Neuropsychological features and risk factors in children with Sturge-Weber syndrome: four case reports.
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.
Zabel
T Andrew
TA
Department of Neuropsychology, Kennedy Krieger Institute, Baltimore, MD 21231, USA. zabela@kennedykrieger.org
Reesman
Jennifer
J
Wodka
Ericka L
EL
Gray
Robert
R
Suskauer
Stacy J
SJ
Turin
Elizabeth
E
Ferenc
Lisa M
LM
Lin
Doris D M
DD
Kossoff
Eric H
EH
Comi
Anne M
AM
D002540:Cerebral Cortex / Q000002:abnormalities*; D002648:Child; D004827:Epilepsy; D005260:Female; D051270:Headache Disorders, Primary; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D019339:Port-Wine Stain; D012307:Risk Factors; D013341:Sturge-Weber Syndrome / Q000175:diagnosis / Q000503:physiopathology* / Q000523:psychology*