Outcomes of Early-Life Focal Cortical Dysplasia–Related Epilepsy

Nathan T. Cohen; Dewi Frances Depositario-Cabacar; Chima O. Oluigbo; Adam P. Ostendorf; Lily Wong-Kisiel; Erin Fedak Romanowski; Nancy A. McNamara; Priyamvada Tatachar; Krista Eschbach; Allyson L. Alexander; Pilar D. Pichon; Michael A. Ciliberto; Ernesto Gonzalez-Giraldo; Danilo Bernardo; Kurtis I. Auguste; Jason Coryell; Kristen H. Arredondo; Edward John Novotny; Shilpa B. Reddy; Abhinaya Ganesh; Ahmad Marashly; Pradeep K. Javarayee; Rani K. Singh; Jeffrey Brian Bolton; Zachary M. Grinspan; Samir Karia; Cemal Karakas; Jenny Lin; Andrew T. Knox; Steven M. Wolf; Taylor J. Abel; Debopam Samanta; Dallas Michael Armstrong; Spriha Pavuluri; Ann Hyslop; Fernando N. Galan; Derryl J. Miller; Jason S. Hauptman; Avery Robert Caraway; M. Scott Perry; William D. Gaillard

doi:10.1212/CPJ.0000000000200539

Outcomes of Early-Life Focal Cortical Dysplasia–Related Epilepsy

Nathan T. Cohen
, Dewi Frances Depositario-Cabacar
, Chima O. Oluigbo
, Adam P. Ostendorf
, Lily Wong-Kisiel
, Erin Fedak Romanowski
, Nancy A. McNamara
, Priyamvada Tatachar
, Krista Eschbach
, Allyson L. Alexander
, Pilar D. Pichon
, Michael A. Ciliberto
, Ernesto Gonzalez-Giraldo
, Danilo Bernardo
, Kurtis I. Auguste
, Jason Coryell
, Kristen H. Arredondo
, Edward John Novotny
, Shilpa B. Reddy
, Abhinaya Ganesh

Ahmad Marashly, Pradeep K. Javarayee, Rani K. Singh, Jeffrey Brian Bolton, Zachary M. Grinspan, Samir Karia, Cemal Karakas, Jenny Lin, Andrew T. Knox, Steven M. Wolf, Taylor J. Abel, Debopam Samanta, Dallas Michael Armstrong, Spriha Pavuluri, Ann Hyslop, Fernando N. Galan, Derryl J. Miller, Jason S. Hauptman, Avery Robert Caraway, M. Scott Perry, William D. Gaillard

Research output: Contribution to journal › Article › peer-review

6 Scopus citations

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.

Original language	English
Journal	Neurology: Clinical Practice
Volume	15
Issue number	6
DOIs	https://doi.org/10.1212/CPJ.0000000000200539
State	Published - 2025
Externally published	Yes

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10.1212/CPJ.0000000000200539

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Cohen, N. T., Depositario-Cabacar, D. F., Oluigbo, C. O., Ostendorf, A. P., Wong-Kisiel, L., Fedak Romanowski, E., McNamara, N. A., Tatachar, P., Eschbach, K., Alexander, A. L., Pichon, P. D., Ciliberto, M. A., Gonzalez-Giraldo, E., Bernardo, D., Auguste, K. I., Coryell, J., Arredondo, K. H., Novotny, E. J., Reddy, S. B., ... Gaillard, W. D. (2025). Outcomes of Early-Life Focal Cortical Dysplasia–Related Epilepsy. Neurology: Clinical Practice, 15(6). https://doi.org/10.1212/CPJ.0000000000200539

