Early infantile epileptic encephalopathy

Key-words Disease name / synonyms Definition / diagnostic criteria Differential diagnosis Abstract 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".