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Outcomes of Early-Life Focal Cortical Dysplasia-Related Epilepsy: A PERC Surgery Study. Neurol Clin Pract 2025 Dec;15(6):e200539

Date

10/10/2025

Pubmed ID

41070137

Pubmed Central ID

PMC12507445

DOI

10.1212/CPJ.0000000000200539

Scopus ID

2-s2.0-105028394146 (requires institutional sign-in at Scopus site)   1 Citation

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.

Author List

Cohen NT, Depositario-Cabacar DF, Oluigbo CO, Ostendorf AP, Wong-Kisiel L, Fedak Romanowski E, McNamara NA, Tatachar P, Eschbach K, Alexander AL, Pichon PD, Ciliberto MA, Gonzalez-Giraldo E, Bernardo D, Auguste KI, Coryell J, Arredondo KH, Novotny EJ, Reddy SB, Ganesh A, Marashly A, Javarayee PK, Singh RK, Bolton JB, Grinspan ZM, Karia S, Karakas C, Lin J, Knox AT, Wolf SM, Abel TJ, Samanta D, Armstrong DM, Pavuluri S, Hyslop A, Galan FN, Miller DJ, Hauptman JS, Caraway AR, Perry MS, Gaillard WD

Author

Pradeep Javarayee MBBS Assistant Professor in the Neurology department at Medical College of Wisconsin