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Familial Creutzfeldt-Jakob disease with a V180I mutation: comparative analysis with pathological findings and diffusion-weighted images.
http://hdl.handle.net/10069/23204
http://hdl.handle.net/10069/23204087d5613-ceff-462b-bba8-dbd7646a8fa4
名前 / ファイル | ライセンス | アクション |
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DGCD28_500.pdf (368.2 kB)
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Item type | 学術雑誌論文 / Journal Article(1) | |||||
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公開日 | 2010-06-16 | |||||
タイトル | ||||||
タイトル | Familial Creutzfeldt-Jakob disease with a V180I mutation: comparative analysis with pathological findings and diffusion-weighted images. | |||||
言語 | ||||||
言語 | eng | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Creutzfeldt-Jakob disease | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Diffusion-weighted imaging | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Magnetic resonance spectroscopy | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Single-photon emission computed tomography | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | Brain biopsy | |||||
資源タイプ | ||||||
資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
資源タイプ | journal article | |||||
著者 |
Mutsukura, Kazuo
× Mutsukura, Kazuo× Satoh, Katsuya× Shirabe, Susumu× Tomita, Itsuro× Fukutome, Takayasu× Morikawa, Minoru× Iseki, Masachika× Sasaki, Kensuke× Shiaga, Yusei× Kitamoto, Tetsuyuki× Eguchi, Katsumi |
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抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | BACKGROUND: Diffusion-weighted imaging (DWI) has been reported to be a useful technique for diagnosing Creutzfeldt-Jakob disease (CJD). The present study reported DWI results in cases of familial CJD with a V180I mutation (CJD180) in the prion protein gene as well as neurological findings. METHODS: A retrospective analysis of 3 patients with V180I was performed. Cerebrospinal fluid (CSF) analysis, brain MRI, single-photon emission computed tomography (SPECT), and magnetic resonance spectroscopy (MRS) were included. CSF was analyzed for biochemical markers, and each patient underwent brain MRI, SPECT, and MRS analysis. A brain biopsy from the frontal cortex, which corresponded to the area of increased DWI signals, was utilized for neuropathological analysis. RESULTS: CSF analysis results revealed elevated total tau protein and the absence of 14-3-3 protein, as well as decreased concentrations of neuron-specific enolase, S100 protein, and prostaglandin E(2). All patients presented with unique MRI features. Brain biopsy showed severe spongiform morphology, but comparatively preserved neurons and mild astrocytic gliosis. Accumulations of PrP(Sc) were not detected using the 3F4 antibody, and microglial activation was subtle. SPECT revealed hypoperfusion throughout both hemispheres. MRS revealed a reduced N-acetyl aspartate/creatine ratio. CONCLUSION: Results from this study suggested that increased DWI signals could reflect severe spongiform changes in CJD180 patients. | |||||
書誌情報 |
Dementia and geriatric cognitive disorders 巻 28, 号 6, p. 550-557, 発行日 2009-12 |
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出版者 | ||||||
出版者 | S. Karger AG | |||||
ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 14208008 | |||||
EISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 14219824 | |||||
書誌レコードID | ||||||
収録物識別子タイプ | NCID | |||||
収録物識別子 | AA11113172 | |||||
PubMed番号 | ||||||
関連タイプ | isVersionOf | |||||
識別子タイプ | PMID | |||||
関連識別子 | 20051687 | |||||
DOI | ||||||
関連タイプ | isVersionOf | |||||
識別子タイプ | DOI | |||||
関連識別子 | 10.1159/000254842 | |||||
権利 | ||||||
権利情報 | Copyright © 2009 S. Karger AG, Basel | |||||
著者版フラグ | ||||||
出版タイプ | AM | |||||
出版タイプResource | http://purl.org/coar/version/c_ab4af688f83e57aa | |||||
引用 | ||||||
内容記述タイプ | Other | |||||
内容記述 | Dementia and geriatric cognitive disorders, 28(6), pp.550-557; 2009 |