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[A case of bilateral auricular chondritis with anti-glutamate receptor (GluRε2) antibody-positive non-herpetic acute limbic encephalitis].

A 62-year-old man experienced pain and swelling in both of his auricles. One and a half months later, he was referred to us because of a mem … a herpes polymerase chain reaction was negative. Thus, he was suspected to have non-herpetic acute limbic encephalitis (NHALE). In addition, relapsing polychondritis (RP) was suspected because of the bilateral auricular chondritis and type-II collagen antibody positivity in the serum. He was treated with high-dose steroid therapy (two cycles of intravenous methylpre …

Impaired consolidation of spatial memory during sleep in patients with leucine-rich glioma-inactivated 1-associated limbic encephalitis.

Sleep promotes the systems consolidation of hippocampus (HC)-dependent spatial memories by reprocessing of previously encoded hippocampal representations. Hippocampal reprocessing involves pattern separation and pattern completion as central hippocampal functions performed by the dentate gyrus (DG) and cornu ammonis region 3 (CA3), respectively. The leucine-rich, glioma inactivated 1 (LGI1)-associated limbic encephalitis (LE) is an autoimmune brain disorder particularly affecting the DG and CA3 regions, thereby impairing hippocampal function. We studied 15 LGI1 patients (and matched healthy co…

Gamma-aminobutiric acid-B receptor antibody-related limbic encephalitis due to small cell lung carcinoma: Two case reports.

BACKGROUND: Paraneoplastic limbic encephalitis (LE) is an inflammatory condition that affects the limbic system, cerebellum, and peripheral nervous system. It causes a range of symptoms including short-term memory loss, impaired cognitive function, behavioral and psychological disorders, and seizures. Paraneoplastic LE can occur when an immune response is activated due to antibodies targeting gamma-aminobutyric acid (GABA) B receptor (GABABR) interacting with antigens on tumor cells and the nervous system, resulting in tumors primarily as small cell lung carcinoma (SCLC). CASE SUMMARY: We dis…

18 F-FDG PET/CT in Seronegative Paraneoplastic Limbic Encephalitis Related to Gastrointestinal Stromal Tumor With Coexisting Sepsis.

Paraneoplastic limbic encephalitis (PLE) is proposed to be triggered by oncoantibodies in response to the malignancies, which cross-react with the self-antigens in the neurons. Patients usually manifest a wide range of clinical presentations and neuropsychiatric symptoms like memory loss, mood changes, behavioral disorders, altered state of consciousness, and seizures. Lung carcinoma is the most common malignancy associated with PLE. Antibodies to common intracellular antigens have been associated with PLE, however, not all patients demonstrate the association. Here the authors demonstrate a r…

Acute Bilateral Vocal Cord Paralysis in a Patient With Anti-Leucine-Rich Glioma-Inactivated 1 (LGI1) Limbic Encephalitis.

Autoimmune encephalitis is a disorder characterized by an autoantibody-mediated process that leads to brain inflammation. It is associated with neurological symptoms including cognitive issues, psychiatric problems, seizures, and autonomic dysfunctions. Anti-leucine-rich glioma-inactivated 1 limbic encephalitis (anti-LGI1 LE) is a rare type of autoimmune LE with a unique presentation, comprising neuropsychiatric disturbances, sleep disorders, and faciobrachial dystonic seizures (FBDS). While the involvement of the larynx is seen in various other autoimmune and neurological diseases, it is not …

Serum neurofilament light chain is associated with disturbed limbic-based functional connectivity in patients with anti-NMDAR encephalitis.

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis shows a predilection for affecting the limbic system, but structural MRI in most patients is usually unremarkable. However, the functional connectivity reorganization of limbic nodes remains unknown. Serum neurofilament light chains (sNfL) are clinically linked with the disease severity and neurological disability of anti-NMDAR encephalitis. However, the relationship between sNfL and limbic-based functional architecture has not been explored. We consecutively recruited 20 convalescent patients with anti-NMDAR encephalitis and 24 healthy c

[Following sensory neuropathy, anti-Hu antibody-positive paraneoplastic neurological syndrome presenting with limbic encephalitis occurs after complete remission].

Paraneoplastic limbic encephalitis is a rare neurological disorder that frequently precedes the detection of malignancy. We report the case of a 68-year-old male with small-cell lung cancer who developed paraneoplastic limbic encephalitis associated with presence of the anti-Hu antibody, after achieving complete remission of the tumor by chemotherapy. The patient visited our hospital because of progressive sensory disturbance of the distal extremities at 65 years of age. Though paraneoplastic sensory neuropathy was suspected, we could not find any tumor and he did not improve with steroids or …

Detection of DNA of six human herpesviruses in the cerebrospinal fluid of immunocompetent non-herpetic acute limbic encephalitis patients.

In order to determine whether six other human herpesviruses, aside from herpes simplex virus, are associated with non-herpetic acute limbic encephalitis in immunocompetent individuals, real-time PCR was used to detect the DNA of herpesviruses in CSF collected from 61 patients with this form of encephalitis. Five of the human herpesviruses tested were not detected in any of the 61 CSF samples. EBV DNA was detected in one CSF sample. The EBV DNA-positive patient was a 36-year-old woman who presented with fever, headache, mild somnolence, and the typical neuroimaging findings.

Limbic encephalitis in a child with ovarian teratoma and influenza B. Case report and critical review of the history of autoimmune anti-N-methyl-d-aspartate receptor encephalitis.

