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Low CSF CD4/CD8+ T-cell proportions are associated with blood-CSF barrier dysfunction in limbic encephalitis.

PURPOSE: Investigating immune cells in autoimmune limbic encephalitis (LE) will contribute to our understanding of its pathophysiology and may help to develop appropriate therapies. The aim of the present study was to analyze immune cells to reveal underlying immune signatures in patients with temporal lobe epilepsy (TLE) with LE. METHODS: We investigated 68 patients with TLE with LE compared with 7 control patients with TLE with no signs of LE screened from 154 patients with suspected LE. From the patients with TLE-LE, we differentiated early seizure onset (<20 years, n = 9) and late seizure…

[Clinical analysis of 9 cases with Anti-leucine-rich glioma inactivated 1 protein antibody associated limbic encephalitis].

Objective: This study was to describe the clinical characteristics of Anti-leucine-rich glioma inactivated 1 protein(LGI1) antibody associated limbic encephalitis. Methods: Clinical data including clinical features, laboratory and radiological findings, treatment and prognosis of the 9 patients were analyzed. Results: In all 9 cases, 6 cases experienced epileptic seizure, 5 cases had psychosis, 7 cases presented with memory impairment, 4 cases showed faciobrachial dystonic seizure, 2 had refractory hyponatremia. One case presented with typically acute Guillain-Barre syndrome (GBS). Anti-LGI1 a…

A Neuropsychiatric Prelude to Unveiling Small Cell Lung Cancer with Suspected Paraneoplastic Limbic Encephalitis: A Case Report.

Small cell lung cancer (SCLC) is an aggressive form of lung cancer characterized by rapid growth and early metastases. As a neuroendocrine tumour, SCLC is especially notorious for various paraneoplastic syndromes, one of which is a rare neurological syndrome called paraneoplastic limbic encephalitis (PLE) that manifests with amnestic cognitive impairment and seizures. Here, we describe a case of a 53-year-old female who presented with neuropsychiatric symptoms of delusions, hallucinations, and cognitive impairment that started months prior to being diagnosed with extensive-stage SCLC. With no …

18FDG PET/CT Tumoral and Neurologic Therapeutic Response in a Case of Anti-GABABR Paraneoplastic Limbic Encephalitis.

A 70-year-old man with a history of small cell lung carcinoma 2 years earlier was addressed for the suspicion of a paraneoplastic limbic encephalitis. Brain 18FDG PET/CT revealed a bilateral amygdalian and hippocampal hypermetabolism, confirming a limbic encephalitis, and concurrent whole-body 18FDG PET/CT showed a small cell lung carcinoma plurifocal metastatic recurrence, consistent with a paraneoplastic limbic encephalitis. 18FDG PET/CT follow-up under chemotherapy revealed an almost complete normalization of brain metabolism and a partial metabolic response of the metastatic recurrence, co…

Bilaterality of temporal EEG findings in limbic encephalitis compared to other mesiotemporal epilepsies - A retrospective cohort study.

BACKGROUND: The current diagnostic criteria for autoimmune limbic encephalitis (LE) stress bilaterality of temporal MRI and EEG findings. This retrospective cohort study aimed to determine whether bilaterality is indeed more common in LE than in other mesiotemporal epilepsies. METHODS: We compared EEG findings mostly from long-term EEG monitoring in patients with LE (n=59), hippocampal sclerosis (HS, n=44), and mesiotemporal tumors (n=24). RESULTS: Bilateral temporal EEG findings including temporal slowing, interictal epileptiform discharges (IED), and seizures were equally frequent with 37%…

Synergistic effect of rituximab with anti-epileptic drugs in treating sero-negative limbic encephalitis: a case report.

Background: Limbic encephalitis can be associated with antibodies directed against intracellular antigens or cell surface antigens, or can be sero-negative. It is still unclear if rituximab can benefit sero-negative limbic encephalitis.Case presentation: Here, we report that a sero-negative limbic encephalitis patient with refractory seizures was treated successfully with rituximab combined with anti-epileptic drugs. Pulsed intravenous methylprednisolone (IVMP) combined with intravenous immunoglobulin (IVIg) in this patient resulted in limited effect.Conclusion: We conclude that rituximab may …

Anti-N-methyl-d-aspartate receptor limbic encephalitis associated with mature cystic teratoma of the fallopian tube.

