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Multi-disciplinary Approach of Pathophysiology of ALS

Research Project

Project/Area Number 05044161
Research Category

Grant-in-Aid for international Scientific Research

Allocation TypeSingle-year Grants
SectionJoint Research
Research InstitutionKyoto University

Principal Investigator

KIMURA Jun  Department of Neurology, Professor, 医学部, 教授 (10204976)

Co-Investigator(Kenkyū-buntansha) DAUBE J.  米国, メイヨー医科大, 教授
MILLS K.  連合王国, オックスフォード大学・医学部, 講師
EISEN A.  カナダ, ブリティシュコロンビア大学・医学部, 教授
KOHARA Nobuo  Department of Neurology, Assistant, 医学部, 助手 (20252439)
KAJI Ryuji  Department of Neurology, Lecturer, 医学部, 助手 (00214304)
EISEN Andrew  British Colimbia University, Professor
MILLS Kerry  Oxford University, Lecturer
DAUBE Jasper  Mayo Medical School, Professor
SEITERBERGER  ウィーン大学, 教授
JOHN C ROTHW  連合国国立神経病院, 教授
ASAO HIRANO  米国モンテフィオーレ病院, 部長
ANDREW EISEN  カナダブリティシュコロンビア大学, 医学部, 教授
萬年 徹  東京大学, 医学部, 名誉教授 (10010208)
秋口 一郎  京都大学, 医学部, 助教授 (30115779)
Project Period (FY) 1993 – 1994
Project Status Completed (Fiscal Year 1994)
Budget Amount *help
¥7,500,000 (Direct Cost: ¥7,500,000)
Fiscal Year 1994: ¥3,500,000 (Direct Cost: ¥3,500,000)
Fiscal Year 1993: ¥4,000,000 (Direct Cost: ¥4,000,000)
KeywordsALS / multifocal motor neuropathy / fasciculation / familial / central motor program / dystonia / K channel / discharge probability / fasciculation / 大脳磁気刺激 / CNV / ジストニア / シナプトフィジン / リルゾール / グルタミン酸
Research Abstract

We have conducted a series of research projects on pathophysiology of ALS and related disorders as well as a basic study on central motor control, which is expected to help understand ALS.
1) Study of pathophysiology of multifocal motor neuropathy (MMN), a disorder simulating ALS.
In collaboration with Professor J Daube, we examined the effect of anti-GM1 antibody, which is pathognomonic of MMN,on saltatory conduction in single myelinated nerve fibers in vivo.
2) Study of fasciculation in ALS
We found that some fasciculations in ALS can be evoked by magnetic cortical stimulation. Lack of their voluntary activation or H-reflex activation favors their origin in the upper motor neurons.
3) Study of discharge probability of spinal motoneurons in ALS.
We studied the discharge probability of spinal motoneurons using peri-stimulus time histograms (PSTHs). Markedly increased firing probability was found for the direct corticospinal tract-evoked discharges (primary peak). This may indicate that excitotoxicity caused by glutamate may be the cause of these fasciculations.
4) Study on central motor physiology
Using contingent negative variation (CNV), We studied the central motor program in patients with dystonia, and found that a specific motor program in lost in this disease condition. We also found the importance of gamma efferent systems in producing symptoms in this disease.
5) Genetic study of familial ALS
We found Leu 106-Val (CTG-GTC) mutation of superoxide dysmutase gene in a Japanese patient with familial ALS.
6) Study on ion channels in ALS
In collaboration with Drs J Rothwell and Bostock in National Institute of Neurology, Queen Square, London, we developed a clinical test of threshold electrotonus which reveals functions of potassium and sodium channels in nerves. We also confirmed that some patients with ALS have abnormal potassium channels in the internodes.

