Anti-N-Methyl-D-Aspartate Receptor Encephalitis in Korea: Clinical Features, Treatment, and Outcome.
10.3988/jcn.2014.10.2.157
- Author:
Jung Ah LIM
1
,
2
;
Soon Tae LEE
;
Keun Hwa JUNG
;
Soyun KIM
;
Jung Won SHIN
;
Jangsup MOON
;
Jung Ick BYUN
;
Tae Joon KIM
;
Yong Won SHIN
;
Keon Joo LEE
;
Young Su KIM
;
Kyung Il PARK
;
Sang Kun LEE
;
Kon CHU
Author Information
1. Department of Neurology, Laboratory for Neurotherapeutics, Comprehensive Epilepsy Center, Biomedical Research Institute, Seoul National University Hospital
2. Program in Neuroscience, Seoul National University College of Medicine, Seoul, Korea. stemcell.snu
- Publication Type:Original Article
- Keywords:
encephalitis;
N-methyl-D-aspartate receptor antibody;
autoimmune encephalitis
- MeSH:
Adult;
Aged;
Anti-N-Methyl-D-Aspartate Receptor Encephalitis*;
Antibodies;
Cerebrospinal Fluid;
Electroencephalography;
Encephalitis;
Fluorescent Antibody Technique, Indirect;
Hospitals, University;
Humans;
Immunotherapy;
Korea;
Magnetic Resonance Imaging;
Memory;
Movement Disorders;
Primary Dysautonomias;
Seizures;
Teratoma
- From:Journal of Clinical Neurology
2014;10(2):157-161
- CountryRepublic of Korea
- Language:English
-
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 > or =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. 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.
