Epilepsy surgery in children with tuberous sclerosis complex: presurgical evaluation and outcome.
OBJECTIVE
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.
Koh
S
S
Comprehensive Epilepsy Center, Miami Children's Hospital, Florida, USA.
Jayakar
P
P
Dunoyer
C
C
Whiting
S E
SE
Resnick
T J
TJ
Alvarez
L A
LA
Morrison
G
G
Ragheb
J
J
Prats
A
A
Dean
P
P
Gilman
J
J
Duchowny
M S
MS
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004827:Epilepsy / Q000601:surgery*; D006801:Humans; D007223:Infant; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000601:surgery*