Myotonic dystrophy: practical issues relating to assessment of strength.

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 wi...

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Main Authors: Whittaker, R, Ferenczi, E, Hilton-Jones, D
Format: Journal article
Language:English
Published: 2006
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author Whittaker, R
Ferenczi, E
Hilton-Jones, D
author_facet Whittaker, R
Ferenczi, E
Hilton-Jones, D
author_sort Whittaker, R
collection OXFORD
description 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.
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spelling oxford-uuid:9ea4f19a-1bae-4165-9d1a-5f866573f0412022-03-27T00:51:25ZMyotonic dystrophy: practical issues relating to assessment of strength.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:9ea4f19a-1bae-4165-9d1a-5f866573f041EnglishSymplectic Elements at Oxford2006Whittaker, RFerenczi, EHilton-Jones, D 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.
spellingShingle Whittaker, R
Ferenczi, E
Hilton-Jones, D
Myotonic dystrophy: practical issues relating to assessment of strength.
title Myotonic dystrophy: practical issues relating to assessment of strength.
title_full Myotonic dystrophy: practical issues relating to assessment of strength.
title_fullStr Myotonic dystrophy: practical issues relating to assessment of strength.
title_full_unstemmed Myotonic dystrophy: practical issues relating to assessment of strength.
title_short Myotonic dystrophy: practical issues relating to assessment of strength.
title_sort myotonic dystrophy practical issues relating to assessment of strength
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AT ferenczie myotonicdystrophypracticalissuesrelatingtoassessmentofstrength
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