title: Benefit of corticosteroid therapy in Angelman syndrome.
abstract: Angelman syndrome is often associated with an intractable seizure disorder. We describe 4 children who demonstrated an excellent response to corticosteroid therapy. The benefits included not only reduction in clinical seizures but also modification of the "typical'' Angelman electroencephalogram. In addition, there was improvement in the myoclonic jerks, sleep pattern, and developmental progress. Corticosteroids appeared to have a broad benefit on the epileptic encephalopathy. We believe that these cases pose a challenge to the conventional management of intractable epilepsy in Angelman syndrome.
authors: Department of Paediatric Neurology, Evelina Children's Hospital at St Thomas' Hospital, Westminster Bridge Road, London, United Kingdom. kforrest@nhs.net
authors: Katharine M L
authors: KM
authors: Forrest
authors: Helen
authors: H
authors: Young
authors: Russell C
authors: RC
authors: Dale
authors: Deepak S
authors: DS
authors: Gill
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D017204:Angelman Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D001921:Brain / Q000187:drug effects / Q000503:physiopathology; D002657:Child Development / Q000187:drug effects; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009207:Myoclonus / Q000188:drug therapy; D012640:Seizures / Q000188:drug therapy; D012890:Sleep / Q000187:drug effects; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones