Electrical status epilepticus in sleep (ESES) - Treatment pattern and EEG outcome in children with very high spike-wave index

Abstract

Objective: The objective of the study was to determine electrical status epilepticus in sleep (ESES) outcome in children with very high spike-wave index (SWI; ≥85%), and assess treatment pattern.

Methods: Medical records of children 1-17 years old with ESES were reviewed. In this study, ESES is defined as SWI in non-rapid eye movement (non-REM) sleep of ≥85%. Electrical status epilepticus in sleep resolution is defined as reduction of SWI to <50%.

Results: Complete data were available in 33 children. Age at ESES diagnosis ranged from 32 to 165 months, median 76 months. The median duration of follow-up was 33 months. Two-thirds of the children were on one or more antiepileptic drugs (AED) at ESES diagnosis. Antiepileptic drugs were used as first treatment for ESES in 24/33 (73%). Electrical status epilepticus in sleep initially resolved in 76%, but 56% had subsequent relapse. The relapse rate was higher for steroids (89%) and benzodiazepines (60%) as compared with nonbenzodiazepine AEDs (29%). At last follow-up, ESES resolved in 21 children (64%). Electrical status epilepticus in sleep resolution was associated with seizure freedom (Fisher's exact, p < 0.05).

Significance: Using electroencephalogram (EEG) criteria, ESES resolved in 64%. We found high failure rate of first-line AEDs in preventing ESES, and high relapse rate. Standardization of ESES management is urgently needed.

Keywords: ESES; Epileptic encephalopathy; Spike–wave index (SWI).