Autoantibodies against glutamate receptor epsilon2-subunit detected in a subgroup of patients with reversible autoimmune limbic encephalitis.
We investigated the presence of autoantibodies against glutamate receptor (GluR) epsilon2 in serum and cerebrospinal fluid (CSF) samples from 12 consecutive patients with acute encephalitis/encephalopathy by immunoblotting using recombinant GluR epsilon2 as antigen. In 4 patients, IgM autoantibodies against GluR epsilon2 were detected in CSF in the early phase of the disease but were not detectable after several months. Seizures and psychiatric symptoms were noted during the acute phase of the disease in these 4 patients, who showed various degrees of residual amnesia. Immunotherapy was performed on 3 patients (patients 1, 3 and 4), and they showed marked improvements. Immunohistochemistry using these patients' sera showed that immunoreactivity is specifically detected in the cytoplasm of rat hippocampal and cortical neurons. The clinical features and neuroimaging findings of patients with IgM autoantibodies against GluR epsilon2 in CSF resemble those of patients with reversible autoimmune limbic encephalitis.
Kimura
A
A
Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Gifu, Japan. kimura1@cc.gifu-u.ac.jp
Sakurai
T
T
Suzuki
Y
Y
Hayashi
Y
Y
Hozumi
I
I
Watanabe
O
O
Arimura
K
K
Takahashi
Y
Y
Inuzuka
T
T
D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood* / Q000134:cerebrospinal fluid; D001327:Autoimmune Diseases / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology* / Q000473:pathology; D001921:Brain / Q000378:metabolism / Q000473:pathology; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D020363:Limbic Encephalitis / Q000097:blood* / Q000134:cerebrospinal fluid / Q000276:immunology* / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009474:Neurons / Q000378:metabolism; D017470:Receptors, Glutamate / Q000276:immunology*; D012189:Retrospective Studies
D001323:Autoantibodies; D007075:Immunoglobulin M; D017470:Receptors, Glutamate; C107913:glutamate receptor delta 2