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Neuropsychological features and risk factors in children with Sturge-Weber syndrome: four case reports.
|Title||Neuropsychological features and risk factors in children with Sturge-Weber syndrome: four case reports.|
|Publication Type||Journal Article|
|Year of Publication||2010|
|Authors||Zabel AT, Reesman J, Wodka EL, Gray R, Suskauer SJ, Turin E, Ferenc LM, Lin DDM, Kossoff EH, Comi AM|
|Journal||The Clinical neuropsychologist|
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.
|Alternate Journal||Clin Neuropsychol|