Outcomes of Early-Life Focal Cortical Dysplasia-Related Epilepsy: A PERC Surgery Study
Neurology Clinical Practice Vol. 15 No. 6 December 2025
AUTHORS
Nathan T Cohen 1, Dewi Frances Depositario-Cabacar 1, Chima O Oluigbo 1, Adam P Ostendorf 2, Lily Wong-Kisiel 3, Erin Fedak Romanowski 4, Nancy A McNamara 4, Priyamvada Tatachar 5, Krista Eschbach 6, Allyson L Alexander 7, Pilar D Pichon 8, Michael A Ciliberto 9, Ernesto Gonzalez-Giraldo 10, Danilo Bernardo 10, Kurtis I Auguste 11, Jason Coryell 12, Kristen H Arredondo 13, Edward John Novotny 14, Shilpa B Reddy 15, Abhinaya Ganesh 15, Ahmad Marashly 16, Pradeep K Javarayee 17, Rani K Singh 18, Jeffrey Brian Bolton 19, Zachary M Grinspan 20, Samir Karia 21, Cemal Karakas 21, Jenny Lin 22, Andrew T Knox 23, Steven M Wolf 24, Taylor J Abel 25, Debopam Samanta 26, Dallas Michael Armstrong 27, Spriha Pavuluri 28, Ann Hyslop 29, Fernando N Galan 30, Derryl J Miller 31, Jason S Hauptman 32, Avery Robert Caraway 33, M Scott Perry 33, William D Gaillard 1
ABSTRACT
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.