Neuropsychological Features and Risk Factors in Children With Sturge-Weber Syndrome: Four Case Reports

作者：Zabel T Andrew^*; Reesman Jennifer; Wodka Ericka L; Gray Robert; Suskauer Stacy J; Turin Elizabeth; Ferenc Lisa M; Lin Doris D M; Kossoff Eric H; Comi Anne M

来源：The Clinical Neuropsychologist, 2010, 24(5): 841-859.

DOI：10.1080/13854046.2010.485133

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

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 otypicalo 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.

• 出版日期2010