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| 題 名 | 路易氏體病與MIBG心肌閃爍造影--織茂智之的三十年足跡=Lewy Body Disease and MIBG Myocardial Scintigraphy: Insights into Clinical and Pathological Significance |
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| 作 者 | 織茂智之; 江孟庭; 郭之凡; 黃文盛; 洪光威; | 書刊名 | Annals of Nuclear Medicine and Molecular Imaging |
| 卷 期 | 38:4 2025.12[民114.12] |
| 頁 次 | 頁109-118 |
| 分類號 | 415.934 |
| 關鍵詞 | 路易氏體病; 路易氏體型失智症; 碘-123 MIBG; 心肌閃爍顯像; 巴金森病; Lewy body disease; Dementia with Lewy bodies; ¹²³I-MIBG; Myocardial scintigraphy; Parkinson's disease; |
| 語 文 | 中文(Chinese) |
| DOI | 10.6332/ANMMI.202512_38(4).0001 |
| 中文摘要 | 路易氏體病 (Lewy body disease, LBD) 包含巴金森病 (Parkinson disease, PD)、路易氏體型失智症 (dementia with Lewy bodies, DLB) 及伴隨失智 的 PDD (PD with dementia),其核心病理為 α-synuclein 聚集。此一病理 改變不僅影響中樞神經,也導致心臟交感神經去神經化。碘 -123 間碘苯 基胍 (metaiodobenzylguanidine, MIBG) 心肌閃爍顯像可反映交感神經功 能,近年被廣泛應用於 LBD 的診斷與鑑別。早期臨床觀察顯示,PD 與 DLB 患者普遍呈現 MIBG 攝取下降,且在疾病初期即出現異常;相反地, 多重系統退化症、進行性核上性麻痺、皮質基底核退化症及阿茲海默症 患者則多維持正常攝取。定量分析顯示,PD 與 DLB 的心臟/縱隔比 (H/ M ratio) 明顯低於對照組,並與病程嚴重度呈相關。病理解剖研究進一 步證實,MIBG 攝取下降與心臟交感神經纖維中 tyrosine hydroxylase 與 neurofilament 陽性纖維顯著減少有關,且與 α-synuclein 沉積呈正相關。 此發現指出,心臟交感神經變性可在 PD 臨床前期即發生,並解釋了臨床 檢查中 MIBG 攝取下降的病理機轉。總結來說,MIBG 心肌閃爍顯像不僅 可協助鑑別 LBD 與其他巴金森症候群或阿茲海默症,更可作為反映疾病 病理變化的重要生物標誌,並已納入 PD 與 DLB 的國際診斷準則之中。 |
| 英文摘要 | Lewy body disease (LBD), encompassing Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and PD with dementia, is characterized by the pathological aggregation of α-synuclein in central and autonomic nervous systems. This neuropathological change leads not only to motor and cognitive symptoms but also to degeneration of cardiac sympathetic nerves. 123I -metaiodobenzylguanidine (MIBG) myocardial scintigraphy is a well-established tool to assess cardiac sympathetic integrity and has become a valuable biomarker for the diagnosis and differentiation of LBD. Clinical studies have consistently demonstrated reduced cardiac MIBG uptake in PD and DLB patients, often detectable at an early disease stage. In contrast, patients with multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, and Alzheimer’s disease typically show preserved uptake. Quantitative analyses reveal that the heart-to-mediastinum (H/ M) ratio is significantly lower in PD and DLB than in controls, correlating with disease severity. Pathological investigations further confirm that reduced MIBG uptake reflects marked loss of tyrosine hydroxylase– and neurofilament–positive fibers in epicardial sympathetic nerves, closely associated with α-synuclein deposition. These findings provide mechanistic evidence that cardiac sympathetic denervation begins even in the preclinical phase of PD, explaining the clinical imaging abnormalities. In summary, 123I-MIBG myocardial scintigraphy serves as a reliable biomarker reflecting both functional and pathological changes in LBD, and it has been incorporated into international diagnostic criteria for PD and DLB. |
本系統中英文摘要資訊取自各篇刊載內容。