Neuropsychological features and risk factors in children with Sturge-Weber syndrome: four case reports.

Mark McIntosh,'s picture
PubMed URL: 
http://www.ncbi.nlm.nih.gov/pubmed/20560093
Author: 
Comi AM
Author List: 
Zabel TA
Reesman J
Wodka EL
Gray R
Suskauer SJ
Turin E
Ferenc LM
Lin DD
Kossoff EH
Comi AM
Journal: 
Clin Neuropsychol
PubMed ID: 
20560093
Pagination: 
841-59
Volume: 
24
Issue: 
5
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.
Published Date: 
January, 2010

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