Regional variation of thigh muscle fat infiltration in patients with neuromuscular diseases compared to healthy controls
Regional variation of thigh muscle fat infiltration in patients with neuromuscular diseases compared to healthy controls
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DOI：
10.21037/qims-20-1098
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发表时间：
2021-06-01
影响因子：
2.8
通讯作者：
Baum, Thomas
中科院分区：
文献类型：
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作者：
Greve, Tobias;Burian, Egon;Baum, Thomas
背景资料：基于化学位移编码的水-脂肪磁共振成像（CSE-MRI）测量定量生物标志物：质子密度脂肪分数（PDFF）。目的是评估与健康对照相比，强直性肌营养不良2型（DM 2）、肢带型肌营养不良2A型（LGMD 2A）和晚发型庞贝氏症（LOPD）患者大腿的区域和近端-远端PDFF变化。方法：7例患者（n=2 DM 2，n=2 LGMD 2A，n= 3 LOPD）和20名对照。使用3D扰相梯度回波序列扫描大腿肌肉组织。手动分割肌肉以生成平均肌肉PDFF。结果：在所有三种疾病实体中，与健康对照组相比，肌肉脂肪替代增加。然而，在每个疾病组中，与对照组相比，症状发作时间较短的患者仅显示轻度PDFF升高（范围为10%至20%）（P=0.05），而症状发作时间较长的患者显示更严重的脂肪替代等级，范围为50%至70%（P = 0.05）。
Background: Chemical shift encoding-based water-fat magnetic resonance imaging (CSE-MRI) measures a quantitative biomarker: the proton density fat fraction (PDFF). The aim was to assess regional and proximo-distal PDFF variations at the thigh in patients with myotonic dystrophy type 2 (DM2), limb-girdle muscular dystrophy type 2A ( LGMD2A), and late-onset Pompe disease (LOPD) as compared to healthy controls. Methods: Seven patients ( n=2 DM2, n=2 LGMD2A, n= 3 LOPD) and 20 controls were recruited. A 3D- spoiled gradient echo sequence was used to scan the thigh musculature. Muscles were manually segmented to generate mean muscle PDFF. Results: In all three disease entities, there was an increase in muscle fat replacement compared to healthy controls. However, within each disease group, there were patients with a shorter time since symptom onset that only showed mild PDFF elevation (range, 10% to 20%) compared to controls (P=0.05), whereas patients with a longer period since symptom onset showed a more severe grade of fat replacement with a range of 50% to 70% (P