Early infantile epileptic encephalopathy: a long-term follow-up study

The authors describe the electroclinical aspects and long-term evolution of five (in one male and four female patients) cases of early infantile epileptic encephalopathy in the neonatal period, the most common seizures were tonic spasms (either generalized or lateralized) that, very often, occurred in series. All newborns showed the typical suppression-burst pattern. The neurological status (initially normal) progressively deteriorated in the subsequent months, and all infants suffered from severe mental insufficiency and daily intractable seizures. Four out of the five children developed hypsarrythmia and one, Lennox-Gastaut syndrome. The authors discuss the main problems related to the suppression-burst pattern and the long-term prognosis of this abnormality.