Neuropsychological features and risk factors in children with sturge-weber syndrome: Four case reports

T. Andrew Zabel, Jennifer Reesman, Ericka L. Wodka, Robert Gray, Stacy J. Suskauer, Elizabeth Turin, Lisa M. Ferenc, Doris D.M. Lin, Eric H. Kossoff, Anne M. Comi

Research output: Contribution to journalArticle

Abstract

Sturge-Weber Syndrome (SWS) is a rare neurocutaneous disorder involving facial capillary malformation (port-wine birthmark) and vascular malformation of the brain that is frequently associated with epilepsy, stroke-like episodes, cognitive deficits, motor impairment, and/or visual field cut. The four cases presented here (ages 8-9, two females) illustrate the broad range of physiologic involvement and associated neuropsychological functioning in SWS, and argue against the idea of a typical SWS neuropsychological presentation. Rather, we highlight a preliminary collection of disease status/severity factors thought to impact neuropsychological presentation in SWS, including degree of cortical involvement (unilateral versus bilateral; posterior only versus posterior/anterior), age at time of seizure onset, extent of seizure control, history of stroke-like episodes, and magnitude of neurologic decline/deficit. We discuss the need for broad-based assessment in this medical population, as various impairment combinations (e.g., perceptual, language, executive) create unique presentations as well as the need for individualized intervention.

Original languageEnglish (US)
Pages (from-to)841-859
Number of pages19
JournalClinical Neuropsychologist
Volume24
Issue number5
DOIs
StatePublished - Jul 1 2010

    Fingerprint

Keywords

  • Cognitive
  • Epilepsy
  • Neuropsychology
  • Pediatric
  • Sturge-Weber

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Developmental and Educational Psychology
  • Clinical Psychology
  • Arts and Humanities (miscellaneous)
  • Psychiatry and Mental health

Cite this