Journal article

AUTOSOMAL-DOMINANT ROLANDIC EPILEPSY AND SPEECH DYSPRAXIA - A NEW SYNDROME WITH ANTICIPATION

IE SCHEFFER, L JONES, M POZZEBON, RA HOWELL, MM SALING, SF BERKOVIC

ANNALS OF NEUROLOGY | LITTLE BROWN CO | Published : 1995

Abstract

We describe a family of 9 affected individuals in three generations with nocturnal oro-facio-brachial partial seizures, secondarily generalized partial seizures, and centro-temporal epileptiform discharges, associated with oral and speech dyspraxia and cognitive impairment. The speech disorder was prominent, but differed from that of Landau-Kleffner syndrome and of epilepsy with continuous spike and wave during slow-wave sleep. The electroclinical features of this new syndrome of autosomal dominant rolandic epilepsy resemble those of benign rolandic epilepsy, a common inherited epilepsy of childhood. This family shows clinical anticipation of the seizure disorder, the oral and speech dysprax..

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