Surgical management of pediatric patients with encephalopathy due to electrical status epilepticus during sleep (ESES)

Abstract

Aims : ESES is a developmental epileptic disorder directly responsible for progressive encephalopathy and neurocognitive regression. The natural history, indications for surgical intervention, and predictors for favorable seizure and neuropsychological outcome remain unclear. Methods : We performed a retrospective review of children who underwent resective or disconnective surgery for ESES between January 2009 and July 2016 at a large tertiary pediatric center. Information on the patients’ demographics, seizure semiology, radiographic and electrographic findings, and surgical management was collected. The primary outcome was seizure freedom at last follow‐up visit, and secondary outcomes were neuropsychological improvement and electrographic ESES resolution. Results : We identified 11 children who underwent surgery for ESES. The mean ages were 3.2 years for seizure onset, 7.1 years for formal ESES diagnosis, and 9.4 years for surgery. Seizure etiologies included cortical malformations (four patients), encephalomalacia and gliosis from prior hemorrhage or tumor resections (three patients), developmental porencephaly (one patient), and Rasmussen's encephalitis (one patient); the etiology was unknown in two children. Preoperatively, nine children had motor deficits, seven had speech and language delay, and three had visual field defects. All children had seizures and neuropsychological regression prior to surgical consideration. Focal cortical resections were performed in seven children, and hemispherectomies in four. Post‐operatively, nine children experienced decreased seizure frequency, eight had neuropsychological improvement, and nine had resolution of electrographic ESES. Patients with poor surgical outcomes had more significant pre‐operative comorbidities, in addition to 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 compare favorably with previous reports for antiepileptic drugs, benzodiazepines, and steroids. As we gain greater understanding into the management of ESES, surgery is an increasingly useful tool for patients with mild or moderate neurodevelopmental delay, focal epileptogenic foci, and hemi‐ESES electrographic findings.

Clinical features of unilateral multilobar and hemispheric polymicrogyria (PMG)‐related epilepsy and seizure outcome with different treatment options

“…Several studies have also shown that PMG is the most common structural cause of ESES. 25 , 30 ESES‐associated epilepsy is generally considered to be age‐dependent and self‐limiting, and similar phenomena have been reported for PMG‐related ESES. 10 , 20 , 31 However, prolonged ESES can lead to DEE‐SWAS or EE‐SWAS with a legacy of severe cognitive impairment and neuropsychological damage.…”

“…A total of 70 patients (42 male, 28 female) were included, 60 of whom were from the 12 included published studies 9 , 15 , 16 , 17 , 19 , 20 , 22 , 23 , 24 , 25 , 26 , 27 (Figure 1 ); the other 10 patients were from our center. Forty‐nine patients (8 from our center) were included in the surgical group, and 21 patients (2 from our center) were included in the nonsurgical (ASM) group (Table 1 ).…”

“…Wang et al. 25 reported that ESES disappeared in 80% of patients after surgery. The studies we included did not describe the age at which patients in either group began to achieve freedom from seizures; thus, it is not clear whether surgery could help patients achieve seizure freedom earlier.…”

Continuous Spike–Waves during Slow Sleep Today: An Update

“…After neurosurgery, nine out of 11 children showed a reduced seizure frequency, eight out of 11 showed neuropsychological improvement, and nine out of 11 had ESES resolution. Children with poor outcomes after surgery showed, in addition to ESES, more severe preoperative comorbidities [143]. Marashly et al studied 14 carefully selected ESES cases who underwent resective neurosurgery for epilepsy and ESES; 12 with magnetic resonance imaging (MRI) suggestive of perinatal suffering and two with normal MRI.…”