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Benign Familial Neonatal Convulsions Followed by Benign Epilepsy with Centrotemporal Spikes in Two Siblings
Author(s) -
Maihara Toshiro,
Tsuji Masahiro,
Higuchi Yoshihisa,
Hattori Haruo
Publication year - 1999
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/j.1528-1157.1999.tb01997.x
Subject(s) - epilepsy , medicine , psychology , pediatrics , audiology , neuroscience
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.