Practical analysis of variability of muscle function measurements in duchenne muscular dystrophy

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Abstract
To determine the possible sources of variation in performance indicators used in therapeutic trials, electrical stimulation techniques were used to measure contractile properties of the adductor pollicis and quadriceps muscles in boys with Duchenne muscular dystrophy. As no therapeutic effects were observed, longitudinal data obtained are taken to indicate changes in disease progress. Variance in voluntary contractions was found to be similar to that with electrically stimulated contractions; thus, variation could not be attributed to motivational changes, but rather to physiologic changes. Dystrophic muscle was slower to relax and less fatiguable than normal. However, such changes are of less significance to the overall disability compared to the loss of muscle bulk (cross-sectional area). Important variations in the function of individual muscles essential to complex performance, such as walking or getting up from the floor, could be masked by combining results from several muscle groups.