1. Upper and Lower Extremities in Duchenne Muscular Dystrophy Evaluated with Quantitative MRI and Proton MR Spectroscopy in a Multicenter Cohort.
- Author
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Forbes SC, Arora H, Willcocks RJ, Triplett WT, Rooney WD, Barnard AM, Alabasi U, Wang DJ, Lott DJ, Senesac CR, Harrington AT, Finanger EL, Tennekoon GI, Brandsema J, Daniels MJ, Sweeney HL, Walter GA, and Vandenborne K
- Subjects
- Adolescent, Case-Control Studies, Child, Cohort Studies, Cross-Sectional Studies, Disease Progression, Feasibility Studies, Humans, Male, Outcome Assessment, Health Care, Prospective Studies, Arm diagnostic imaging, Leg diagnostic imaging, Magnetic Resonance Imaging methods, Muscle, Skeletal diagnostic imaging, Muscular Dystrophy, Duchenne diagnostic imaging, Proton Magnetic Resonance Spectroscopy methods
- Abstract
Background Upper extremity MRI and proton MR spectroscopy are increasingly considered to be outcome measures in Duchenne muscular dystrophy (DMD) clinical trials. Purpose To demonstrate the feasibility of acquiring upper extremity MRI and proton (
1 H) MR spectroscopy measures of T2 and fat fraction in a large, multicenter cohort (ImagingDMD) of ambulatory and nonambulatory individuals with DMD; compare upper and lower extremity muscles by using MRI and1 H MR spectroscopy; and correlate upper extremity MRI and1 H MR spectroscopy measures to function. Materials and Methods In this prospective cross-sectional study, MRI and1 H MR spectroscopy and functional assessment data were acquired from participants with DMD and unaffected control participants at three centers (from January 28, 2016, to April 24, 2018). T2 maps of the shoulder, upper arm, forearm, thigh, and calf were generated from a spin-echo sequence (repetition time msec/echo time msec, 3000/20-320). Fat fraction maps were generated from chemical shift-encoded imaging (eight echo times). Fat fraction and1 H2 O T2 in the deltoid and biceps brachii were measured from single-voxel1 H MR spectroscopy (9000/11-243). Groups were compared by using Mann-Whitney test, and relationships between MRI and1 H MR spectroscopy and arm function were assessed by using Spearman correlation. Results This study evaluated 119 male participants with DMD (mean age, 12 years ± 3 [standard deviation]) and 38 unaffected male control participants (mean age, 12 years ± 3). Deltoid and biceps brachii muscles were different in participants with DMD versus control participants in all age groups by using quantitative T2 MRI ( P < .001) and1 H MR spectroscopy fat fraction ( P < .05). The deltoid, biceps brachii, and triceps brachii were affected to the same extent ( P > .05) as the soleus and medial gastrocnemius. Negative correlations were observed between arm function and MRI (T2: range among muscles, ρ = -0.53 to -0.73 [ P < .01]; fat fraction, ρ = -0.49 to -0.70 [ P < .01]) and1 H MR spectroscopy fat fraction (ρ = -0.64 to -0.71; P < .01). Conclusion This multicenter study demonstrated early and progressive involvement of upper extremity muscles in Duchenne muscular dystrophy (DMD) and showed the feasibility of MRI and1 H MR spectroscopy to track disease progression over a wide range of ages in participants with DMD. © RSNA, 2020 Online supplemental material is available for this article.- Published
- 2020
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