Use of quantitative EEG in infants with port-wine birthmark to assess for Sturge-Weber brain involvement
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Joshua B Ewen1, Eric H Kossoff, Nathan E Crone
1Department of Neurology and Developmental Medicine, Kennedy Krieger Institute, Baltimore, MD 21205, USA. Ewen@kennedykrieger.org
Insights
Quantitative EEG (qEEG) can accurately predict Sturge-Weber syndrome (SWS) brain involvement in infants with facial port-wine birthmarks. This non-invasive method shows promise for early identification and management of SWS.
Area of Science:
- Neurology
- Pediatrics
- Medical Imaging
Background:
- Facial port-wine birthmarks can indicate Sturge-Weber syndrome (SWS), a condition affecting the brain.
- Previous research linked asymmetric quantitative EEG (qEEG) to SWS severity in older patients.
- Early identification of SWS brain involvement in infants is crucial for timely intervention.
Purpose of the Study:
- To evaluate the efficacy of qEEG in predicting SWS brain involvement in infants with facial port-wine birthmarks.
- To determine if qEEG can differentiate infants with SWS brain involvement from those without.
Main Methods:
- An observational study involving infants with facial port-wine birthmarks.
- Recording standard clinical EEGs and deriving an asymmetry measure.
- Validating the qEEG asymmetry threshold in a separate cohort.
Main Results:
- Quantitative EEG correctly identified SWS brain involvement in all infants in the validation cohort.
- The qEEG method demonstrated performance comparable to experienced pediatric electroencephalographers.
- This technique shows high accuracy in differentiating SWS brain involvement.
Conclusions:
- Quantitative EEG is a reliable tool for discriminating between infants with and without SWS brain involvement.
- This qEEG technique represents a significant advancement in predicting SWS development in infants with port-wine birthmarks.
Objective:
Many infants born with a facial port-wine (PW) birthmark will not develop brain involvement of Sturge-Weber syndrome (SWS). Previous studies have shown asymmetry in quantitative EEG (qEEG) correlates with degree of clinical impairment in children and adults with known SWS. We hope to determine if quantitative qEEG can be used as a method to predict which infants are most likely to develop SWS brain involvement on MRI. The current study looks at the ability of qEEG to differentiate between infants with radiographically demonstrated SWS and those without.
Methods:
We first performed an observational study of qEEG results on eight infants with facial PW birthmark (four had SWS brain involvement). We recorded standard clinical EEGs and then derived a measure of asymmetry. We subsequently validated this threshold through a study of an additional nine infants with PW birthmark (five with SWS brain involvement).
Results:
Quantitative EEG correctly identified infants with SWS brain involvement in all cases in the Validation cohort. This technique was at least as good as a pediatric electroencephalographer with extensive experience reading SWS EEGs.
Conclusions:
This study demonstrates the ability for qEEG to discriminate between those infants with SWS brain involvement and those with neurologically asymptomatic PW birthmark.
Significance:
This study represents an important step toward the development of a qEEG technique able to predict which infants with PW birthmark will develop SWS brain involvement.
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