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Muscle Nerve. 2019 Dec;60(6):679-686. doi: 10.1002/mus.26717. Epub 2019 Oct 23.

Respiratory muscle weakness in facioscapulohumeral muscular dystrophy.

Author information

1
Respiratory Physiology Laboratory, Department of Neurology with Institute of Translational Neurology, University of Münster, Münster, Germany.
2
Department of Pneumology, Cardiology and Intensive Care Medicine, Klinikum Konstanz, Konstanz, Germany.
3
Bethanien Hospital gGmbH Solingen, Solingen, Germany.
4
Institute for Pneumology, University of Cologne, Solingen, Germany.
5
Institute of Medical Informatics, University of Münster, Münster, Germany.
6
Institute for Biostatistics and Clinical Research, University Hospital, Münster, Germany.
7
Medical Park Klinik Reithofpark, Bad Feilnbach, Germany.

Abstract

INTRODUCTION:

The purpose of this study was to comprehensively evaluate respiratory muscle function in adults with facioscapulohumeral muscular dystrophy (FSHD).

METHODS:

Fourteen patients with FSHD (9 men, 53 ± 16 years of age) and 14 matched controls underwent spirometry, diaphragm ultrasound, and measurement of twitch gastric and transdiaphragmatic pressures (twPgas and twPdi; n = 10) after magnetic stimulation of the lower thoracic nerve roots and the phrenic nerves. The latter was combined with recording of diaphragm compound muscle action potentials (CMAPs; n = 14).

RESULTS:

The following parameters were significantly lower in patients vs controls: forced vital capacity (FVC); maximum inspiratory and expiratory pressure; peak cough flow; diaphragm excursion amplitude; and thickening ratio on ultrasound, twPdi (11 ± 5 vs 20 ± 6 cmH2 O) and twPgas (7 ± 3 vs 25 ± 20 cmH2 O). Diaphragm CMAP showed no group differences. FVC correlated inversely with the clinical severity scale score (r = -0.63, P = .02).

DISCUSSION:

In FSHD, respiratory muscle weakness involves both the diaphragm and the expiratory abdominal muscles.

KEYWORDS:

diaphragm ultrasound; facioscapulohumeral muscular dystrophy; phrenic nerve conduction studies; respiratory muscle strength; transdiaphragmatic pressure

PMID:
31566774
DOI:
10.1002/mus.26717
[Indexed for MEDLINE]

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