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Multi-parametric MR in Becker muscular dystrophy patients.
NMR in Biomedicine ( IF 2.7 ) Pub Date : 2020-08-05 , DOI: 10.1002/nbm.4385
Melissa T Hooijmans 1, 2 , Martijn Froeling 3 , Zaida Koeks 4 , Jan J G M Verschuuren 4, 5 , Andrew Webb 1 , Erik H Niks 4, 5 , Hermien E Kan 1, 5
Affiliation  

Quantitative MRI and MRS of muscle are increasingly being used to measure individual pathophysiological processes in Becker muscular dystrophy (BMD). In particular, muscle fat fraction was shown to be highly associated with functional tests in BMD. However, the muscle strength per unit of contractile cross‐sectional area is lower in patients with BMD compared with healthy controls. This suggests that the quality of the non‐fat‐replaced (NFR) muscle tissue is lower than in healthy controls. Consequently, a measure that reflects changes in muscle tissue itself is needed. Here, we explore the potential of water T2 relaxation times, diffusion parameters and phosphorus metabolic indices as early disease markers in patients with BMD. For this purpose, we examined these measures in fat‐replaced (FR) and NFR lower leg muscles in patients with BMD and compared these values with those in healthy controls. Quantitative proton MRI (three‐point Dixon, multi‐spin‐echo and diffusion‐weighted spin‐echo echo planar imaging) and 2D chemical shift imaging 31P MRS data were acquired in 24 patients with BMD (age 18.8‐66.2 years) and 13 healthy controls (age 21.3‐63.6 years). Muscle fat fractions, phosphorus metabolic indices, and averages and standard deviations (SDs) of the water T2 relaxation times and diffusion tensor imaging (DTI) parameters were assessed in six individual leg muscles. Phosphodiester levels were increased in the NFR and FR tibialis anterior, FR peroneus and FR gastrocnemius lateralis muscles. No clear pattern was visible for the other metabolic indices. Increased T2 SD was found in the majority of FR muscles compared with NFR and healthy control muscles. No differences in average water T2 relaxation times or DTI indices were found between groups. Overall, our results indicate that primarily muscles that are further along in the disease process showed increases in T2 heterogeneity and changes in some metabolic indices. No clear differences were found for the DTI indices between groups.

中文翻译:

Becker 型肌营养不良症患者的多参数 MR。

肌肉的定量 MRI 和 MRS 越来越多地用于测量贝克尔肌营养不良症 (BMD) 的个体病理生理过程。特别是,肌肉脂肪分数被证明与 BMD 的功能测试高度相关。然而,与健康对照组相比,BMD 患者单位收缩横截面积的肌肉力量较低。这表明非脂肪替代 (NFR) 肌肉组织的质量低于健康对照。因此,需要一种反映肌肉组织本身变化的措施。在这里,我们探索水T 2的潜力松弛时间、扩散参数和磷代谢指标作为 BMD 患者的早期疾病标志物。为此,我们检查了 BMD 患者脂肪替代 (FR) 和 NFR 小腿肌肉的这些测量值,并将这些值与健康对照组的值进行了比较。定量质子MRI(三点Dixon,多自旋回波和扩散加权自旋回波回波平面成像)和2D化学位移成像31个P MRS数据是在24名患者获得的与BMD(年龄18.8-66.2岁)和13健康对照(年龄 21.3-63.6 岁)。肌肉脂肪分数、磷代谢指数以及水T 2 的平均值和标准偏差 (SD)在六个单独的腿部肌肉中评估了松弛时间和扩散张量成像 (DTI) 参数。NFR 和 FR 胫骨前肌、FR 腓骨肌和 FR 腓肠外侧肌中的磷酸二酯水平增加。其他代谢指标没有可见的清晰模式。与 NFR 和健康对照肌肉相比,在大多数 FR 肌肉中发现T 2 SD增加。组间没有发现平均水T 2弛豫时间或 DTI 指数的差异。总体而言,我们的结果表明,主要是疾病过程中更远的肌肉显示T 2一些代谢指标的异质性和变化。组间 DTI 指数未发现明显差异。
更新日期:2020-10-05
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