We report the appearance of clinical symptoms and signs of N-methyl-d-Aspartate (NMDA) receptor encephalitis in a patient presenting just days after contraction of influenza B. The offending mature ovarian teratoma was identified and removed on the 10th day after the appearance of symptoms, with subsequent nearly complete resolution of symptoms over the subsequent 6 months. We provide a focused literature review of the clinical and pathophysiologic literature of anti-NMDA receptor encephalitis pertaining to influenza B virus and the pediatric population. Taken together, this study contributes

A Case of Anti-LGI1 Limbic Encephalitis Presenting With Non-convulsive Status Epilepticus and Requiring a Long Period of Hospitalization.

An 81-year-old man was brought to our hospital with tonic-clonic seizures and a gradual progression of cognitive dysfunction. Four months pr … the right temporal lobe. Serum anti-LGI1 antibody was detected. Based on these findings, anti-LGI1 limbic encephalitis (LE) was diagnosed. His symptoms improved with corticosteroid treatment; however, six months of hospitalization were necessary for him to regain independence in daily living. Although the prognosis of anti-LGI1 LE is generally favorable, older age a …

Beyond the limbic system: disruption and functional compensation of large-scale brain networks in patients with anti-LGI1 encephalitis.

OBJECTIVE: Hippocampal inflammation in anti-LGI1 encephalitis causes memory deficits, seizures and behavioural abnormalities. Recent findings suggest that extralimbic brain areas are additionally affected and that patients also suffer from non-limbic cognitive symptoms. Moreover, up to 60% of patients show no structural MRI abnormalities in the acute disease stage. We therefore investigated whether functional connectivity analyses can identify brain network changes underlying disease-related symptoms. METHODS: We studied 27 patients and a matched healthy control group using structural and fun

Hypothermia due to limbic system involvement and longitudinal myelitis in a case of Japanese encephalitis: a case report from India.

Japanese encephalitis (JE) is an infectious encephalitis prevalent in Asia. It usually presents with fever, headache, convulsions and extrapyramidal symptoms. Limbic system involvement and hypothermia though common in autoimmune encephalitis have never been reported in JE. We report a case of an 18-year-old girl with no previous comorbidities who presented to us with a history of fever and headache for 1 week duration. She developed bilateral lateral rectus palsy and asymmetric flaccid weakness of all four limbs, after 2 days of admission, which was followed by altered sensorium and intermitte

Limbic encephalitis associated with anti-Hu antibody induced by atezolizumab treatment for small cell lung cancer: A case report.

With the emergence and frequent use of immune checkpoint inhibitors (ICIs), reports of immunotherapy-induced limbic encephalitis have begun to appear. The present study describes the case of a patient with small cell lung cancer who developed Hu-associated limbic encephalitis while being treated with atezolizumab. After treatment with high-dose steroids and intravenous immunoglobulin, the neurological symptoms of the patient (including confusion, short-term memory loss, disorientation, and impaired calculation ability) remained stable for 3 months, after which the patient was lost to follow-up…

A Case of Anti-Leucine-Rich Glioma-Inactivated Protein 1 (Anti-LGI1) Limbic Encephalitis With New-Onset Panic Attacks.

Anti-leucine-rich glioma-inactivated protein 1 (anti-LGI1) limbic encephalitis is a rare autoimmune neurologic disorder with antibodies against LGI1. It was first recognized as a disease in 2010 and represents the second most common cause of autoimmune encephalitis. Clinically, it is characterized by subacute changes in cognition, memory, and behavior, associated with hyponatremia and faciobrachial dystonic seizures (FBDS). This report discusses a unique onset of anti-LGI1 limbic encephalitis where an elderly female presented with symptoms of new-onset panic attacks and rhythmic facial movemen…

Фальсифицированный лимфатический энцефалит с гипертонической комой, брюксизмом, изоэлектрической электроэнцефалограммой и временной кортикальной атрофией с восстановлением.

Аутоиммунные энцфалитиды представляют собой потенциально разрушительную группу излечимых расстройств с широким спектром клинических презентаций. Наиболее изученный аутоиммунный энцефалит вызывается антителами рецептора глютамата N-метил-D. Более редкой причиной является то, что антитела противостоят эволюционно связанному α-амино-3-гидрокси-5-метил-4-изоксазолпропионовому кислотному рецептору (АМПАРЕ). Полная асортация электроэнцефалограммы (ЭГЭ) и клиническое описание последних еще не полностью описаны. 44-летняя женщина с расстройством сознания и последующей комой, характерной для изоэлектри

Совпадение в аннотации оригинала
Autoimmune encephalitides are a potentially devastating group of treatable disorders with a wide variety of clinical presentations. The most … ciousness and subsequent coma characterised by an isoelectric EEG was diagnosed with AMPAR-antibody limbic encephalitis. MRI revealed temporal T2 hyperintensities that improved with immunosuppression, although leaving marked cortical atrophy. Gradual clinical improvement saw the development of aggressive bruxism requiring botulinum toxin injection with eventual meani …

Caspr2 antibody-associated limbic encephalitis: contribution of visual aided analysis of 18F-FDG PET images using normal database comparison.

Early immunotherapy is of paramount importance for a positive outcome in patients suffering acute encephalitis of autoimmune origin (AIE). A … nt and FDG-PET patterns have not been well characterized. In our experience, FDG-PET in anti-Caspr2 limbic encephalitis shows medial temporal hypermetabolism and diffuse cortical hypometabolism, even in the absence of findings in these tests. However, it is necessary to standardize PET image analysis by means of visual and voxel-based methods compared to normal datab …