Myoclonic-astatic epilepsy

Definition:

Myoclonic astatic epilepsy (MAE), also known as Doose syndrome, is a generalized epilepsy syndrome of young children that includes multiple different seizure types, of which myoclonic and astatic seizures were the most prominent. The electro- encephalogram (EEG) tracings may be normal initially, but later develop a characteristic biparietal theta background rhythm, and irregularly generalized spike wave, and polyspike wave discharges. In general, children are developmentally normal before the onset of epilepsy and organic brain abnormalities are absent. Long-term prognosis varies from cessation of seizures with normal developmental outcome to intractable epilepsy with mental retardation. Favorable outcomes were reported in half to two-thirds of cases. Treatment strategies such as corticosteroids, ethosuximide, and valproate have been described as only partially effective, but newer anticonvulsants, such as levetiracetam and zonisamide, may provide additional seizure control. The most effective treatment reported to date appears to be the ketogenic diet.

References:

[1]. Sarah A Kelley, et al. Doose syndrome (myoclonic-astatic epilepsy): 40 years of progress. Dev Med Child Neurol. 2010 Nov;52(11):988-93. [Content Brief]

[2]. Sudha Kilaru, et al. Current treatment of myoclonic astatic epilepsy: clinical experience at the Children's Hospital of Philadelphia. Epilepsia. 2007 Sep;48(9):1703-1707. [Content Brief]

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