Outcomes of Early-Life Focal Cortical Dysplasia–Related Epilepsy

Authors

• Nathan T. Cohen, The George Washington University School of Medicine and Health Sciences
• Dewi Frances Depositario-Cabacar, The George Washington University School of Medicine and Health Sciences
• Chima O. Oluigbo, The George Washington University School of Medicine and Health Sciences
• Adam P. Ostendorf, Nationwide Children’s Hospital
• Lily Wong-Kisiel, Mayo Clinic
• Erin Fedak Romanowski, C.S. Mott Children's Hospital
• Nancy A. McNamara, C.S. Mott Children's Hospital
• Priyamvada Tatachar, Northwestern University Feinberg School of Medicine
• Krista Eschbach, University of Colorado Anschutz Department of Pediatrics
• Allyson L. Alexander, University of Colorado Anschutz School of Medicine
• Pilar D. Pichon, CHOC Children‘s UC Irvine School of Medicine
• Michael A. Ciliberto, University of Iowa Carver College of Medicine
• Ernesto Gonzalez-Giraldo, University of California, San Francisco
• Danilo Bernardo, University of California, San Francisco
• Kurtis I. Auguste, UCSF Benioff Children‘s Hospital
• Jason Coryell, Oregon Health & Science University
• Kristen H. Arredondo, Dell Medical School
• Edward John Novotny, University of Washington
• Shilpa B. Reddy, Monroe Carell Jr. Children's Hospital at Vanderbilt
• Abhinaya Ganesh, Monroe Carell Jr. Children's Hospital at Vanderbilt
• Ahmad Marashly, Johns Hopkins University
• Pradeep K. Javarayee, Medical College of Wisconsin
• Rani K. Singh, Wake Forest University School of Medicine
• Jeffrey Brian Bolton, Massachusetts General Hospital
• Zachary M. Grinspan, Weill Cornell Medicine
• Samir Karia, University of Louisville School of Medicine
• Cemal Karakas, University of Louisville School of Medicine
• Jenny Lin, Emory University School of Medicine
• Andrew T. Knox, University of Wisconsin School of Medicine and Public Health
• Steven M. Wolf, New York Medical College
• Taylor J. Abel, Swanson School of Engineering
• Debopam Samanta, UAMS College of Medicine

DOI

10.1212/CPJ.0000000000200539

Journal Title

Neurology Clinical Practice

Document Type

Article

Publication Date

1-1-2025

Department

Pediatrics

Disciplines

Medicine and Health Sciences

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.

Recommended Citation

Cohen, N., Depositario-Cabacar, D., Oluigbo, C., Ostendorf, A., Wong-Kisiel, L., Fedak Romanowski, E., McNamara, N., Tatachar, P., Eschbach, K., Alexander, A., Pichon, P., Ciliberto, M., Gonzalez-Giraldo, E., Bernardo, D., Auguste, K., Coryell, J., Arredondo, K., Novotny, E., Reddy, S., Ganesh, A., Marashly, A., Javarayee, P., Singh, R., Bolton, J., Grinspan, Z., Karia, S., Karakas, C., Lin, J., Knox, A., Wolf, S., Abel, T., & Samanta, D. (2025). Outcomes of Early-Life Focal Cortical Dysplasia–Related Epilepsy. Neurology Clinical Practice, 15 (6). https://doi.org/10.1212/CPJ.0000000000200539