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Making database of information of patients with Kii ALS/parkinsonism-dementia complex (ALS/PDC) for research on pathophysiology and pathogenesis

Research Project

Project/Area Number 15K09364
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Neurology
Research InstitutionSuzuka University of Medical Science

Principal Investigator

Kuzuhara Shigeki  鈴鹿医療科学大学, 看護学部, 教授 (70111383)

Co-Investigator(Kenkyū-buntansha) 小久保 康昌  三重大学, 地域イノベーション学研究科, 招へい教授 (60263000)
Research Collaborator Mimuro Maya  愛知医科大学, 加齢医学研究所
Sasaki Ryogen  三重大学, 医学部
Morimoto Satoru  慶応大学, 医学部
Project Period (FY) 2015-04-01 – 2019-03-31
Project Status Completed (Fiscal Year 2018)
Budget Amount *help
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2017: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2016: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2015: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Keywords紀伊半島 / 神経変性疾患 / 筋萎縮性側索硬化症 / パーキンソン・認知症複合 / タウオパチー / TDP-43, / α-シヌクレイノパチー / 神経風土病 / 臨床症状 / 神経病理 / TDP-43 / α-シヌクレイン / TDP-43 proteinopathy / α-シヌクレインパチー / データベース / 臨床資料 / 生体試料 / 臨床所見 / 神経病理所見 / tauopathy
Outline of Final Research Achievements

Amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) is an endemic neurodegenerative disease in Guam, Kii peninsula and Papua. The incidence of ALS of the 3 foci was extremely high, but has gradually declined in these decades and ALS/PDC is expected to disappear in the near future with the pathogenesis unclarified. The purpose of this study is to make database of the important information of the patients with this peculiar disease to use them for future research.
Database of clinical history, physical findings and laboratory data of 50 patients with ALS/PDC of the H focus of Kii peninsula were made. Additionally, brains and spinal cords of 18 autopsy cases were examined, and neuropathological and immunohistochemical diagnoses were made. Database of neuropathological findings with microscopic pictures was made. Data from this database has been used by the internal researchers, and we plan to make it available for use by outside researchers under certain restrictions.

Academic Significance and Societal Importance of the Research Achievements

アルツハイマー病、パーキンソン病、筋萎縮性側索硬化症(ALS)などの神経変性疾患は、遺伝素因と後天的な環境因子の相互作用によって発症すると考えられている。紀伊半島南部、グアム島、西ニューギニアの特定地域に多発するALS/PDC(パーキンソン・認知症複合)は、この相互関係を解明するのに適したモデルである。しかし、1970年頃から患者発生が激減し、近い将来に消滅が予想されている。多発の原因が明らかでないが、環境要因の影響が大きいと推定される。将来は消滅するかも知れないALS/PDCの多発と激減の原因解明研究に備えて、貴重な症例の情報と試料を正確に記録し保管して置くことに本研究の意義がある。

Report

(5 results)
  • 2018 Annual Research Report   Final Research Report ( PDF )
  • 2017 Research-status Report
  • 2016 Research-status Report
  • 2015 Research-status Report
  • Research Products

    (27 results)

All 2018 2017 2016 2015

All Journal Article (16 results) (of which Int'l Joint Research: 3 results,  Peer Reviewed: 9 results,  Open Access: 7 results) Presentation (9 results) (of which Int'l Joint Research: 5 results,  Invited: 2 results) Book (2 results)

  • [Journal Article] Nitrative Stress and Tau Accumulation in Amyotrophic Lateral Sclerosis/Parkinsonism-Dementia Complex (ALS/PDC) in the Kii Peninsula, Japan2018

    • Author(s)
      Hata Yukiko、Ma Ning、Yoneda Misao、Morimoto Satoru、Okano Hideyuki、Murayama Shigeo、Kawanishi Shosuke、Kuzuhara Shigeki、Kokubo Yasumasa
    • Journal Title

      Frontiers in Neuroscience

      Volume: 11 Pages: 751-751

    • DOI

      10.3389/fnins.2017.00751

    • Related Report
      2018 Annual Research Report 2017 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] 手指の異常連合運動(Souquesの指徴候、Watenberg徴候、指折り数え試験)2018

    • Author(s)
      葛原茂樹
    • Journal Title

      神経内科

      Volume: 88 Pages: 51-54

    • Related Report
      2018 Annual Research Report
    • Open Access
  • [Journal Article] Tau imaging detects distinctive distribution of tau pathology in ALS/PDC on the Kii Peninsula2018

    • Author(s)
      Shinotoh Hitoshi、Shimada Hitoshi、Kokubo Yasumasa、Tagai Kenji、Niwa Fumitoshi、Kitamura Soichiro、Endo Hironobu、Ono Maiko、Kimura Yasuyuki、Hirano Shigeki、Mimuro Maya、Ichise Masanori、Sahara Naruhiko、Zhang Ming-Rong、Suhara Tetsuya、Higuchi Makoto
    • Journal Title

      Neurology

      Volume: 92 Issue: 2

    • DOI

      10.1212/wnl.0000000000006736

    • Related Report
      2018 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Optineurin pathology in the spinal cord of amyotrophic lateral sclerosis/parkinsonism-dementia complex patients in Kii Peninsula, Japan2018

