is crucial to be mindful of the broad range of potential diagnoses for isolated hand weakness and to consider acute ischemic or embolic stroke as an infrequent but underlying cause of such clinical presentations.

Abstract ID: 1147: An Unusual Case of Idiopathic Hypertrophic Pachymeningitis Responsive Only to Steroids

Aare Sidhartha, E. Srikanth Reddy, G. Kehshava Anand

Yashoda Hospital, Malakpet, Hyderabad, Telangana, India

Background and Aim: Hypertrophic cranial pachymeningitis (HCP) is a rare chronic fibrosing inflammatory disease characterized by marked diffuse thickening of cranial dura matter, resulting in progressive neurological deficits. Chronic headaches and multiple cranial palsies are most common clinical manifestations. Various clinicopathological entities produce HCP, and idiopathic HCP is a diagnosis of exclusion.

Case report: A 45-year-old female, with no prior comorbid illnesses or addictions, presented initially with complaints of headache, hoarseness of voice, and mild facial deviation to the right. She was diagnosed clinically with Left lower motor neuron (LMN) VII, VIII, and X cranial nerve palsies.

Discussion: Magnetic resonance imaging (MRI) brain (plain/contrast) showed T2 FLAIR hyperintensity (with post-contrast enhancement) posterior to the nasopharynx, involving clivus and extending to bilateral adjacent parapharayngeal space. Pachymeningeal enhancement was noted along the right anterior temporal lobe. Vasculitic workup was negative transnasal endoscopic biopsy, serum. IgG4 levels and cerebrospinal fluid (CSF) analysis were normal. She was treated with oral steroids and immunosuppressants (MMF). Patient symptoms gradually improved but any attempts to gradually taper and stop steroids (as the patient developed chronic steroid usage-related complications like cushingoid features, and pathological vertebral body fractures) resulted in a flare of HCP and multiple cranial nerve palsies (subsequently, she developed bilateral LMN VII, VIII, and X, Left XII; Left III and VI, Right II, V1, III, IV, VI cranial nerve palsies). Later, she developed pulmonary tuberculosis (TB) and was started on antituberculous treatment (ATT) (rituximab is planned in review).

Conclusions: Idiopathic HCP is very rare and very difficult to diagnose, and treatment modalities steroids, immunosuppressive drugs, and other supportive treatments are required.

Abstract ID: 1148: Hyperhomocysteinemia in a Juvenile: A Treatable Metabolic Condition

Sufiya Almas, Surisetti Bharath Kumar

Apollo Hospital Chennai, Tamil Nadu, India

Background and Aim: Hyperhomocysteinemia is a metabolic condition characterized by elevated levels of homocysteine serving as a potential risk factor for serious complications. It has been associated with increased cardiovascular, cerebrovascular, and thromboembolic diseases.

Case report: A 15-year-old female presented with a history of difficulty in walking for the past 1 year, gradually progressive with mild behavioral disturbances, recent onset of headache, neck pain, and vomiting. On examination, spasticity of all limbs was noted with exaggerated reflexes, bulk and power were normal, spastic gait was observed, sensory examination was normal, and no cerebellar features were noted. There was no history of any delayed milestones. Previous magnetic resonance imaging (MRI) brain showed T2/FLAIR hyperintensities with corresponding hypointensities in T1 in the deep white matter of bilateral fronto-pareito-occipital regions, no contrast enhancement, MRI C spine showed insignificant changes. Now in view of the headache, an MRI with magnetic resonance venography (MRV) brain was repeated which showed evidence of cerebral venous sinus thrombosis (CSVT) with superior sagittal and left transverse sinus thrombosis. Coagulation workup showed elevated levels of homocysteine (251 micromol/L), rest was normal. Nerve conduction study (NCS) study was normal. Serum folate and vitamin B12 levels were normal. To rule out other genetic causes, an methylenetetrahydrofolate-reductase (MTHFR) genetic study was sent which turned out to be positive. Started on heparin and other supportive care. No new deficits during the hospital stay, headache, and neck pain gradually subsided, and repeat computed tomography (CT) brain did not show any new changes. The patient was discharged on DABIGATRAN and HOMIN.

Discussion: The patient on follow-up has partial improvement.

