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Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
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Strength Testing in Motor Neuron Diseases.

Jeremy M Shefner1

  • 1Department of Neurology, Barrow Neurological Institute, Phoenix, AZ, 85013, USA. Jeremy.shefner@dignityhealth.org.

Neurotherapeutics : the Journal of the American Society for Experimental Neurotherapeutics
|September 8, 2016
PubMed
Summary
This summary is machine-generated.

Accurate measurement of limb muscle strength is crucial for motor neuron disease trials. Quantitative strength measures are reliable indicators of disease progression and therapeutic impact.

Keywords:
Hand held dynamometryManual muscle testingMotor neuron diseaseSpinal muscular atrophyTQNE

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Area of Science:

  • Neurology
  • Rehabilitation Medicine
  • Biomedical Engineering

Background:

  • Muscle strength loss is a primary characteristic of motor neuron diseases (MNDs).
  • Functional decline in MNDs, including respiratory and mobility issues, is largely attributed to diminished muscle strength.
  • Accurate strength measurement is vital for assessing therapeutic efficacy in clinical trials.

Purpose of the Study:

  • To review qualitative and quantitative methods for measuring limb muscle strength in motor neuron diseases.
  • To examine the utility and limitations of various strength measurement techniques.
  • To highlight the importance of reliable strength assessment in therapeutic trials for MNDs.

Main Methods:

  • Review of existing literature on qualitative and quantitative strength measurement techniques.
  • Analysis of the reproducibility, linearity, and sensitivity of different measurement approaches.
  • Examination of data from clinical trials involving amyotrophic lateral sclerosis (ALS), spinobulbar muscular atrophy (SBMA), and spinal muscular atrophy (SMA).

Main Results:

  • Qualitative strength measurements lack reproducibility and linearity, making them inadequate for tracking disease progression or therapeutic effects.
  • Quantitative strength measurements, with proper training and reliability testing, are reliable and sensitive indicators.
  • Quantitative measures have shown potential therapeutic effects in ALS and SBMA and are feasible in pediatric SMA.

Conclusions:

  • Quantitative limb muscle strength measurement is essential for evaluating disease progression and therapeutic interventions in motor neuron diseases.
  • Reliable quantitative methods are critical for the successful design and execution of clinical trials for MNDs.
  • The careful application of quantitative strength assessment provides valuable insights into disease dynamics and treatment outcomes.