Making database of information of patients with Kii ALS/parkinsonism-dementia complex (ALS/PDC) for research on pathophysiology and pathogenesis
| Project/Area Number |
15K09364
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Neurology
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| Research Institution | Suzuka University of Medical Science |
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Principal Investigator |
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| 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)
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| 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
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| Project Period (FY) |
2015-04-01 – 2019-03-31
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| Project Status |
Completed (Fiscal Year 2018)
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| 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)
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| 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 |
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| 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.
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| 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。
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Report
(5 results)
Research Products
(27 results)
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[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.
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Journal Title
Brain Pathol.
Volume: -
Issue: 2
Pages: 287-291
DOI
Related Report
Peer Reviewed / Open Access / Int'l Joint Research
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[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.
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Journal Title
Biol Trace Elem Res.
Volume: 164
Issue: 1
Pages: 36-42
DOI
Related Report
Peer Reviewed / Open Access
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