Pediatric Focal Epilepsy Syndromes

Abstract

Benign epilepsy with centrotemporal spikes, early-onset childhood occipital epilepsy (Panayiotopoulos syndrome [PS]) and late-onset childhood occipital epilepsy (Gastaut type [LOCE-G]) are the principal pediatric focal epilepsy syndromes. They share major common characteristics: the appearance and resolution of electroclinical features are age related, there is a strong genetic predisposition, the clinical course is often mild with infrequent and easy to control seizures, interictal epileptiform activity is disproportionately abundant when compared with the clinical correlate, and tends to potentiate and generalize during sleep. In this review, we outline the relevant pathophysiology underlying this electroclinical spectrum. Then, the initial description of individual syndromes is followed by a summary of overlapping features and intermediate presentations that question the boundaries between these entities and provide the basis for the concept of a childhood seizure susceptibility syndrome. Additionally, we outline the main features of the related epileptic encephalopathies. An outlook on potential future lines of research completes this review.