| Co-Investigator(Kenkyū-buntansha) |
UCHIHARA Toshiki Tokyo Metropolitan Institute for Neuroscience, Dept. of Neuropathology, Researcher, 東京都神経科学総合研究所, 主任研究員 (10223570)
KAWAKAMI Emiko Tokyo Metropolitan Institute for Neuroscience, Researcher, 東京都神経科学総合研究所, 主事研究員 (00301797)
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| Research Abstract |
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.
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