[Myoclonic encephalopathy (Kinsbourne syndrome)].
Three children with myoclonic encephalopathy (Kinsbourne's disease) are described, in which one of them was shown to have ganglioneuroblastoma. Symptoms were opsoclonus, polymyoclonia of the striated muscles and cerebellar ataxia. Treatment consisted in corticosteroids and adrenocorticotropic hormone respectively in all patients, the patient with ganglioneuroblastoma also had a resection of the tumor. All patients responded to therapy, however recurrence of myoclonia and of the opsoclonus were seen after discontinuation or reduction of the corticosteroid dose, as well as following the course of intercurrent viral infections. Neurologic symptoms eventually disappeared after 3 1/2-5 1/2 years, however in two children behavioural abnormalities and disorders of speech and cognitive development remained.
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001927:Brain Diseases / Q000150:complications*; D002524:Cerebellar Ataxia / Q000150:complications; D002648:Child; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009207:Myoclonus / Q000150:complications* / Q000188:drug therapy; D009759:Nystagmus, Pathologic / Q000150:complications; D013577:Syndrome
D000305:Adrenal Cortex Hormones
Myoklonische Enzephalopathie (Kinsbourne-Syndrom).