[Landau-Kleffner sydnrome].
The Landau-Kleffner syndrome consists of the association of paroxysmal EEG abnormalities, more marked during sleep, acquired aphasia usually of receptive type, and epileptic seizures in three quarters of cases. Additional features include behavioral disturbances that may even present with autistic features, cognitive regression of variable degree, and sometimes motor difficulties, indicating the pervasive nature of the disorder. The epileptic activity, as manifested by the EEG, seems responsible for the pervasive dysfunction. Treatment with antiepileptic drugs is often ineffective. ACTH and corticosteroids are currently regarded as the best therapy but surgical treatment by subpial transection is being actively studied.
Aicardi
J
J
Institute of Child Health, University College, Londres, Gran Bretaña. alexis.arzimanoglou@rdb.aphopparis.fr
D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D012307:Risk Factors; D013256:Steroids
D000893:Anti-Inflammatory Agents; D000927:Anticonvulsants; D013256:Steroids