Benign Familial Neonatal Convulsions Followed by Benign Epilepsy with Centrotemporal Spikes in Two Siblings

Summary: Purpose: To report on sibling cases with benign familial neonatal convulsions (BFNC) followed by benign epilepsy with centrotemporal spikes (BECT).

Methods: Case histories and EEGs were obtained for the two siblings with neonatal and subsequent epileptic seizures in one pedigree with BFNC.

Results: The family included six affected cases of BFNC in two generations: the proband, the proband's mother and two sisters, and the proband's maternal uncle and his daughter. The proband developed a generalized tonic convulsion 2 days after birth with no apparent cause and normal interictal EEG, and experienced a total of 18 episodes of tonic or clonic seizures or both by age 9 months. In the follow-up course, an EEG recording showed rolandic discharges at 2 years, and a sylvian seizure occurred at 4 years during sleep. On carbamazepine therapy, the last seizure was recorded at 9 years after a total of 11 episodes of sylvian seizures, with normal EEGs after 12 years. The proband's sister experienced nine episodes of brief tonic seizures between 7 and 9 days after birth, and also developed eight episodes of sylvian seizures from 4 to 7 years, with rolandic discharges on EEG until age 9 years. All of the family members had normal psychomotor development, with no neurologic sequelae.

Conclusions: This report of BFNC followed by BECT in sibling cases is significant in view of the genetic analysis and the classification of epilepsies and epileptic syndromes.