[Atypical early infantile epileptic encephalopathy with suppression-burst].

We report an 8-month-old infant with tonic spasms that started at the age of 38 days and occurred in clusters. He had no myoclonic seizures, or erratic myoclonus. Repeated simultaneous video-EEG monitoring revealed abrupt flexion of the neck and extremities associated with high voltage slow waves preceded by multiple spikes and/or sharps, which resembled spasms seen in West syndrome. Interictal EEGs, before and after the onset of epilepsy, showed suppression-burst. The bursts consisted of irregular high voltage slow waves mixed with poorly developed spike before the onset of epilepsy. On the other hand, duration of suppression phase ranged from 1 to 27 seconds, and burst-burst interval from 2 to 30 seconds after the onset of epilepsy. He was considered as having an atypical early infantile epileptic encephalopathy with suppression burst.