NYMC Faculty Publications

Epilepsy Surgery in Children With Genetic Etiologies: A Prospective Evaluation of Current Practices and Outcomes

Jason Coryell, Department of Pediatrics, Oregon Health & Sciences University, CDRC-P, 707 SW Gaines Rd, Portland, OR 97239, USA. Electronic address: coryellj@ohsu.edu.
Rani Singh, Division of Neurology, Department of Pediatrics, Atrium Health/Levine Children's Hospital, Charlotte, NC, USA.
Adam P. Ostendorf, Department of Pediatrics, Nationwide Children's Hospital, Ohio State University, Columbus, OH, USA.
Mariah Eisner, Biostatistics Resource at Nationwide Children's Hospital, Columbus, Ohio, USA.
Allyson Alexander, Department of Neurosurgery, University of Colorado Anschutz Medical Campus, Aurora, CO, USA; Division of Pediatric Neurosurgery, Children's Hospital Colorado, Aurora, CO, USA.Follow
Krista Eschbach, Department of Neurology, Children's Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Daniel W. Shrey, Children's Hospital of Orange County, Orange, CA, USA.
Joffre Olaya, Children's Hospital of Orange County, Orange, CA, USA.
Michael A. Ciliberto, Department of Pediatrics, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.
Cemal Karakas, Department of Neurology, Division of Child Neurology, Norton Children's Hospital, University of Louisville School of Medicine, Louisville, KY, USA.
Samir Karia, Department of Neurology, Division of Child Neurology, Norton Children's Hospital, University of Louisville School of Medicine, Louisville, KY, USA.
Nancy McNamara, Department of Pediatrics, Section of Pediatric Neurology, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA.
Erin Fedak Romanowski, Department of Pediatrics, Section of Pediatric Neurology, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA.
Ammar Kheder, Department of Pediatrics, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, GA, USA.
Javarayee Pradeep, Department of Pediatric Neurology, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI, USA.
Shilpa B. Reddy, Department of Pediatrics, Vanderbilt University Medical Center, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN, USA.
Michael J. McCormack, Department of Pediatrics, Vanderbilt University Medical Center, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN, USA.
Jeffrey Bolton, Department of Neurology, Boston Children's Hospital, Boston, MA, USA.
Steven Wolf, Boston Children's Health Physicians of New York and Connecticut, Maria Fareri Children's Hospital, New York Medical College, Valhalla, NY, USA.
Patricia McGoldrick, Boston Children's Health Physicians of New York and Connecticut, Maria Fareri Children's Hospital, New York Medical College, Valhalla, NY, USA.
Jason S. Hauptman, Division of Pediatric Neurosurgery, University of Washington/Seattle Children's Hospital, Seattle, WA, USA.
Debopam Samanta, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
Priya Tatachar, Department of Pediatrics, Ann and Robert H Lurie Children's Hospital, Chicago, IL, USA.
Joseph Sullivan, University of California San Francisco Weill Institute for Neurosciences, Benioff Children's Hospital, San Francisco, CA, USA.
Kurtis Auguste, University of California San Francisco Weill Institute for Neurosciences, Benioff Children's Hospital, San Francisco, CA, USA.Follow
Ernesto Gonzalez-Giraldo, University of California San Francisco Weill Institute for Neurosciences, Benioff Children's Hospital, San Francisco, CA, USA.
Ahmad Marashly, Department of Neurology, Johns Hopkins, Baltimore, MD, USA.
Dewi F. Depositario-Cabacar, Center for Neuroscience, Children's National Hospital, George Washington University School of Medicine, Washington, DC, USA.
Lily C. Wong-Kisiel, Department of Neurology, Divisions of Child Neurology and Epilepsy, Mayo Clinic College of Medicine, Rochester, MN, USA.
Scott Perry, Jane and John Justin Institute for Mind Health, Cook Children's Medical Center, Fort Worth, TX, USA.

Author Type(s)

Faculty

DOI

10.1016/j.seizure.2023.10.011

Journal Title

Seizure

First Page

6

Last Page

12

Document Type

Article

Publication Date

12-1-2023

Department

Pediatrics

Abstract

OBJECTIVE: This study assesses current practices and outcomes of epilepsy surgery in children with a genetic etiology. It explores the pre-surgical workup, types of surgeries, and post-surgical outcomes in a broad array of disorders. METHODS: Patients ≤18 years who completed epilepsy surgery and had a known genetic etiology prior to surgical intervention were extrapolated from the Pediatric Epilepsy Research Consortium (PERC) surgery database, across 18 US centers. Data were assessed univariably by neuroimaging and EEG results, genetic group (structural gene, other gene, chromosomal), and curative intent. Outcomes were based on a modified International League Against Epilepsy (ILAE) outcome score. RESULTS: Of 81 children with genetic epilepsy, 72 % had daily seizures when referred for surgery evaluation, which occurred a median of 2.2 years (IQR 0.3, 5.2) after developing drug resistance. Following surgery, 68 % of subjects had >50 % seizure reduction, with 33 % achieving seizure freedom [median follow-up 11 months (IQR 6, 17). Seizure freedom was most common in the monogenic structural group, but significant palliation was present across all groups. Presence of a single EEG focus was associated with a greater likelihood of seizure freedom (p=0.02). SIGNIFICANCE: There are meaningful seizure reductions following epilepsy surgery in the majority of children with a genetic etiology, even in the absence of a single structural lesion and across a broad spectrum of genetic causes. These findings highlight the need for expedited referral for epilepsy surgery and support of a broadened view of which children may benefit from epilepsy surgery, even when the intent is palliative.

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