| A type of epilepsy that presents with myoclonic-atonic seizures usually between 2 to 6 years of age. Other generalized seizure types which may be seen in this syndrome include atonic, myoclonic, generalized tonic-clonic seizures, tonic and absence seizures. Nonconvulsive status epilepticus is common. Development prior to seizure onset is normal in two thirds of cases. These children typically show developmental stagnation or even regression during the active seizures (stormy) phase, which improves once seizures are controlled. The electroencephalogram shows generalized 2 to 6 Hz spike-wave or polyspike-and-wave abnormalities, with normal background. |
