Neurotherapeutics

, Volume 14, Issue 1, pp 154–160

Strength Testing in Motor Neuron Diseases

Review

DOI: 10.1007/s13311-016-0472-0

Cite this article as:
Shefner, J.M. Neurotherapeutics (2017) 14: 154. doi:10.1007/s13311-016-0472-0

Abstract

Loss of muscle strength is a cardinal feature of all motor neuron diseases. Functional loss over time, including respiratory dysfunction, inability to ambulate, loss of ability to perform activities of daily living, and others are due, in large part, to decline in strength. Thus, the accurate measurement of limb muscle strength is essential in therapeutic trials to best understand the impact of therapy on vital function. While qualitative strength measurements show declines over time, the lack of reproducibility and linearity of measurement make qualitative techniques inadequate. A variety of quantitative measures have been developed; all have both positive attributes and limitations. However, with careful training and reliability testing, quantitative measures have proven to be reliable and sensitive indicators of both disease progression and the impact of experimental therapy. Quantitative strength measurements have demonstrated potentially important therapeutic effects in both amyotrophic lateral sclerosis and spinobulbar muscular atrophy, and have been shown feasible in children with spinal muscular atrophy. The spectrum of both qualitative and quantitative strength measurements are reviewed and their utility examined in this review.

Keywords

Motor neuron disease Spinal muscular atrophy Manual muscle testing TQNE Hand held dynamometry 

Supplementary material

13311_2016_472_MOESM1_ESM.pdf (1.2 mb)
ESM 1(PDF 1225 kb)

Copyright information

© The American Society for Experimental NeuroTherapeutics, Inc. 2016

Authors and Affiliations

  1. 1.Department of NeurologyBarrow Neurological InstitutePhoenixUSA

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