On the Specific Age Dependent Epileptic Syndrome: The Early-Infantile Epileptic Encephalopathy with Suppression-Burst

Abstract

An early-infantile epileptic syndrome is reported. This syndrome is characterized by its early onset (less than 3 months of age), frequent tonic spasms, suppression-burst on EEG, extremely poor response to treatment and grave outcome. Out of 8 cases (5 boys and 3 girls) experienced thus far, 6 had thir onset before 1 month of age. Tonic spasms appeared in series in 4 cases, and were seen during awake and sleep period. The underlying pathologies, known in 4 cases, were porencephaly (in 2), Aicardi syndrome (in one), and subacute diffuse encephalopathy (in one). In all cases the EEG showed suppression-burst as a common denominator of this syndrome when awake or asleep, in addition to desynchronization during seizure. It is worthy of mention that hypsarhythmia was observed in 5 patients during the follow up period, and the transition to the West's syndrome in 4 cases. In most cases the suppression-burst pattern disappeared after 3 months of age. Follow up study of up to 3 years indicated the extreme ref actoriness to therapy; 4 are dead, and the remaining 4 have been suffering from severe mental and physical handicaps. Supposed pathophysiology of this syndrome studied from the burst-burst intervals was also discussed. In conclusion we would like to list this syndrome as the third specific, age-dependent childhood epileptic encephalopathy, to be entirely separated from the West's and the Lennox syndromes. © 1976, The Japanese Society of Child Neurology. All rights reserved.