Medical Journal of The Islamic Republic of Iran (MJIRI)

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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.I Imaging shows anatomic defects such as migration disorders and generalized atrophy" with essentially normal metabolic tests. The seizures often change to West's syndrome (WS) or Lennox G austaut syndrome (LG S) pattern.3 Ohtahara et a1. first described this syndrome in 1976.3 We observed ten cases of this syndrome during a period of I 2 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 used 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

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