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OBJECTIVE: To determine the prevalence of serum antibodies to the ionotropic glutamate receptor 3 (GluR3) in patients with Rasmussen encephalitis (RE), a severe epileptic disorder, and to compare with serum from control subjects and patients with intractable epilepsy (IE). METHODS: The authors looked for serum immunoglobulin (Ig) G antibodies to GluR3 in 30 patients with RE, including two patients who had plasma exchange and 12 who had been treated with IV Igs with varying results, and 49 patients with IE and 23 healthy individuals, using ELISA with GluR3B peptide, Western blot analysis of recombinant full-length GluR3, immunoprecipitation of [35S]- and [125I]-labeled GluR3 extracellular domains, immunohistochemistry on rat brain sections, and electrophysiology of GluR3 expressed in Xenopus oocytes. RESULTS: Low levels of antibodies to the GluR3B peptide were detected using ELISA in only 4 of the 79 patients with epilepsy (2 with RE and 2 with IE); binding to GluR3B in other sera was shown to be nonspecific. One other patient with IE had antibodies to recombinant GluR3 on Western blot analysis. However, none of the sera tested precipitated either the [35S]- or the [125I]-labeled GluR3 domains; none bound to rat brain sections in a manner similar to rabbit antibodies to GluR3; and none of the nine sera tested affected the electrophysiologic function of GluR3. CONCLUSIONS: GluR3 antibodies were only infrequently found in Rasmussen encephalitis or intractable epilepsy.

Type

Journal article

Journal

Neurology

Publication Date

13/07/2004

Volume

63

Pages

43 - 50

Keywords

Adolescent, Adult, Aged, Amino Acid Sequence, Animals, Antibodies, Anticardiolipin, Antibody Specificity, Autoantibodies, Autoantigens, Brain, Cell Line, Child, Child, Preschool, Encephalitis, Epilepsy, Epitopes, Female, G(M1) Ganglioside, Glutamate Decarboxylase, Humans, Infant, Middle Aged, Models, Molecular, Molecular Sequence Data, Protein Conformation, Rabbits, Rats, Receptors, AMPA, Receptors, Nicotinic, Recombinant Proteins, Sodium Channels, alpha7 Nicotinic Acetylcholine Receptor