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As muscle contracts, the overlap between the thin and thick filaments increases, decreasing the length of the sarcomere—the contractile unit of the muscle—using energy in the form of ATP. At the molecular level, this is a cyclic, multistep process that involves binding and hydrolysis of ATP, and movement of actin by myosin.
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Related Experiment Video

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In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
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Cortical function and corticomotoneuronal adaptation in monomelic amyotrophy.

José Manuel Matamala1, Nimeshan Geevasinga2, William Huynh3

  • 1Brain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, NSW 2050, Australia.

Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology
|June 19, 2017
PubMed
Summary

Threshold tracking transcranial magnetic stimulation (TT-TMS) reveals normal cortical function in monomelic amyotrophy, differentiating it from early-onset amyotrophic lateral sclerosis (ALS). This suggests adaptive changes in the central nervous system for monomelic amyotrophy patients.

Keywords:
Amyotrophic lateral sclerosisCorticomotoneuronal plasticityMonomelic amyotrophyMotor evoked potentialsShort-interval intracortical inhibitionThreshold tracking transcranial magnetic stimulation

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Area of Science:

  • Neuroscience
  • Neurology
  • Neurophysiology

Background:

  • Monomelic amyotrophy (MMA) is a rare motor neuron disease with unclear pathophysiology.
  • Distinguishing MMA from other motor neuron diseases like amyotrophic lateral sclerosis (ALS) is crucial for accurate diagnosis and management.

Purpose of the Study:

  • To investigate corticomotoneuronal integrity in MMA using threshold tracking transcranial magnetic stimulation (TT-TMS).
  • To differentiate MMA from early-onset ALS by assessing cortical excitability.

Main Methods:

  • Prospective study involving 8 MMA patients, 21 early-onset ALS patients, and 40 healthy controls.
  • Cortical excitability assessed using TT-TMS, recording motor evoked potentials (MEPs) over abductor pollicis brevis.
  • Analyzed MEP/CMAP ratio and short-interval intracortical inhibition (SICI).

Main Results:

  • Monomelic amyotrophy patients showed a significantly increased MEP/CMAP ratio compared to controls.
  • Short-interval intracortical inhibition (SICI) was similar in MMA patients and controls.
  • SICI was significantly reduced in early-onset ALS patients compared to MMA patients, indicating SICI as a potential discriminator.

Conclusions:

  • TT-TMS demonstrates normal cortical function in MMA, distinguishing it from early-onset ALS.
  • Enhanced corticomotoneuronal projections may represent adaptive changes in the central nervous system in MMA.
  • Corticomotoneuronal dysfunction is unlikely to be the primary cause of lower motor neuron loss in MMA.