Diaphragmatic dysfunction in neuromuscular disease, an MRI study

Research output: Contribution to journal › Journal article › Research › peer-review

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Diaphragmatic dysfunction in neuromuscular disease, an MRI study. / Harlaar, Laurike; Ciet, Pierluigi; van Tulder, Gijs; Brusse, Esther; Timmermans, Remco G M; Janssen, Wim G M; de Bruijne, Marleen; van der Ploeg, Ans T; Tiddens, Harm A W M; van Doorn, Pieter A; van der Beek, Nadine A M E.

In: Neuromuscular Disorders, Vol. 32, No. 1, 2022, p. 15-24.

Research output: Contribution to journal › Journal article › Research › peer-review

Harvard

Harlaar, L, Ciet, P, van Tulder, G, Brusse, E, Timmermans, RGM, Janssen, WGM, de Bruijne, M, van der Ploeg, AT, Tiddens, HAWM, van Doorn, PA & van der Beek, NAME 2022, 'Diaphragmatic dysfunction in neuromuscular disease, an MRI study', Neuromuscular Disorders, vol. 32, no. 1, pp. 15-24. https://doi.org/10.1016/j.nmd.2021.11.001

APA

Harlaar, L., Ciet, P., van Tulder, G., Brusse, E., Timmermans, R. G. M., Janssen, W. G. M., de Bruijne, M., van der Ploeg, A. T., Tiddens, H. A. W. M., van Doorn, P. A., & van der Beek, N. A. M. E. (2022). Diaphragmatic dysfunction in neuromuscular disease, an MRI study. Neuromuscular Disorders, 32(1), 15-24. https://doi.org/10.1016/j.nmd.2021.11.001

Vancouver

Harlaar L, Ciet P, van Tulder G, Brusse E, Timmermans RGM, Janssen WGM et al. Diaphragmatic dysfunction in neuromuscular disease, an MRI study. Neuromuscular Disorders. 2022;32(1):15-24. https://doi.org/10.1016/j.nmd.2021.11.001

Author

Harlaar, Laurike ; Ciet, Pierluigi ; van Tulder, Gijs ; Brusse, Esther ; Timmermans, Remco G M ; Janssen, Wim G M ; de Bruijne, Marleen ; van der Ploeg, Ans T ; Tiddens, Harm A W M ; van Doorn, Pieter A ; van der Beek, Nadine A M E. / Diaphragmatic dysfunction in neuromuscular disease, an MRI study. In: Neuromuscular Disorders. 2022 ; Vol. 32, No. 1. pp. 15-24.

Bibtex

@article{b11ebe7a04ce49929921b3673a0de007,
title = "Diaphragmatic dysfunction in neuromuscular disease, an MRI study",
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.",
author = "Laurike Harlaar and Pierluigi Ciet and {van Tulder}, Gijs and Esther Brusse and Timmermans, {Remco G M} and Janssen, {Wim G M} and {de Bruijne}, Marleen and {van der Ploeg}, {Ans T} and Tiddens, {Harm A W M} and {van Doorn}, {Pieter A} and {van der Beek}, {Nadine A M E}",
year = "2022",
doi = "10.1016/j.nmd.2021.11.001",
language = "English",
volume = "32",
pages = "15--24",
journal = "Journal of Neuromuscular Diseases",
issn = "0960-8966",
publisher = "Elsevier",
number = "1",

}

RIS

TY - JOUR

T1 - Diaphragmatic dysfunction in neuromuscular disease, an MRI study

AU - Harlaar, Laurike

AU - Ciet, Pierluigi

AU - van Tulder, Gijs

AU - Brusse, Esther

AU - Timmermans, Remco G M

AU - Janssen, Wim G M

AU - de Bruijne, Marleen

AU - van der Ploeg, Ans T

AU - Tiddens, Harm A W M

AU - van Doorn, Pieter A

AU - van der Beek, Nadine A M E

PY - 2022

Y1 - 2022

N2 - 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.

AB - 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.

U2 - 10.1016/j.nmd.2021.11.001

DO - 10.1016/j.nmd.2021.11.001

M3 - Journal article

C2 - 34973872

VL - 32

SP - 15

EP - 24

JO - Journal of Neuromuscular Diseases

JF - Journal of Neuromuscular Diseases

SN - 0960-8966

IS - 1

ER -

ID: 290451837