Syndrome of Ohtahara

Abstract A literature review using the term Ohtahara syndrome and early infantile epileptic encephalopathy revealed 51 cases. The characteristics of these cases were: 1) early onset of seizures; 2) tonic seizures; 3) poor response to treatment; 4) mental retardation; 5) poor prognosis; 6) burst-suppression EEG pattern; 7) evolution to West syndrome, and 8) multiple causes. These characteristics are non specific. No cause was found in most cases. The onset of seizures was between 7 hours and 86 days of age. The most frequent brain imaging abnormality was diffuse atrophy. ACTH was effective in a few cases and hemispherectomy was successful in one case. We believe that Ohtahara syndrome and early myoclonic encephalopathy are the same entity.