J Child Neurol. 2009 Aug;24(8):952-8.

Benefit of corticosteroid therapy in Angelman syndrome.

Source

Department of Paediatric Neurology, Evelina Children's Hospital at St Thomas' Hospital, Westminster Bridge Road, London, United Kingdom. kforrest@nhs.net

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.

PMID:: 19666884; [PubMed - indexed for MEDLINE]

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Adrenal Cortex Hormones

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