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Establishment of electrophysiological criteria for early diagnosis of amyotrophic lateral sclerosis with high sensitivity and specificity

Research Project

Project/Area Number 19K07966
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Review Section Basic Section 52020:Neurology-related
Research InstitutionTeikyo University

Principal Investigator

SONOO Masahiro  帝京大学, 医療技術学部, 教授 (40231386)

Project Period (FY) 2019-04-01 – 2024-03-31
Project Status Completed (Fiscal Year 2023)
Budget Amount *help
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2021: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2020: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2019: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Keywords筋萎縮性側索硬化症 / 選択的筋障害 / 徒手筋力テスト / 反復神経刺激試験 / 針筋電図 / 僧帽筋 / 診断基準 / 表面筋電図 / 遠隔電場電位 / 体性感覚誘発電位 / 神経反復刺激試験
Outline of Research at the Start

筋萎縮性側索硬化症(ALS)においては、近年新しい治療薬の治験が盛んに行われていますが、既存の診断基準では早期の感度が低く、治験に入れないケースがあることが問題となっています。本研究では、研究代表者がこれまでALSでの感度の高いことを見出した、神経反復刺激試験・針筋電図などの電気生理学的検査におけるいくつかのパラメータを組み合わせて、発症早期から感度・特異度の高い診断基準を創設することを目指します。

Outline of Final Research Achievements

With the aid from this grant, we completed following researches. 1) We found three new signs of selective involvement in amyotrophic lateral sclerosis (ALS): split finger, weak shoulder and arm sparing. These achieved higher diagnostic yields than signs reported by previous authors. 2) We proposed new electrophysiologic parameters to diagnose ALS combining repetitive nerve stimulation (RNS) of the trapezius and deltoid muscles and spontaneous activities in needle EMG of the trapezius muscle. Decrement in RNS and spontaneous activities in the trapezius muscle were highly specific for ALS, and negative findings in all three indicies in patients with upper-limb symptoms were highly specific for non-ALS. 3) We also conducted other researches related to electrophysiological diagnosis of ALS.

Academic Significance and Societal Importance of the Research Achievements

本研究で見出されたALSの臨床的・電気生理学的徴候はいずれもこれまで報告されていないもので、かつ感度ないし特異度が非常に高いものが含まれることが特徴的である。これらを広く世に知らしめることによって、ALSの早期診断、早期の他疾患、とりわけ頸椎症などの脊椎疾患との鑑別が可能となり、早期の治療導入、意思決定の援助、不要な手術の回避(脊椎手術はALSの進行を促進することが示されている)に役立つことが期待される。

Report

(6 results)
  • 2023 Annual Research Report   Final Research Report ( PDF )
  • 2022 Research-status Report
  • 2021 Research-status Report
  • 2020 Research-status Report
  • 2019 Research-status Report
  • Research Products

    (28 results)

All 2023 2022 2021 2020 2019

All Journal Article (11 results) (of which Int'l Joint Research: 2 results,  Peer Reviewed: 11 results,  Open Access: 5 results) Presentation (17 results) (of which Int'l Joint Research: 7 results,  Invited: 3 results)

  • [Journal Article] Weak gluteus maximus and weak iliopsoas with normal gluteus maximus: Two complementary new signs to diagnose lower limb functional weakness2023

    • Author(s)
      Sonoo M, Kanbayashi T, Kobayashi S, Matsuno H, Nakayama T, Imafuku I, et al.
    • Journal Title

      Brain Behav

      Volume: 13 Issue: 8

    • DOI

      10.1002/brb3.3135

    • Related Report
      2023 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Far-field potential of the compound muscle action potential as a reliable marker in amyotrophic lateral sclerosis2023

    • Author(s)
      Higashihara M, Yamazaki H, Izumi Y, Kobayashi M, Nodera H, Sonoo M, et al.
    • Journal Title

      Muscle Nerve

      Volume: 68 Issue: 3 Pages: 257

    • DOI

      10.1002/mus.27829

    • Related Report
      2023 Annual Research Report 2022 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Tibial nerve SEPs in diagnosing lumbar spinal stenosis: The utility of segmental evaluation using P15 and N212023

    • Author(s)
      Matsukura K, Hokkoku K, Mukai T, Oishi C, Kanbayashi T, Takahashi T, Sonoo M
    • Journal Title

      Clin Neurophysiol Pract

      Volume: 8 Pages: 49

    • DOI

      10.1016/j.cnp.2023.03.001

    • Related Report
      2022 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Weak shoulder and arm sparing signs in amyotrophic lateral sclerosis2022

    • Author(s)
      Hamada Y, Kanbayashi T, Takahashi K, Kamiya H, Kobayashi S, Sonoo M
    • Journal Title

