Cortical Tubers, Cognition, and Epilepsy in Tuberous Sclerosis

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...

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Published in:Pediatric neurology 2011-05, Vol.44 (5), p.328-332
Main Authors: Kassiri, Janani, PhD, MD, Snyder, Thomas J., PhD, Bhargava, Ravi, MD, Wheatley, B. Matt, PhD, MD, Sinclair, D. Barry, MD
Format: Article
Language:English
Subjects:
Biological and medical sciences
Cerebral Cortex - abnormalities
Cerebral Cortex - pathology
Child
Child, Preschool
Cognition Disorders - etiology
Electroencephalography - methods
Epilepsy - etiology
Female
Functional Laterality
Humans
Infant
Intelligence Tests
Magnetic Resonance Imaging
Male
Medical sciences
Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis
Neurology
Neuropsychological Tests
Pediatrics
Retrospective Studies
Statistics as Topic
Tuberous Sclerosis - complications
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Summary: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.
ISSN:0887-8994
1873-5150
DOI:10.1016/j.pediatrneurol.2011.01.001