Report

(2 results)
  • 1994 Final Research Report Summary
  • 1993 Annual Research Report
  • Research Products

    (24 results)

All Other

All Publications (24 results)

  • [Publications] Kaji R et al: "Tonic vibration veflex and muscle attereut block in writer's cramp" Ann Neurol. (in press). (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Kaji R et al.: "Physiological.study of cervicai; dustonia:Task-spccific abnormality in contingent nogative variation" Brain. 118(in press). (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Mezaki T et al.: "Comparison of therapeutic efficacies of types A and F botulinum toxins for blepharospasm" Neurology. 45(in press). (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Watanabe T et al.: "Ultra-high dose methylcobalamin promotes nerve regeueration in experimental acrylamide neuropathy" J.Neurol.Sci. 122. 140-143 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Kaji R et al.: "Anti-GM_1 antibodies and impairey blood-nerve barrier may interfere with remyelination in multifocal motor neu・yiatl." Muscle Nerve. 17. 108-110 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Kawamata J et al: "Leu 106-Val(CTG-GTC) mutation of superoxide dismutuse in a natient with famialinl ALS in Japan" Lancet. 343. 1501 (1994)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Kaji R et al: "Tonic vibration reflex and muscle afferent block in writer's cramp" Ann Neurol. (in press). (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Kaji R et al: "Physiological study on cervical dystonia : task specific abnormality in contingent negative variatio" Brain. 118 (in press). (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Mezaki T et al: "Compalison of therapeutic efficacies of types A and botulinum toxins for blepharospasm" Neurology. 45 (in press). (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Watanabe T et al: "Ultra-high dose methylcobamin promotes nerve regeneration in experimental acrylamide neuropathy" J.Neurol.Sci.122. 140-143 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Kaji R et al: "Anti-GM1 antibodies and impaired bloot-nerve barrior may interfere with remyelination in multifocal motor neruopathy" Muscle Nerve. 17. 108-110 (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Kawamata J et al: "Len 106-Val (CTG-GTC) mutation of superoxide dysmutase in a patient with familial ALS in Japan" Lancet. 343. 1501- (1994)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1994 Final Research Report Summary
  • [Publications] Kaji R.: "Fasciculations evoked by covtical stimulation in amyotrophic lateral sclerosis." Ann Neurol. (in press). (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] Hamano T: "Lack of prolonged cerebral blood flow change after transcranial magnetic stimulation" Electroenceph Clin Neurophysiol. 89. 207-210 (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] Kaji R: "Pathological findings at the site of conduction block in multifocal motor neuropathy." Ann Neurol. 33. 152-158 (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] Kaji R: "Amyotrophic lateral sclerosis:what makes the direct corticospinal tract so vulnerable?" Muscle Nerve. 16. 872-874 (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] Kaji R: "Anti-GM_1 Antibodies and Impaired Blood-Nerve Barvier May Interfere with Remyelination in Multifocal Motor Neuropathy." Muscle Nerve. 17. 108-110 (1994)

    • Related Report
      1993 Annual Research Report
  • [Publications] Hamano T: "Vibration-evoked sensory nerve action potentials derived from Paccinian corpuscles." Electroencephalogr Clin Neurophysiol. 89. 278-286 (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] Kaji R: "Fasciculations evoked by covtical stimulation in amyotrophic lateral sclerosis." Ann Neurol. (in press). (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] Hamano T: "Lack of prolonged cerebral blood flow change after transcranial magnetic stimulation" Electroenceph Clin Neurophysiol. 89. 207-210 (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] Kaji R: "Pathological findings at the site of conduction block in multifocal motor neuropathy" Ann Neurol. 33. 152-158 (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] Kaji R: "Amyotrophic lateral sclerosis:what makes the direct corticospinal tract so vnlnerable?" Muscle Nerve. 16. 872-874 (1993)

    • Related Report
      1993 Annual Research Report
  • [Publications] Kaji R: "Anti-GM_1 Antibodies and Impaired Blood-Nerve Barvier May Interfere with Remyelination in Multifocal Motor Neuropathy." Muscle Nerve. 17. 108-110 (1994)

    • Related Report
      1993 Annual Research Report
  • [Publications] Hamano T: "Vibration-evoked sensory nerve action potentials derived from Paccinian corpuscles." Electroencephalogr Clin Neurophysiol. 89. 278-286 (1993)

    • Related Report
      1993 Annual Research Report

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Published: 1993-04-01   Modified: 2016-04-21  

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