Early infantile epileptic encephalopathy (EIEE) or Ohtahara syndrome is the earliest form of age-dependent encephalopathies, which include also West syndrome and Lennox-Gastaut syndrome. This rare syndrome is characterized by a very early onset, during the first months of life, with frequent tonic spasms and a suppression-burst pattern on electroencephalogram. Partial motor seizures may occur. Brain imaging usually discloses gross structural abnormalities in the majority of cases. Metabolic disorders were present in a few cases. The course is severe with early death or marked psychomotor retardation and intractable seizures with frequent evolution to West syndrome. Antiepileptic drugs remain as first-line treatment. EIEE constitutes along with the neonatal or early myoclonic encephalopathy the group of 'epileptic encephalopathies with suppression-burst pattern'' or 'severe neonatal epilepsies with suppression-burst pattern''.
Last update: July 2004