Gerstmann-Sträussler-Scheinker症候群(Pro102Leu)の患者の臨床的特徴と放射線学的特徴との相関

Material type: 博士論文

Author: 吉村, 道由

Publisher: 鹿児島大学

Publication date: 2018-08-15

Material Format: Digital

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Name of awarding university/degree: 鹿児島大学,博士(医学),Doctor of Philosophy in Medical Science

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Note (General)：: 博士論文全文, 最終試験結果の要旨, 論文審査の要旨, 博士論文要旨Background and purpose: Gerstmann-Sträussler-Scheinker syndrome is a rare hereditary neurodegenerative disorder with...

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Material Type: 博士論文
Title: Gerstmann-Sträussler-Scheinker症候群(Pro102Leu)の患者の臨床的特徴と放射線学的特徴との相関
Author/Editor: 吉村, 道由
Author Heading: 吉村, 道由ヨシムラ, ミチヨシ
Publication, Distribution, etc.: 鹿児島大学
Publication Date: 2018-08-15
Publication Date (W3CDTF): 2018-08-15
Alternative Title: Correlation between clinical and radiologic features of patients with Gerstmann-Sträussler-Scheinker syndrome (Pro102Leu)
Periodical title: Journal of the Neurological Sciences
No. or year of volume/issue: 391
Volume: 391
Pages: 15-21
Degree grantor/type: 鹿児島大学
Date Granted: 2018-12-27
Dissertation Number: 甲総研第487号
Degree Type: 博士(医学)
Doctor of Philosophy in Medical Science
Text Language Code: eng
Target Audience: 一般
Note (General): 博士論文全文, 最終試験結果の要旨, 論文審査の要旨, 博士論文要旨
Background and purpose: Gerstmann-Sträussler-Scheinker syndrome is a rare hereditary neurodegenerative disorder with clinical heterogeneity. This study is aim to demonstrate the clinical spectrum and radiologic characteristics of patients caused by Pro102Leu mutation in PRNP. Materials and methods: We retrospect clinical manifestations of five patients from four Japanese families, and comprehensively analyzed their brain MRI, SPECT (N-isopropyl-p-[123I] iodoamphetamine), and PET (18F-2-fluoro-2-deoxy-D-glucose) images. Results: All patients developed ataxia of lower limbs and trunk, gait disturbance, dysesthesia in legs, and lower limb hyporeflexia. In the early clinical stage before dementia began, no noticeable abnormalities could be observed from brain MRI, but SPECT and PET revealed mosaic-like pattern of blood flow and glucose metabolism of the brain. Predominant abnormalities were found in the occipital and frontal lobes on SPECT and PET analysis, respectively. In SPECT analysis, blood flow of the anterior cerebellar lobes was lower than that of the posterior cerebellar lobes. Conclusions: Clinical symptoms resulting from failure of dorsal horn of spinal cord and spinocerebellar tracts were observed in all cases. Radiologic findings revealed individual differences of involved region in their brain, which could produce clinical diversity. We identified a downtrend of blood flow in the anterior cerebellar lobes, a projection field of the spinocerebellar tracts, which is an important feature of Gerstmann-Sträussler-Scheinker syndrome.
Format (IMT): application/pdf
Source: Diss_吉村_道由_ISK487_2018 （fulltext）
https://ir.kagoshima-u.ac.jp/record/14527/files/Diss_%E5%90%89%E6%9D%91_%E9%81%93%E7%94%B1_ISK487_2018.pdf
Access Restrictions: インターネット公開
Related Material: Journal of the Neurological Sciences
https://www.elsevier.com/locate/jns
Related Material (DOI): 10.1016/j.jns.2018.05.012
Data Provider (Database): 国立情報学研究所 : 学術機関リポジトリデータベース（IRDB）（機関リポジトリ）
https://irdb.nii.ac.jp
Original Data Provider (Database): 鹿児島大学 : 鹿児島大学リポジトリ