Imaging respiratory muscle quality and function in Duchenne muscular dystrophy
Imaging respiratory muscle quality and function in Duchenne muscular dystrophy
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DOI：
10.1007/s00415-019-09481-z
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发表时间：
2019-11-01
影响因子：
6
通讯作者：
Walter, Glenn A.
中科院分区：
文献类型：
--
作者：
Barnard, Alison M.;Lott, Donovan J.;Walter, Glenn A.
目的Duchenne型肌营养不良症（DMD）是一种以呼吸肌为主要表现的肌源性疾病。营养不良的肌肉变得虚弱并被脂肪组织浸润，导致进行性呼吸障碍。本研究的目的是使用磁共振成像评估DMD患者的呼吸肌质量和功能，并确定其与临床呼吸功能的关系。方法对36例DMD患者和12例正常对照者进行横断面磁共振成像研究。参与者进行胸部动态成像，以评估隔膜和胸壁的流动性和胸部和腹部的化学位移编码成像，以确定副呼吸肌的脂肪浸润。此外，还获得了临床肺功能指标。结果在潮气呼吸和最大呼吸时，DMD患者的胸腔面积较对照组减少。DMD患者在最大吸气和呼气时胸壁前后方向的运动减少，但最大吸气时横膈膜下降（标准化为身高）仅在最大吸气压低于60%预测值的一部分患者中减少。在评估的所有三个呼气肌中，肌肉脂肪分数升高（p < 0.001），并且脂肪浸润程度与预测的最大呼气压百分比相关（r =-0.70，p < 0.001）。肋间肌显示出极轻微的可见脂肪浸润;然而，该分析是定性的，分辨率有限。通过对膈肌运动、胸壁运动和副呼吸肌脂肪浸润的磁共振成像研究，为疾病进展与临床呼吸功能之间的关系提供了新的见解。
Objective Duchenne muscular dystrophy (DMD) is characterized by damage to muscles including the muscles involved in respiration. Dystrophic muscles become weak and infiltrated with fatty tissue, resulting in progressive respiratory impairment. The objective of this study was to assess respiratory muscle quality and function in DMD using magnetic resonance imaging and to determine the relationship to clinical respiratory function. Methods Individuals with DMD (n = 36) and unaffected controls (n = 12) participated in this cross sectional magnetic resonance imaging study. Participants underwent dynamic imaging of the thorax to assess diaphragm and chest wall mobility and chemical shift-encoded imaging of the chest and abdomen to determine fatty infiltration of the accessory respiratory muscles. Additionally, clinical pulmonary function measures were obtained. Results Thoracic cavity area was decreased in individuals with DMD compared to controls during tidal and maximal breathing. Individuals with DMD had reduced chest wall movement in the anterior-posterior direction during maximal inspirations and expirations, but diaphragm descent during maximal inspirations (normalized to height) was only decreased in a subset of individuals with maximal inspiratory pressures less than 60% predicted. Muscle fat fraction was elevated in all three expiratory muscles assessed (p < 0.001), and the degree of fatty infiltration correlated with percent predicted maximal expiratory pressures (r = - 0.70, p < 0.001). The intercostal muscles demonstrated minimal visible fatty infiltration; however, this analysis was qualitative and resolution limited. Interpretation This magnetic resonance imaging investigation of diaphragm movement, chest wall movement, and accessory respiratory muscle fatty infiltration provides new insights into the relationship between disease progression and clinical respiratory function.