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

TitleNeuropsychological features and risk factors in children with Sturge-Weber syndrome: four case reports.
Publication TypeJournal Article
Year of Publication2010
AuthorsZabel AT, Reesman J, Wodka EL, Gray R, Suskauer SJ, Turin E, Ferenc LM, Lin DDM, Kossoff EH, Comi AM
JournalThe Clinical neuropsychologist
Volume24
Issue5
Pagination841-59
Date Published2010
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.

DOI10.1016/j.neuroimage.2010.06.058
Alternate JournalClin Neuropsychol