| Neuroimaging findings in a brain with niemann-pick type C disease. | |
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MedLine Citation:
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PMID: 21783023 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Niemann-Pick type C disease (NPC) is a rare autosomal recessive lipid storage disorder caused by impaired cellular functions in processing and transporting low-density lipoprotein-cholesterol. In this report, we present magnetic resonance imaging (MRI), magnetic resonance spectrography (MRS) and 18-fluoro-2-deoxyglucose positron emission tomography (PET) imaging results for a 22-year-old male NPC patient. The patient's two MRI studies (at age 19 years and 22 years) demonstrated progressive changes of brain atrophy that were more prominent at the frontal lobes, and hyperintense signals in bilateral parietal-occipital periventricular white matter. MRS (at age 19 years) revealed no significant decrease in N-acetyl aspartate/choline ratio in the left frontal central white matter. PET (at age 22 years) showed significant bilateral hypometabolism in the prefrontal cortex and dorsomedial thalamus, and hypermetabolism in the parietal-occipital white matter, lenticular nucleus of the basal ganglia, cerebellum and pons. The imaging findings noted by MRI, MRS and 18-fluoro-2-deoxyglucose PET offered a possible supplementary explanation for the clinical neurological symptoms of this NPC patient. |
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Authors:
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Jei-Yie Huang; Sing-Fung Peng; Chih-Chao Yang; Kuo-Yang Yen; Kai-Yuan Tzen; Ruoh-Fang Yen |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of the Formosan Medical Association = Taiwan yi zhi Volume: 110 ISSN: 0929-6646 ISO Abbreviation: J. Formos. Med. Assoc. Publication Date: 2011 Aug |
Date Detail:
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Created Date: 2011-07-25 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9214933 Medline TA: J Formos Med Assoc Country: China (Republic : 1949- ) |
Other Details:
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Languages: eng Pagination: 537-42 Citation Subset: IM |
Copyright Information:
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Copyright © 2011 Formosan Medical Association & Elsevier. Published by Elsevier B.V. All rights reserved. |
Affiliation:
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Department of Nuclear Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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