Document Detail

Neuropsychological features and risk factors in children with Sturge-Weber syndrome: four case reports.
MedLine Citation:
PMID:  20560093     Owner:  NLM     Status:  MEDLINE    
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.
T Andrew Zabel; Jennifer Reesman; Ericka L Wodka; Robert Gray; Stacy J Suskauer; Elizabeth Turin; Lisa M Ferenc; Doris D M Lin; Eric H Kossoff; Anne M Comi
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-06-14
Journal Detail:
Title:  The Clinical neuropsychologist     Volume:  24     ISSN:  1744-4144     ISO Abbreviation:  Clin Neuropsychol     Publication Date:  2010  
Date Detail:
Created Date:  2010-06-28     Completed Date:  2010-11-10     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8806548     Medline TA:  Clin Neuropsychol     Country:  England    
Other Details:
Languages:  eng     Pagination:  841-59     Citation Subset:  IM    
Department of Neuropsychology, Kennedy Krieger Institute, Baltimore, MD 21231, USA.
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MeSH Terms
Cerebral Cortex / abnormalities*
Headache Disorders, Primary
Magnetic Resonance Imaging
Neurologic Examination
Neuropsychological Tests
Port-Wine Stain
Risk Factors
Sturge-Weber Syndrome / diagnosis,  physiopathology*,  psychology*

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