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Myotonic dystrophy: Practical issues relating to assessment of strength

Lookup NU author(s): Professor Roger Whittaker


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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.

Publication metadata

Author(s): Whittaker RG, Ferenczi E, Hilton-Jones D

Publication type: Article

Publication status: Published

Journal: Journal of Neurology, Neurosurgery and Psychiatry

Year: 2006

Volume: 77

Issue: 11

Pages: 1282-1283

ISSN (print): 0022-3050

ISSN (electronic): 1468-330X

Publisher: BMJ Group


DOI: 10.1136/jnnp.2006.099051

PubMed id: 17043296


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