Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia

Encephalopathy with electrical status epilepticus during sleep or ESES is a n age-dependent and self-limited syndrome whose distinctive features includ e a characteristic age of onset (with a peak around 4-5 years), heterogeneo us seizures types (mostly partial motor or unilateral seizures during sleep and absences or falls while awake), a typical EEG pattern (with continuous and diffuse paroxysms occupying at least 85% of slow wave sleep) and a var iable neuropsychological regression consisting of IQ decrease, reduction of language (as in acquired aphasia or Landau-Kleffner syndrome), disturbance of behaviour (psychotic states) and motor impairment (in the form of ataxi a, dyspraxia, dystonia or unilateral deficit). Despite the long-term favour able outcome of epilepsy and status epilepticus during sleep (SES), the pro gnosis is guarded because of the persistence of severe neuropsychological a nd/or motor deficits in approximately half of the patients. No specific tre atment has been advocated for this syndrome, but valproate sodium, benzodia zepines and ACTH have been shown to control the seizures and the SES patter n in many cases, although often only temporarily. Subpial transection is pr oposed in some instances as in non-regressive acquired aphasia. Recent data support the concept that ESES syndrome may include a large subset of devel opmental or acquired regressive conditions of infancy. (C) 2000 Elsevier Sc ience Ireland Ltd. All rights reserved.