| Intracerebral involvement in scleroderma en coup de sabre: report of a case with neuropathologic findings. | |
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MedLine Citation:
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PMID: 7755364 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Linear scleroderma en coup de sabre (LScs) is a rare disorder not infrequently associated with neurologic symptoms, notably epilepsy. However, histopathologic documentation of intracerebral lesions in LScs is very limited and the etiology of the central nervous system symptoms has therefore never been convincingly established. We describe a 27-year-old woman with LScs and a longstanding history of epilepsy. Radiographic studies demonstrated a focal, intraparenchymal lesion in the left frontal lobe directly subjacent to the area of scleroderma on the forehead and scalp. The resected cerebral lesion revealed localized band-like sclerosis of the leptomeninges and associated vessels, as well as intraparenchymal calcifications and anomalous, ectatic vessels. These findings suggest that LScs may represent a neurocutaneous syndrome of vascular dysplasia similar to the Sturge-Weber syndrome, rather than a localized form of collagen vascular disease, as suggested by some. |
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Authors:
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M H Chung; J Sum; M J Morrell; D S Horoupian |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Annals of neurology Volume: 37 ISSN: 0364-5134 ISO Abbreviation: Ann. Neurol. Publication Date: 1995 May |
Date Detail:
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Created Date: 1995-06-16 Completed Date: 1995-06-16 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 7707449 Medline TA: Ann Neurol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 679-81 Citation Subset: IM |
Affiliation:
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Division of Neuropathology, Stanford Comprehensive Epilepsy Center, Stanford University Medical Center, CA 94305-5324, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Brain / pathology Epilepsy / complications, pathology* Female Frontal Lobe / pathology Humans Magnetic Resonance Imaging Scleroderma, Localized / complications, pathology* |
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