Im Yu Jin, Choe Yumi, Lee Jiwon, Lee Jeehun, Do Jong Geol, Kwon Jeong-Yi
Department of Physical and Rehabilitation Medicine, Samsung Medical Center, Seoul, Republic of Korea.
Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
Muscle Nerve. 2025 Oct;72(4):606-615. doi: 10.1002/mus.28469. Epub 2025 Jul 16.
INTRODUCTION/AIMS: Quantitative muscle ultrasound (QMUS) shows promise as a non-invasive biomarker for monitoring functional status in Duchenne muscular dystrophy (DMD). We evaluated the correlation between QMUS in various muscles and functional capacity assessments. Additionally, we compared the utility of composite QMUS scores as a comprehensive measure of disease progression to that of single-muscle analyses.
We analyzed baseline data from an ongoing prospective study involving 23 patients with DMD (aged 5-18 years; mean 10.7; SD 4.2). High-resolution ultrasound scans of muscle groups across the upper and lower limbs, head and neck, and diaphragm were assessed for muscle echogenicity using mean grayscale values (MGV). Functional capacity assessments included the 6-min walk test (6MWT), North Star Ambulatory Assessment (NSAA), Performance of Upper Limb (PUL) module, timed function tests, quantitative muscle strength tests, and respiratory muscle strength tests.
The composite MGV for upper and lower limbs demonstrated moderate to strong negative correlations with the 6MWT and NSAA scores (correlation coefficient [CC]: -0.44 to -0.74; p < 0.01) and strong to very strong positive correlations with rise-from-floor, 4-stair climb, and 10-m walk/run times (CC: 0.65 to 0.87; p < 0.01). For upper limb function, the MGV of the flexor carpi radialis muscle was negatively correlated with PUL scores (CC = -0.35, p = 0.02). However, no significant correlations were observed between QMUS parameters and muscle strength test (p > 0.05).
QMUS offers a non-invasive method for assessing muscle changes and tracking disease progression in DMD.
ClinicalTrials.gov identifier: NCT05249361.
引言/目的：定量肌肉超声（QMUS）有望成为监测杜氏肌营养不良症（DMD）功能状态的一种非侵入性生物标志物。我们评估了不同肌肉的QMUS与功能能力评估之间的相关性。此外，我们比较了复合QMUS评分作为疾病进展综合指标与单肌肉分析的效用。
我们分析了一项正在进行的前瞻性研究的基线数据，该研究涉及23名DMD患者（年龄5 - 18岁；平均10.7岁；标准差4.2）。使用平均灰度值（MGV）对上肢、下肢、头颈部和膈肌的肌肉群进行高分辨率超声扫描，以评估肌肉回声。功能能力评估包括6分钟步行试验（6MWT）、北极星动态评估（NSAA）、上肢功能（PUL）模块、定时功能测试、定量肌肉力量测试和呼吸肌力量测试。
上肢和下肢的复合MGV与6MWT和NSAA评分呈中度至强负相关（相关系数[CC]：-0.44至-0.74；p < 0.01），与从地面起身、爬4级楼梯和10米步行/跑步时间呈强至非常强正相关（CC：0.65至0.87；p < 0.01）。对于上肢功能，桡侧腕屈肌的MGV与PUL评分呈负相关（CC = -0.35，p = 0.02）。然而，未观察到QMUS参数与肌肉力量测试之间存在显著相关性（p > 0.05）。
QMUS为评估DMD患者的肌肉变化和追踪疾病进展提供了一种非侵入性方法。
ClinicalTrials.gov标识符：NCT05249361。