Conclusions: The diagnosis of disorders affecting homocysteine metabolism faces delay due to insufficient awareness of its clinical presentation and unique biochemical features. Nutritional approach targeting vitamin supplementations like vitamin B6, B12, and folate is necessary along with lifestyle modifications.

Abstract ID: 1150: The Clinical Profile of Migraine in Elderly versus Young – A Cross-Sectional Study

Neerupaka Venkatachalam, pooja Mailankody, Girish Baburao Kulkarni, Sivakumar P T, Seshagiri D V

National Institute of Mental Health and Neuro-Sciences (NIMHANS), Bengaluru, Karnataka, India

Background and Aim: The migraine types, presentation, and treatment options change with advancing age. Depression, anxiety, sleep issues, medication overuse, and co-existing medical comorbidities make the elderly migraine different from that in the younger age. We aimed to compare the clinical profile of migraine, associated symptoms, treatment profile, and comorbidities like depression, anxiety, and sleep issues in migraine between younger age and older age groups.

Methodology: The prospective cross-sectional study was conducted in the Department of Neurology at the National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India, after approval by the Institute Ethics Committee. We included 154 patients, fulfilling the International Classification of Headache Disorders (ICHD-3) criteria for migraine. They were assessed with history, clinical examination, severity of migraine (Migraine Disability Assessment Scale, MIDAS), headache impact test-6 (HIT-6), depression (Hamilton Depression Rating Scale, HAM-D), anxiety (Hamilton Anxiety Rating Scale (HAM-A), and sleep disturbances (Pittsburgh Sleep Quality Index, PSQI).

Results: In the old age group (age > 60 years, n = 81), females constituted 46 (56.8%), and males were 35 (42%). In the young age group (age 20–40 years, n = 73), 50 (68.5%) were females, and 23 (31%) were males. In the old age group, throbbing ( P value = 0.009) and vomiting ( P value = 0.003) are less common compared to the young age group. Depression was seen in 65.4% of the old age group compared to 42% seen in the young age group ( P value = 0.004). Anxiety was seen in 63% of the old age group and 78% of the young age group ( P value of 0.041).

Conclusions: Older patients with migraine have more frequent headaches, higher depression, and sleep disturbances along with less disability, anxiety, throbbing, and vomiting compared to young migraine patients. Our findings have implications for the management of migraine in the elderly, both from diagnostic and therapeutic perspectives.

Abstract ID: 1151: Impact of Audiovisual Brainwave Entrainment on Alpha Wave Activity: A Real-Time EEG Assessment

Om Prasad, Francisco J. Cidral

Gobind Ballabh Pant Hospital, Delhi University, New Delhi, India

Background and Aim: In this study, we aimed to investigate the impact of a 20 min audiovisual brainwave entrainment session on alpha brainwave activity using continuous real-time Electroencephalogram (EEG) monitoring.

Methodology: The study was conducted at the Electrophysiology Laboratory of Sri Sri Neuro Center in Warangal, Telangana. The research included 30 healthy participants aged 18 to 65 (male and female), with no neurological or psychiatric conditions. The BrainTap headset was used for audiovisual brain entrainment targeting alpha waves, and wave activity was measured before, during (EEG recording began 2 min after the beginning of the session), and after the session. The EEG data, recorded with a Nihon Kohden EEG machine, was preprocessed to ensure quality by removing artifacts and applying a notch filter. We used power spectral analysis and density spectral array (DSA) to evaluate the power spectrum within a frequency band of 8 to 13 Hz over time. Statistical analysis, including ANOVA or Kruskal-Wallis followed by multiple comparison tests, was conducted to compare alpha wave activity across the three phases.

Results: A significant increase in alpha amplitude was observed during ( P ≤ 0.0001) and after ( P ≤ 0.0001) the session with large effect sizes (1.52 and 1.73, respectively), as well as a significant increase in alpha power during ( P ≤ 0.0001) and after ( P ≤ 0.0001) the session also with large effect sizes (1.72 and 1.91, respectively).

Conclusions: The findings of this study demonstrate that a 20 min audiovisual brainwave entrainment session significantly enhances alpha brainwave activity in healthy adults. Both alpha amplitude and alpha power showed substantial increases during and after the session, as evidenced by the highly significant P values and large effect sizes. These results suggest that audiovisual brainwave entrainment is an effective method for increasing alpha brainwave activity, which could have implications for relaxation, mental clarity, and overall cognitive function. Further research may explore the long-term benefits and potential applications of this technique in various clinical and non-clinical settings.

