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McMaster University (Dr. Ronen), Hamilton, ON, Canada; Memorial University of Newfoundland (Dr. Rosales), St. John's, NF, Canada; The Jane-way Child Health Centre (M. Connolly), St. John's, NF, Canada; the University of Minnesota (Dr. Anderson), Minneapolis, MN; and the Howard Hughes Medical Institute (Dr. Leppert), University of Utah Health Science Center, Salt Lake City, UT.
We studied a kindred of 69 affected individuals with the autosomal dominant epileptic syndrome of benign familial neonatal convulsions, linked to chromosome 20. Forty-two percent had their seizure onset on day 3, while remission took place in 68% during the first 6 weeks. Seizures were brief and the phenotype was of a mixed seizure type, starting with tonic posture, ocular symptoms, apnea, and other autonomic features. The seizure often progressed to clonic movements and motor automatisms. The postictal state was brief, and interictally the neonates looked well. The ictal EEG pattern with generalized suppression of amplitude on onset may be relatively unique. Neurocognitive outcome was usually normal, but the risk for subsequent epilepsy was 16%. Most of the later epilepsy was generalized tonic or tonic-clonic, and some seizures were provoked, raising the possibility of an unusual form of reflex epilepsy.
Address correspondence and reprint requests to Dr. G.M. Ronen, Department of Pediatrics, McMaster University Medical Centre, 1200 Main Street West, Room 4U1, Hamilton, ON, L8N 3Z5, Canada.
Supported by a grant from The Janeway Child Health Centre Foundation, St. John's, NF.
Received August 5, 1992. Accepted for publication in final form November 17, 1992.
Presented at the American Epilepsy Society Meeting, Philadelphia, PA, December 1991.
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