The spectrum of neuropsychiatric abnormalities associated with electrical status epilepticus in sleep

Electrical status epilepticus in sleep (ESES) is an electrographic pattern consisting of an almost continuous presence of spike-wave discharges in slo w wave sleep. ESES is frequently encountered in pediatric syndromes associa ted with epilepsy or cognitive and language dysfunction. It can be present in various evolutionary stages of a spectrum of diseases, the prototypes of which are the 'continuous spikes and waves during slow wave sleep' syndrom e (CSWS), the Landau-Kleffner syndrome (LKS), as well as in patients initia lly presenting as benign childhood epilepsy with centrotemporal spikes (BEC TS). The purpose of this article is to review the literature data on the se miology, electrographic findings, prognosis, therapeutic options, as well a s the current theories on the pathophysiology of these disorders. The frequ ent overlap of CSWS, LKS, and BECTS urges an increased level of awareness f or the occasional transition from benign conditions such as BECTS to more d evastating syndromes such as LKS and CSWS. Identification of atypical signs and symptoms, such as high discharge rates, prolonged duration of ESES, ne uropsychiatric and cognitive dysfunction, lack of responsiveness to medicat ions, and pre-existing neurologic conditions is of paramount importance in order to initiate the appropriate diagnostic measures. Prolonged and if nee ded repetitive sleep electroencephalographs (EEGs) are warranted for proper diagnosis. (C) 2000 Elsevier Science B.V. All rights reserved.