Encephalopathy Associated with Electrical Status Epilepticus of Sleep (ESES): A Practical Approach

Encephalopathy associated with electrical status epilepticus of sleep (ESES) is an underdiagnosed epileptic encephalopathy syndrome. Though there is considerable controversy regarding the terminology of this condition, there is increasing acceptance of the term, ‘encephalopathy associated with electrical status epilepticus of sleep,’ as it implies a clinical component as well as EEG feature. Core EEG feature of sleep activation of epileptiform discharges temporarily associated with cognitive or behavioral regression in a child with epilepsy is sufficient to make a diagnosis of ESES. It is important to quantify the spike wave index (SWI) during awake state and sleep to assess for sleep activation. Since focal spikes are also included in the counting of SWI, some grading to assess the distribution of epileptiform discharges is suggested to obtain the complete sleep EEG picture. MRI of the brain does not show abnormality in a number of patients. But common structural lesions include perinatal vascular insults and cortical malformations. Response to treatment is usually variable, but highest success rate is reported with the steroids. Prognosis usually depends on the duration of ESES.