Atypical Evolutions of Idiopathic Focal Epilepsies in Childhood

Abstract

Clinical and electroencephalographic (EEG) features of the three well-defined idiopathic focal epilepsies in childhood (IFEC)—benign childhood epilepsy with centrotemporal spikes (BCECTS), Panayiotopoulos syndrome, and the Gastaut type of idiopathic occipital epilepsy of childhood (IOEC-G)—have been clearly described and reported. It is also known that a significant percentage of children with IFEC present what were named atypical features in seizures and EEG abnormalities. We are studying here the small number of patients with IFEC who not only present atypical features, but also evolve into the spectrum of epileptic encephalopathies related with continuous spike-and-wave during sleep (CSWSS) or electrical status epilepticus during sleep (ESES), which includes atypical benign focal epilepsy of childhood, status of BCECTS, Landau-Kleffner syndrome, and CSWSS or ESES syndrome. We also emphasize that some patients with these encephalopathic course of IFEC present a mix or a sequence in time of the four mentioned subsyndromes.