Anti-N-Methyl-D-Aspartate Receptor Encephalitis in Korea: Clinical Features, Treatment, and Outcome
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Background and Purpose Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitisis 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. Methods Serum and/or cerebrospinal fluid (CSF) of adult patients (aged ≥18 years) with encephalitis of undetermined cause were screened for anti-NMDAR antibodies using a cell-basedindirect immunofluorescence assay. The patients came from 41 university hospitals. Results Of the 721 patients screened, 40 were identified with anti-NMDAR antibodies andclinical details of 32 patients were obtained (median age, 41.5 years; 15 females). Twenty-twopatients (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 onlyavailable 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 wereanalyzed in 21 patients, of which 14 (66.7%) achieved favorable functional outcomes (score onthe modified Rankin Scale of 0–2). 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 willimprove the early detection of anti-NMDAR encephalitis in patients both young and old.
Abstract
Background and Purpose Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitisis 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. Methods Serum and/or cerebrospinal fluid (CSF) of adult patients (aged ≥18 years) with encephalitis of undetermined cause were screened for anti-NMDAR antibodies using a cell-basedindirect immunofluorescence assay. The patients came from 41 university hospitals. Results Of the 721 patients screened, 40 were identified with anti-NMDAR antibodies andclinical details of 32 patients were obtained (median age, 41.5 years; 15 females). Twenty-twopatients (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 onlyavailable 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 wereanalyzed in 21 patients, of which 14 (66.7%) achieved favorable functional outcomes (score onthe modified Rankin Scale of 0–2). 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 willimprove the early detection of anti-NMDAR encephalitis in patients both young and old.
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