[Myoclonic-astatic seizures (Lennox syndrome) in the course of juvenile neuronal ceroid-lipofuscinosis (M. Batten-Spielmeyer-Vogt) (author's transl)].
EEG findings and the course of epileptic seizures in two patients with neuronal ceroid lipofuscinosis (Batten Spielmeyer Vogt syndrome) are presented. Both patients, during the course of disease, developed therapy resistant epileptic reactions with myoclonicastatic seizures. These seizures in connection with diffuse encephalopathy and EEG pattern with 2.5 to 3.5/sec slow-spike-wave meet the criteria of the Lennox syndrome. Pathogenetic questions regarding possible additional genetic predisposition for epileptic seizures are discussed. Since therapeutic effect of different medications is uncertain hormonal therapy may be considered.
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D005260:Female; D005733:Gangliosidoses / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008297:Male
D000324:Adrenocorticotropic Hormone
Myoklonisch-astatische Anfälle (Lennox-Syndrom) im Verlauf der juvenilen neuronalen Ceroidlipofuscinose (M. Batten-Spielmeyer-Vogt).