Diaphragmatic dysfunction in neuromuscular disease, an MRI study

Research output: Contribution to journalArticleAcademicpeer-review

3 Citations (Scopus)
2 Downloads (Pure)

Abstract

The aim of this exploratory study was to evaluate diaphragmatic function across various neuromuscular diseases using spirometry-controlled MRI. We measured motion of the diaphragm relative to that of the thoracic wall (cranial-caudal ratio vs. anterior posterior ratio; CC-AP ratio), and changes in the diaphragmatic curvature (diaphragm height and area ratio) during inspiration in 12 adults with a neuromuscular disease having signs of respiratory muscle weakness, 18 healthy controls, and 35 adult Pompe patients – a group with prominent diaphragmatic weakness. CC-AP ratio was lower in patients with myopathies (n=7, 1.25±0.30) and motor neuron diseases (n=5, 1.30±0.10) than in healthy controls (1.37±0.14; p=0.001 and p=0.008), but not as abnormal as in Pompe patients (1.12±0.18; p=0.011 and p=0.024). The mean diaphragm height ratio was 1.17±0.33 in patients with myopathies, pointing at an insufficient diaphragmatic contraction. This was also seen in patients with Pompe disease (1.28±0.36), but not in healthy controls (0.82±0.33) or patients with motor neuron disease (0.82±0.24). We conclude that spirometry-controlled MRI enables us to investigate respiratory dysfunction across neuromuscular diseases, suggesting that the diaphragm is affected in a different way in myopathies and motor neuron diseases. Whether MRI can also be used to evaluate progression of diaphragmatic dysfunction requires additional studies.

Original languageEnglish
Pages (from-to)15-24
Number of pages10
JournalNeuromuscular Disorders
Volume32
Issue number1
Early online date9 Nov 2021
DOIs
Publication statusPublished - Jan 2022

Bibliographical note

Publisher Copyright:
© 2021 The Authors

Fingerprint

Dive into the research topics of 'Diaphragmatic dysfunction in neuromuscular disease, an MRI study'. Together they form a unique fingerprint.

Cite this