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Benefit of Corticosteroid Therapy in Angelman SyndromeEvelina Children's Hospital, Westminster Bridge Road, London, United Kingdom, kforrest{at}nhs.net
T.Y. Nelson Department of Neurology, The Children's Hospital at Westmead, Sydney, New South Wales, Australia, Royal North Shore Hospital and Northern Clinical School, University of Sydney, Sydney, New South Wales, Australia
T.Y. Nelson Department of Neurology, The Children's Hospital at Westmead, Sydney, New South Wales, Australia
T.Y. Nelson Department of Neurology, The Children's Hospital at Westmead, Sydney, New South Wales, Australia 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.
Key Words: Angelman syndrome • corticosteroid • epilepsy • sleep
Journal of Child Neurology, Vol. 24, No. 8,
952-958 (2009) |
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