Anti-NMDA Receptor Antibody-Related Encephalitis Shigemi Nagayama 1 , Keiko Tanaka 2 1Department of Neurology, Kanazawa Medical University 2Department of Cellular Neurobiology, Brain Research Institute, Niigata University Keyword: 抗NMDA受容体抗体 , 自己免疫性脳炎 , 卵巣奇形腫 , 免疫療法 , 単純ヘルペス脳炎 , anti-NMDA receptor antibody , autoimmune encephalitis , ovarian teratoma , immunotherapy , herpes simplex encephalitis pp.1001-1009
Published Date 2016/9/1
DOI https://doi.org/10.11477/mf.1416200546
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Recently, the search for diagnostic antibody markers has drawn considerable attention in relation to autoimmune encephalitis. Among the antibody markers, the most frequently detected is the anti-N-methyl-D-aspartate receptor (NMDAR)antibody. Patients with this antibody develop characteristic clinical features. This disease tends to affect young women, and starts with psychiatric symptoms followed by seizures, involuntary movements, autonomic failure, and respiratory failure. Nearly half of these female patients have ovarian teratoma.

Some of the patients with anti-NMDAR antibody show atypical clinical features. Approximately 4% show only psychiatric symptoms, which might lead to a diagnosis of malignant catatonia. Other reports describe patients experiencing refractory seizures to have the anti-NMDAR antibody. Some of the antibody-positive patients are associated with demyelinating disorders, and some develop anti-NMDAR encephalitis after recovery from herpes simplex encephalitis. It is important to test the anti-NMDAR antibody in these groups since immunotherapy ameliorates their symptoms.

The anti-NMDAR antibody binds to the constitutional epitope at the extracellular domain of GluN1 and disrupts its function. Early introduction of immunotherapy together with tumor resection will results in improvement of neurological symptoms.

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