SURVEY OF TEN CASES OF EARLY INFANTILE EPILEPTIC ENCEPHALOPATHY (OHTAHARA SYNDROME)

Early infantile epileptic encephalopathy or EIEE (Ohtahara syndrome; OS) is a kind of intractable seizure that begins in neonatal age with sudden onset of tonic spasms in series or single suppression-burst S-B in EEG.¹ Imaging shows anatomic defects such as migration disorders and generalized atrophy² with essentially normal metabolic tests. The seizures often change to West’s syndrome (WS) or Lennox Gaustaut syndrome (LGS) pattern.³ Ohtahara et al. first described this syndrome in 1976.3 We observed ten cases of this syndrome during a period of 12 months. 70% of the patients were male. The mean age at the time of onset of seizures was 8 days; the mean age at the time of admission was 6.8 months. In 20% of cases the first seizures were generalized, in 80% tonic. EEG at the time of referring showed suppression-burst S-B in 50%, hypsarrhythmia in another and in 60% of them there were scattered sharp, spike or spike and wave in one or both sides. The drugs that have been u! sed in multiple therapy were clonazepam 50%, prednisone 60%, sodium valproate 30%, nitrazepam 20%, vigabatrin 30% and acetazolamide in 10%. Response to treatment was good in 70% and moderate in 30%. At the end of the course all patients showed severe mental and motor retardation. Prognosis was shown to be poor in these patients.

Keywords
Early infantile epilepsy with suppression-bursts; EIEE; Intractable infantile seizure; Ohtahara syndrome; OS; Early myoclonic encephalopathy.