Limited Utility of Structural MRI to Identify the Epileptogenic Zone in Young Children With Tuberous Sclerosis

Authors

Maaike Nijman
Edward Yang
Camilo Jaimes
Anna K Prohl
Mustafa Sahin
Darcy A Krueger
Joyce Y Wu
Hope Northrup
Scellig S D Stone
Joseph R Madsen
Aria Fallah
Jeffrey P Blount
Howard L Weiner
Leslie Grayson
E Martina Bebin
Brenda E Porter
Simon K Warfield
Sanjay P Prabhu
Jurriaan M Peters
TACERN Study Group

Publication Date

9-1-2022

Journal

Journal of Neuroimaging

DOI

10.1111/jon.13016

PMID

35729081

PMCID

PMC11267633

PubMedCentral® Posted Date

7-24-2024

PubMedCentral® Full Text Version

Author MSS

Published Open-Access

yes

Keywords

Child, Child, Preschool, Electroencephalography, Epilepsy, Humans, Magnetic Resonance Imaging, Malformations of Cortical Development, Retrospective Studies, Treatment Outcome, Tuberous Sclerosis, children, epilepsy, epilepsy surgery, MRI, tuberous sclerosis complex

Abstract

BACKGROUND AND PURPOSE: The success of epilepsy surgery in children with tuberous sclerosis complex (TSC) hinges on identification of the epileptogenic zone (EZ). We studied structural MRI markers of epileptogenic lesions in young children with TSC.

METHODS: We included 26 children with TSC who underwent epilepsy surgery before the age of 3 years at five sites, with 12 months or more follow-up. Two neuroradiologists, blinded to surgical outcome data, reviewed 10 candidate lesions on preoperative MRI for characteristics of the tuber (large affected area, calcification, cyst-like properties) and of focal cortical dysplasia (FCD) features (cortical malformation, gray-white matter junction blurring, transmantle sign). They selected lesions suspect for the EZ based on structural MRI, and reselected after unblinding to seizure onset location on electroencephalography (EEG).

RESULTS: None of the tuber characteristics and FCD features were distinctive for the EZ, indicated by resected lesions in seizure-free children. With structural MRI alone, the EZ was identified out of 10 lesions in 31%, and with addition of EEG data, this increased to 48%. However, rates of identification of resected lesions in non-seizure-free children were similar. Across 251 lesions, interrater agreement was moderate for large size (κ = .60), and fair (κ = .24) for all other features.

CONCLUSIONS: In young children with TSC, the utility of structural MRI features is limited in the identification of the epileptogenic tuber, but improves when combined with EEG data.