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[Formula: see text]Intellectual and adaptive functioning in Sturge-Weber Syndrome
Brian Kavanaugh1, Aditya Sreenivasan2, Catherine Bachur2
1Department of Behavioral Psychology, Kennedy Krieger Institute.
Summary
Children and young adults with Sturge-Weber Syndrome (SWS) show significantly impaired intellectual and adaptive functioning. Neurological factors like seizures and hemiparesis impact these abilities, with younger individuals functioning better.
Area of Science:
- Neuroscience
- Developmental Psychology
- Clinical Psychology
Background:
- Sturge-Weber Syndrome (SWS) is a rare neurological disorder.
- Intellectual and adaptive functioning are critical aspects of development and quality of life.
Purpose of the Study:
- To assess intellectual and adaptive functioning in individuals with SWS.
- To identify predictors of cognitive and adaptive deficits in SWS.
- To evaluate a composite neurological status measure for screening.
Main Methods:
- Neuropsychological evaluations were conducted on 29 participants with SWS.
- Intellectual and adaptive functioning were assessed using standardized measures.
- Neurological status, including seizure frequency and hemiparesis, was recorded.
Main Results:
- The SWS sample showed significantly lower intellectual and adaptive functioning compared to normative data.
- Impaired intellectual functioning was observed in 32% and adaptive functioning in 58% of the sample.
- Seizure frequency predicted intellectual functioning, while hemiparesis predicted adaptive functioning.
Conclusions:
- Individuals with SWS experience significant challenges in intellectual and adaptive domains.
- Neurological factors play a crucial role in cognitive and adaptive outcomes.
- A composite neurological status measure shows potential for identifying individuals needing targeted support.
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