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

Research Project

Project/Area Number 10680714
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Nerve anatomy/Neuropathology
Research InstitutionTokyo Metropolitan Institute for Neuroscience

Principal Investigator

OYANAGI Kiyomitsu  Tokyo Metropolitan Institute for Neuroscience, Dept. of Neuropathology, Director, 東京都神経科学総合研究所, 副参事研究員 (00134958)

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)
Project Period (FY) 1998 – 1999
Project Status Completed (Fiscal Year 1999)
Budget Amount *help
¥3,100,000 (Direct Cost: ¥3,100,000)
Fiscal Year 1999: ¥900,000 (Direct Cost: ¥900,000)
Fiscal Year 1998: ¥2,200,000 (Direct Cost: ¥2,200,000)
Keywordsparkinsonism-dementia complex / progressive supranuclear palsy / corticobasal degeneration / substantia nigra / neuron / neurofibrillary tangle / glial tangle / morphometry / 細胞原線維変化
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.

Report

(3 results)
  • 1999 Annual Research Report   Final Research Report Summary
  • 1998 Annual Research Report
  • Research Products

    (23 results)

All Other

All Publications (23 results)

  • [Publications] Yamazaki M, Arai Y, et al.: "α-synuclein inclusion in amygdala in the brains of patients with the parkinsonism-dementia complex of Guam"Journal of Neuropathology and Experimental Neurology. (印刷中).

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Wada M, Uchihara T, et al.: "Bunina bodies in amyotrophic lateral sclerosis on Guam : a histochemical, immunohistochemical and ultrastractural investigation"Acta Neuropathologica. 98. 150-156 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Oyanagi K, Chen K-M, et al.: "Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial depeneration"Acta Neuropathologica. 99. 73-80 (2000)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Oyanagi K, Wada M: "Neuropathology of parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam : an update"Jorunal of Neurology. 246(Suppl). II/9-II/27 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Oyanagi K, Nagao K, Ono S: "Amyotrophic lateral sclerosis : increased hyaluronic acid in the media of the basilar artery"Jorunal of Neurology. 167. 112-116 (1999)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] 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)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Oyanagi K: "Diagnosis of Guam ALS/PDC and its Relationship to Parkinson's Disease,Alzheimer's Disease,and Amyotrophic Lateral Sclerosis Elsewhere in the World"American Association of Neuropathologists. 12 (1998)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Yamazaki M, Arai Y, Baba M, Iwatsubo T, Mori O, Katayama Y, Oyanagi K: "α-synuclein inclusion in amygdala in the brains of patients with the parkinsonism-dementia complex of Guam"Journal of Neuropathology and Experimental Neurology.

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Oyanagi K, Chen K-M, Craig UK, Yamazaki M, Perl DP: "Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial degeneration"Acta Neuropathologica. 99. 73-80 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Wada M, Uchihara T, Nakamura A, Oyanagi K: "Bunina bodies in amyotrophic lateral sclerosis on Guam: a histo-chemical, immunohistochemical and ultrastractural investigation"Acta Neuropathologica. 98. 150-156 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Oyanagi K, Wada M: "Neuropathology of parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam: an update"Journal of Neurology. 246(Suppl). II/9-II/27 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] 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)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Oyanagi K, Kawakami E, Morita T, Takahashi H: "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 lateral sclerosis"Acta Neuropathologica. 98. 635-640 (1999)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Oyanagi K: "Diagnosis of Guam ALS/PDC and its Relationship to Parkinson's Disease, Alzheimer's Disease, and Amyo-trophic Lateral Sclerosis Elsewhere in the world"American Association of Neuropathologists. 50-62 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1999 Final Research Report Summary
  • [Publications] Wada M., Uchihara T., et al.: "Bunina bodies in amyotrophic lateral sclerosis on Guam : a histochemical, immunohistochemical and ultrastractural investigation"Acta Neuropathologica. 98. 150-156 (2000)

    • Related Report
      1999 Annual Research Report
  • [Publications] Oyanagi K., Chen K-M., et al.: "Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial degeneration"Acta Neuropathologica. 99. 73-80 (2000)

    • Related Report
      1999 Annual Research Report
  • [Publications] Oyanagi K., Wada M.: "Neuropathology of parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam : an update"Journal of Neurology. 246 (Suppl). II/9-II/27 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] 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)

    • Related Report
      1999 Annual Research Report
  • [Publications] Oyanagi K., Kawakami E., et al.: "Pursuit of the origin of the large myellnated fibers of the anterolateral funiculus in the spinal cord in humans in relation to the pathomechanism in amyotrophic lateral sclerosis"Acta Neuropathologica. 98. 635-640 (1999)

    • Related Report
      1999 Annual Research Report
  • [Publications] 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)

    • Related Report
      1999 Annual Research Report
  • [Publications] Oyanagi K,et al.: "Neuropathology of parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam: an update" Journal of Neurology. 発表予定(印刷中).

    • Related Report
      1998 Annual Research Report
  • [Publications] Oyanagi K,et al.: "Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial degeneration" Acta Neuropathologica. 発表予定(印刷中).

    • Related Report
      1998 Annual Research Report
  • [Publications] Wada M,et al.: "Bunina bodies in amyotrophic lateral sclerosis on Guam: a histochemical, immunohistochemical and ultrastractural investigation" Acta Neuropathologica. 発表予定(印刷中).

    • Related Report
      1998 Annual Research Report

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Published: 1998-04-01   Modified: 2016-04-21  

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