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Paper Information

Journal:   MEDICAL JOURNAL OF THE ISLAMIC REPUBLIC OF IRAN (MJIRI)   AUGUST 2004 , Volume 18 , Number 2; Page(s) 135 To 139.
 
Paper: 

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

 
 
Author(s):  NASIRIAN A.*
 
* DEPARTMENT OF PEDIATRIC NEUROLOGY, CHILDREN S MEDICAL CENTER, TEHRAN UNIVERSITY OF MEDICAL SCIENCES, TEHRAN, I.R. IRAN
 
Abstract: 

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.l Imaging shows anatomic defects such as migration disorders and generalized atrophyl with essentially nonllal 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 12months. 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 ofthe course all patients showed severe mental and motor retardation. Prognosis was shown to be poor in these patients.

 
Keyword(s): EARLY INFANTILE EPILEPSY WITH SUPPRESSION-BURSTS; EIEE; INTRACTABLE INFANTILE SEIZURE; OHTAHARA
 
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