Doose syndrome (myoclonic–astatic epilepsy): 40 years of progress
Article first published online: 16 AUG 2010
© The Authors. Journal compilation © Mac Keith Press 2010
Developmental Medicine & Child Neurology
Volume 52, Issue 11, pages 988–993, November 2010
How to Cite
KELLEY, S. A. and KOSSOFF, E. H. (2010), Doose syndrome (myoclonic–astatic epilepsy): 40 years of progress. Developmental Medicine & Child Neurology, 52: 988–993. doi: 10.1111/j.1469-8749.2010.03744.x
- Issue published online: 7 OCT 2010
- Article first published online: 16 AUG 2010
- PUBLICATION DATA Accepted for publication 8th June 2010. Published online 16th August 2010.
Doose syndrome, otherwise traditionally known as myoclonic–astatic epilepsy, was first described as a unique epilepsy syndrome by Dr Hermann Doose in 1970. In 1989, the International League Against Epilepsy classified it formally as a symptomatic generalized epilepsy, and 20 years later it was renamed ‘epilepsy with myoclonic–atonic seizures’. In this review, we discuss the components of this unique disorder including its incidence, clinical features, and electroencephalographic findings. Recent evidence has suggested possible genetic links to the GEFS+ (generalized epilepsy with febrile seizures plus) family, and, additionally, some children with structural brain lesions can mimic the Doose syndrome phenotype. 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. Prognosis is quite varied in this disorder; however, many children can have a remarkably normal neurodevelopmental outcome.