Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex

Charles M. Zaroff, William B. Barr, Chad Carlson, Josiane LaJoie, Deepak Madhavan, Daniel K. Miles, Ruth Nass, Orrin Devinsky

Research output: Contribution to journalArticle

42 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)558-562
Number of pages5
JournalSeizure
Volume15
Issue number7
DOIs
StatePublished - Oct 1 2006

Fingerprint

Tuberous Sclerosis
Intellectual Disability
Seizures
Age of Onset
Infantile Spasms
Neuroimaging
Anticonvulsants
Sample Size

Keywords

  • Autism
  • Bilateral
  • Epilepsy
  • Infantile spasms
  • Mental retardation
  • Neuropsychology
  • Tuberous sclerosis complex
  • Tubers

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Zaroff, C. M., Barr, W. B., Carlson, C., LaJoie, J., Madhavan, D., Miles, D. K., ... Devinsky, O. (2006). Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex. Seizure, 15(7), 558-562. https://doi.org/10.1016/j.seizure.2006.06.010

Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex. / Zaroff, Charles M.; Barr, William B.; Carlson, Chad; LaJoie, Josiane; Madhavan, Deepak; Miles, Daniel K.; Nass, Ruth; Devinsky, Orrin.

In: Seizure, Vol. 15, No. 7, 01.10.2006, p. 558-562.

Research output: Contribution to journalArticle

Zaroff, CM, Barr, WB, Carlson, C, LaJoie, J, Madhavan, D, Miles, DK, Nass, R & Devinsky, O 2006, 'Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex', Seizure, vol. 15, no. 7, pp. 558-562. https://doi.org/10.1016/j.seizure.2006.06.010
Zaroff CM, Barr WB, Carlson C, LaJoie J, Madhavan D, Miles DK et al. Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex. Seizure. 2006 Oct 1;15(7):558-562. https://doi.org/10.1016/j.seizure.2006.06.010
Zaroff, Charles M. ; Barr, William B. ; Carlson, Chad ; LaJoie, Josiane ; Madhavan, Deepak ; Miles, Daniel K. ; Nass, Ruth ; Devinsky, Orrin. / Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex. In: Seizure. 2006 ; Vol. 15, No. 7. pp. 558-562.
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