Faculty Collaboration Database

Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex. Seizure 2006 Oct;15(7):558-62

Date

08/29/2006

Pubmed ID

16935530

DOI

10.1016/j.seizure.2006.06.010

Scopus ID

2-s2.0-33748060502 (requires institutional sign-in at Scopus site)   51 Citations

Abstract

In patients with tuberous sclerosis complex (TSC), the high rates of mental retardation are associated with cortical tubers, seizure activity, and genetic factors. The goal of the study was to investigate the relationship between bilateral cortical tubers and seizure variables and mental retardation in individuals with TSC. The records of 27 patients with TSC (age 6 months to 33 years) undergoing neuropsychological assessment and the following clinical variables were examined: bilateral versus non-bilateral cortical tubers, the age of seizure onset, and presence of infantile spasms. Results were statistically analyzed. Bilateral cortical tubers (p=0.02) and early age of seizure onset (p=0.04) were significantly related to impaired cognitive functioning. Only one of the seven patients with normal cognitive functioning had bilateral tubers, whereas 13/21 patients with intellectual impairment had bilateral tubers. Patients with normal cognitive functioning experienced a mean age of seizure onset after 6 years. A trend was observed between infantile spasms and cognitive functioning (p=0.06); the lack of statistical significance likely reflects the small sample size. Neither age nor gender was related to cognitive status. Further investigation incorporating additional neuroimaging factors, antiepileptic treatment effects, and genetic variables, is needed.

Author List

Zaroff CM, Barr WB, Carlson C, LaJoie J, Madhavan D, Miles DK, Nass R, Devinsky O

Author

Chad Carlson MD Professor in the Neurology department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adolescent
Adult
Child
Child, Preschool
Cognition Disorders
Epilepsy
Female
Humans
Infant
Intellectual Disability
Intelligence
Male
Neuropsychological Tests
Retrospective Studies
Spasms, Infantile
Tuberous Sclerosis