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Epilepsy Surgery in Children with Tuberous Sclerosis Complex: Presurgical Evaluation and Outcome
Author(s) -
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.
Publication year - 2000
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/j.1528-1157.2000.tb00327.x
Subject(s) - tuberous sclerosis , ictal , epilepsy surgery , magnetic resonance imaging , epilepsy , electrocorticography , medicine , ictal interictal spect analysis by spm , electroencephalography , nuclear medicine , radiology , surgery , psychiatry
Summary: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.