    • Author(s)
      Morimoto Satoru、Hatsuta Hiroyuki、Motoyama Rie、Kokubo Yasumasa、Ishiura Hiroyuki、Tsuji Shoji、Kuzuhara Shigeki、Murayama Shigeo
    • Journal Title

      Brain Pathology

      Volume: 28 Issue: 3 Pages: 422-426

    • DOI

      10.1111/bpa.12558

    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Amyotrophic lateral sclerosis and parkinsonism-dementia complex of the Hohara focus of the Kii Peninsula: A multiple proteinopathy?2017

    • Author(s)
      Mimuro Maya、Yoshida Mari、Kuzuhara Shigeki、Kokubo Yasumasa
    • Journal Title

      Neuropathology

      Volume: 38 Issue: 1 Pages: 98-107

    • DOI

      10.1111/neup.12434

    • Related Report
      2018 Annual Research Report 2017 Research-status Report
    • Peer Reviewed
  • [Journal Article] 多発から消滅へ-紀伊半島・グアム・パプアをつなぐ特異な神経風土病ALS/PDCの不思議-2017

    • Author(s)
      葛原茂樹
    • Journal Title

      學士會会報3月 No.923

      Volume: 2017-Ⅱ

    • NAID

      40021126780

    • Related Report
      2017 Research-status Report
  • [Journal Article] 進行性核上性麻痺(PSP)歴史と概要2017

    • Author(s)
      葛原茂樹
    • Journal Title

      月刊臨床神経科学

      Volume: 35 Pages: 271-274

    • Related Report
      2017 Research-status Report
  • [Journal Article] さまざまな認知症との出会い-パーキンソン・認知症複合など2017

    • Author(s)
      葛原茂樹
    • Journal Title

      高次脳機能研究/日本高次脳機能障害会誌

      Volume: 23 Pages: 271-274

    • Related Report
      2017 Research-status Report
  • [Journal Article] β-N-methylamino-l-alanine analysis in the brains of patients with Kii ALS/PDC2017

    • Author(s)
      Kokubo Yasumasa、Banack Sandra Anne、Morimoto Satoru、Murayama Shigeo、Togashi Tatsuya、Metcalf James S.、Cox Paul Alan、Kuzuhara Shigeki
    • Journal Title

      Neurology

      Volume: 89 Issue: 10 Pages: 1091-1092

    • DOI

      10.1212/wnl.0000000000004310

    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] A migration case of Kii amyotrophic lateral sclerosis/parkinsonism dementia complex with the shortest stay in the endemic area and the longest incubation to develop the disease2017

    • Author(s)
      Tsunoda Keiichiro、Yamashita Toru、Shimada Hitoshi、Nomura Emi、Takahashi Yoshiaki、Shang Jingwei、Sato Kota、Takemoto Mami、Hishikawa Nozomi、Ohta Yasuyuki、Higuchi Makoto、Suhara Tetsuya、Kokubo Yasumasa、Kuzuhara Shigeki、Abe Koji
    • Journal Title

      Journal of Clinical Neuroscience

      Volume: 46 Pages: 64-67

    • DOI

      10.1016/j.jocn.2017.08.057

    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Unusual tau pathology of the cerebellum in patients with amyotrophic lateral sclerosis/parkinsonism-dementia complex from the kii peninsula, Japan.2017

    • Author(s)
      Morimoto S, Hatsuta H, Kokubo Y, Nakano Y, Hasegawa M, Yoneda M, Hirokawa Y, Kuzuhara S, Shiraishi T, Murayama S.
    • Journal Title

      Brain Pathol.

      Volume: - Issue: 2 Pages: 287-291

    • DOI

      10.1111/bpa.12500

    • Related Report
      2017 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Journal Article] 認知症を呈する様々な神経疾患との出会い~GSSD,DLB,薬剤性パ-キンソニズム、ALS/PDCなど~2017

    • Author(s)
      葛原茂樹
    • Journal Title

      月刊難病と在宅ケア

      Volume: 23 Pages: 59-63

    • Related Report
      2017 Research-status Report
  • [Journal Article] 多発から消滅へ-紀伊半島・グアム・パプアをつなぐ特異な神経風土病ALS/PDCの不思議-2017

    • Author(s)
      葛原茂樹
    • Journal Title

      學士會会報

      Volume: 923(Ⅱ)

    • NAID

      40021126780

    • Related Report
      2016 Research-status Report
  • [Journal Article] 紀伊パーキンソン認知症複合2015

    • Author(s)
      三室マヤ、吉田眞里、小久保康昌、葛原茂樹
    • Journal Title

      Clinical Neuroscience

      Volume: 134 Pages: 1450-1451

    • Related Report
      2015 Research-status Report
  • [Journal Article] Neutron activation analysis of scalp hair from ALS patients and residents in the Kii Peninsula, Japan.2015

    • Author(s)
      Kihira T, Sakurai I, Yoshida S, Wakayama I, Takamiya K, Okumura R, Iinuma Y, Iwai K, Kajimoto Y, Hiwatani Y, Kohmoto J, Okamoto K, Kokubo Y, Kuzuhara S.
    • Journal Title

      Biol Trace Elem Res.

