Award: American Academy of Pediatrics Award

Authors: Shelly Wang, MD; Alexander Weil, MD; George Ibrahim, MD, PhD, FRCSC; Aria Fallah; Brandon Korman, PhD; Michael Duchowny, MD; Sanjiv Bhatia, MD; John Ragheb, MD (Miami, FL)

Introduction : Electrical status epilepticus during sleep (ESES) is a developmental epileptic condition directly responsible for progressive encephalopathy and neurocognitive regression. The natural history and predictors of favorable outcome remain unclear. Methods : We performed a retrospective review of children who underwent surgery for ESES at Nicklaus (Miami) Children's Hospital, with at least 24 months of post-operative follow-up. Patient demographic, radiographic, EEG, and neuropsychological information was collected. The primary outcome was seizure-freedom, and secondary outcomes were neuropsychological improvement and electrographic ESES resolution. Results : During the study period, 58 children with ESES were identified; of these, 11 children (9 girls, 2 boys) underwent surgery. The average age of seizure onset was 3.2 ± 1.9 years, and the average age at surgery was 9.4 ± 3.4 years. The seizure etiologies included malformations of cortical development (4), encephalomalacia and gliosis from prior hemorrhage or tumor resections (3), developmental porencephaly (1), and Rasmussen’s encephalitis (1); the etiology was unknown in 2 children. All of the children had seizures and neuropsychological regression prior to surgical consideration, and 9 children had focal neurologic deficits. Focal cortical resections were performed on 7 children, and hemispherectomies in 4. Post-operatively, 10 children experienced decrease in seizure frequency, 8 of whom had complete electrographic resolution of ESES. Post-operative formal neuropsychological testing and parental reports revealed improved cognitive, language, and academic performance in 8 children. Children with unfavorable outcomes had increased pre-operative morbidities and evidence of bilateral ESES activity. Conclusion : In this case series, surgery for a carefully selected group of children with ESES is safe and feasible, yielding rates of seizure freedom and neuropsychological improvement that compares favorably to those reported in literature for antiepileptic drugs, benzodiazepines, and steroids. Surgery is an ideal treatment modality for patients with moderate neurodevelopmental delay, focal epileptogenic foci, and hemi-ESES electrographic findings.