Video/EEG aspects of early-infantile epileptic encephalopathy with suppression-bursts (Ohtahara syndrome)

Early-infantile epileptic encephalopathy (EIEE) with suppression-bursts is a severe neonatal epileptic encephalopathy. The etiology is multiple, with cerebral malformations as the more frequent. We review the clinical and vid co/EEG aspects of eight infants with EIEE. These infants, aged between 4 an d 70 days at the time of video/EEG recordings, were studied in relation to their clinical and video/EEG characteristics, evolution, persistence of sup pression-burst pattern and etiology. Seven of the eight infants showed an i ctal clinical sign correlated to the burst of the suppression-burst pattern , four of whom died within 11 months of age. The other three are alive. One , now aged 4 years, underwent surgery for hemimegalencephaly and is seizure -free, with good neurological outcome. One, now aged 9 months, was pyridoxi ne-dependent and she is seizure-free, and with normal neurological evolutio n under pyridoxine therapy. One, now aged 3 years and 9 months, is seizure- free, but with severe neurological and cognitive impairment. The only child who did not show a clinical ictal correlation of burst is also alive, now aged 3 years and 9 months, with drug-resistant epilepsy, and severe neurolo gical and cognitive deficits. With regard to the etiology, three showed str uctural abnormalities, two more showed some signs of prenatal origin of neu rological disease, and three had metabolic etiology. Our study confirms tha t EIEE is a severe age-dependent early epileptic encephalopathy. The etiolo gy is mostly malformative. The prognosis is poor regarding motor and cognit ive development, seizures, as well as life expectancies. The presence of an ictal burst of the suppression-burst pattern usually correlates with a neg ative outcome. (C) 2001 Elsevier Science B.V. All rights reserved.