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.