Antibodies against GluR3 peptides are not specific for Rasmussen's encephalitis but are also present in epilepsy patients with severe, early onset disease and intractable seizures.
Rasmussen's encephalitis (RE) is a rare condition characterized by drug-resistant seizures, recurrent status epilepticus and progressive lateralized neurological deterioration. There is evidence of autoimmune involvement in the pathogenesis. We investigated the presence of anti-GluR3 antibodies against peptides A and B in patients with RE (n=11), partial and generalized epilepsy (n=85) and other neurological diseases (n=30). The antibodies were specific for epilepsy and are thus not a marker of RE, while particularly high antibody titers characterized a subgroup of non-RE patients with "catastrophic" epilepsy. Antibodies against GluR3B peptide were significantly associated with frequent seizures compared to occasional or drug-controlled seizures.
Mantegazza
Renato
R
Immunology and Muscular Pathology Unit, Istituto Nazionale Neurologico Carlo Besta, via Celoria 11, Milan, Italy. rmantegazza@istituto-besta.it
Bernasconi
Pia
P
Baggi
Fulvio
F
Spreafico
Roberto
R
Ragona
Francesca
F
Antozzi
Carlo
C
Bernardi
Gaetano
G
Granata
Tiziana
T
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000918:Antibody Specificity; D001323:Autoantibodies / Q000134:cerebrospinal fluid / Q000276:immunology*; D020274:Autoimmune Diseases of the Nervous System / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D004660:Encephalitis / Q000276:immunology*; D004827:Epilepsy / Q000175:diagnosis / Q000276:immunology*; D000939:Epitopes / Q000276:immunology; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009422:Nervous System Diseases / Q000276:immunology; D010455:Peptides / Q000276:immunology; D018091:Receptors, AMPA; D017470:Receptors, Glutamate / Q000737:chemistry / Q000276:immunology*
D001323:Autoantibodies; D000939:Epitopes; D010455:Peptides; D018091:Receptors, AMPA; D017470:Receptors, Glutamate; C104723:glutamate receptor ionotropic, AMPA 3