BENIGN MYOCLONIC EPILEPSY IN INFANTS - VIDEO-EEG FEATURES AND LONG-TERM FOLLOW-UP

We report video-EEG findings and a long-term follow-up study in 10 pat ients with benign myoclonic epilepsy in infants (BMEI). A high inciden ce of a past and family history of febrile convulsions was noted. Six of the 10 patients manifested characteristic vocalization associated w ith myoclonic seizures. It consisted of a sudden, brief expiratory noi se and is considered to be characteristic of BMEI. Afebrile convulsion s occurred before the onset of myoclonic seizures or during the clinic al course in six patients, but the accurate type of these seizures rem ains to be clarified. Monotherapy with valproatic acid (VPA) was very effective, but plasma VPA levels over 100 mu g/ml were initially neces sary in most of the cases, although they did not need to be maintained for further seizure control. All patients showed a favorable longterm seizure outcome, although one showed moderate mental retardation.