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Predictors of Cognitive Functions in Children With Sturge-Weber Syndrome: A Longitudinal Study

Edit Bosnyák1, Michael E Behen1, William C Guy1

  • 1Department of Pediatrics, Wayne State University and Children's Hospital of Michigan, Detroit, Michigan.

Pediatric Neurology
|June 30, 2016
PubMed

Insights

Cognitive function in children with Sturge-Weber syndrome varies greatly. Early EEG abnormalities and frequent seizures predict lower intelligence quotient (IQ) outcomes, highlighting a target for intervention.

Area of Science:

  • Neuroscience
  • Developmental Pediatrics
  • Cognitive Science

Background:

  • Sturge-Weber syndrome (SWS) often leads to seizures and cognitive decline.
  • Early brain plasticity may mitigate cognitive issues in unilateral SWS.
  • Mechanisms of plasticity in SWS are not well understood.

Purpose of the Study:

  • To evaluate longitudinal changes in intelligence quotient (IQ) in children with unilateral SWS.
  • To identify clinical, electroencephalography (EEG), and magnetic resonance imaging (MRI) predictors of IQ outcomes.
  • To understand the rules governing brain plasticity in SWS.

Main Methods:

  • 33 young children with unilateral SWS underwent serial MRI, scalp EEG, and neuropsychological evaluations over 2 years.
  • Longitudinal IQ changes were calculated.
  • Seizure variables, EEG abnormalities, and MRI findings were correlated with follow-up IQ.

Main Results:

  • Global IQ trajectories were highly variable, with both increases and decreases observed.
  • Lower baseline IQ predicted interval IQ increase.
  • Baseline EEG abnormalities, early seizure onset, high seizure frequency, and frontal lobe MRI involvement predicted lower outcome IQ.

Conclusions:

  • Cognitive development in unilateral SWS is highly variable.
  • Effective functional reorganization may lead to IQ improvement.
  • Early seizures and EEG abnormalities impede cognitive function, identifying a high-risk group for targeted interventions.
Abstract

Keywords:
EEGMRISturge–Weber syndromecognitive functionsepilepsylongitudinal study

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