Résumé :
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Assessment of the neuromuscular function during the time course of neuromuscular disorders is much informative for patient follow-up either during the natural history of his disease or during a therapeutic intervention. Within this last frame, the choice of the evaluation criteria is crucial for many reasons. Many methods exist to assess the various aspects of the neuromuscular function. This was the aim of this study to perform and to analyze a selection of different tests for the follow-up of patients during the natural history of their disease. This study is a small part of a larger multicentric study about a multi-parametric approach to natural history knowledge of calpainopathies for efficacy assessment of therapies. Twenty one patients suffering from LGMD 2A (calpainopathy) were followed every six months during two years. Each patient visit included Manuel Muscle Testing (MMT), Quantified Muscle Testing (QMT), Muscle Function Measure (MFM), isometric dynamometry (Biodex) and timed functional tests. Elbow and knee flexion and extension were particularly analyzed. Their reproducibility and variability were assessed. Correlations between the measurements obtained using different methods were analysed. This study revealed that the results obtained with QMT or Biodex were not always highly correlated and may differ depending on the muscle function studied. For instance, the evaluation of elbow and knee extensions seems to be much more reliable than the evaluation of elbow and knee flexions for this patient population. Moreover, both QMT and Biodex were unsuitable to detect small strengths in very weak patients. From longitudinal data, it was possible to compute percentage of variations to be considered significant of true strength changes. Natural history of disorders may help to identify the muscle function to be studied in further therapeutic trials and to provide the amount of change significant of a true evolution.
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