Electro-clinic features and outcome in children with “pure” epilepsy with myoclonic atonic seizures (EMAS)

bjective: Epilepsy with myoclonic-atonic seizures (EMAS), previously known as epilepsy with myoclonic astatic seizures, or Doose syndrome, is a rare epileptic syndrome in early childhood. The aim of this study is to elucidate the electro-clinical features and outcome in patients with “ pure “ MAE Method: From out-patient setting between 1994-2004 four boy with “pure” EMAS syndrome were included into study. Results: Seizure types include myoclonic; in 4 patients, myoclonic-astatic seizures; in 4 patients and myoclonic-astatic with associated absence,clonic, and tonic components; in 2 patients. Episodes of status epilepticus were seen in two of patients (50%). The outcome was favorable in three patients and unfavorable in one patients who had long episodes of status epilepticus. The antiepileptic drug treatment was variably succesful; two patients responded well to dualtherapy with valproate and clonazepam, one patient needed lamotrigine add-on therapy to dualtherapy, the remaining patient had unfavorable response to multiple drug therapy (valproate, clonazepam, lamotrigine and topramate respectively). Serial EEG examinations of patient with unfavorable outcome revealed generalized, irregular burst of spike-wave discharges lasting 5-10 sec during ictal and interictal recordings. Conclusion: This study provide additional support for the significance of electro-clinical features in patients with “ pure” EMAS for outcome.
Keywords: myoclonic atonic seizures: Doose syndrome: electroclinical syndrome: outcome.