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Quadriceps strength and timed motor performances in myotonic dystrophy, Charcot–Marie–Tooth disease, and healthy subjects

Department of Rehabilitation, University Hospital Utrecht/Rehabilitation Centre ‘de Hoogstraat’, The Netherlands

Pieters Leffers

Department of Epidemiology, University of Maastricht, The Netherlands

Jos Reulen

Frank Spaans

Department of Clinical Neurophysiology, University Hospital Maastricht, The Netherlands

Jan Drukker

Department of Anatomy and Embryology, University of Maastricht, The Netherlands

Background and purpose: The leading hypothesis was that a relation exists between muscular strength and functional abilities. Therefore a study was undertaken to quantify such a relationship in a population of subjects with different muscular strengths. This population consisted of healthy subjects and subjects with slowly progressive neuromuscular disorders.

Methods: The study included 33 patients with myotonic dystrophy, 29 patients with Charcot–Marie–Tooth disease and 20 healthy subjects. Isokinetic and isometric knee torques were measured on an isokinetic dynamometer at various velocities. The following activities were timed: descending and ascending stairs, rising from a chair, rising from supine, walking at natural speed and walking at maximum speed.

Results: The population covered a wide range of the variables: whereas the healthy subjects performed best (i.e. had the highest knee torques and performed the activities most quickly), the myotonic dystrophy group included the subjects with the lowest knee torques. The natural logarithms (ln) of isokinetic extension torque at the highest velocity (120°/s) and those of the time taken to perform the described activities showed the highest levels of correlation. It was found that after correction for age and weight, 56% (walking at natural speed) to 73% (descending stairs) of the variance in the ln of the time taken could be attributed to the variance in the ln of the torques.

Conclusion and discussion: A strong relation between quadriceps strength and timed motor performances were demonstrated. The impact of strength reduction on time taken was most obvious in subjects with considerably decreased strength. Therefore, it is feasible to try to influence muscle strength in patients with relevant strength reduction in order to achieve better functional ability.

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