| Neuropsychological features and risk factors in children with Sturge-Weber syndrome: four case reports. | |
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MedLine Citation:
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PMID: 20560093 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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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:
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Type: Case Reports; Journal Article Date: 2010-06-14 |
Journal Detail:
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Title: The Clinical neuropsychologist Volume: 24 ISSN: 1744-4144 ISO Abbreviation: Clin Neuropsychol Publication Date: 2010 |
Date Detail:
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Created Date: 2010-06-28 Completed Date: 2010-11-10 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8806548 Medline TA: Clin Neuropsychol Country: England |
Other Details:
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Languages: eng Pagination: 841-59 Citation Subset: IM |
Affiliation:
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Department of Neuropsychology, Kennedy Krieger Institute, Baltimore, MD 21231, USA. zabela@kennedykrieger.org |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Cerebral Cortex
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abnormalities* Child Epilepsy Female Headache Disorders, Primary Humans Magnetic Resonance Imaging Male Neurologic Examination Neuropsychological Tests Port-Wine Stain Risk Factors Sturge-Weber Syndrome / diagnosis, physiopathology*, psychology* |
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