Cortical Tubers, Cognition, and Epilepsy in Tuberous Sclerosis

Comprehensive Epilepsy Program, University of Alberta Hospital, Edmonton, Alberta, Canada.
Pediatric Neurology (Impact Factor: 1.7). 05/2011; 44(5):328-32. DOI: 10.1016/j.pediatrneurol.2011.01.001
Source: PubMed


Tuberous sclerosis complex is an autosomal-dominant genetic disorder characterized by hamartomatous growth in various organs. Patients who have this disorder exhibit a high rate of epilepsy and cognitive problems. We investigated number of tubers, location, seizure types, and cognitive outcome, and we analyzed the relationships among them in our tuberous sclerosis patients in the Comprehensive Epilepsy Program at the University of Alberta. We also examined the seizure outcome after tuber resection. Our study cohort included 24 patients with tuberous sclerosis complex. We obtained seizure history, electroencephalogram, and neuropsychologic parameters. Magnetic resonance imaging was used to examine tuber numbers and locations. Ten patients underwent surgical removal of tubers responsible for intractable epilepsy. A negative correlation was found between the number of tubers and intelligent quotient score. Epilepsy surgery led to freedom from seizures in this patient group. We demonstrated that the total number and location of cortical tubers play a significant role in the extent of mental retardation in patients with tuberous sclerosis complex. In addition, patients with intractable seizures and well-defined epileptic focus had excellent surgical outcome.

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    • "Cortical tubers are mostly supratentorial, in the frontal lobes, although infratentorial cerebellar tubers have been described as well.[25] The number and location of these tubers is associated with the degree of cerebral dysfunction including cognitive impairment, seizures, and autism.[26–28] There have been attempts to describe clinical severity based on MRI imaging characteristics [T1, T2, and fluid attenuated inversion recovery (FLAIR) signal] of cortical tubers,[29] but larger studies are needed to establish reliable criteria. "
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