Benefit of corticosteroid therapy in Angelman syndrome.
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.
Forrest
Katharine M L
KM
Department of Paediatric Neurology, Evelina Children's Hospital at St Thomas' Hospital, Westminster Bridge Road, London, United Kingdom. kforrest@nhs.net
Young
Helen
H
Dale
Russell C
RC
Gill
Deepak S
DS
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
D000305:Adrenal Cortex Hormones