| The cutaneous manifestations of Sturge-Weber syndrome. | |
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MedLine Citation:
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PMID: 6226707 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The Sturge-Weber syndrome is characterized by the presence of a leptomeningeal hemangioma variably associated with a facial nevus flammeus, seizure disorder, mental retardation, hemiparesis, homonymous hemianopsia, glaucoma, and other signs and symptoms. The relationship between the cutaneous manifestations and the other features of this disorder was studied in 35 patients. We observed that all patients with the facial nevus flammeus had involvement of the upper eye lid or forehead. Bilateral cutaneous involvement was common and when present, was often associated with extensive lesions of the trunk and extremities. The distribution of the cutaneous lesions appeared to be unrelated to that of the trigeminal nerve. A subgroup of patients, the syndrome's "forme fruste," were identified. There appeared to be no relationship between the presence and extent of the cutaneous lesions and the observed seizure disorder, difficulty of seizure control, intellectual deficit, hemiparesis, homonymous hemianopsia, and intracranial calcification. Notably, the "forme fruste" patients appeared to be spared from glaucomatous involvement and from choroidal hemangioma. We will also discuss other aspects of this syndrome. |
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Authors:
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M Uram; C Zubillaga |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of clinical neuro-ophthalmology Volume: 2 ISSN: 0272-846X ISO Abbreviation: J Clin Neuroophthalmol Publication Date: 1982 Dec |
Date Detail:
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Created Date: 1983-12-20 Completed Date: 1983-12-20 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 8109051 Medline TA: J Clin Neuroophthalmol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 245-8 Citation Subset: IM |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Angiomatosis / diagnosis* Brain Diseases / diagnosis Calcinosis / diagnosis Child Child, Preschool Eye Diseases / diagnosis Female Hemangioma / diagnosis* Humans Infant Infant, Newborn Male Nevus / diagnosis* Seizures / diagnosis Skin Neoplasms / diagnosis* Sturge-Weber Syndrome / classification, diagnosis* |
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