Lower limb muscle impairment in myotonic dystrophy type 1: the need for better guidelines

Muscle Nerve. 2015 Apr;51(4):473-8. doi: 10.1002/mus.24521. Epub 2015 Feb 25.

Abstract

In myotonic dystrophy type 1 (DM1), leg muscle weakness is a major impairment. There are challenges to obtaining a clear portrait of muscle strength impairment. A systematic literature review was conducted on lower limb strength impairment in late-onset and adult phenotypes to document variables which affect strength measurement. Thirty-two articles were reviewed using the COSMIN guidelines. Only a third of the studies described a reproducible protocol. Only 2 muscle groups have documented reliability for quantitative muscle testing and only 1 total score for manual muscle testing. Variables affecting muscle strength impairment are not described in most studies. This review illustrates the variability in muscle strength assessment in relation to DM1 characteristics and the questionable validity of the results with regard to undocumented methodological properties. There is therefore a clear need to adopt a consensus on the use of a standardized muscle strength assessment protocol.

Keywords: Lower Extremity; Muscle Strength; Myotonic Dystrophy; Outcome Measure; Physiotherapy.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Animals
  • Humans
  • Lower Extremity / innervation
  • Lower Extremity / physiopathology
  • Muscle Strength / physiology*
  • Muscle Weakness / physiopathology*
  • Muscle, Skeletal / innervation
  • Muscle, Skeletal / physiopathology
  • Myotonic Dystrophy / diagnosis
  • Myotonic Dystrophy / physiopathology
  • Myotonic Dystrophy / therapy*
  • Practice Guidelines as Topic*