Neuroradiology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, via Francesco Sforza 35, 20122 Milan, Italy.
Neuromuscular and Rare Diseases Unit, Department of Neuroscience, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, via Francesco Sforza 35, 20122 Milan, Italy.
Br J Radiol. 2024 May 7;97(1157):947-953. doi: 10.1093/bjr/tqae070.
Becker muscular dystrophy (BMD) is a relatively less investigated neuromuscular disease, partially overlapping the phenotype of Duchenne dystrophy (DMD). Physiopathological and anatomical patterns are still not comprehensively known, despite recent effort in the search of early biomarkers. Aim of this study was to selectively compare normal appearing muscles of BMD with healthy controls.
Among a pool of 40 BMD patients and 20 healthy controls, Sartorius and gracilis muscles were selected on the basis of a blinded clinical quantitative/qualitative evaluation, if classified as normal (0 or 1 on Mercuri scale) and subsequently segmented on diffusion tensor MRI scans with a tractographic approach. Diffusion derived parameters were extracted.
Non-parametric testing revealed significant differences between normal and normal appearing BMD derived parameters in both muscles, the difference being more evident in sartorius. Bonferroni-corrected P-values (<.05) of Mann-Whitney test could discriminate between BMD and controls for standard deviation of all diffusion parameters (mean diffusivity, fractional anisotropy, axial and radial diffusivity) in both sartorius and gracilis, while in sartorius the significant difference was found also in the average values of the same parameters (with exception of RD).
This method could identify microstructural alterations in BMD normal appearing sartorius and gracilis.
Diffusion based MRI could be able to identify possible early or subclinical microstructural alterations in dystrophic patients with BMD.
贝克型肌营养不良症(BMD)是一种研究相对较少的神经肌肉疾病,其表型部分与杜氏肌营养不良症(DMD)重叠。尽管最近在寻找早期生物标志物方面做出了努力,但生理病理和解剖模式仍不完全清楚。本研究的目的是选择性地比较 BMD 正常肌肉与健康对照组。
在 40 名 BMD 患者和 20 名健康对照组中,根据盲法临床定量/定性评估,如果被归类为正常(Mercuri 量表上为 0 或 1),则选择股薄肌和缝匠肌,并随后在弥散张量 MRI 扫描上进行基于轨迹的分割。提取弥散衍生参数。
非参数检验显示,在这两种肌肉中,正常和正常外观的 BMD 衍生参数之间存在显著差异,股薄肌中的差异更为明显。Mann-Whitney 检验的 Bonferroni 校正 P 值(<0.05)可以区分 BMD 和对照组在股薄肌和缝匠肌中所有弥散参数(平均弥散度、各向异性分数、轴向和径向弥散度)的标准差,而在股薄肌中,同样参数的平均值也存在显著差异(除 RD 外)。
这种方法可以识别 BMD 正常外观的股薄肌和缝匠肌的微观结构改变。
基于弥散的 MRI 可能能够识别 BMD 患者可能的早期或亚临床微观结构改变。