| Radiologic and neuropathologic findings in patients in a family with dentatorubral-pallidoluysian atrophy. | |
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MedLine Citation:
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PMID: 20966051 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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SUMMARY: We describe the cases of 2 patients, a father and his son, with DRPLA who underwent MR examinations prior to death and in whom postmortem examinations of the brain were obtained. MR imaging findings had the following features: 1) atrophy of the cerebellum and brain stem were the common findings, 2) high-signal-intensity lesions in the cerebral white matter and brain stem were observed on T2-weighted images in the patient with adult-onset DRPLA, 3) signal-intensity changes in the cerebral white matter were restricted to the periventricular white matter in the patient with juvenile-onset DRPLA, but these changes appear in the advanced stage, and 4) progressive cerebral atrophy was more marked in the patient with juvenile-onset DRPLA. In the patients with DRPLA, the abnormal high signal intensity of the cerebral white matter or brain stem on MR images reflect the loss of myelinated fibers. Cerebral atrophy mainly reflects atrophy of the neuropile. |
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Authors:
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Y Sunami; R Koide; N Arai; M Yamada; T Mizutani; K Oyanagi |
Publication Detail:
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Type: Journal Article Date: 2010-10-21 |
Journal Detail:
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Title: AJNR. American journal of neuroradiology Volume: 32 ISSN: 1936-959X ISO Abbreviation: AJNR Am J Neuroradiol Publication Date: 2011 Jan |
Date Detail:
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Created Date: 2011-01-14 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8003708 Medline TA: AJNR Am J Neuroradiol Country: United States |
Other Details:
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Languages: eng Pagination: 109-14 Citation Subset: IM |
Affiliation:
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Departments of Neurology. |
Export Citation:
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Descriptor/Qualifier:
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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