Early infantile epileptic encephalopathy
Early infantile epileptic encephalopathy (EIEE) with burst-suppression (Ohtaharas syndrome) is a rare type of epileptic encephalopathy in infancy and represents the earliest type of age-related symptomatic generalised epilepsy. We present 4 cases of EIEE fulfilling the classic diagnostic criteria, excluding the cases with segmentary and erratic myoclonic, characteristic features of Early Myoclonic Encephalopathy. All the patients were females. Pregnancy and delivery were normal and no other cases were observed in the families. One of the patients had a normal twin brother. Seizures began in all the cases before the 10th day of life. The initial EEG showed burst-suppression pattern, lasting for an average of 2.3 months of age (1.5-4 months). Seizures were initially resistant to therapy, although 2 cases showed partial response. None of the patients evolved to West syndrome. A case died at 5 months of age. No etiologic factors were found in two cases, but hemimegalencephaly was detected in the other two patients; in one of them seizures disappeared after corticectomy. We believe EIEE constitutes a type of epileptic encephalopaty which can be distinguished from other types of early onset epilepsy and should be included in the International Classification of Epilepsies with the West and Lennox-Gastaut syndromes.