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Updated: Jul 6, 2025
Author Spotlight: Advancing Pediatric Epilepsy Surgery in Children Through Novel Biomarkers and Enhanced Localization
Published on: September 20, 2024
Epilepsy surgery in children with genetic etiologies: A prospective evaluation of current practices and outcomes
Jason Coryell1, Rani Singh2, Adam P Ostendorf3
1Department of Pediatrics, Oregon Health & Sciences University, CDRC-P, 707 SW Gaines Rd, Portland, OR 97239, USA.
Insights
Epilepsy surgery offers significant seizure reduction in children with genetic causes, with 33% achieving freedom. Expedited surgical referral is recommended for broader patient benefit, even for palliative intent.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Genetics
Background:
- Epilepsy surgery is a treatment option for drug-resistant epilepsy.
- Genetic etiologies represent a significant portion of pediatric epilepsy cases.
- Understanding surgical outcomes in genetically diverse pediatric epilepsy populations is crucial.
Purpose of the Study:
- To evaluate current practices and outcomes of epilepsy surgery in children with known genetic causes.
- To explore pre-surgical workup, surgical interventions, and post-operative results across various genetic epilepsy types.
- To identify factors influencing seizure control after surgery in this cohort.
Main Methods:
- Retrospective analysis of 81 pediatric patients (≤18 years) with genetic epilepsy who underwent surgery.
- Data extracted from the Pediatric Epilepsy Research Consortium (PERC) surgery database across 18 US centers.
- Outcomes assessed using a modified International League Against Epilepsy (ILAE) score, analyzing neuroimaging, EEG, genetic group, and curative intent.
Main Results:
- 72% of children had daily seizures pre-surgery, with a median delay of 2.2 years post-drug resistance.
- 68% experienced >50% seizure reduction, and 33% achieved seizure freedom post-operatively (median follow-up 11 months).
- Seizure freedom was highest in the monogenic structural group; a single EEG focus correlated with better seizure freedom (p=0.02).
Conclusions:
- Epilepsy surgery provides substantial seizure reduction in most children with genetic epilepsy, irrespective of a single structural lesion.
- Significant seizure palliation is achievable across a wide spectrum of genetic epilepsy etiologies.
- Findings support expedited surgical referral and a broadened eligibility criteria for epilepsy surgery in pediatric genetic epilepsy.
Objective:
This study assesses current practices and outcomes of epilepsy surgery in children with a genetic etiology. It explores the pre-surgical workup, types of surgeries, and post-surgical outcomes in a broad array of disorders.
Methods:
Patients ≤18 years who completed epilepsy surgery and had a known genetic etiology prior to surgical intervention were extrapolated from the Pediatric Epilepsy Research Consortium (PERC) surgery database, across 18 US centers. Data were assessed univariably by neuroimaging and EEG results, genetic group (structural gene, other gene, chromosomal), and curative intent. Outcomes were based on a modified International League Against Epilepsy (ILAE) outcome score.
Results:
Of 81 children with genetic epilepsy, 72 % had daily seizures when referred for surgery evaluation, which occurred a median of 2.2 years (IQR 0.3, 5.2) after developing drug resistance. Following surgery, 68 % of subjects had >50 % seizure reduction, with 33 % achieving seizure freedom [median follow-up 11 months (IQR 6, 17). Seizure freedom was most common in the monogenic structural group, but significant palliation was present across all groups. Presence of a single EEG focus was associated with a greater likelihood of seizure freedom (p=0.02).
Significance:
There are meaningful seizure reductions following epilepsy surgery in the majority of children with a genetic etiology, even in the absence of a single structural lesion and across a broad spectrum of genetic causes. These findings highlight the need for expedited referral for epilepsy surgery and support of a broadened view of which children may benefit from epilepsy surgery, even when the intent is palliative.
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