Epilepsy surgery in children with tuberous sclerosis complex: presurgical evaluation and outcome.

Abstract

PURPOSE:

Children with tuberous sclerosis complex (TSC) benefit from excisional surgery if seizures can be localized to a single tuber. We evaluated the role of noninvasive studies to localize the epileptogenic tuber/region (ET/R) and the outcome of focal resection.

METHODS:

We identified 21 children with TSC, ages 3 months to 15 years (mean 4.8 years). All had video-(electroencephalogram) EEG and magnetic resonance imaging (MRI) scans, and 18 also had ictal single photon emission-computed tomography (SPECT) studies. An ET/R was localized in 17 patients. Thirteen patients underwent resection guided by intraoperative electrocorticography (n = 7) or subdural monitoring (n = 6).

RESULTS:

Interictal EEG revealed a principal spike focus (PSF) that corresponded to the ET/R in 14 children. In seven, PSFs occurred in rhythmic runs. PSFs were not observed remote from the ET/R. Focal polymorphic slowing and attenuation occurred in the region of the PSF in 11 patients. Sixteen patients demonstrated an ictal focus corresponding to the ET/R. Ictal SPECT revealed focal hyperperfusion correlating with the ET/R in 10 patients. Although the MRIs in all children revealed multiple tubers, the ET/R corresponded to a large discrete tuber in 8 patients and a calcified tuber in 13 patients. Patchy calcified tubers were also seen elsewhere in six patients. At a mean follow-up of 26 months, 9 of the 13 children who underwent surgery were seizure-free, one had greater than 75% reduction in seizures, two were unchanged, and one was lost to follow-up. New seizures developed in one child from a contralateral tuber.

CONCLUSIONS:

Surgical resection of an ET/R alleviates seizures in most children with TSC and intractable epilepsy. The scalp EEG and MRI help define the ET/R and improve case selection when ictal SPECT is nonlocalizing.

• Authors: S Koh, P Jayakar, C Dunoyer, S E Whiting, T J Resnick, L A Alvarez, G Morrison, J Ragheb, A Prats, P Dean, J Gilman, M S Duchowny
• Journal: Epilepsia (Epilepsia) Vol. 41 Issue 9 Pg. 1206-13 (Sep 2000) ISSN: 0013-9580 [Print] United States
• PMID: 10999561 (Publication Type: Journal Article)

Topics

• Adolescent
• Child
• Child, Preschool
• Epilepsy (surgery)
• Humans
• Infant
• Treatment Outcome
• Tuberous Sclerosis (surgery)