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.

Highlights

  • Respiratory insufficiency is a well-known problem in patients with neuromuscular diseases and has been described in various types of myopathies and in motor neuron diseases [1, 2]

  • The mean postural drop between forced vital capacity (FVC) upright and FVC supine (Δ FVC), indicating diaphragmatic weakness, was only 3 ± 4 % in patients with myopathies compared to 15 ± 12 % in patients with Pompe disease (p=0.007)

  • No other pulmonary function test results in patients with motor neuron diseases were significantly different compared to healthy controls or Pompe patients

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Summary

Introduction

Respiratory insufficiency is a well-known problem in patients with neuromuscular diseases and has been described in various types of myopathies and in motor neuron diseases [1, 2]. We recently showed that MRI can be used to investigate early impairment of the diaphragm in patients with Pompe disease [11] It is unknown, whether these findings are specific for Pompe disease, or whether the diaphragm is affected in a similar way in other types of neuromuscular diseases. Whether these findings are specific for Pompe disease, or whether the diaphragm is affected in a similar way in other types of neuromuscular diseases It was shown in patients with Duchenne muscular dystrophy that both diaphragmatic motion and thoracic wall motion were decreased [12-

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