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

Jennifer Reesman, Robert Gray, Stacy Jennifer M Suskauer, Lisa M. Ferenc, Eric H Kossoff, Doris Lin, Elizabeth Turin, Anne Marie Spalding Comi, Patrick J. Brice, Theodore A Zabel

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)701-708
Number of pages8
JournalJournal of Child Neurology
Volume24
Issue number6
DOIs
StatePublished - 2009

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Keywords

  • Activities of daily living
  • Adaptive functioning
  • Hemiparesis
  • Neuropsychology
  • Sturge-Weber syndrome

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health

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