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

对进行性核上性瘫痪(PSP)、皮质基底性变性(CBD)和关岛帕金森病-痴呆复合体(PDC)的黑质病变进行了神经病理学检查。PDC黑质内有色神经元丢失非常严重，而无色素神经元相对较少。而在PSP和CBD，黑质中的有色和无色神经元数量明显减少。在地形图上，PSP的非色素神经元被耗尽，尤其是在网状部。PDC和PSP内可见大量神经纤维缠结(NFT)。虽然NFT的数量很少，但在CBD的神经元中可见许多网状的嗜银胞浆结构，即所谓的“椒盐”。颗粒模糊的星形细胞包涵体仅见于PDC，且线数较少。PSP和CBD中可见大量泡沫状的球形小体和盘绕小体和线状结构，而PDC中则很少。结论：PDC是一种与PSP和CBD明显不同的疾病，对于PSP和CBD，神经病理表现中的一些共性表明存在共同的发病机制，但特征表现显示PSP和CBD在神经病理过程中存在表型差异。

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.