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强直性肌营养不良:与肌力评估相关的实际问题。

Myotonic dystrophy: practical issues relating to assessment of strength.

作者信息

Whittaker R G, Ferenczi E, Hilton-Jones D

机构信息

Mitochondrial Research Group, School of Neurology, Neurobiology and Psychiatry, University of Newcastle upon Tyne, Framlington Place, Newcastle upon Tyne NE2 4HH, UK.

出版信息

J Neurol Neurosurg Psychiatry. 2006 Nov;77(11):1282-3. doi: 10.1136/jnnp.2006.099051.

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.

摘要

背景

1型强直性肌营养不良症是一种缓慢进展的多系统疾病,其中骨骼肌受累较为突出。随着新型物理和药物治疗方法的出现,准确测量其疗效至关重要。

方法

在一家专业肌肉诊所,每年对158例连续性强直性肌营养不良症患者进行评估。使用医学研究委员会(MRC)量表和手持测力计测量肌力。从108名正常受试者(对照组)获取测力计读数。

结果

肌力变化率最大的动作是踝背屈和捏力。在MRC量表上,这两个动作每年仅下降0.06分。使用手持测力计,检测到女性肌力每年变化1.18千克牛顿,男性为每年1.61千克牛顿。

结论

MRC量表不适用于检测强直性肌营养不良症等缓慢进展性疾病中出现的微小肌力变化。测力法提供了一种简单的替代方法,可在典型临床试验期间提供有意义的数据。

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本文引用的文献

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Aerobic training in patients with myotonic dystrophy type 1.
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