A role of autoimmunity in the etiopathogenesis of Landau-Kleffner syndrome?
Four children, 3 boys and 1 girl, with the syndrome of acquired aphasia Landau-Kleffner were followed up during the last 10 years. In 3 children an episodic form of the disease with a good response to corticosteroid treatment and with a favourable prognosis was observed. In the other 1 no improvement of speech function was observed and also epileptic fits were reduced only with difficulty. A positive autoimmune reaction to central (MOZAN) and peripheral (LISAN) myelin was observed repeatedly during the attacks of clinical worsening. On the contrary, during the periods of improvement of language disturbances during the treatment with corticosteroids this hypersensitivity to central as well as peripheral myelin disappeared. Possible changes in myelinization and the role of autoimmune reactions in the etiopathogenesis of the disease are discussed.
D000293:Adolescent; D001037:Aphasia / Q000209:etiology* / Q000503:physiopathology; D015551:Autoimmunity; D001921:Brain / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006967:Hypersensitivity / Q000150:complications / Q000276:immunology; D008297:Male; D009186:Myelin Sheath / Q000276:immunology; D010525:Peripheral Nerves / Q000276:immunology; D013577:Syndrome