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Outcomes of Early-Life Focal Cortical Dysplasia-Related Epilepsy: A PERC Surgery Study
Nathan T Cohen1, Dewi Frances Depositario-Cabacar1, Chima O Oluigbo1
1Center for Neuroscience, Children's National Hospital, The George Washington University School of Medicine, Washington, DC.
Insights
Epilepsy surgery for early-onset focal cortical dysplasia (FCD) in children yields high seizure freedom rates, especially for FCD type IIB. Early intervention shows promising outcomes with minimal complications for drug-resistant epilepsy (DRE).
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Epileptology
Background:
- Focal cortical dysplasia (FCD) is a leading cause of pediatric drug-resistant epilepsy (DRE) requiring surgery.
- Outcomes of epilepsy surgery for early-onset FCD-DRE (seizure onset <4 months) are not well-defined.
- This study investigates clinical and presurgical factors influencing surgical success in infants with FCD-DRE.
Purpose of the Study:
- To evaluate surgical outcomes in early-life FCD-DRE.
- To identify clinical and presurgical predictors of seizure freedom.
- To assess the safety of epilepsy surgery in this pediatric population.
Main Methods:
- A multicenter prospective cohort analysis of the PERC Surgery Database.
- Inclusion criteria: pathologically confirmed FCD-DRE, seizure onset <4 months.
- Data collected: clinical characteristics, presurgical workup, surgical intervention, and outcomes.
Main Results:
- Sixty-five percent (20/31) of patients achieved seizure freedom.
- Focal cortical dysplasia type IIB pathology showed an 89% seizure-free rate.
- Epileptic spasms were associated with a lower seizure-free outcome (45%).
- No deaths occurred; complication rates were minimal.
Conclusions:
- Epilepsy surgery offers high seizure-free rates and safety for early-onset FCD-DRE.
- FCD type IIB is strongly associated with favorable surgical outcomes.
- Early surgical intervention should be considered for eligible pediatric patients with FCD-DRE.
Background And Objectives:
Focal cortical dysplasia (FCD) is the most common cause of surgically treatable drug-resistant epilepsy (DRE) in children. Surgical outcomes are poorly defined in early-onset FCD-DRE. The purpose of this study was to evaluate clinical and presurgical characteristics relating to surgical outcomes in early-life (seizure onset <4 months old) FCD-DRE.
Methods:
A multicenter prospective cohort was analyzed from the Pediatric Epilepsy Research Consortium (PERC) Surgery Database to identify patients with pathologically confirmed FCD-DRE and seizure onset younger than 4 months old. Clinical characteristics, presurgical workup, and surgical intervention and outcomes data were collected and analyzed. Primary outcome was to evaluate whether earlier surgery is associated with seizure freedom; secondary outcomes evaluated clinical/presurgical predictors of seizure freedom and safety.
Results:
Thirty-one patients with FCD-DRE were identified from 18 PERC centers. Median age at seizure onset was 2.4 months (interquartile range 1.2-3.6 months). Four patients had focal to bilateral tonic-clonic seizures (FBTCS); 35% (n = 11) had epileptic spasms. Median age at phase 1 referral was 2.0 years (0.7-4.0 years). Median age at surgery was 2.6 years (1.1-5.5 years). Pathology was type II, 65% (n = 20); type I, 26% (n = 8); and type III, 6% (n = 2). Seizure freedom was achieved in 65% (n = 20) with median follow-up 2.5 years (1.3-4 years). Age at seizure onset/referral/surgery, surgery type, and experiencing FBTCS were not associated with seizure-free outcome. Type IIB pathology had 89% (n = 8) seizure-free outcome. Epileptic spasms had 45% seizure-free outcome. Transient neurologic deficits occurred in 2 patients, and an expected neurologic deficit in 1 (visual field cut from occipital lobectomy). There were no deaths.
Discussion:
This study finds high rates of seizure-free outcome in epilepsy surgery for early-onset FCD-DRE across all pathologies and procedures with minimal complication rates and no deaths. Focal cortical dysplasia type IIB is associated with very high rates of seizure-free outcome. Epileptic spasms were associated with lower seizure-free outcome. The study also fails to confirm a high rate of multilobar unilateral hypoplasia with severe epilepsy in children, a type I FCD variant that has been reported as a common etiology of early-life FCD.