Anti-N-methyl-d-aspartate receptor (NMDAR) limbic encephalitis is the most common form of paraneoplastic encephalitis that is associated with teratomas. Because tumor removal leads to better clinical outcomes, it is essential to reveal the location of the teratomas. This is the first reported case of anti-NMDAR encephalitis associated with teratoma of the fallopian tube. Salpingo-oophorectomy improved neurological symptoms and immunohistochemical examinations indicated the expression of NMDAR on neuroglial cells within the fallopian tube teratoma. Teratomas of the fallopian tube cause anti-NMD…

Limbic encephalitis associated with anti-NH2-terminal of α-enolase antibodies: A clinical subtype of Hashimoto encephalopathy.

Several types of autoantibodies have been reported in autoimmune limbic encephalitis (LE), such as antibodies against the voltage-gated potassium channel (VGKC) complex including leucine-rich glioma inactivated 1 (LGI1). We recently reported a patient with autoimmune LE and serum anti-NH2-terminal of α-enolase (NAE) antibodies, a specific diagnostic marker for Hashimoto encephalopathy (HE), who was diagnosed with HE based on the presence of antithyroid antibodies and responsiveness to immunotherapy. This case suggests that LE patients with antibodies to both the thyroid and NAE could be diagno…

Auditory hallucinations as ictal phenomena in a patient with voltage-gated potassium channel antibody-associated limbic encephalitis.

Limbic encephalitis involving anti-voltage-gated potassium channel antibodies (VGKC-LE) has become increasingly recognised, with seizures and psychotic features, such as hallucinations being typical clinical manifestations. Though the literature supports auditory hallucinations as ictal phenomena, there are no reported cases of these hallucinations correlating with electrographic seizure for this disease entity. Early recognition of auditory hallucinations as seizures could alter treatment and subsequently affect short-term outcomes in these patients. We report the case of a patient with audit…

[The analysis of cytokines in cerebrospinal fluid (CSF) in two cases of non-herpetic acute limbic encephalitis (NHALE)].

We report two cases of non-herpetic acute limbic encephalitis (NHALE) which showed elevation of interleukin (IL)-6 in the cerebrospinal fluid (CSF). [Case 1] The patient was a 25-year-old woman who was admitted to another hospital because of fever and severe headache, following common cold. After the admission, she developed severe disturbance of consciousness and suffered from generalized convulsions, and was then transferred to our hospital. The CSF examination revealed neither pleocytosis nor elevation of total protein. Her consciousness improved by intravenous administration of high-dose m…

Case Report: Value of MR relaxometry and spectroscopy in multimodal imaging characterization of limbic encephalitis in systemic sclerosis.

Central nervous system involvement is uncommon in systemic sclerosis, and inflammatory temporomesial syndromes in this context are rarely re … bilateral mesial temporal criterion required by current consensus criteria for definite autoimmune limbic encephalitis, MR relaxometry revealed bilateral temporomesial abnormalities. This finding is consistent with available neuropathological and experimental evidence and suggests that routine imaging may underestimate disease extent. This case highlights a rare pre …

Differentiation between viral and autoimmune limbic encephalitis: a prospective cohort study with development and validation of a diagnostic model.

BACKGROUND: Distinguishing between viral encephalitis (VE) and autoimmune limbic encephalitis (ALE) presents a clinical challenge due to the overlap in symptoms. We aimed to develop and validate a diagnostic prediction model to differentiate VE and ALE. METHODS: A prospective observational multicentre cohort study, which continuously enrolled patients diagnosed with either ALE or VE from October 2011 to April 2023. The demographic data, clinical features, and laboratory test results were collected and subjected to logistic regression analyses. The model was displayed as a web-based nomogram a…

New-Onset Seizure With Possible Limbic Encephalitis in a Patient With COVID-19 Infection: A Case Report and Review.

With the outbreak of COVID-19 (coronavirus disease 2019) as a global pandemic, various of its neurological manifestations have been reported. We report a case of a 54-year-old male with new-onset seizure who tested positive for severe acute respiratory syndrome coronavirus 2 from a nasopharyngeal swab sample. Investigative findings, which included contrast-enhancing right posterior temporal lobe T2-hyperintensity on brain magnetic resonance imaging, right-sided lateralized periodic discharges on the electroencephalogram, and elevated protein level on cerebrospinal fluid analysis, supported the

A discrepancy between clinical course and magnetic resonance imaging in a case of non-herpetic acute limbic encephalitis.

We report the case of a 64-year old man who presented memory disturbance, low-grade fever, weight loss, and bilateral hand tremors for three months. He was diagnosed with non-herpetic acute limbic encephalitis (NHALE). Follow-up magnetic resonance imaging (MRI) revealed new lesions after symptomatic improvement following steroid pulse therapy. This may indicate that there is a time lag between the disturbance or recovery of neurons and astrocytes. Thus, other lesions might occasionally appear during convalescence in patients with NHALE, even if only minimal lesions were found on the initial MR…

Syndrome of inappropriate secretion of antidiuretic hormone associated with limbic encephalitis in a patient with drug-induced hypersensitivity syndrome.

