Substantia Nigra in Progressive Supranuclear Palsy, Corticobasal Degeneration and Parkinsonism-Dementia Complex of Guam: Specific Pathological Features

关岛进行性核上性麻痹、皮质基底节变性和帕金森痴呆症中的黑质：具体病理特征

• 批准号: 10680714
• 负责人: OYANAGI Kiyomitsu
• 金额: $ 1.98万
• 依托单位: Tokyo Metropolitan Institute for Neuroscience
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 1998
• 资助国家: 日本
• 起止时间: 1998 至 1999
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-10680714/
• 关键词: parkinsonism-dementia complex; progressive supranuclear palsy; corticobasal degeneration; substantia nigra; neuron; neurofibrillary tangle; glial tangle; morphometry; 細胞原線維変化

Disease specific findings in the substantia nigra was examined neuropathologically in the progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and parkinsonism-dementia complex of Guam (PDC), which are the diseases showing dementia and parkinsonism with neurofibrillary and glial tangles composed of hyperphospholyrated tau. Loss of the pigmented neurons was extremely severe in the substantia nigra in PDC, but the nonpigmented neurons was relatively spared. On the other hand in PSP and CBD, both the pigmented and nonpigmented neurons were correlatively and markedly decreased in number in the substantia nigra. Topographically the nonpigmented neurons was depleted especially in the pars reticulata in PSP. Many neurofibrillary tangles (NFTs) were observed in PDC and PSP. Though the number of NFTs was small, reticular argyrophilic intraneurocytoplasmic structures, so-called "pretangles", were seen many in the neurons in CBD. Granular hazy astocytic inclusions were identified exclusively in PDC, and the number of threads were small. Many foamy spheroid bodies as well as coiled bodies and threads were observed in PSP and CBD, but rare in PDC. Conclusively, PDC is a disease being distinctly different from PSP and CBD, and as for PSP and CBD, some commonalities in the neuropathological findings indicate the presence of common pathogenesis, but characteristic findings observed show a presence of phenotypic differences in the neuropathological processes between PSP and CBD.

在进行性核上性麻痹 (PSP)、皮质基底节变性 (CBD) 和关岛帕金森痴呆复合体 (PDC) 中对黑质的疾病特异性发现进行了神经病理学检查，这些疾病表现为痴呆和帕金森病，伴有由过度磷酸化的 tau 蛋白组成的神经原纤维和神经胶质缠结。 PDC 黑质中色素神经元的损失极其严重，但非色素神经元相对幸免。另一方面，在 PSP 和 CBD 中，黑质中色素和非色素神经元的数量均相关且显着减少。从地形上看，PSP 中的非色素神经元尤其是网状部被耗尽。在 PDC 和 PSP 中观察到许多神经原纤维缠结 (NFT)。尽管 NFT 的数量很少，但在 CBD 的神经元中发现了许多网状嗜银神经细胞质内结构，即所谓的“前缠结物”。仅在 PDC 中发现了颗粒状的模糊星形细胞包涵体，并且线的数量很少。在 PSP 和 CBD 中观察到许多泡沫球体以及卷绕体和线，但在 PDC 中很少见。总之，PDC 是一种与 PSP 和 CBD 明显不同的疾病，对于 PSP 和 CBD，神经病理学结果的一些共同点表明存在共同的发病机制，但观察到的特征性发现表明 PSP 和 CBD 之间的神经病理过程存在表型差异。

项目成果

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山崎峰雄、新井裕至 ら: "Guam parkinsonism-dementia complexの扁桃体に認められたα-synuclein陽性封入体"Neuropathology. 19 (Suppl). 119 (1999)

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Oyanagi K., Nagao K., Ono S.: "Amyotrophic lateral sclerosis : Increased hyaluronic acid in the media of the basilar artery"Journal of Neurology. 167. 112-116 (1999)

Oyanagi K.、Nagao K.、Ono S.：“肌萎缩侧索硬化症：基底动脉中层透明质酸增加”神经病学杂志。

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Oyanagi K, Kawakami E, et al.: "Pursuit of the origin of the large myelinated fibers of the anterolateral funiculus in the spinal cord in humans in relation to the pathomechanism in amyotrophic"Acta Neuropathologica. 98. 635-640 (1999)

Oyanagi K、Kawakami E 等人：“追寻人类脊髓前外侧索大有髓纤维的起源与肌萎缩症病理机制的关系”《神经病理学报》。