      Muscle Nerve

      Volume: 65 Issue: 3 Pages: 311

    • DOI

      10.1002/mus.27480

    • Related Report
      2021 Research-status Report
    • Peer Reviewed
  • [Journal Article] Estimation of the true infection rate and infection fatality rate of coronavirus disease 2019 in each country2022

    • Author(s)
      Sonoo Masahiro、Kanbayashi Takamichi、Shimohata Takayoshi、Kobayashi Masahito、Idogawa Masashi、Hayashi Hideyuki
    • Journal Title

      Journal of Infection and Public Health

      Volume: 15 Issue: 2 Pages: 210

    • DOI

      10.1016/j.jiph.2021.12.010

    • Related Report
      2021 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Determining C5, C6 and C7 myotomes through comparative analyses of clinical, MRI and EMG findings in cervical radiculopathy2021

    • Author(s)
      Furukawa Yuichi、Miyaji Yosuke、Kadoya Akiko、Kamiya Hisao、Chiba Takashi、Hokkoku Kei-ichi、Hatanaka Yuki、Imafuku Ichiro、Miyoshi Kota、Sonoo Masahiro
    • Journal Title

      Clinical Neurophysiology Practice

      Volume: 6 Pages: 88

    • DOI

      10.1016/j.cnp.2021.02.002

    • URL

      https://pure.teikyo.jp/en/publications/71a5bfd7-a345-452f-9f53-41358839c218

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Split-finger syndrome in amyotrophic lateral sclerosis2020

    • Author(s)
      Sonoo Masahiro、Takahashi Kazusa、Hamada Yuichi、Hokkoku Keiichi、Kobayashi Shunsuke
    • Journal Title

      Journal of Neurology, Neurosurgery & Psychiatry

      Volume: 91 Issue: 11 Pages: 1235

    • DOI

      10.1136/jnnp-2020-323986

    • Related Report
      2020 Research-status Report
    • Peer Reviewed
  • [Journal Article] Updated size index valid for both neurogenic and myogenic changes2020

    • Author(s)
      Sonoo Masahiro、Ogawa Go、Hokkoku Keiichi、St?lberg Erik
    • Journal Title

      Muscle & Nerve

      Volume: 62 Issue: 6 Pages: 735

    • DOI

      10.1002/mus.27072

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Appropriate window width for the “clustering index method” in the tibialis anterior muscle2020

    • Author(s)
      Sonoo Masahiro、Uesugi Haruo、Ogawa Go、Hokkoku Keiichi、Kanbayashi Takamichi、Higashihara Mana、St?lberg Stefan、St?lberg Erik
    • Journal Title

      Muscle & Nerve

      Volume: 63 Issue: 1 Pages: 89

    • DOI

      10.1002/mus.27082

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Int'l Joint Research
  • [Journal Article] Far-field potentials in the compound muscle action potential2020

    • Author(s)
      Sonoo M
    • Journal Title

      Muscle Nerve

      Volume: 61 Issue: 3 Pages: 271

    • DOI

      10.1002/mus.26743

    • Related Report
      2019 Research-status Report
    • Peer Reviewed
  • [Journal Article] A new method to define cutoff values in nerve conduction studies for carpal tunnel syndrome considering the presence of false-positive cases2020

    • Author(s)
      Miyaji Y, Kobayashi M, Oishi C, Mizoi Y, Tanaka F, Sonoo M
    • Journal Title

      Neurol Sci

      Volume: 41 Issue: 3 Pages: 669

    • DOI

      10.1007/s10072-019-04145-2

    • URL

      https://pure.teikyo.jp/en/publications/99ca8084-1eea-4c94-870e-63b8c15092bc

    • Related Report
      2019 Research-status Report
    • Peer Reviewed
  • [Presentation] Weak gluteus maximus and weak iliopsoas with normal gluteus maximus: new signs to diagnose functional weakness of the lower limbs2023

    • Author(s)
      Sonoo M, Kanbayashi T
    • Organizer
      70th Annual Meeting of American Association of Neuromuscular and Electrodiagnostic Medicine
    • Related Report
      2023 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Far-field potential of CMAP (FFP-CMAP) as a reliable neurophysiological marker in ALS.2022

    • Author(s)
      Higashihara M, Yamazaki H, Izumi Y, Oishi C, Chiba T, Nodera H, Iwata A, Murayama S, Kaji R, Sonoo M
    • Organizer
      32nd International Congress of Clinical Neurophysiology
    • Related Report
      2022 Research-status Report
    • Int'l Joint Research
  • [Presentation] Where to place the reference electrode for recording the compound muscle action potential of the Deltoid muscle?2022

