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Epilepsy surgery in children with tuberous sclerosis complex: presurgical evaluation and outcome
1Comprehensive Epilepsy Center, Miami Children's Hospital, Florida, USA.
Insights
Focal resection of the epileptogenic tuber/region (ET/R) in children with tuberous sclerosis complex (TSC) significantly reduces seizures. Noninvasive studies like EEG and MRI effectively localize the ET/R for successful surgical intervention.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neurosurgery
Background:
- Tuberous sclerosis complex (TSC) is a genetic disorder associated with epilepsy.
- Surgical resection of the epileptogenic tuber/region (ET/R) can be effective for intractable seizures in children with TSC.
- Accurate localization of the ET/R is crucial for successful surgical outcomes.
Purpose of the Study:
- To evaluate the role of noninvasive studies in localizing the ET/R in children with TSC.
- To assess the outcomes of focal resection guided by these localization methods.
Main Methods:
- Twenty-one children with TSC underwent video-electroencephalogram (EEG) and magnetic resonance imaging (MRI).
- Ictal single photon emission-computed tomography (SPECT) was performed in 18 patients.
- Localization of the ET/R was achieved in 17 patients, with 13 undergoing surgical resection.
Main Results:
- Scalp EEG identified a principal spike focus (PSF) corresponding to the ET/R in 14 children.
- MRI revealed tubers, with the ET/R correlating to large discrete or calcified tubers in most cases.
- Post-surgery, 9 of 13 children were seizure-free, with significant seizure reduction in others.
Conclusions:
- Surgical resection of the ET/R is effective in alleviating intractable epilepsy in most children with TSC.
- Scalp EEG and MRI are valuable tools for defining the ET/R and guiding surgical selection.
- These noninvasive methods improve case selection, especially when ictal SPECT is nonlocalizing.
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.