Purpose

Aim of this study is to compare Quantitative Magnetic Resonance Imaging (qMRI) measures between Becker Muscular Dystrophy (BMD) and Healthy Subjects (HS) and to correlate these parameters with clinical scores.

Methods

Ten BMD patients (mean age ±standard deviation: 38.7 ± 15.0 years) and ten age-matched HS, were investigated through magnetic resonance imaging (MRI) at thigh and calf levels, including: 1) a standard axial T1-weighted sequence; 2) a volumetric T2-weighted sequence; 3) a multiecho spin-echo sequence; 4) a 2-point Dixon sequence; 5) a Diffusion Tensor Imaging (DTI) sequence.

Results

Mean Fat Fraction (FF), T2-relaxation time and Fractional Anisotropy (FA) DTI at thigh and calf levels were significantly higher in BMD patients than in HS (p-values < 0.01). FF at thigh and calf levels significantly correlated with North Star Ambulatory Assessment (NSAA) score (p-values < 0.01) and6 Minutes Walking Test (6MWT) (p-values < 0.01), whereas only calf muscle FF was significantly associated with time to get up from floor (p-value = 0.01). T2 significantly correlated with NSAA score (p-value < 0.01), 6MWT (p-value = 0.02) and time to get up from floor (p-value < 0.01) only at calf level. Among DTI values, only FA in thigh and calf muscles significantly correlated with NSAA score, 6MWT and 10-m walk (all p-values < 0.05); only FA in calf muscles significantly correlated with time to get up from floor (p = 0.01).

Conclusions

Muscle FF, T2-relaxometry and DTI, seem to be a promising biomarker to assess BMD disease severity, although further studies are needed to evaluate changes over the time.

中文翻译：

---

定量肌肉 MRI 协议作为贝克尔肌营养不良症的可能生物标志物

目的

本研究的目的是比较贝克尔肌营养不良症 (BMD) 和健康受试者 (HS) 之间的定量磁共振成像 (qMRI) 测量，并将这些参数与临床评分相关联。

方法

通过大腿和小腿水平的磁共振成像 (MRI) 对 10 名 BMD 患者（平均年龄 ± 标准差：38.7 ± 15.0 岁）和 10 名年龄匹配的 HS 进行了调查，包括：1）标准轴向 T1 加权序列；2) 体积T2加权序列；3) 一个多回波自旋回波序列；4）一个2点狄克逊序列；5) 扩散张量成像 (DTI) 序列。

结果

BMD 患者的大腿和小腿水平的平均脂肪分数 (FF)、T2 松弛时间和分数各向异性 (FA) DTI 显着高于 HS（p 值< 0.01）。大腿和小腿水平的 FF 与北极星动态评估 (NSAA) 评分（p 值< 0.01）和 6 分钟步行测试（6MWT）（p 值< 0.01）显着相关，而只有小腿肌肉 FF 与到达时间显着相关从地板上站起来（p值 = 0.01）。T2 与 NSAA 评分 ( p值 < 0.01)、6MWT ( p值 = 0.02) 和从地板起床的时间 ( p-value < 0.01) 仅在犊牛水平。在 DTI 值中，只有大腿和小腿肌肉的 FA 与 NSAA 评分、6MWT 和 10 米步行显着相关（所有p 值< 0.05）；只有小腿肌肉中的 FA 与从地板上起床的时间显着相关 ( p  = 0.01)。

结论

肌肉 FF、T2 松弛测量和 DTI 似乎是评估 BMD 疾病严重程度的有希望的生物标志物，尽管需要进一步研究来评估随时间的变化。