Epilepsy Surgery in Children with Tuberous Sclerosis Complex: Presurgical Evaluation and Outcome

• Published in: Epilepsia (Copenhagen) 2000-09, Vol.41 (9), p.1206-1213
• Main Authors: Koh, Susan, Jayakar, Prasanna, Dunoyer, Catalina, Whiting, Sharon E., Resnick, Trevor J., Alvarez, Luis A., Morrison, Glenn, Ragheb, John, Prats, Antonio, Dean, Patricia, Antel, Samson B., Gilman, Jamie, Duchowny, Michael S.
• Format: Article
• Language: English
• Subjects: Adolescent; Biological and medical sciences; Child; Child, Preschool; Childhood epilepsy; Epilepsy - surgery; Humans; Infant; Medical sciences; Neurology; Treatment Outcome; Tuberous sclerosis; Tuberous Sclerosis - surgery; Tumors of the nervous system. Phacomatoses
• ISSN: 0013-9580; 1528-1167
• DOI: 10.1111/j.1528-1157.2000.tb00327.x

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.