Rasmussen's syndrome.
Rasmussen's encephalitis (RE) is characterized by focal seizures, often associated with epilepsia partialis continua, progressive neurological deterioration, hemispheric atrophy, and inflammatory histopathology. Surgical exclusion of the affected hemisphere is the only treatment that halts progression of the disease. The possible role played by serum antibodies against the glutamate receptor GluR3 in the animal model and in a number of affected patients supports a disimmune component in the pathogenesis of the disease and provides a rational basis for considering the use of immunomodulatory treatments in selected cases. The use of intravenous immunoglobulins has produced encouraging results in adult-onset RE but further studies are needed to assess the effect, dosage, and schedules in childhood-onset RE.
Department of Child Neurology, National Neurological Institute C. Besta, Milan, Italy.
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D000818:Animals; D001323:Autoantibodies / Q000097:blood*; D004195:Disease Models, Animal; D004660:Encephalitis / Q000188:drug therapy* / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D018091:Receptors, AMPA / Q000276:immunology*
D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D018091:Receptors, AMPA; C104723:glutamate receptor ionotropic, AMPA 3