Anti-N-Methyl-D-Aspartate Receptor Encephalitis in Korea: Clinical Features, Treatment, and Outcome

作者：Lim Jung Ah; Lee Soon Tae; Jung Keun Hwa; Kim Soyun; Shin Jung Won; Moon Jangsup; Byun Jung Ick; Kim Tae Joon; Shin Yong Won; Lee Keon Joo; Kim Young Su; Park Kyung Il; Lee Sang Kun; Chu Kon^*

来源：Journal of Clinical Neurology, 2014, 10(2): 157-161.

DOI：10.3988/jcn.2014.10.2.157

• encephalitis
• N-methyl-D-aspartate receptor antibody
• autoimmune encephalitis

Background and Purpose Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune synaptic encephalitis and it often responds to treatment. We analyzed the clinical characteristics of anti-NMDAR encephalitis in Korea. %26lt;br%26gt;Methods Serum and/or cerebrospinal fluid (CSF) of adult patients (aged %26gt;= 18 years) with encephalitis of undetermined cause were screened for anti-NMDAR antibodies using a cell-based indirect immunofluorescence assay. The patients came from 41 university hospitals. %26lt;br%26gt;Results Of the 721 patients screened, 40 were identified with anti-NMDAR antibodies and clinical details of 32 patients were obtained (median age, 41.5 years; 15 females). Twenty-two patients (68.8%) presented with psychiatric symptoms, 16 (50%) with seizures, 13 (40.6%) with movement disorders, 15 (46.9%) with dysautonomia, 11(34.4%) with memory disturbance, and 11(34.4%) with speech disturbance. Magnetic resonance imaging, electroencephalography, and CSF examinations yielded nonspecific findings. Tumor information was only available for 22 patients: 5 patients had tumors, and 2 of these patients had ovarian teratomas. Twenty-two patients received immunotherapy and/or surgery, and therapeutic responses were analyzed in 21 patients, of which 14 (66.7%) achieved favorable functional outcomes (score on the modified Rankin Scale of 0-2). %26lt;br%26gt;Conclusions This study investigated the clinical characteristics of adult anti-NMDAR encephalitis in Korea. Currently, elderly patients who do not have tumors are commonly diagnosed with this condition. Understanding the detailed clinical characteristics of this disease will improve the early detection of anti-NMDAR encephalitis in patients both young and old.

• 出版日期2014-4