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定量肌肉 MRI 方案可作为贝克型肌营养不良症的生物标志物。

Quantitative Muscle MRI Protocol as Possible Biomarker in Becker Muscular Dystrophy.

机构信息

Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.

Neurology IV-Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico "Carlo Besta", Via Celoria 11, 20133, Milan, Italy.

出版信息

Clin Neuroradiol. 2021 Mar;31(1):257-266. doi: 10.1007/s00062-019-00875-0. Epub 2020 Jan 23.

Abstract

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.

摘要

目的

本研究旨在比较贝克型肌营养不良症(BMD)患者和健康受试者(HS)的定量磁共振成像(qMRI)测量值,并将这些参数与临床评分相关联。

方法

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 进行了大腿和小腿水平的磁共振成像(MRI)检查,包括:1)标准轴位 T1 加权序列;2)容积 T2 加权序列;3)多回波自旋回波序列;4)两点 Dixon 序列;5)弥散张量成像(DTI)序列。

结果

BMD 患者大腿和小腿水平的平均脂肪分数(FF)、T2 弛豫时间和分数各向异性(FA)DTI 值均显著高于 HS(p 值均<0.01)。大腿和小腿水平的 FF 与北星日常活动评估(NSAA)评分(p 值均<0.01)和 6 分钟步行测试(6MWT)(p 值均<0.01)显著相关,而仅小腿肌肉 FF 与从地板上站起来的时间显著相关(p 值=0.01)。T2 仅与 NSAA 评分(p 值<0.01)、6MWT(p 值=0.02)和从地板上站起来的时间(p 值<0.01)在小腿水平显著相关。在 DTI 值中,只有大腿和小腿肌肉的 FA 与 NSAA 评分、6MWT 和 10 米步行显著相关(所有 p 值均<0.05);只有小腿肌肉的 FA 与从地板上站起来的时间显著相关(p=0.01)。

结论

肌肉 FF、T2 弛豫测量和 DTI 似乎是评估 BMD 疾病严重程度的有前途的生物标志物,尽管需要进一步的研究来评估随时间的变化。

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