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Changes in motor unit numbers in patients with ALS: a longitudinal study using the adapted multiple point stimulation

F C Wang1, O Bouquiaux, V De Pasqua

  • 1University Department of Physical Medicine and Rehabilitation, Hôpital du Sart Tilman, Liège, Belgium. fc.wang@chu.ulg.ac.be

Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders : Official Publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases
|June 14, 2002
PubMed
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The adapted multiple point stimulation (AMPS) method effectively tracks motor unit number (MUNE) and compound muscle action potential (CMAP) changes in amyotrophic lateral sclerosis (ALS) patients over time. This technique helps differentiate disease progression rates and informs treatment monitoring.

Area of Science:

  • Neurology
  • Electrophysiology

Background:

  • Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons.
  • Monitoring disease progression and treatment effects in ALS is crucial for patient management.

Purpose of the Study:

  • To evaluate the utility of the adapted multiple point stimulation (AMPS) method for quantifying changes in motor unit number (MUNE) and related electrophysiological parameters in ALS patients undergoing riluzole therapy.
  • To assess the correlation between MUNE, compound muscle action potential (CMAP) size, and surface motor unit potential (SMUP) size over a 12-month period.

Main Methods:

  • The AMPS method was employed to measure MUNE, CMAP size, and SMUP size in 12 ALS patients.
  • Measurements were taken at baseline (T(0)) and at 4, 8, and 12 months after initiating riluzole treatment.

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  • Patients were categorized into two groups based on the rate of motor unit loss at 4 months.

    • Main Results:

    • A significant decrease in thenar MUNE and CMAP size was observed over the 12 months, while SMUP size remained stable.
    • Patients with rapid motor unit loss (>20% at 4 months) showed a dramatic reduction in CMAP size compared to those with slower loss.
    • Percent changes in MUNE and CMAP size were significantly different between the rapid and slow motor unit loss groups.

    Conclusions:

    • The AMPS technique is valuable for documenting longitudinal changes in MUNE, SMUP size, and CMAP size in ALS patients.
    • AMPS can help differentiate disease progression rates and may aid in monitoring therapeutic responses.
    • The study highlights the utility of AMPS in clinical trials and patient management for ALS.