Brain damage and IQ in unilateral Sturge-Weber syndrome: support for a "fresh start" hypothesis

Epilepsy Behav. 2011 Oct;22(2):352-7. doi: 10.1016/j.yebeh.2011.07.010.


We tested the hypothesis that extent of severe hypometabolism measured by fluorodeoxyglucose PET has a U-shaped (nonlinear) relationship to IQ in children with unilateral Sturge-Weber syndrome. Thirty-five consecutive children (age range: 30-153 months) with Sturge-Weber syndrome and unilateral brain involvement were enrolled in the study. Participants underwent cognitive assessment and interictal fluorodeoxyglucose PET scans. Regression analyses tested whether a quadratic model best accounted for the relationship between extent of severe cortical hypometabolism and IQ, controlling for seizure variables. A significant quadratic relationship was found between IQ and extent of severe (but not total) hypometabolism. Seizure variables also contributed significant variance to cognitive functions. Results suggest that intermediate size of severe hemispheric hypometabolism is associated with the worst cognitive outcomes, and small or absent lesions, with the best cognitive outcomes. Children in whom a very large extent of the hemisphere is severely affected are likely to have relatively preserved cognitive function.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Brain / diagnostic imaging
  • Brain / pathology
  • Brain Injuries / diagnostic imaging
  • Brain Injuries / etiology*
  • Brain Injuries / pathology
  • Brain Mapping
  • Child
  • Child, Preschool
  • Cognition Disorders / diagnosis
  • Cognition Disorders / etiology
  • Female
  • Fluorodeoxyglucose F18
  • Functional Laterality*
  • Humans
  • Intelligence*
  • Magnetic Resonance Imaging
  • Male
  • Neuropsychological Tests
  • Positron-Emission Tomography
  • Seizures / diagnosis
  • Seizures / etiology
  • Statistics, Nonparametric
  • Sturge-Weber Syndrome / complications*
  • Sturge-Weber Syndrome / diagnostic imaging
  • Sturge-Weber Syndrome / pathology
  • Sturge-Weber Syndrome / psychology*


  • Fluorodeoxyglucose F18