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• W4387302595 abstract "Becker Muscular Dystrophy (BMD) results in impaired function of dystrophin and leads to fat replacement of skeletal muscle impacting function. There is a growing interest in drug development for BMD, and biomarkers that characterize muscle quality and their relationships to function are needed. Quantitative magnetic resonance (qMR) imaging of muscle fat fraction (FF) is highly reliable and sensitive to disease progression in muscular dystrophy. Whole-body quantitative three-point Dixon (3PD) imaging (WBI) has efficient acquisition and the ability to analyze multiple muscles to develop body segment-specific FF composites. Using WBI qMR, we aimed to 1) characterize skeletal muscle using FF composites, and 2) identify relationships among FF composites and function in men with BMD. We hypothesized that FF composites of the upper (UE) and lower (LE) extremity and the trunk would be moderately correlated with the Performance of Upper Limb (PUL), North Star Assessment for Dysferlinopathy (NSAD), and pulmonary function tests (PFTs), respectively. In 29 men with BMD (18 to 61 years), we measured FF composites of the muscles of the right proximal UE (deltoid and biceps brachii), LE (gluteus maximus, gluteus medius/minimus, psoas, quadriceps femoris, and hamstrings), and trunk (rectus abdominus, obliques, and paraspinals). Relationship between FF and function was assessed using the Spearman Rank correlation coefficient (rs). Men with BMD had FF composites ranging from 9% to 78% (median: 20%) for UE, 7% to 81% (median: 57%) for LE, and 9% to 76% (median: 41%) for trunk. Relationships were identified between UE FF composite and PUL scores (rs=-0.76; p<0.001), LE FF composite and NSAD scores (rs=-0.93; p<0.001), and trunk FF composite and PFTs (forced vital capacity [FVC], forced expiratory volume [FEV1], and peak cough flow [PCF]; rs=-0.47 to -0.48; p=0.03). Body segment-specific relationships with function suggest that WBI qMR FF is a meaningful biomarker in men with BMD. Becker Muscular Dystrophy (BMD) results in impaired function of dystrophin and leads to fat replacement of skeletal muscle impacting function. There is a growing interest in drug development for BMD, and biomarkers that characterize muscle quality and their relationships to function are needed. Quantitative magnetic resonance (qMR) imaging of muscle fat fraction (FF) is highly reliable and sensitive to disease progression in muscular dystrophy. Whole-body quantitative three-point Dixon (3PD) imaging (WBI) has efficient acquisition and the ability to analyze multiple muscles to develop body segment-specific FF composites. Using WBI qMR, we aimed to 1) characterize skeletal muscle using FF composites, and 2) identify relationships among FF composites and function in men with BMD. We hypothesized that FF composites of the upper (UE) and lower (LE) extremity and the trunk would be moderately correlated with the Performance of Upper Limb (PUL), North Star Assessment for Dysferlinopathy (NSAD), and pulmonary function tests (PFTs), respectively. In 29 men with BMD (18 to 61 years), we measured FF composites of the muscles of the right proximal UE (deltoid and biceps brachii), LE (gluteus maximus, gluteus medius/minimus, psoas, quadriceps femoris, and hamstrings), and trunk (rectus abdominus, obliques, and paraspinals). Relationship between FF and function was assessed using the Spearman Rank correlation coefficient (rs). Men with BMD had FF composites ranging from 9% to 78% (median: 20%) for UE, 7% to 81% (median: 57%) for LE, and 9% to 76% (median: 41%) for trunk. Relationships were identified between UE FF composite and PUL scores (rs=-0.76; p<0.001), LE FF composite and NSAD scores (rs=-0.93; p<0.001), and trunk FF composite and PFTs (forced vital capacity [FVC], forced expiratory volume [FEV1], and peak cough flow [PCF]; rs=-0.47 to -0.48; p=0.03). Body segment-specific relationships with function suggest that WBI qMR FF is a meaningful biomarker in men with BMD." @default.
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• W4387302595 date "2023-10-01" @default.
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• W4387302595 title "P132 Quantifying skeletal muscle fat fraction and function using whole body magnetic resonance imaging (MRI) in men with Becker muscular dystrophy" @default.
• W4387302595 doi "https://doi.org/10.1016/j.nmd.2023.07.067" @default.
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