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

Susan Koh, Prasanna Jayakar, Catalina Dunoyer, Sharon E. Whiting, Trevor J. Resnick, Luis A. Alvarez, Glenn Morrison, John Ragheb, Antonio Prats, Patricia Dean, Samson B. Antel, Jamie Gilman, Michael S. Duchowny

Research output: Contribution to journalArticle

166 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1206-1213
Number of pages8
JournalEpilepsia
Volume41
Issue number9
DOIs
StatePublished - Jan 1 2000

    Fingerprint

Keywords

  • Childhood epilepsy
  • Epilepsy surgery
  • Tuberous sclerosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Koh, S., Jayakar, P., Dunoyer, C., Whiting, S. E., Resnick, T. J., Alvarez, L. A., Morrison, G., Ragheb, J., Prats, A., Dean, P., Antel, S. B., Gilman, J., & Duchowny, M. S. (2000). Epilepsy surgery in children with tuberous sclerosis complex: Presurgical evaluation and outcome. Epilepsia, 41(9), 1206-1213. https://doi.org/10.1111/j.1528-1157.2000.tb00327.x