title: Antibodies against peptides of NMDA-type GluR in cerebrospinal fluid of patients with epileptic spasms.
abstract: OBJECTIVE
  We investigated the contribution of antibodies against N-methyl-d-aspartate (NMDA)-type glutamate receptor (GluR) in cerebrospinal fluid (CSF) to the clinical features of patients with epileptic spasms (ES).
  
  
  METHODS
  CSF samples were collected from 33 patients with ES with median (range) age 1.8 (0.2-8.5) years. Thirty patients without ES with 3.5 (0.5-7.0) years were also studied as disease controls. The CSF levels of antibodies against peptides of NMDA-type GluR subunits (GluN2B & GluN1) were measured by enzyme-linked immunosorbent assay.
  
  
  RESULTS
  The levels of antibodies against the n-terminal of GluN2B (GluN2B-NT2), c-terminal of GluN2B (GluN2B-CT) and n-terminal of GluN1 (GluN1-NT), were significantly higher in patients with ES than in disease controls (p < 0.01, p < 0.01 & p = 0.03). Levels of antibodies to GluN2B-NT2 & CT were not related with ACTH therapy nor conventional CSF factors (cell counts, protein level, etc). Levels of antibodies to GluN2B-NT2 & CT showed evidence of correlation within a linear regression model with intervals from the onset to the examination of CSF until 25 months (p = 0.01 & p = 0.01). The correlation was significant in patients with unknown cause (p = 0.01). Five of 33 patients (four unknown cause & one chromosomal anomaly) had higher level of antibodies to GluN2B-NT2 exceeding mean + 1 SD of all ES patients, and they had poor motor (score 0) and cognitive outcomes (score 0 or 1).
  
  
  CONCLUSIONS
  The CSF level of antibodies against GluN2B in ES patients with unknown cause was estimated to increase after onset. We hypothesize that some ES patients may have immune process after the onset of ES.
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan. Electronic address: mori.tatsuo@tokushima-u.ac.jp.
authors: Tatsuo
authors: T
authors: Mori
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Yukitoshi
authors: Y
authors: Takahashi
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Nami
authors: N
authors: Araya
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Taikan
authors: T
authors: Oboshi
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Hirokazu
authors: H
authors: Watanabe
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Kazuki
authors: K
authors: Tsukamoto
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Tokito
authors: T
authors: Yamaguchi
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Shinsaku
authors: S
authors: Yoshitomi
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Hirosato
authors: H
authors: Nasu
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Hiroko
authors: H
authors: Ikeda
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Hideyuki
authors: H
authors: Otani
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Katsumi
authors: K
authors: Imai
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Hideo
authors: H
authors: Shigematsu
authors: National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, NHO, 886 Urushiyama, Aoi-ku, Shizuoka 420-8688, Japan.
authors: Yushi
authors: Y
authors: Inoue
mesh_terms: D001323:Autoantibodies / Q000134:cerebrospinal fluid*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D021122:Protein Subunits / Q000276:immunology*; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D013036:Spasms, Infantile / Q000134:cerebrospinal fluid / Q000276:immunology*
chemical_list: D001323:Autoantibodies; C121001:NR2B NMDA receptor; D021122:Protein Subunits; D016194:Receptors, N-Methyl-D-Aspartate
keywords: Autoantibodies; Cognitive outcome; Infantile spasms; Motor outcome; West syndrome