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Journal of Child Neurology
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0883073808329529v1
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Article

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

Jennifer Reesman, PhD1, Robert Gray, PhD, ABPP-CN2, Stacy J. Suskauer, MD3, Lisa M. Ferenc, MA4, Eric H. Kossoff, MD3, Doris D. M. Lin, MD, PhD3, Elizabeth Turin, MD5, Anne M. Comi, MD5, Patrick J. Brice, PhD1, and T. Andrew Zabel, PhD, ABPP-CN5*

1 Gallaudet University, Washington, D.C.
2 Advanced Neurobehavioral Health of Southern California, California
3 Johns Hopkins University School of Medicine, Baltimore, Maryland
4 Kennedy Krieger Institute, Baltimore, Maryland
5 Kennedy Krieger Institute, and Johns Hopkins University School of Medicine, Baltimore, Maryland

* To whom correspondence should be addressed. E-mail: zabela{at}kennedykrieger.org.


  Abstract

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.

First published on April 8, 2009, doi:10.1177/0883073808329529

Journal of Child Neurology 2009;24:701.

A more recent version of this article appeared on June 1, 2009

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