Identical Twin Discordant for Electrical Status Epilepticus of Sleep (ESES)

Abstract

While some experts use the terms interchangeably, other refer to ESES strictly as the electrographic pattern of frontally dominant spike wave discharges that comprise 85% of nonREM sleep, while CSWSS comprise this EEG pattern, together with the clinical syndrome of regression and epilepsy. The discovery of ESES is considered to be a neurological emergency as permanent intellectual deterioration is associated with the ESES EEG pattern, when refractory. Age of onset has been reported between 3-14 years. The EEG pattern of sleepinduced generalized spike-and wave discharges (1.5-3.5 Hz), occurring continuously or discontinuously, and lasts for months to years. The International League against Epilepsy (ILAE) has determined that ESES is neither a focal nor generalized syndrome [1]. Indeed, this condition has been determined symptomatic, idiopathic (presumed genetic), or cryptogenic (unknown).