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From the Rudolf Magnus Institute of Neuroscience, Departments of Child Neurology (F.E.J., O.v.N.), Neurology (F.E.J., A.A.), Neuropsychology (O.B., A.J.-S.), and Image Sciences Institute (K.L.V., P.A.), Department of Clinical Epidemiology (A.A.), Julius Centre for Health Sciences and Primary Care, Departments of Internal Medicine (B.A.Z.), TSC Clinic and Clinical Neurophysiology (A.C.v.H.), University Medical Centre, Utrecht, Department of Clinical Genetics (M.N., A.v.d.O., D.H.), Erasmus Medical Centre, Rotterdam, the Netherlands.
* To whom correspondence should be addressed. E-mail: f.e.jansen{at}umcutrecht.nl.
ABSTRACT
Objective: In patients with tuberous sclerosis complex (TSC), associations between tuber number, infantile spasms, and cognitive impairment have been proposed. We hypothesized that the tuber/brain proportion (TBP), the proportion of the total brain volume occupied by tubers, would be a better determinant of seizures and cognitive function than the number of tubers. We investigated tuber load, seizures, and cognitive function and their relationships.
Methods: Tuber number and TBP were characterized on three-dimensional fluid-attenuated inversion recovery MRI with an automated tuber segmentation program. Seizure histories and EEG recordings were obtained. Intelligence equivalents were determined and an individual cognition index (a marker of cognition that incorporated multiple cognitive domains) was calculated.
Results: In our sample of 61 patients with TSC, TBP was inversely related to the age at seizure onset and to the intelligence equivalent and tended to be inversely related to the cognition index. Further, a younger age at seizure onset or a history of infantile spasms was related to lower intelligence and lower cognition index. In a multivariable analysis, only age at seizure onset and cognition index were related.
Conclusions: Our systematic analysis confirms proposed relationships between tuber load, epilepsy and cognitive function in tuberous sclerosis complex (TSC), but also indicates that tuber/brain proportion is a better predictor of cognitive function than tuber number and that age at seizure onset is the only independent determinant of cognitive function. Seizure control should be the principal neurointervention in patients with TSC.
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