Outcomes of Early-Life Focal Cortical Dysplasia-Related Epilepsy: A PERC Surgery Study

Nathan T. Cohen, Center for Neuroscience, Children's National Hospital, The George Washington University School of Medicine, Washington, DC.
Dewi Frances Depositario-Cabacar, Center for Neuroscience, Children's National Hospital, The George Washington University School of Medicine, Washington, DC.
Chima O. Oluigbo, Center for Neuroscience, Children's National Hospital, The George Washington University School of Medicine, Washington, DC.
Adam P. Ostendorf, Nationwide Children's Hospital and the Ohio State University, Columbus, OH.
Lily Wong-Kisiel, Division of Epilepsy, Department of Neurology, Mayo Clinic, Rochester, MN.
Erin Fedak Romanowski, Michigan Medicine Pediatric Neurology, CS Mott Children's Hospital, Ann Arbor, MI.
Nancy A. McNamara, Michigan Medicine Pediatric Neurology, CS Mott Children's Hospital, Ann Arbor, MI.
Priyamvada Tatachar, Division of Neurology-Epilepsy Center, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, IL.
Krista Eschbach, Department of Pediatrics, Section of Neurology, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora.
Allyson L. Alexander, Department of Neurosurgery, Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora.
Pilar D. Pichon, Department of Neurology, Children's Hospital of Orange County, CA.
Michael A. Ciliberto, Child Neurology, Department of Pediatrics, University of Iowa, Iowa City.
Ernesto Gonzalez-Giraldo, Department of Neurology, Division of Epilepsy, University of California-San Francisco.
Danilo Bernardo, Department of Neurology, Division of Epilepsy, University of California-San Francisco.
Kurtis I. Auguste, University of California San Francisco Weill Institute for Neurosciences, Benioff Children's Hospital.
Jason Coryell, Department of Pediatrics, Oregon Health and Sciences University, Portland.
Kristen H. Arredondo, Department of Neurology, Dell Medical School, The University of Texas at Austin.
Edward John Novotny, Division of Pediatric Neurology, Departments of Neurology and Pediatrics, Seattle Children's Hospital, Neurosciences, University of Washington.
Shilpa B. Reddy, Division of Pediatric Neurology, Department of Pediatrics, Monroe Carell Jr. Children's Hospital at Vanderbilt, Vanderbilt University Medical Center, Nashville, TN.
Abhinaya Ganesh, Division of Pediatric Neurology, Department of Pediatrics, Monroe Carell Jr. Children's Hospital at Vanderbilt, Vanderbilt University Medical Center, Nashville, TN.
Ahmad Marashly, Epilepsy Division, Neurology Department, The Johns Hopkins University, Baltimore, MD.
Pradeep K. Javarayee, Division of Child Neurology, Department of Neurology, Medical College of Wisconsin, Milwaukee.
Rani K. Singh, Department of Pediatrics, Atrium Health-Levine Children's Hospital, Wake Forest University School of Medicine, Charlotte, NC.
Jeffrey Brian Bolton, Department of Neurology, Boston Children's, MA.
Zachary M. Grinspan, Department of Pediatrics, Department of Neurology Department of Population Health Sciences, Weill Cornell Medicine, New York, NY.
Samir Karia, Division of Child Neurology, Department of Pediatrics, Norton Children's Neuroscience Institute, University of Louisville, KY.
Cemal Karakas, Division of Child Neurology, Department of Pediatrics, Norton Children's Neuroscience Institute, University of Louisville, KY.
Jenny Lin, Department of Pediatric Neurology, Children's Healthcare of Atlanta, Emory University School of Medicine, GA.
Andrew T. Knox, Department of Neurology, University of Wisconsin School of Medicine and Public Health, Madison.
Steven M. Wolf, Boston Children's Health Physicians of New York and Connecticut, Maria Fareri Children's Hospital, New York Medical College, Valhalla.
Taylor J. Abel, Department of Neurological Surgery, Department of Bioengineering, University of Pittsburgh, PA.
Debopam Samanta, Child Neurology Division, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock.

Document Type

Journal Article

Publication Date

12-1-2025

Journal

Neurology. Clinical practice

Volume

15

Issue

6

DOI

10.1212/CPJ.0000000000200539

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.

Department

Neurology

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