    • Author(s)
      Oishi C, Hamada Y, Mukai T, Takahashi K, Chiba A, Sonoo M
    • Organizer
      32nd International Congress of Clinical Neurophysiology
    • Related Report
      2022 Research-status Report
    • Int'l Joint Research
  • [Presentation] Concern regarding the interpretation of the updated Awaji criteria for the diagnosis of amyotrophic lateral sclerosis. , web, 4, 2021.2021

    • Author(s)
      Takahashi K, Hamada Y, Sonoo M
    • Organizer
      17th Asian Oceanian Congress of Neurology
    • Related Report
      2021 Research-status Report
    • Int'l Joint Research
  • [Presentation] Clinical and electrophysiological assessment for ALS2021

    • Author(s)
      Sonoo M
    • Organizer
      PACTALS
    • Related Report
      2021 Research-status Report
    • Int'l Joint Research / Invited
  • [Presentation] 脊椎・脊髄疾患の電気診断2021

    • Author(s)
      園生雅弘
    • Organizer
      第39回日本神経治療学会
    • Related Report
      2021 Research-status Report
    • Invited
  • [Presentation] 筋萎縮性側索硬化症診断(ALS)における神経反復刺激試験(RNS)の有用性2021

    • Author(s)
      高橋和沙, 濵田雄一, 園生雅弘
    • Organizer
      第62回日本神経学会学術大会
    • Related Report
      2021 Research-status Report
  • [Presentation] Arm sparing, Forearm sparingの感度と特異度2021

    • Author(s)
      濱田雄一, 高橋和沙, 神林隆道, 神谷久雄, 園生雅弘
    • Organizer
      第51回日本臨床神経生理学会学術大会
    • Related Report
      2021 Research-status Report
  • [Presentation] 頚椎症の臨床と電気生理:ALSとの鑑別を含めて2021

    • Author(s)
      園生雅弘
    • Organizer
      Kagoshima Spinal Cord Club 2021
    • Related Report
      2020 Research-status Report
    • Invited
  • [Presentation] Concern regarding the interpretation of the updated Awaji criteria for the diagnosis of amyotrophic lateral sclerosis2021

    • Author(s)
      Takahashi K, Hamada Y, Sonoo M
    • Organizer
      17th Asian Oceanian Congress of Neurology
    • Related Report
      2020 Research-status Report
    • Int'l Joint Research
  • [Presentation] 重症筋無力症と筋萎縮性側索硬化症における神経反復刺激試験の漸減パターンの違い2020

    • Author(s)
      上田優樹, 高橋和沙, 宮地洋輔, 神谷久雄, 畑中裕己, 田口丈士, 相澤仁志, 園生雅弘
    • Organizer
      第61回日本神経学会学術大会
    • Related Report
      2020 Research-status Report
  • [Presentation] Utility of modified Awaji criteria for diagnosis of amyotrophic lateral sclerosis2020

    • Author(s)
      高橋和沙, 濱田雄一, 園生雅弘
    • Organizer
      第61回日本神経学会学術大会
    • Related Report
      2020 Research-status Report
  • [Presentation] 遠位潜時延長、伝導速度定価より多巣性運動ニューロパチーが疑われていたALSの1例2020

    • Author(s)
      立山佳祐, 神林隆道, 田中園子, 千葉隆司, 園生雅弘
    • Organizer
      第31回日本末梢神経学会学術集会
    • Related Report
      2020 Research-status Report
  • [Presentation] 筋萎縮性側索硬化症(ALS)診断におけるUpdated Awaji基準の解釈について2020

    • Author(s)
      高橋和沙, 濱田雄一, 園生雅弘
    • Organizer
      第50回日本臨床神経生理学会学術大会
    • Related Report
      2020 Research-status Report
  • [Presentation] ALSにおけるsplit-finger syndrome2020

    • Author(s)
      園生雅弘, 高橋和沙, 濱田雄一, 北國圭一, 小林俊輔
    • Organizer
      第50回日本臨床神経生理学会学術大会
    • Related Report
      2020 Research-status Report
  • [Presentation] ALS におけるarm/forearm sparing2019

    • Author(s)
      濱田雄一, 高橋和沙, 神谷久雄, 園生雅弘
    • Organizer
      第49回日本臨床神経生理学会学術大会
    • Related Report
      2019 Research-status Report
  • [Presentation] Split finger syndrome in ALS2019

    • Author(s)
      Sonoo M, Takahashi K
    • Organizer
      30th International symposium on ALS/MND
    • Related Report
      2019 Research-status Report
    • Int'l Joint Research

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Published: 2019-04-18   Modified: 2025-01-30  

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