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EEG evolution in Sturge-Weber syndrome.

Eric H Kossoff1, Catherine D Bachur2, Angela M Quain3

  • 1The Johns Hopkins Hospital, United States.

Epilepsy Research
|February 25, 2014
PubMed
Summary

Electroencephalogram (EEG) findings in Sturge-Weber syndrome (SWS) evolve over time, becoming more abnormal with age. However, these EEG changes do not correlate with neurologic function or seizure frequency in children with SWS.

Keywords:
AsymmetryChildrenEEGEpilepsySturge-Weber

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Area of Science:

  • Neurology
  • Pediatrics
  • Medical Imaging

Background:

  • The electroencephalogram (EEG) in Sturge-Weber syndrome (SWS) was theorized to change over time from normal to focal asymmetry, and finally to epileptiform activity.
  • Previous studies suggested an evolution of EEG patterns in SWS, but validation in larger cohorts was needed.

Purpose of the Study:

  • To retrospectively validate the hypothesized temporal evolution of EEG abnormalities in a larger cohort of children with SWS.
  • To correlate EEG scores with patient age, neurologic function, and seizure frequency.

Main Methods:

  • Retrospective evaluation of 81 EEGs from 44 pediatric patients with confirmed SWS.
  • Development of an EEG scoring system (0-3) to quantify abnormality (normal, focal slowing, focal sharp waves, frequent spike-wave bursts).
  • Correlation analysis of EEG scores with patient age, neurologic neuroscore, and seizure subscore.

Main Results:

  • Patients with more abnormal EEG scores (2-3) were significantly older (mean age 8.7 years) than those with less abnormal scores (0-1, mean age 3.2 years) (p=0.006).
  • EEG abnormalities in SWS do appear to progress over time, with increasing epileptiform activity.
  • No significant correlation was found between EEG scores and overall neurologic function or seizure frequency.

Conclusions:

  • The study confirms that EEG findings in SWS evolve over time, becoming more abnormal with age.
  • Despite the progression of EEG abnormalities, this evolution does not correlate with the child's neurological status or seizure frequency.
  • These findings support the long-standing theory of EEG changes in SWS and provide quantitative data from a larger cohort.