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  3. Muscle Ultrasound Is A Sensitive Outcome Measure In Als.
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  3. Muscle Ultrasound Is A Sensitive Outcome Measure In Als.

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Published on: August 25, 2022

Muscle Ultrasound Is a Sensitive Outcome Measure in ALS.

Andrew M Hannaford1, Isabella E Supnet2, Nathan Pavey1

  • 1Brain and Nerve Research Centre, Concord Clinical School, University of Sydney, Concord Hospital, Sydney, New South Wales, Australia.

Muscle & Nerve
|June 22, 2026

View abstract on PubMed

Summary
This summary is machine-generated.

Muscle ultrasound echogenicity is a sensitive biomarker for amyotrophic lateral sclerosis (ALS) progression. This method shows greater responsiveness than clinical and neurophysiological measures in ALS clinical trials.

Keywords:
amyotrophic lateral sclerosisdisease progressionechogenicitymuscle ultrasoundoutcome measures

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Area of Science:

  • Neurology
  • Biomarkers
  • Medical Imaging

Background:

  • Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease.
  • Muscle ultrasound is a developing outcome measure for ALS.
  • Prospective, multicenter longitudinal studies are needed to validate its use.

Purpose of the Study:

  • To evaluate muscle ultrasound as an outcome measure in ALS.
  • To compare the sensitivity of muscle ultrasound with clinical and neurophysiological metrics.

Main Methods:

  • Prospective, two-center cohort study of adults with ALS.
  • Clinical assessments included ALS Functional Rating Scale-Revised (ALSFRS-R) and MRC sum scores.
  • Neurophysiological measures involved median and ulnar nerve compound motor action potential (CMAP) amplitudes.
  • Muscle ultrasound of 11 muscles analyzed thickness and echogenicity using harmonized protocols.

    • Main Results:

    • Muscle echogenicity showed the largest effect size (1.09), indicating significant ALS progression.
    • ALSFRS-R declined (-0.7%/month, effect size 0.84) and CMAP amplitude decreased (-1.2%/month, effect size 0.77).
    • Onset-specific muscle selection, particularly biceps brachii and gastrocnemius, enhanced ultrasound responsiveness (effect sizes 1.12 and 1.18).

    Conclusions:

    • Muscle ultrasound echogenicity is a sensitive structural biomarker for ALS progression.
    • It demonstrates greater responsiveness than ALSFRS-R and CMAP over 3-12 months.
    • Its accessibility and sensitivity support its use as an outcome measure in ALS clinical trials.