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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.

Original publication

DOI

10.1136/jnnp.2006.099051

Type

Journal article

Journal

J Neurol Neurosurg Psychiatry

Publication Date

11/2006

Volume

77

Pages

1282 - 1283

Keywords

Adult, Aged, Endpoint Determination, Female, Hand Strength, Humans, Male, Middle Aged, Muscle Weakness, Myotonic Dystrophy, Reference Values, Severity of Illness Index, Sex Factors