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2011 Fiscal Year Annual Research Report

TDP-43proteinopathyの神経病理学的研究

Research Project

Project/Area Number 21500339
Research InstitutionAichi Medical University

Principal Investigator

吉田 眞理  愛知医科大学, 加齢医科学研究所, 教授 (60288545)

Keywords筋萎縮性側索硬化症(ALS) / 前頭側頭葉変性症(FTLD-TDP) / TDP-43 / 変性突起 / 神経細胞胞体内封入体
Research Abstract

目的:愛知医科大学加齢医科学研究所の筋萎縮性側索硬化症(ALS)と前頭側頭葉変性症(FTLD)の連続剖検例にTDP-43免疫染色を施行し、封入体のサブタイプ、臨床病理像との相関を検討した。
結果:2011年は8例を解析した。臨床診断は孤発性ALS7例、FTLD1例、ALSの平均死亡時年齢は71歳(65~76歳)、男女比は7:0、ALS7例中6例はTDP-43 proteinopathyを示した。6例のALSはほぼ運動ニューロン系に限局した変性を認めたが、1例にはパーキンソン病の合併を認めた。6例の脊髄前角細胞には全例でBunina小体、skein-like inclusions, round inclusionを認めた。大脳皮質には辺縁系を含めてTDP-43陽性構造物の優位な出現は乏しかった。1例は、家族歴は確認されていないが遺伝子診断でSOD1の遺伝子異常が確認され、病理学的にTDP-43 proteinopathyはみられなかった。FTLDの1例は、病理学的に前頭側頭葉の変性、多数のNCIと短いdystrophic neurites、核内封入体を伴うTDP-43 proteinopathyを認めFTLD-TDP typeAと診断された。
考察と結論:TDP-43 proteinopathyを示すALS~FTLDは8例中7例88%、ALS7例中6例はTDP-43 proteinopathyを示す孤発性ALSであった。FTLD-TDP例は前頭側頭型認知症の臨床像を呈し、臨床的には運動ニューロン障害はみられず、病理学的には脊髄は採取されていないが舌下神経核の変性、延髄錐体の変性はみられなかった。TDP-43 proteinopathyに占める運動ニューロン疾患の頻度はきわめて高いが、FTLD-TDP typeAでは下位運動ニューロンの変性が乏しい可能性が示唆された。

  • Research Products

    (16 results)

All 2011

All Journal Article (11 results) (of which Peer Reviewed: 11 results) Presentation (5 results)

  • [Journal Article] Brainstem and spinal cord motor neuron involvement with optineurin inclusions in proximal-dominant hereditary motor and sensory neuropathy2011

    • Author(s)
      Fujita K, Yoshida M, Sako W, et al
    • Journal Title

      J Neurol Neurosurg Psychiatry

      Volume: 82 Pages: 1402-1403

    • DOI

      DOI:10.1136/jnnp-2011-300783

    • Peer Reviewed
  • [Journal Article] Molecular Dissection of TDP-43 Proteinopathies2011

    • Author(s)
      Hasegawa M, Nonaka T, Tsuji H, et al
    • Journal Title

      J Mol Neurosci

      Volume: 45(3) Pages: 480-5

    • DOI

      DOI:10.1007/s12031-011-9571-x

    • Peer Reviewed
  • [Journal Article] RGMa modulates T cell responses and is involved in autoimmune encephalomyelitis2011

    • Author(s)
      Muramatsu R, et al
    • Journal Title

      Nat Med

      Volume: 17 Pages: 488-494

    • DOI

      10.1038/nm.2321

    • Peer Reviewed
  • [Journal Article] An autopsy case of lymphomatosis cerebri showing pathological changes of intravascular large B-cell lymphoma in visceral organs2011

    • Author(s)
      Hishikawa N, et al
    • Journal Title

      Neuropathology

      Volume: 31 Pages: 612-619

    • DOI

      10.1111/j.1440-1789.2011.01203.x

    • Peer Reviewed
  • [Journal Article] An autopsy case of progressive multifocal leukoencephalopathy : comparison of magnetic resonance imaging findings with the pathological findings2011

