Nihon rinsho. Japanese journal of clinical medicine
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Review
[Clinical diagnosis and treatment of anti-NMDA (N-methyl-D-aspartate) receptor encephalitis].
Recent clinical management of anti-NMDA receptor encephalitis is reviewed. This illness is required the management of the neurological emergency. Typical symptoms of anti-NMDA receptor encephalitis develop in several stages that progresses from psychosis, memory deficits, seizures, and language disintegration into a state of unresponsiveness with catatonic features often associated with abnormal movements, and autonomic and respiratory instability. ⋯ The disorder predominantly affects children and young adults, occurs with or without tumor association. The presence of a tumor (usually an ovarian teratoma) is dependent on age and sex, being more frequent in women older than 18 years. Anti-NMDA receptor encephalitis should be treated with tumor resection and immunotherapy (corticosteroids, intravenous immunoglobulin, or plasma exchange) responded faster to treatment and less frequently needed second-line immunotherapy (cyclophosphamide or rituximab, or both).
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The author reviewed the epidemiological and clinical aspects of Bickerstaff brainstem encephalitis (BBE) based on the data from the nationwide survey, which was planned in Japanese population between 2006 and 2009. Annual BBE onset was roughly estimated as 100 cases in Japan, which accounted for 43% of brainstem encephalitis, and corresponded to 6.8% of Guillain-Barré syndrome incidence. BBE consisted of typical and atypical cases, and typical BBE had the similar neurological and serological features to Fisher syndrome as well as with good recovery, whereas atypical BBE was characterized by delayed recovery, negative anti-GQ1b antibodies, and abnormal CSF and brain MRI findings with possible other pathogeneses.