Abstract | PURPOSE: To evaluate antibody-mediated and cytotoxic T cell-mediated pathogenicity that has been implicated as the autoimmune pathophysiological mechanism in Rasmussen's encephalitis. METHODS: RESULTS: All nine definitive patients (five patients with EPC and four without EPC), and 10 of 11 clinical Rasmussen's encephalitis patients had the autoantibodies. In four patients, the autoantibodies were absent in early stage when epileptic seizures had already become frequent, and appeared subsequently. In two patients, the autoantibodies persisted in the serum after frontal lobe resection or functional hemispherectomy, although epileptic seizures were completely controlled. Autoantibodies to the C2 epitope predominated, while autoantibodies to the extracellular N epitope were rare. The mean 3H-thymidine uptake ratios (stimulation by GluRepsilon2-containing homogenates/stimulation by PHA) were significantly higher in definitive and clinical Rasmussen encephalitis patients than in controls. The mean 3H-thymidine uptake ratios (relative to PHA) were significantly higher for GluRepsilon2-containing homogenate than for control homogenate or GluRdelta2-containing homogenate. CONCLUSIONS:
Autoantibodies against GluRepsilon2 may be one of the diagnostic markers for Rasmussen's encephalitis with and without EPC. Patients have activated T cells stimulated by GluRepsilon2 in peripheral blood circulation. We speculate that cellular autoimmunity and the subsequent humoral autoimmunity against GluRepsilon2 may contribute to the pathophysiological processes in Rasmussen's encephalitis.
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Authors | Yukitoshi Takahashi, Hisashi Mori, Masayoshi Mishina, Masahiko Watanabe, Naomi Kondo, Jiro Shimomura, Yuko Kubota, Kazumi Matsuda, Katsuyuki Fukushima, Naohide Shiroma, Noriyuki Akasaka, Hiroshi Nishida, Atsushi Imamura, Hiroo Watanabe, Nobuyoshi Sugiyama, Makoto Ikezawa, Tateki Fujiwara |
Journal | Epilepsia
(Epilepsia)
Vol. 46 Suppl 5
Pg. 152-8
( 2005)
ISSN: 0013-9580 [Print] United States |
PMID | 15987271
(Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Autoantibodies
- Biomarkers
- NR2B NMDA receptor
- Receptors, N-Methyl-D-Aspartate
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Topics |
- Adolescent
- Adult
- Autoantibodies
(blood, cerebrospinal fluid, immunology)
- Autoimmunity
(immunology)
- Biomarkers
- Child
- Encephalitis
(blood, cerebrospinal fluid, diagnosis, immunology)
- Epilepsia Partialis Continua
(blood, cerebrospinal fluid, immunology)
- Female
- Humans
- Immunity, Cellular
(immunology)
- Male
- Receptors, N-Methyl-D-Aspartate
(analysis, blood, immunology)
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