Abstract ID: 1152: Two Cases of Atypical Presentation of Rabies – GBS Mimic/Post Vaccination Myelitis

Sai Sandeep Dumpa, Nikith A

Kasturba Medical College (KMC), Manipal, Karnataka, India

Background and Aim: Rabies is transmitted through the rabies-infected animal bite, primarily dogs. Furious type and paralytic type of rabies are the two clinically recognized forms. Paralytic rabies should be considered as a potential differential diagnosis in patients presenting with acute flaccid paralysis with atypical features. Rabies needs to be considered even in patients with acute progressive asymmetrical quadriparesis; despite receiving prior vaccination.

Case report: A 38-year-old male presented with h/o acute-onset progressive quadriparesis with bulbar involvement was treated initially as Guillain-Barré syndrome (GBS) and h/odog bite 2 months ago surfaced 3 days before death. A 25-year-old male, with h/o dog bite 1 month ago, completed five doses of rabies vaccination, now presented with acute progressive asymmetrical quadriparesis since 1 week. The diagnostic dilemma of vaccination-induced myelitis vs GBS was considered.

Discussion: In the first case, a nuchal biopsy was positive for rabies polymerase chain reaction (PCR). In the second case due to the rapid downhill course associated with delirium, an altered sensorium repeat magnetic resonance imaging (MRI) was performed which progressed to encephalomyelitis; repeat rabies PCR was sent and showed rising titers, and hence, rabies infection was considered and both patients succumbed to their illness.

Conclusions: In a rabies endemic region, any patient presenting with acute-onset quadriparesis with atypical features, paralytic rabies should be considered as a potential differential diagnosis. A patient with H/O of rabid animal bite should receive adequate post-exposure prophylaxis as stated by world health organization (WHO). Early diagnosis of paralytic rabies is crucial to prevent unnecessary treatment along the lines of GBS.

Abstract ID: 1153: Comparison of Motor and Non-Motor Symptoms in Young-Onset Parkinson S and Late-Onset Parkinson’s Disease

Sai Sandeep Dumpa, Aparna Pai

Kasturba Medical College (KMC), Manipal, Karnataka, India

Background and Aim: Parkinson’s disease (PD) is usually a disease of the elderly but a small percentage of the population develops young-onset Parkinson’s disease (below 50 years). Patients can be divided into several subtypes, and one of them is depending on the age of symptom onset. There is a heterogeneity in clinical presentation and prognosis between young onset PD (YOPD) and late-onset PD (LOPD). To compare clinical characteristics in YOPD and LOPD cases.

Methodology: This prospective study was conducted from September 2023 to May 2024 at the Neurology Department of Kasturba Medical College, Manipal. To evaluate motor and non-motor symptoms, we used Unified Parkinson’s Disease Rating Scale III (UPDRS III), non-motor symptoms questionnaire (NMSQ), and for the disease stage, we used the Hoehn and Yahr Scale.

Results: A total of 40 patients were studied. The median age in YOPD was 43. 3 years, and in LOPD, it was 61. 9 years with a higher prevalence in males. Tremor and bradykinesia were the most frequent predominant initial symptoms in both groups, while gait instability was more frequent in LOPD. The average UPDRS III score was lower in YOPD. The average Hoehn and Yahr Scale are lower in YOPD. The predominant non-motor clinical features in YOPD were depression and low mood, and in LOPD, predominant non-motor clinical features were urinary symptoms and constipation; fatigability is equal in both groups.

Conclusions: In our study, tremors and bradykinesia were the most frequent predominant initial presenting symptoms in both YOPD and LOPD, while gait instability was frequent in LOPD, and non-motor features constipation followed by sleep disturbance was more frequent in LOPD, while restless leg was the least reported in our study.

