Hemiparesis is a clinical correlate of general adaptive dysfunction in children and adolescents with Sturge-Weber syndrome

This study sought to identify neurologic correlates of adaptive functioning in individuals with Sturge-Weber syndrome. A total of 18 children, adolescents, and young adults with Sturge-Weber syndrome with brain involvement were recruited from our Sturge-Weber center. All underwent neurologic examina...

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Published inJournal of child neurology Vol. 24; no. 6; p. 701
Main Authors Reesman, Jennifer, Gray, Robert, Suskauer, Stacy J, Ferenc, Lisa M, Kossoff, Eric H, Lin, Doris D M, Turin, Elizabeth, Comi, Anne M, Brice, Patrick J, Zabel, T Andrew
Format Journal Article
LanguageEnglish
Published United States 01.06.2009
Subjects
Adaptation, Psychological
Adolescent
Brain - pathology
Child
Child, Preschool
Cognition Disorders
Female
Functional Laterality
Humans
Intelligence Tests
Magnetic Resonance Imaging
Male
Neuropsychological Tests
Paresis - pathology
Paresis - psychology
Pilot Projects
Severity of Illness Index
Sturge-Weber Syndrome - pathology
Sturge-Weber Syndrome - psychology
Young Adult
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Summary:This study sought to identify neurologic correlates of adaptive functioning in individuals with Sturge-Weber syndrome. A total of 18 children, adolescents, and young adults with Sturge-Weber syndrome with brain involvement were recruited from our Sturge-Weber center. All underwent neurologic examination (including review of clinical brain magnetic resonance imaging) and neuropsychological assessment. Neuropsychological assessment included measures of intellectual ability and standardized parent report of adaptive functioning. Overall, Full Scale IQ and ratings of global adaptive functioning were both lower than the population-based norms (P < .05). Negative correlations were identified between adaptive functioning ratings, clinician ratings of cortical abnormality, and ratings of neurologic status. Hemiparesis (minimal versus prominent) was the only individual component of the rating scales that differentiated between individuals with nonimpaired and impaired adaptive functioning scores. Information obtained during neurological examination of children and adolescents with Sturge-Weber syndrome particularly hemiparetic status is useful for identifying children who may need additional intervention.
ISSN:1708-8283
DOI:10.1177/0883073808329529