Auteurs
Lesca G - Rudolf G - Labalme A - Hirsch E - Arzimanoglou A - Genton P - Motte J - de Saint Martin A - Valenti MP - Boulay C - De Bellescize J - Kéo-Kosal P - Boutry-Kryza N - Edery P - Sanlaville D - Szepetowski P
Journal
Epilepsia
Abstract
The continuous spike and waves during slow-wave sleep syndrome (CSWSS) and the Landau-Kleffner (LKS) syndrome are two rare epileptic encephalopathies sharing common clinical features including seizures and regression. Both CSWSS and LKS can be associated with the electroencephalography pattern of electrical status epilepticus during slow-wave sleep and are part of a clinical continuum that at its benign end also includes rolandic epilepsy (RE) with centrotemporal spikes. The CSWSS and LKS patients can also have behavioral manifestations that overlap the spectrum of autism disorders (ASD). An impairment of brain development and/or maturation with complex interplay between genetic predisposition and nongenetic factors has been suspected. A role for autoimmunity has been proposed but the pathophysiology of CSWSS and of LKS remains uncharacterized.