    • Author(s)
      Iida M, et al
    • Journal Title

      Brain Nerve

      Volume: 63 Pages: 1001-1007

    • Peer Reviewed
  • [Journal Article] FUS immunoreactivity of neuronal and glial intranuclear inclusions in intranuclear inclusion body disease2011

    • Author(s)
      Mori, F, et al
    • Journal Title

      Neuropathol.Appl.Neurobiol

      Volume: 38(Epub ahead of print) Pages: 322-328

    • DOI

      10.1111/j.1365-2990.2011.01217.x

    • Peer Reviewed
  • [Journal Article] Nationwide survey of Alexander disease in Japan and proposed new guidelines for diagnosis2011

    • Author(s)
      Yoshida, T, et al
    • Journal Title

      J.Neurol

      Volume: 258 Pages: 1998-2008

    • DOI

      10.1007/s00415-011-6056-3

    • Peer Reviewed
  • [Journal Article] Glial fibrillary acidic protein mutations in adult-onset Alexander disease : clinical features observed in 12 Japanese patients2011

    • Author(s)
      Yoshida, T, et al
    • Journal Title

      Acta Neurol Scand

      Volume: 124 Pages: 104-108

    • DOI

      10.1111/j.1600-0404.2010.01427.x

    • Peer Reviewed
  • [Journal Article] Longitudinal study on MRI intensity changes of Machado-Joseph disease : correlation between MRI findings and neuropathological changes2011

    • Author(s)
      Horimoto, Y, et al
    • Journal Title

      J.Neurol.

      Volume: 258 Pages: 1657-1664

    • DOI

      10.1007/s00415-011-5992-2

    • Peer Reviewed
  • [Journal Article] An autopsied case of V180I Creutzfeldt-Jakob disease presenting with panencephalopathic-type pathology and a characteristic prion protein type2011

    • Author(s)
      Iwasaki, Y, et al
    • Journal Title

      Neuropathology

      Volume: 31 Pages: 540-548

    • DOI

      10.1111/j.1440-1789.2010.01192.x

    • Peer Reviewed
  • [Journal Article] An autopsy case of MM2-cortical+thalamic-type sporadic Creutzfeldt-Jakob disease2011

    • Author(s)
      Saito, Y, et al
    • Journal Title

      Neuropathology

      Volume: 31 Pages: 523-530

    • DOI

      10.1111/j.1440-1789.2010.01181.x

    • Peer Reviewed
  • [Presentation] Neuropathological diversity of corticobasal degeneration (CBD) in 27 autopsied cases2011

    • Author(s)
      Yoshida M
    • Organizer
      XX^<th> World Congress of Neurology
    • Place of Presentation
      Marrakesh, Morocco
    • Year and Date
      2011-11-17
  • [Presentation] Multiple system atrophy : glial and neuronal synuleinopathy2011

    • Author(s)
      Yoshida M
    • Organizer
      The 2nd Congress of Asian Society of Neuropathology
    • Place of Presentation
      Beijing
    • Year and Date
      2011-11-05
  • [Presentation] Neuropathological diversity of corticobasal degeneration (CBD) in 27 autopsied cases2011

    • Author(s)
      Yoshida M
    • Organizer
      The 2nd Congress of Asian Society of Neuropathology
    • Place of Presentation
      Beijing
    • Year and Date
      2011-11-05
  • [Presentation] よくわかる中枢神経病理2011

    • Author(s)
      吉田眞理
    • Organizer
      第52回日本神経学会学術大会
    • Place of Presentation
      名古屋
    • Year and Date
      2011-05-20
  • [Presentation] 多系統萎縮症-シヌクレインと神経細胞変性-2011

    • Author(s)
      吉田眞理
    • Organizer
      第52回日本神経学会学術大会
    • Place of Presentation
      名古屋
    • Year and Date
      2011-05-18

URL: 

Published: 2013-06-26  

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