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

Journal of Clinical Neurology 2014³â 10±Ç 2È£ p.157 ~ p.161

ÀÓÁ¤¾Æ(Lim Jung-Ah) - Seoul National University College of Medicine Department of Neurology
À̼øÅÂ(Lee Soon-Tae) - Seoul National University College of Medicine Department of Neurology
Á¤±ÙÈ­(Jung Keun-Hwa) - Seoul National University College of Medicine Department of Neurology
±è¼Ò¿¬(Kim So-Yun) - Seoul National University College of Medicine Department of Neurology
½ÅÁ¤¿ø(Shin Jung-Won) - Seoul National University College of Medicine Department of Neurology
¹®Àå¼·(Moon Jang-Sup) - Seoul National University College of Medicine Department of Neurology
º¯Á¤ÀÍ(Byun Jung-Ick) - Seoul National University College of Medicine Department of Neurology
±èÅÂÁØ(Kim Tae-Joon) - Seoul National University College of Medicine Department of Neurology
½Å¿ë¿ø(Shin Yong-Won) - Seoul National University College of Medicine Department of Neurology
À̰ÇÁÖ(Lee Keon-Joo) - Seoul National University College of Medicine Department of Neurology
±è¿µ¼ö(Kim Young-Su) - Sungkyunkwan University School of Medicine Samsung Changwon Hospital Department of Neurology
¹Ú°æÀÏ(Park Kyung-Il) - Inje University College of Medicine Seoul Paik Hospital Department of Neurology
ÀÌ»ó°Ç(Lee Sang-Kun) - Seoul National University College of Medicine Department of Neurology
ÁÖ°Ç(Chu Kon) - Seoul National University College of Medicine Department of Neurology

Abstract

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.

Methods: Serum and/or cerebrospinal fluid (CSF) of adult patients (aged ¡Ã18 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.

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).

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.

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