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

在进行性核上性麻痹（PSP）、皮质基底变性（CBD）和关岛帕金森-痴呆复合体（PDC）中检查了黑质疾病的特异性发现，这些疾病表现为痴呆和帕金森病，由高磷酸化tau组成的神经原纤维和胶质缠结。PDC黑质中有色素的神经元损失极为严重，而非色素的神经元相对较少。另一方面，在PSP和CBD中，黑质中有色素和无色素的神经元数量均呈相关性，且显著减少。在地形上，非色素神经元被耗尽，特别是在PSP的网状部。在PDC和PSP中观察到许多神经原纤维缠结。虽然nft的数量很少，但在CBD的神经元中可以看到许多网状亲银胞浆内结构，即所谓的“前缠结”。PDC中仅鉴定出粒状模糊星形包裹体，且螺纹数量较少。在PSP和CBD中观察到许多泡沫球状体、盘绕体和螺纹，而在PDC中很少见到。综上所述，PDC是一种明显不同于PSP和CBD的疾病，PSP和CBD在神经病理表现上的一些共性表明存在共同的发病机制，但观察到的特征性表现表明PSP和CBD在神经病理过程上存在表型差异。

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.