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Hemiparesis Is a Clinical Correlate of General Adaptive Dysfunction in Children and Adolescents with Sturge-Weber Syndrome

Kennedy Krieger Institute, Baltimore, Maryland, Gallaudet University, Washington, D.C.

Robert Gray, PhD, ABPP-CN

Advanced Neurobehavioral Health of Southern California, California

Stacy J. Suskauer, MD

Johns Hopkins University School of Medicine, Baltimore, Maryland, Kennedy Krieger Institute, Baltimore, Maryland

Lisa M. Ferenc, MA

Kennedy Krieger Institute, Baltimore, Maryland

Eric H. Kossoff, MD

Johns Hopkins University School of Medicine, Baltimore, Maryland

Doris D. M. Lin, MD, PhD

Johns Hopkins University School of Medicine, Baltimore, Maryland

Elizabeth Turin, MD

Johns Hopkins University School of Medicine, Baltimore, Maryland, Kennedy Krieger Institute, Baltimore, Maryland

Anne M. Comi, MD

Kennedy Krieger Institute, Baltimore, Maryland, Johns Hopkins University School of Medicine, Baltimore, Maryland

Patrick J. Brice, PhD

Gallaudet University, Washington, D.C.

T. Andrew Zabel, PhD, ABPP-CN

Kennedy Krieger Institute, Baltimore, Maryland, zabela{at}kennedykrieger.org, Johns Hopkins University School of Medicine, Baltimore, Maryland

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.

Key Words: Sturge-Weber syndrome • adaptive functioning • activities of daily living • hemiparesis • neuropsychology

This version was published on June 1, 2009

Journal of Child Neurology, Vol. 24, No. 6, 701-708 (2009)
DOI: 10.1177/0883073808329529


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