Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Clinical Trial
. 2006 Nov;77(11):1282-3.
doi: 10.1136/jnnp.2006.099051.

Myotonic dystrophy: practical issues relating to assessment of strength

R G Whittaker  1 , E Ferenczi, D Hilton-Jones
Affiliations
Clinical Trial

Myotonic dystrophy: practical issues relating to assessment of strength

R G Whittaker et al. J Neurol Neurosurg Psychiatry. 2006 Nov.

Abstract

Background: Myotonic dystrophy type 1 is a slowly progressive multisystem disease in which skeletal muscle involvement is prominent. As novel physical and pharmacological treatments become available, it is crucial to be able to measure their efficacy accurately.

Methods: 158 consecutive patients with myotonic dystrophy were assessed annually in a specialist muscle clinic. Strength was measured using both the Medical Research Council (MRC) scale and a hand-held dynamometer. Dynamometer readings were obtained from 108 normal subjects (controls).

Results: The movements showing the greatest rate of change in strength were ankle dorsiflexion and pinch grip. Both of these showed a decline of only 0.06 points/year on the MRC scale. Using a hand-held dynamometer, a change in strength of 1.18 kgN/year for women and 1.61 kgN/year for men was detected.

Conclusions: The MRC scale is unsuitable for detecting the small changes in strength seen in a slowly progressive disease such as myotonic dystrophy. Dynamometry provides a simple alternative that can give meaningful data over the duration of a typical clinical trial.

PubMed Disclaimer

Conflict of interest statement

Competing interests: None.

References

    1. Harper P S.Myotonic dystrophy. 3rd edn. London: Saunders, 2001
    1. Harper P S, van Engelen B, Eymard B.et alMyotonic dystrophy: present management future therapy. Oxford: Oxford University Press, 2004 - PubMed
    1. Orngreen M C, Olsen D B, Vissing J. Aerobic training in patients with myotonic dystrophy type 1. Ann Neurol 200557754–757. - PubMed
    1. Tollback A, Eriksson S, Wredenberg A.et al Effects of high resistance training in patients with myotonic dystrophy. Scand J Rehabil Med 1999319–16. - PubMed
    1. Mathieu J, Boivin H, Richards C L. Quantitative motor assessment in myotonic dystrophy. Can J Neurol Sci 200330129–136. - PubMed

Publication types