[Formula: see text]Intellectual and adaptive functioning in Sturge-Weber Syndrome. Child Neuropsychol 2016;22(6):635-648
Date
05/09/2015Pubmed ID
25952468Pubmed Central ID
PMC4868126DOI
10.1080/09297049.2015.1028349Scopus ID
2-s2.0-84929011807 (requires institutional sign-in at Scopus site) 14 CitationsAbstract
The present study examined the intellectual and adaptive functioning in a sample of children and young adults with Sturge-Weber Syndrome (SWS). A total of 80 research participants from a SWS study database underwent full neurological evaluation as part of their participation or concurrent medical care. Twenty-nine of the participants received neuropsychological evaluations. Analyses indicated no significant demographic or neurological differences between those who did and did not receive neuropsychological evaluations. Overall, the neuropsychological evaluation sample displayed significantly lower functioning relative to published normative data across domains of intellectual and adaptive functioning. Thirty-two percent of the sample displayed impaired performance (standard score ≤ 75) in intellectual functioning and 58% displayed impaired performance in adaptive functioning. Hemiparesis status independently predicted overall adaptive functioning while seizure frequency independently predicted overall intellectual functioning. Younger participants displayed significantly higher (more intact) ratings in adaptive functioning compared to older participants, specifically in overall adaptive functioning, motor skills, and community living skills. A composite measure of neurological status (SWS-NRS) incorporating seizure and hemiparesis status effectively distinguished between individuals with impaired or nonimpaired adaptive and intellectual functioning and showed promise as a screening method for identifying individuals with more involved intellectual and/or adaptive needs.
Author List
Kavanaugh B, Sreenivasan A, Bachur C, Papazoglou A, Comi A, Zabel TAAuthor
Catherine Bachur MD, MA Maternal Fetal Medicine Fellow in the Obstetrics and Gynecology department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
ChildFemale
Humans
Male
Neurologic Examination
Neuropsychological Tests
Sturge-Weber Syndrome