Epileptic syndromes with focal seizures of childhood and adolescence.

Abstract The first description of benign focal epilepsy of childhood likely dates back to the 16th century. Further delineation over the past 60 years led to the identification “benign” rolandic epilepsy and subsequently of occipital subtypes and other variants, including epilepsy with continuous spike-and-wave activity during slow-wave sleep and Landau–Kleffner syndrome. The pattern of spontaneous occurrence and resolution with age, frequently in association with cognitive deficits, has led to the term benign seizure susceptibility syndrome. In this review we outline epidemiological features, clinical and EEG presentations in relationship to diurnal patterns and to sleep, neuropsychological findings, genetics, pathophysiology, management, and prognosis of childhood seizure susceptibility syndromes. Interactions between brain development and maturation processes as well as genetic influences may play a role in the development of various childhood epileptic syndromes associated with language and cognitive deficits.