A syndrome of nocturnal oro-facio-brachial partial seizures, secondarily generalized partial seizures, centro-temporal epileptiform discharges, associated with oral and speech dyspraxia and cognitive impairment, is described in a family of 9 affected members in three generations reported from Austin Hospital, Heidelberg (Melbourne), the University of Melbourne, and the Royal Children’s Hospital, Melbourne, Australia. All affected individuals had nocturnal rolandic seizures limited to midchildhood. Inheritance of epilepsy and speech dyspraxia was autosomal dominant, with 100% penetrance for speech dyspraxia. Clinical anticipation was noted across the three generations of affected individuals, with increasingly severe speech dyspraxia, epilepsy as well as cognitive impairment. The syndrome has features resembling benign rolandic epilepsy (BRE) and may help in identifying the gene for BRE which is unassociated with clinical anticipation. It differs from the syndromes of Landau-Kleffner and epilepsy with continuous spike and wave during slow-wave sleep. 
COMMENT. The authors suggest that this new syndrome, with its known genetic basis, may help to clarify the relationship between benign rolandic epilepsy, a benign syndrome, and Landau-Kleffner and CSWSS, more severe syndromes.
Symptoms and findings in the Landau-Kleffner (LKS) and continuous spike-and-wave during slow sleep (CSWSS) syndromes are compared in a report from the Department of Child Neurology, University of Helsinki, Children’s Castle Hospital, Helsinki, Finland . Bilateral epileptiform activity was found in sleep EEGs in 5 of 6 LKS children and in all 11 children with CSWSS. All LKS children had auditory agnosia and deterioration of expressive language, 4 had attention deficit disorders, and 2 became clumsy or ataxic. Six children with CSWSS had deterioration of expressive language, motor skills and general intelligence, and 4 had hyperkinesia and delayed development. Epileptic seizures occurred in all LKS and in 8 CSWSS children. Mean age at diagnosis was 5 years for LKS and 6 and 1/2 years for CSWSS. MRI/CT was abnormal in 1 LKS and 5 CSWSS patients. LKS usually affects previously normal children whereas CSWSS occurs in children with pre- or perinatal pathology and previously abnormal development. An overnight EEG is recommended in children with developmental arrest, loss of speech, and/or major behavioral problems. Four additional papers on Landau-Kleffner syndrome will be presented at the Annual Meeting of the American Epilepsy Society, Baltimore, Dec 1-6, 1995.