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題 名 | Regional Decrease in Brain Glucose Metabolism in Asymptomatic Gene Carriers of Machado-Joseph Disease: A Preliminary Report=Machado-Joseph病無症狀帶因者腦局部葡萄糖代謝減少研究初報 |
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作 者 | 宋秉文; 劉仁賢; | 書刊名 | 中華醫學雜誌 |
卷 期 | 61:3 民87.03 |
頁 次 | 頁121-126 |
分類號 | 415.826 |
關鍵詞 | 無症狀之帶因者; Machado-Joseph氏病; 正子攝影術; 三核甘酸重複擴張; Asymptomatic gene carriers; Machado-joseph disease; Positron emission tomography; Trinucleotide repeat expansion; |
語 文 | 英文(English) |
中文摘要 | 背景:Machado-Joseph氏病為一神經退化性疾病,其分子機制最近才被發現。我們以前普經使用正子攝影術證明,這些患者的腦部有特殊的代謝變化;本研究嘗試使用正子攝影術,探討Machado-Joseph氏病無症狀之帶因者,腦部是否也有代謝變化。 方法:經由分子診斷方法,篩選出三位Machado-Joseph氏病無症狀之帶因者及十位正常人參與這項研究。我們使用[18F] fluorodcoxyglcuose研究受試者腦局部葡萄糖代謝的變化,並與正常人的值比較。 結果:Machado-Joseph氏病無症狀之帶因者,在小腦半球及枕葉已有局部葡萄糖代謝減少的現象,表示疾病的變化已經在進行;換言之,這些人在表現臨床症狀以前,腦子巳先有特殊的、異常的變化。正子攝影術對這些變化偵測的敏感度可達100%。 結論:以正子攝影術評估局部腦代謝活性,為一非侵犯之檢查,而小腦半球及枕葉之局部葡萄糖代謝的減少,可做為疾病的活性之客觀標記。本研究之樣本數不多,但已顯示正子攝影術有極高的敏感度,可用來研究Machado-Joseph氏病無症狀之帶因者。 |
英文摘要 | BACKGROUND: The molecular abnormality responsible for Machado-Joseph disease (MJD), a neurodegenerative disorder, has recently been described. It has also been previously documented using positron emission tomography (PET) with [18F]-2-fluoro-2-deoxy-D-glucose (FDG) to record the metabolic changes in the brains of symptomatic subjects affected with MJD. The present study evaluated these changes in asymptomatic MJD gene carriers, previously identified by molecular analysis. METHODS: PET with FDG was used to study glucose metabolism in the brains of three asymptomatic MJD gene carriers who were identified by molecular analysis, and in 10 normal control subjects. RESULTS: By comparison with data obtained from normal control subjects, significantly decreased glucose utilization was found in the cerebellar hemisphere and occipital cortex of these three asymptomatic MJD gene carriers, suggesting preclinical disease activity. The sensitivity of diagnosis based on PET examination alone was 100% in this study, using significant decrease in glucose metabolism in the cerebellar hemisphere as a criterion. CONCLUSIONS: Measurement of regional brain metabolism by PET is noninvasive, and subclinical reduction of FDG consumption in the cerebellar hemisphere and occipital cortex is found to act as an objective marker of disease activity. Based on the results from a limited number of patients, the sensitivity of PET is sufficient to justify its routine use in the investigation of asymptomatic at-risk MJD subjects. |
本系統中英文摘要資訊取自各篇刊載內容。