Magnetic resonance imaging of the proximal upper extremity musculature in boys with Duchenne muscular dystrophy.
Magnetic resonance imaging of the proximal upper extremity musculature in boys with Duchenne muscular dystrophy.
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DOI：
10.1007/s00415-016-8311-0
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发表时间：
2017-01
影响因子：
6
通讯作者：
Vandenborne K
中科院分区：
文献类型：
--
作者：
Willcocks RJ;Triplett WT;Forbes SC;Arora H;Senesac CR;Lott DJ;Nicholson TR;Rooney WD;Walter GA;Vandenborne K
There is a pressing need for biomarkers and outcomes that can be used across disease stages in Duchenne muscular dystrophy (DMD), to facilitate the inclusion of a wider range of participants in clinical trials and to improve our understanding of the natural history of DMD. Quantitative magnetic resonance imaging (qMRI) and spectroscopy (MRS) biomarkers show considerable promise in both the legs and forearms of individuals with DMD, but have not yet been examined in functionally important proximal upper extremity muscles such as the biceps brachii and deltoid. The primary objective of this study was to examine the feasibility of implementing qMRI and MRS biomarkers in the proximal upper extremity musculature, and the secondary objective was to examine the relationship between MR measures of arm muscle pathology and upper extremity functional endpoints. Biomarkers included MRS and MRI measures of fat fraction and transverse relaxation time (T2). The MR exam was well tolerated in both ambulatory and nonambulatory boys. qMR biomarkers differentiated affected and unaffected participants and correlated strongly with upper extremity function (r=0.91 for biceps brachii T2 versus Performance of Upper Limb score). These qMR outcome measures could be highly beneficial to the neuromuscular disease community, allowing measurement of the quality of functionally important muscles across disease stages to understand the natural history of DMD and particularly to broaden the opportunity for clinical trial participation.
