| Scheie Syndrome Diagnosed After Cerebral Infarction. | |
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MedLine Citation:
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PMID: 21167740 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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We report a 41-year-old woman with Scheie syndrome diagnosed after cerebral infarction. She presented with acute onset dysarthria and right upper limb weakness. The neurologic findings revealed dysarthria, right central facial paralysis, mild right hemiparesis, and mild sensory impairment in the right arm and leg. Diffusion-weighted magnetic resonance imaging (MRI) showed subtle high signal lesions in the left corona radiata and posterior limb of the internal capsule. The diagnosis was made by a coarse facial appearance, claw hands, pigmentary degeneration of the bilateral retinas, and a deficiency of the enzymatic activity of lysosomal α-L-iduronidase. The patient was successfully treated with intravenous recombinant tissue plasminogen activator (rtPA) followed by enzyme replacement therapy. The prognosis of this disease would improve with enzyme replacement therapy. It is necessary to be aware of cerebral infarction in patients with Scheie syndrome. |
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Authors:
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Daiki Fujii; Yasuhiro Manabe; Tomotaka Tanaka; Syoichiro Kono; Yasuko Sakai; Hisashi Narai; Nobuhiko Omori; Mahoko Furujyo; Koji Abe |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2010-12-15 |
Journal Detail:
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Title: Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association Volume: - ISSN: 1532-8511 ISO Abbreviation: - Publication Date: 2010 Dec |
Date Detail:
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Created Date: 2010-12-20 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9111633 Medline TA: J Stroke Cerebrovasc Dis Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2010 National Stroke Association. Published by Elsevier Inc. All rights reserved. |
Affiliation:
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Department of Neurology, National Hospital Organization Okayama Medical Center, Okayama, Japan. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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