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Cerebral tuber count and its impact on mental outcome of patients with tuberous sclerosis complex
Magdalena Kaczorowska1, Elżbieta Jurkiewicz, Dorota Domańska-Pakieła
1Department of Neurology Radiology, MR Unit Pediatric Rehabilitation Psychology, The Children's Memorial Health Institute, Warsaw, Poland.
Purpose:
The aim of the study was to reveal the relationships between the tuber count of the brain found in patients with tuberous sclerosis complex (TSC) and their cognitive outcome.
Methods:
A single-center, retrospective analysis was performed of patients with documented TSC seen from 1988 to 2010 at the Children's Memorial Health Institute, Warsaw, Poland.
Key Findings:
Sixty-two patients were analyzed, and there was a significant correlation between younger age at the first seizure and developmental delay. The patients who did not develop seizures had normal development, despite some presenting with higher tuber load than those with seizures. There was a statistically significant negative correlation between the number of tubers within the right temporal lobe and cognition.
Significance:
Our findings confirm our hypothesis that the cognitive outcome in TSC is more dependent on the age of the seizure onset rather than on the tuber count.
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