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Characteristics of respiratory muscle involvement in myotonic dystrophy type 1.
- Source :
-
Neuromuscular disorders : NMD [Neuromuscul Disord] 2020 Jan; Vol. 30 (1), pp. 17-27. Date of Electronic Publication: 2019 Nov 05. - Publication Year :
- 2020
-
Abstract
- The pathophysiology of respiratory muscle weakness in myotonic dystrophy type 1 (DM1) remains incompletely understood. 21 adult patients with DM1 (11 men, 42 ± 13 years) and 21 healthy matched controls underwent spirometry, manometry, and diaphragm ultrasound. In addition, surface electromyography of the diaphragm and the obliquus abdominis muscle was performed following cortical and posterior cervical magnetic stimulation (CMS) of the phrenic nerves or magnetic stimulation of the lower thoracic nerve roots. Magnetic stimulation was combined with invasive recording of the twitch transdiaphragmatic and gastric pressure (twPdi and twPgas) in 10 subjects per group. The following parameters were reduced in DM1 patients compared to control subjects: maximum inspiratory pressure (MIP; 40.3 ± 19.2 vs. 95.8 ± 28.5 cmH <subscript>2</subscript> O, p < 0.01), diaphragm thickening ratio (DTR; 2.0 ± 0.4 vs. 2.7 ± 0.6, p < 0.01), twPdi following CMS (10.8 ± 8.3 vs. 21.4 ± 10.1 cmH2O, p = 0.03), and amplitude of diaphragm compound muscle action potentials (0.10 ± 0.25 vs. 0.46 ± 0.35 mV; p = 0.04). MIP and DTR were significantly correlated with the muscular impairment rating scale (MIRS) score. Maximum expiratory pressure (MEP) was reduced in DM1 patients compared to controls (41.3 ± 13.4 vs. 133.8 ± 28.0 cmH2O, p < 0.01) and showed negative correlation with the MIRS score. Pgas following a maximum cough was markedly lower in patients than in controls (71.9 ± 43.2 vs. 102.4 ± 35.5 cmH <subscript>2</subscript> O) but without statistical significance (p = 0.06). In DM1, respiratory muscle weakness relates to clinical disease severity and involves inspiratory and probably expiratory muscle strength. Axonal phrenic nerve pathology may contribute to diaphragm dysfunction.<br /> (Copyright © 2019 Elsevier B.V. All rights reserved.)
- Subjects :
- Adult
Case-Control Studies
Cross-Sectional Studies
Diaphragm diagnostic imaging
Diaphragm physiopathology
Electromyography
Female
Humans
Male
Middle Aged
Muscle Strength physiology
Muscle Weakness etiology
Myotonic Dystrophy complications
Neural Conduction physiology
Respiratory Function Tests
Respiratory Muscles diagnostic imaging
Severity of Illness Index
Spirometry
Ultrasonography
Muscle Weakness physiopathology
Myotonic Dystrophy physiopathology
Phrenic Nerve physiopathology
Respiratory Muscles physiopathology
Subjects
Details
- Language :
- English
- ISSN :
- 1873-2364
- Volume :
- 30
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Neuromuscular disorders : NMD
- Publication Type :
- Academic Journal
- Accession number :
- 31839403
- Full Text :
- https://doi.org/10.1016/j.nmd.2019.10.011