Drug-induced hypersensitivity syndrome (DIHS)/drug rash with eosinophilia and systemic symptoms (DRESS) is a severe multiorgan reaction with reactivation of herpesviruses. Various features are often seen during the course of the disease. Many aspects of this syndrome suggest close similarities between DIHS/DRESS and graft-versus-host disease. We describe a patient with phenobarbital-induced hypersensitivity syndrome who revealed syndrome of inappropriate secretion of antidiuretic hormone (SIADH) associated with limbic encephalitis during the course of the disease. In view of previous reports t…

The late effects of necrotizing encephalitis of the temporal lobes and limbic areas: a clinico-pathological study of 10 cases.

The clinical and neuropathological features are reported of 10 patients who had suffered, usually for several years, from the after effects of an acute or sub-acute necrotizing encephalitis of the limbic grey matter and of the adjacent temporal lobes. Emphasis is laid on the memory disturbance and on the behavioural and emotional aberrations that tended to occur and that are in some ways reminiscent of the Klüver-Bucy syndrome.

Anti-Leucine-Rich Glioma-Inactivated 1 (Anti-LGI 1) Limbic Encephalitis and New-Onset Psychosis: A Case Report.

Anti-leucine-rich glioma-inactivated 1 limbic encephalitis (Anti-LGI 1 LE) is a subtype of autoimmune encephalitis (AE) and the most common cause of limbic encephalitis (LE). Clinically, it can have an acute to sub-acute onset of confusion and cognitive impairment, facial-brachial dystonic seizures (FDBS), and psychiatric disturbances. The clinical manifestations are varied, and its diagnosis requires high clinical suspicion to avoid delay in the treatment. When patients manifest mostly psychiatric symptoms, the disease may not be immediately recognized. We aim to report a case of Anti-LGI 1 L…

Публичное изображение TSPO показывает ярко выраженную воспаление воспаления в человеке и мурине CD8 T клетчатого лимфатического энцефалита.

Аутоиммунный лимфатический энцефалит (ALE) представляет собой новые мезиальные припадки височной доли, прогрессивное нарушение памяти и другие поведенческие и когнитивные изменения. Считается, что клетки CD8 T играют ключевую роль в тех случаях, когда аутоантифиды (АБ) нацелены на внутриклеточные антигены или нет АБ. Оценка таких пациентов сопряжена с клинической проблемой, и срочно необходимы новые биомаркеры неинвазивных изображений. Здесь мы показываем, что визуализация белка транслокатора (TSPO) с [18F]DPA-714-PET-MRI показывает ярко выраженную микроглию активации и реактивный глиоз в гипп

Совпадение в аннотации оригинала
Autoimmune limbic encephalitis (ALE) presents with new-onset mesial temporal lobe seizures, progressive memory disturbance, and other behavioral and cognitive changes. CD8 T cells are considered to play a key role in those cases where autoantibodies (ABs) target intracellular antigens or no ABs were found. Assessment of such patients presents a clinical chal…

Autoimmune Limbic Encephalitis in Patients with Hematologic Malignancies after Haploidentical Hematopoietic Stem Cell Transplantation with Post-Transplant Cyclophosphamide.

Autoimmune limbic encephalitis (LE) is a rare, but devastating complication of allogeneic hematopoietic stem cell transplantation (HSCT). There is currently limited evidence describing the risk factors, laboratory features, and underlying mechanisms of this neurologic adverse event. We retrospectively reviewed available clinical, imaging, and laboratory data from adult patients with hematological malignancies who underwent haploidentical HSCT with post-transplant cyclophosphamide (PTCy) at Chungnam National University Hospital from June 2016 to May 2020. Patients who developed LE were compared…

A case report of limbic encephalitis in a metastatic colon cancer patient during first-line bevacizumab-combined chemotherapy.

RATIONALE: Paraneoplastic limbic encephalitis (PLE) is one of the most common causes of neurologic paraneoplastic syndromes, with unclear pathogenesis. While several reports published in the last decades showed the occurrence of PLE in a variety of cancers, only a few cases have been associated with colon cancer. PATIENT CONCERNS: In February 2017, a 54-year-old man with clinical history of radically resected colon cancer started first line chemotherapy with FOLFOXIRI plus bevacizumab, after radiological diagnosis of multiple liver and bone metastases. During the third cycle of treatment, the…