Electrical status epilepticus during sleep (ESES). Different clinical syndromes: towards a unifying view?

Abstract

'Electrical status epilepticus during sleep' (ESES) is a typical childhood process of generalization of paroxysmal activity. Notwithstanding a number of intermediate forms, three syndromes included in the 1989 ILAE classification can be considered as prototypes: the 'continuous spike-waves during sleep' (CSWS syndrome), the 'acquired aphasia with convulsive disorder in children' (L-Kl syndrome) and the 'benign epilepsy of childhood with rolandic spikes' (BECRS), which can be considered as the benign end of the spectrum. The pathognomonic clinical and EEG features of these conditions are described. They can probably be considered, in a unifying view, to be based on a common pathogenetic factor. They are associated with neuropsychological and/or mental disturbances with differences probably due to the idiopathic or symptomatic origin of the underlying epileptic condition, the cortical area of the primary focal paroxysmal activity, the patient's age and the severity and duration of the paroxysmal dysfunction. Possible hypotheses on the physiopathogeneses of ESES and correlated neuropsychological disorders are summarized. Short cycles (3-4 weeks) of relatively high daily doses of diazepam (DZP) (0.5 mg/kg body weight) following a rectal DZP bolus of 1 mg/kg b.w. seem to be effective in the majority of ESES conditions. The somewhat underestimated problem of neuropsychological disorders correlated with ESES in BECRS is also considered.