Abstract ID: 1154: Topographical Anatomy and Cytoarchitectonic Organization of Speech Areas of the Cerebrum in Tamil and Non-Tamil-Speaking Subjects

Nishal Thellakula, Sunil K Narayan 1 , Sulochana Shaktivel 2 , Vinod A Chaudhari 3 , Nagarajan L 4 , Rajeswari Aghoram 1 , Pawan Sabale 5

Departments of, 1 Neurology, 2 Anatomy, 3 Forensic Medicine and Toxicology and 4 Radiodiagnosis, JIPMER, Pondicherry, 5 Department of Forensic Medicine, B.Y.L Nayar Charitable Hospital, Mumbai, Maharashtra, India

Background and Aim : Tamil is arguably the oldest surviving classical language in the world and is considered to be the mother of all the Dravidian languages. Hindi is the most common language among most of the population of North India. Few studies are available on the topographical anatomy of speech areas and none on the cytoarchitectonic histology of speech areas. The present study focused on exploring any differences between the persons with Tamil and Hindi as their mother tongue.

Methodology: This was a single-blinded and single-center study that was conducted in Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER). The study tried to standardize brain autopsy and histopathological techniques for language areas using standard stains and to identify and distinguish the topographical and cytotechtonic differences in the language areas between two major linguistic and subracial ethnic groups. Tamil-speaking and non-Tamil-speaking (Hindi) groups were chosen for this pilot study. JIPMER: The ethics committee (Human) had approved study No. JIP/IEC/2021/187 dated 21/7/21. Based on the inclusion and exclusion criteria, brain samples from patients who were cognitively intact at death and died from clearly non-neurological causes were collected from the Department of Forensic Medicine & Toxicology, JIPMER, Puducherry, and the samples were transferred to the Department of Anatomy, JIPMER where the later part of the processing of the brain tissues, histology (Luxol fast blue and Toluidine blue stains were used), and images of the slides were taken. Written informed consent was taken from the legally accepted representative of the deceased before the start of the autopsy for the study. The brain weight was measured before fixation by using an electronic weighing machine and the volume of the brain was measured using the Archimedes principle (by immersing the brain in a measuring glass jar filled with water to measure the displaced volume of water before and after the immersing of the brain into the glass jar). The brain was put in a plastic jar containing 10% formalin in an upside-down position suspended on the basilar artery with the other end of the thread tied to the rod kept on the jar for carrying out the later processes on the brains. Gross morphology parameters like fronto-occipital length, lengths of lateral sulci, the distance between the Anterior Sylvian point and inferior Rolandic point, length of the posterior rami, anterior ascending, anterior horizontal rami of the lateral sulci, presence of diagonal and triangular sulcus, and the shapes of the rami of lateral sulcus were measured, and intra and intercomparisons of the measurements were performed. Using Image J software, the histology slides were analyzed, and statistical analysis was performed using SPSS software v.20. For the radiological part of the study, magnetic resonance imaging (MRI) scans were performed on a different set of samples with an equal representation of Tamil and non-Tamil (Hindi) speaking individuals. The volume of the Broca’s area was calculated using Syngovia Neuro 3D software and the vertical and horizontal lengths of the cerebellum were compared between the samples.

Results : Males had more neuronal density than females in the motor speech area. In contrast, the females had more neuronal density when both the motor and sensory speech areas were considered. The fronto-occipital length showed a significant difference between the left and right hemispheres with the left side being longer than the right. U/V-shaped configuration was more commonly observed on the hemispheres than the Y-shaped configuration. Hindi-speaking individuals exhibited a higher average neuronal count of 9.2 million neurons while Tamil-speaking individuals showed a lower average count of 8.1 million neurons, and this finding is possibly due to the share of participants among Tamil-speaking and non-Tamil-speaking cadavers. The imaging studies failed to show the differences between the language-speaking groups.

Conclusions : Dravidian languages remained relatively insulated for centuries due to geographical peculiarities of the region and are considered to have evolved from the proto-Dravidian roots and are relatively independent of influences from Sanskrit/Prakrit believed to have evolved from the Aryan languages Tamil-speaking individuals had insignificantly lesser number of neurons in relevant brain regions. However, neuronal density count alone does not correlate with functional efficiency, and more important are factors like synaptic density, synaptic arborization density, and neurohumoral receptor/channel density. Our study did not look into those finer differences. In this preliminary study, with limited study samples, there were only statistically insignificant differences in the neuronal density and neuronal count between the brains of Tamil and non-Tamil-speaking persons.