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• W4214697281 abstract "Quantitative MRI (qMRI) of skeletal muscles is a promising method to objectively assess muscle involvement in Duchenne muscular dystrophy (DMD) and a potential outcome measure in clinical trials. qMRI data of the upper extremity (UE) muscles in non-ambulant DMD patients are limited. Therefore, we longitudinally assessed qMRI and UE functional outcome measures in this stage of the disease. 4-point Dixon MRI scans (3T) of the right upper arm, performance of the upper limb (PUL) version 2.0 and elbow flexion and grip strength were measured at baseline, 12 and 18 months follow-up. Whole muscle contractile volume (CV) and mean weighed fat fraction (FFw) and center slice contractile cross-sectional area (cCSA) and fat fraction (FFc) were obtained from the UE flexor muscles (m. biceps brachii and m. brachialis). Correlations between qMRI variables and function outcomes at baseline were assessed with Spearman's rank coefficient. Differences between baseline and follow-up were compared using Wilcoxon signed rank test. Data were obtained for 20 boys at baseline (median age 13.4y), 15 at 12 months (median age 14.5y) and 11 at 18 months (median age 14.9y). CV and cCSA showed strong correlations with PUL2.0 (ρ 0.891; ρ 0.839) and elbow flexion strength (ρ 0.950; ρ 0.948) at baseline. For FFw and FFc these correlations were ρ -0.667; ρ -0.659 and ρ -0.671; ρ -0.704 respectively. PUL2.0 and elbow flexion strength showed a median decline of 3 points (range -10.0 – 0.0; p=0.005) and 4.5 N (range -9.3 – +3.6; p=0.013) over 18 months respectively. CV decreased by 7.1 cm3 (range -20.0 – +6.3; p=0.013) and FFw increased by 9.5% (range 1.7 – 24.3; p=0.003) over 18 months, where cCSA decreased by -75.8mm2 (range -296.5 – +113.0; p=0.131) and FFc increased by 6.7% (range 1.1 – 21.7; p=0.003) during this interval. Both functional measures and qMRI variables CV, FFm and FFc measured significant disease progression over 18 months. cCSA did not show a significant decline, likely due to the larger range in changes over time in this single slice based assessment. Both CV and cCSA correlated more strongly to elbow flexion strength and PUL2.0 than FF parameters. These results support the use of qMRI in UE muscles in non-ambulant DMD patients." @default.
• W4214697281 created "2022-03-02" @default.
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• W4214697281 date "2021-10-01" @default.
• W4214697281 modified "2023-09-24" @default.
• W4214697281 title "IMAGING" @default.
• W4214697281 doi "https://doi.org/10.1016/j.nmd.2021.07.361" @default.
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