| High prevalence of bihemispheric structural and functional defects in Sturge-Weber syndrome. | |
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MedLine Citation:
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PMID: 9881530 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Abnormal cerebral venous drainage is associated with hypoxia and glucose deprivation, which can account for progressive neurologic deterioration in Sturge-Weber syndrome. Although developmental delay is common in Sturge-Weber syndrome, bihemispheric calcification is uncommon. Computed tomography (CT) and magnetic resonance imaging (MRI) were used to study the neuroanatomy, while single photon emission computed tomography (SPECT) was used concurrently to evaluate perfusion and glucose metabolism using 99mTc hexamethylpropyleneamine oxime (HMPAO) and [18F] fluorodeoxyglucose (FDG), respectively. Ten patients (10 to 22 years of age) with previously diagnosed Sturge-Weber syndrome, port-wine nevi, and clinical evidence of seizures or stroke-like episodes were studied. Five children with onset of seizures in the first year of life had overall clinical severity comparable to that of children with later-onset seizures. Calcification was present in both hemispheres in one patient; six additional patients had other radiologic evidence of bihemispheric disease; SPECT studies detected bihemispheric disease in four cases. Our study is the first to concurrently evaluate structure, perfusion, and glucose metabolism in Sturge-Weber syndrome and to show a mismatch between functional and structural brain imaging in both cerebral hemispheres. Widespread abnormalities of cerebral perfusion and glucose metabolism might explain the high prevalence of developmental delay associated with Sturge-Weber syndrome. Longitudinal studies are needed to define better the natural history of neurologic deterioration and radiologic progression that relates to central nervous system circulatory dysfunction in Sturge-Weber syndrome. |
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Authors:
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B L Maria; J A Neufeld; L C Rosainz; K Ben-David; W E Drane; R G Quisling; L M Hamed |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Journal of child neurology Volume: 13 ISSN: 0883-0738 ISO Abbreviation: J. Child Neurol. Publication Date: 1998 Dec |
Date Detail:
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Created Date: 1999-03-17 Completed Date: 1999-03-17 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 8606714 Medline TA: J Child Neurol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 595-605 Citation Subset: IM |
Affiliation:
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Department of Pediatrics, University of Florida College of Medicine, Gainesville, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Brain / blood supply Brain Diseases / pathology*, radiography Calcinosis / physiopathology* Child Female Fluorodeoxyglucose F18 / diagnostic use Glucose / metabolism* Humans Magnetic Resonance Imaging Male Radiopharmaceuticals / diagnostic use Seizures / physiopathology Sturge-Weber Syndrome / complications, physiopathology* Tomography, Emission-Computed, Single-Photon |
| Chemical | |
Reg. No./Substance:
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0/Radiopharmaceuticals; 50-99-7/Glucose; 63503-12-8/Fluorodeoxyglucose F18 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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