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ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
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Lower motor neuron dysfunction in ALS.

Mamede de Carvalho1, Michael Swash2

  • 1Department of Neurosciences, Hospital de Santa Maria-Centro Hospitalar de Lisboa Norte, Lisbon, Portugal; Institute of Physiology, Faculty of Medicine, Instituto de Medicina Molecular, University of Lisbon, Portugal.

Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology
|April 28, 2016
PubMed
Summary
This summary is machine-generated.

Lower motor neuron (LMN) loss in motor neuron disease/amyotrophic lateral sclerosis (MND/ALS) can be estimated using compound muscle action potential (CMAP) measures. Fasciculation potentials (FPs) indicate early LMN hyperexcitability and are crucial for ALS diagnosis.

Keywords:
Amyotrophic lateral sclerosisElectromyographyFasciculation potentialsLower motor neuronMotor neuron disease

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Area of Science:

  • Neuroscience
  • Motor Neuron Physiology
  • Electromyography

Background:

  • The motor system involves complex interactions between cortical structures and spinal cord lower motor neurons (LMNs).
  • LMN loss is a key indicator of motor neuron disease/amyotrophic lateral sclerosis (MND/ALS).
  • Conventional needle electromyography (EMG) cannot quantify LMN loss.

Purpose of the Study:

  • To explore neurophysiological markers for quantifying LMN loss and assessing reinnervation in MND/ALS.
  • To highlight the significance of fasciculation potentials (FPs) and motor unit potential analysis in electrodiagnosis.
  • To evaluate the role of LMN excitability studies and emerging techniques in understanding disease progression and treatment efficacy.

Main Methods:

  • Measurement of compound muscle action potential (CMAP) amplitude/area and neurophysiological index to estimate functional motor units.
  • Analysis of fasciculation potentials (FPs) as an early indicator of LMN hyperexcitability.
  • Utilizing needle EMG for investigating reinnervation and its temporal profile; quantitative motor unit potential analysis.
  • Considering advanced techniques like electrical impedance myography, ultrasound, and spinal cord imaging.

Main Results:

  • CMAP amplitude/area and neurophysiological index serve as surrogate estimates for functional motor units.
  • Fasciculation potentials (FPs) represent the earliest observable changes in affected muscles, signifying LMN hyperexcitability.
  • Needle EMG provides crucial data on the temporal dynamics of reinnervation.
  • FPs are recognized in the Awaji criteria, enhancing the electrodiagnosis of ALS.

Conclusions:

  • Quantitative neurophysiological measures, including CMAP analysis and FPs, are vital for assessing LMN loss and reinnervation in ALS.
  • Fasciculation potentials are critical early markers of LMN hyperexcitability and are integral to ALS diagnostic criteria.
  • Further research into LMN functionality, excitability, and novel imaging techniques may advance understanding and treatment of MND/ALS.