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 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.
Kennedy Krieger Institute, Baltimore, Maryland 21231, USA.
Jennifer
J
Reesman
Robert
R
Gray
Stacy J
SJ
Suskauer
Lisa M
LM
Ferenc
Eric H
EH
Kossoff
Doris D M
DD
Lin
Elizabeth
E
Turin
Anne M
AM
Comi
Patrick J
PJ
Brice
T Andrew
TA
Zabel
D000223:Adaptation, Psychological; D000293:Adolescent; D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders; D005260:Female; D007839:Functional Laterality; D006801:Humans; D007361:Intelligence Tests; D008279:Magnetic Resonance Imaging; D008297:Male; D009483:Neuropsychological Tests; D010291:Paresis / Q000473:pathology / Q000523:psychology*; D010865:Pilot Projects; D012720:Severity of Illness Index; D013341:Sturge-Weber Syndrome / Q000473:pathology / Q000523:psychology*; D055815:Young Adult