@article{c1a18e159b2a4711a8e4ee7e1a0a7e86,

title = "Outcomes of Early-Life Focal Cortical Dysplasia{\textendash}Related Epilepsy",

abstract = "Background and Objectives {\textendash} 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 {\textendash} 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 {\textendash} 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{\textendash}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{\textendash}4.0 years). Median age at surgery was 2.6 years (1.1{\textendash}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 {\textendash} 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 = "Cohen, \{Nathan T.\} and Depositario-Cabacar, \{Dewi Frances\} and Oluigbo, \{Chima O.\} and Ostendorf, \{Adam P.\} and Lily Wong-Kisiel and \{Fedak Romanowski\}, Erin and McNamara, \{Nancy A.\} and Priyamvada Tatachar and Krista Eschbach and Alexander, \{Allyson L.\} and Pichon, \{Pilar D.\} and Ciliberto, \{Michael A.\} and Ernesto Gonzalez-Giraldo and Danilo Bernardo and Auguste, \{Kurtis I.\} and Jason Coryell and Arredondo, \{Kristen H.\} and Novotny, \{Edward John\} and Reddy, \{Shilpa B.\} and Abhinaya Ganesh and Ahmad Marashly and Javarayee, \{Pradeep K.\} and Singh, \{Rani K.\} and Bolton, \{Jeffrey Brian\} and Grinspan, \{Zachary M.\} and Samir Karia and Cemal Karakas and Jenny Lin and Knox, \{Andrew T.\} and Wolf, \{Steven M.\} and Abel, \{Taylor J.\} and Debopam Samanta and Armstrong, \{Dallas Michael\} and Spriha Pavuluri and Ann Hyslop and Galan, \{Fernando N.\} and Miller, \{Derryl J.\} and Hauptman, \{Jason S.\} and Caraway, \{Avery Robert\} and Perry, \{M. Scott\} and Gaillard, \{William D.\}",

note = "Publisher Copyright: {\textcopyright} 2025 American Academy of Neurology",

year = "2025",

doi = "10.1212/CPJ.0000000000200539",

language = "English",

volume = "15",

journal = "Neurology: Clinical Practice",

issn = "2163-0402",

publisher = "Lippincott Williams and Wilkins",

number = "6",

}

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 2025, 'Outcomes of Early-Life Focal Cortical Dysplasia–Related Epilepsy', Neurology: Clinical Practice, vol. 15, no. 6. https://doi.org/10.1212/CPJ.0000000000200539

TY - JOUR

T1 - Outcomes of Early-Life Focal Cortical Dysplasia–Related Epilepsy

AU - Cohen, Nathan T.

AU - Depositario-Cabacar, Dewi Frances

AU - Oluigbo, Chima O.

AU - Ostendorf, Adam P.

AU - Wong-Kisiel, Lily

AU - Fedak Romanowski, Erin

AU - McNamara, Nancy A.

AU - Tatachar, Priyamvada

AU - Eschbach, Krista

AU - Alexander, Allyson L.

AU - Pichon, Pilar D.

AU - Ciliberto, Michael A.

AU - Gonzalez-Giraldo, Ernesto

AU - Bernardo, Danilo

AU - Auguste, Kurtis I.

AU - Coryell, Jason

AU - Arredondo, Kristen H.

AU - Novotny, Edward John

AU - Reddy, Shilpa B.

AU - Ganesh, Abhinaya

AU - Marashly, Ahmad

AU - Javarayee, Pradeep K.

AU - Singh, Rani K.

AU - Bolton, Jeffrey Brian

AU - Grinspan, Zachary M.

AU - Karia, Samir

AU - Karakas, Cemal

AU - Lin, Jenny

AU - Knox, Andrew T.

AU - Wolf, Steven M.

AU - Abel, Taylor J.

AU - Samanta, Debopam

AU - Armstrong, Dallas Michael

AU - Pavuluri, Spriha

AU - Hyslop, Ann

AU - Galan, Fernando N.

AU - Miller, Derryl J.

AU - Hauptman, Jason S.

AU - Caraway, Avery Robert

AU - Perry, M. Scott

AU - Gaillard, William D.

PY - 2025

Y1 - 2025

N2 - 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.

AB - 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.

UR - https://www.scopus.com/pages/publications/105028394146

U2 - 10.1212/CPJ.0000000000200539

DO - 10.1212/CPJ.0000000000200539

M3 - Article

AN - SCOPUS:105028394146

SN - 2163-0402

VL - 15

JO - Neurology: Clinical Practice

JF - Neurology: Clinical Practice

IS - 6

ER -

Outcomes of Early-Life Focal Cortical Dysplasia–Related Epilepsy

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