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A novel analysis of the disease progression patterns in ALS using electrophysiological data

Research Project

Project/Area Number 16K09710
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Neurology
Research InstitutionTokyo Medical and Dental University

Principal Investigator

Kanouchi Tadashi  東京医科歯科大学, 医学部附属病院, 講師 (50345287)

Project Period (FY) 2016-04-01 – 2020-03-31
Project Status Completed (Fiscal Year 2019)
Budget Amount *help
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2018: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2017: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2016: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Keywords筋委縮性側索硬化症 / プリオン様伝播仮説 / 病変進展 / 定量的疾患進行パターン / Local progression / Regional spread / 電気生理学的検査 / 筋萎縮性側索硬化症 / 局所進行 / 領域進展 / 複合筋活動電位 / 針筋電図 / 経頭蓋磁気刺激(三重刺激法) / プリオン様伝搬仮説 / 疾患進行パターン / 電気生理 / local progression / regional spread / 脳神経疾患 / 進行パターン / 臨床電気生理学的手法
Outline of Final Research Achievements

The speed of symptom spreading from the onset site to the next body region tended to be positively correlated with the speed of CMAP amplitude reduction in the onset limb in 8 of 21 ALS patients, in whom the involved spinal segments with acute denervation in needle EMG are contiguously distributed from the onset segment. In 7 ALS patients with the upper limb onset, the smaller the response of onset limb to transcranial magnetic stimulation using the triple stimulation tecnique is, the faster the inter-regional symptom spreading relative to the CMAP amplitude reduction speed in the onset limb is. The first finding seems to be consistent with the hypothesis in which the ALS lesion would spread out with domino-like cell-to-cell propagation of the pathogenic proteins. The second finding suggests that impairment of the upper motor neurons may affect the regional spreading of ALS lesion. But both findings are not sufficiently certain and further research will be necessary in the future.

Academic Significance and Societal Importance of the Research Achievements

プリオン様伝播仮説などの病態機序を視野に入れたALSの疾患進行パターンを新しく考案した。電気生理学的検査により得られる上位および下位運動ニューロンの情報を入れ込むことで疾患進行パターンを立体的かつ定量的に扱うことができるようになった。これにより、病変の進展が病因因子のドミノ倒し的な神経細胞間伝播によるという仮説と合うような進行パターンを示している可能性のある一群を見出すことができた。

Report

(5 results)
  • 2019 Annual Research Report   Final Research Report ( PDF )
  • 2018 Research-status Report
  • 2017 Research-status Report
  • 2016 Research-status Report
  • Research Products

    (3 results)

All 2017 2016

All Presentation (3 results) (of which Int'l Joint Research: 1 results)

  • [Presentation] Temporal relation between local disease progression in the onset site and regional spread of motor symptoms in amyotrophic lateral sclerosis.2017

    • Author(s)
      Kanouchi T, Sekiguchi T, Iida S, Akaza M, Yokota T.
    • Organizer
      The XXIII World Congress of Neurology
    • Related Report
      2017 Research-status Report
    • Int'l Joint Research
  • [Presentation] 新たな視点による筋萎縮性側索硬化症(ALS)の進行様式に関する定量解 析の試み2016

    • Author(s)
      叶内 匡,関口輝彦,横田隆徳
    • Organizer
      第57 回日本神経学会学術大会
    • Place of Presentation
      神戸コンペンションセンター・神戸ポートピアホテル(兵庫県神戸市)
    • Related Report
      2016 Research-status Report
  • [Presentation] 筋萎縮性側索硬化症(ALS)における下位運動神経の障害速度 に上位運動神経障害は影響するか2016

    • Author(s)
      叶内 匡,関口輝彦,東田修二,横田隆徳
    • Organizer
      第46 回日本臨床神経生理学会学術大会
    • Place of Presentation
      ホテルハマツ(福島県郡山市)
    • Related Report
      2016 Research-status Report

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Published: 2016-04-21   Modified: 2021-02-19  

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