Résumé :
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In the Natural History of Calpainopathies study (F Fougerousse's communication), 37 patients were subjected to Computed Tomography (CT) scans of lower limbs at the beginning of the investigation and 2 years later. The objective was to quantify muscle loss for a better characterization of the muscle damage specificity known in Limb Girdle Muscle Dystrophy and its progression over two years. We applied a methodology based on manual determination of Regions Of Interest (ROI) of individual muscles on 4 cross sections per patient. Cross sectional areas and mean pixel intensity were obtained for each muscle enabling respectively the calculation of the level of atrophy and the degree of adiposity in muscle which is measured using changes in density based on Hounsfield unit. Through this approach, we could recover the proximal pattern of muscle deficiency and were able to correlate it with the muscle strength data (JY Hogrel's communication). Most importantly, we showed evidence of a significant decrease of density in most lower limb muscles in all patients over two years. This study indicates that CT-scans can be a valuable tool for a precise follow-up of muscle disease and could be useful in the context of a clinical assay. Presentation of the Calpain Study Group Promoter - Généthon : P Borel, N Bousquet, F Fougerousse, E Laurent, I Richard, D Stockholm Centre 1 - Institute of Myology : A Canal, B Eymard, V Doppler, M Fardeau, J Y Hogrel, P Laforêt, G Ollivier, C Payan, A Urtizberea Centre 2 - Hospital San Sebastian : V Bahon, A Lopez de Munain, JJ Pozza, D Salicio Centre 3 - Hospital Saint Pierre : la Reunion. JM Begue, P Boué, C Mignard
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