      Volume: 164 Issue: 1 Pages: 36-42

    • DOI

      10.1007/s12011-014-0202-6

    • Related Report
      2015 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] 紀伊半島の筋萎縮性側索硬化症/Parkinson認知症複合におけるedaravoneを用いた臨床研究2015

    • Author(s)
      小久保康昌、中川十夢、宮崎光一、森本悟、葛原茂樹
    • Journal Title

      神経治療学

      Volume: 31 Pages: 50-53

    • Related Report
      2015 Research-status Report
    • Peer Reviewed
  • [Presentation] The Three Subtypes in Kii ALS/PDC of the H focus : Clinicopathological Differences of Each Subtype2018

    • Author(s)
      Maya Mimuro, Mari Yoshida, Ryogen Sasaki, Keita Sakurai, Masato Hasegawa, Shigeki Kuzuhara, Yasumasa Kokubo
    • Organizer
      19th Internatiional Congress of Neuropathology (19th ICN2018 TOKYO)
    • Related Report
      2018 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Tau imaging in patients with ALS/PDC Complex in the Kii peninsula2017

    • Author(s)
      Shinotoh H.Kokubu Y.Kitamura S.Niwa F.Tagai K.Hirano A.morimoto S.Yamashita T.sahara N.Zhang MR.Kuzuhara S. .Higuchi M
    • Organizer
      The 23rd World Congrss of Neurology, Kyoto, Japan
    • Related Report
      2017 Research-status Report
    • Int'l Joint Research
  • [Presentation] Common epidemiological changes of amyotrophic lateral sclerosis and parkinsonism dementia complex in Guam, Papua and Kii peninsula.2017

    • Author(s)
      Satoru Morimoto1, Kiyohito Okumiya2, Indrajaya Manuaba 3, Shigeki Kuzuhara 4, Yasumasa Kokubo5
    • Organizer
      The 23rd World Congrss of Neurology, Kyoto, Japan
    • Related Report
      2017 Research-status Report
    • Int'l Joint Research
  • [Presentation] 様々な認知症との出会い-GSSD,DLB,ALS/PDCなど2017

    • Author(s)
      葛原茂樹
    • Organizer
      第40回日本高次脳機能障害学会
    • Related Report
      2017 Research-status Report
    • Invited
  • [Presentation] エキスパートに聞く1 様々な認知症との出会い2016

    • Author(s)
      葛原茂樹
    • Organizer
      第40回日本高次脳機能障害学会学術総会
    • Place of Presentation
      松本市
    • Related Report
      2016 Research-status Report
    • Invited
  • [Presentation] Clinicopathological study of familial ALS/parkinsonism-dementia complex(ALS/PDC)cases in the Kii peninsula2015

    • Author(s)
      Kuzuhara S, Kokubo Y
    • Organizer
      XXII World Congress of Neurology
    • Place of Presentation
      Santiago,Chili
    • Year and Date
      2015-10-31
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] Clinical database of amyotrophic lateral sclerosis/parkinsonism-dementia complex of kii, Japan2015

    • Author(s)
      Kokubo Y, Morimoto S, Kuzuhara S
    • Organizer
      XXII World Congress of Neurology
    • Place of Presentation
      Santiago,Chili
    • Year and Date
      2015-10-31
    • Related Report
      2015 Research-status Report
    • Int'l Joint Research
  • [Presentation] 紀伊半島に多発するパーキンソン認知症複合5例の病理2015

    • Author(s)
      三室マヤ、吉田眞里、佐々木良元、赤木明星、岩崎靖、葛原茂樹、小久保康昌
    • Organizer
      第56回日本神経病理学会総会学術研究会 (岩城徹会長)
    • Place of Presentation
      福岡市
    • Year and Date
      2015-06-03
    • Related Report
      2015 Research-status Report
  • [Presentation] PDDとの鑑別を要した紀伊のPDCの1剖検例2015

    • Author(s)
      小久保康昌、三室マヤ、森本悟、藤原雅也、広川佳史、白石泰三、長谷川成人、吉田真理、葛原茂樹
    • Organizer
      第56回日本神経病理学会総会学術研究会 (岩城徹会長)
    • Place of Presentation
      福岡市
    • Year and Date
      2015-06-03
    • Related Report
      2015 Research-status Report
  • [Book] Guam島・紀伊半島の筋萎縮性側索硬化症(臨床神経内科学第6版【平山惠造 監修】)2016

    • Author(s)
      葛原茂樹
    • Publisher
      南山堂
    • Related Report
      2016 Research-status Report
  • [Book] Guam島・紀伊半島の筋萎縮性側索硬化症、臨床神経内科学改定第6版(監修・平山恵造、編集・廣瀬源二郎、田代邦夫、葛原茂樹)2016

    • Author(s)
      葛原茂樹
    • Publisher
      南山堂、東京
    • Related Report
      2015 Research-status Report

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Published: 2015-04-16   Modified: 2020-03-30  

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