17 May 2021

Jeroen van Doorn and colleagues published a comprehensive review on imaging of the respiratory muscles in neuromuscular disorders in the European Respiratory Journal.

Many individuals with a neuromuscular disorder (NMD), such as amyotrophic lateral sclerosis  or Duchenne muscular dystrophy  develop respiratory muscle weakness. This can lead to dyspnea, sleep problems, lung infections, and ultimately respiratory failure. Therefore, reliable  assessment of respiratory muscle function in NMDs is crucial. For patients it is of vital importance to identify early signs of respiratory insufficiency, to monitor disease progression, and to guide individual respiratory management. From a scientific perspective, relevant and reliable respiratory outcome measures are urgently needed to evaluate new gene-modifying and drug therapies. In the last decade, ultrasound (US) and magnetic resonance (MR) imaging emerged as techniques to evaluate the respiratory muscles. In their review, they discuss the latest developments in imaging of the respiratory muscles by US and MR, and its clinical application and limitations. Their goal is to increase understanding of respiratory muscle imaging and facilitate its use as an outcome measure in daily practice and clinical trials.

Through an extensive literature search they found that different US and MR imaging techniques have been successfully demonstrated to detect impaired respiratory muscle structure and function in patients with NMDs. However, a lack of standardization in measurement procedures, data on extra-diaphragmatic respiratory muscles and clinical data from natural history studies impede its widespread clinical use. In addition, new technological developments in US and MR imaging will further increase its value. Currently, they are planning a large follow-up study in patients with congenital myopathies to evaluate the value of new US techniques, such as strain imaging to measure muscle deformation and ultrafast shear wave elastography to measure muscle elasticity.


van Doorn, J. L. M., Pennati, F., Hansen, H. H. G., van Engelen, B. G. M., Aliverti, A., & Doorduin, J. (2021). Respiratory muscle imaging by ultrasound and MRI in neuromuscular disorders. Eur Respir J. doi:10.1183/13993003.00137-2021


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