Henke, Carolin, Spiesshoefer, Jens ORCID: 0000-0001-8205-1749, Kabitz, Hans-Joachim
ORCID: 0000-0002-0080-7125, Herkenrath, Simon, Randerath, Winfried, Brix, Tobias, Goerlich, Dennis, Young, Peter and Boentert, Matthias
ORCID: 0000-0001-6133-1397
(2019).
Respiratory muscle weakness in facioscapulohumeral muscular dystrophy.
Muscle Nerve, 60 (6).
S. 679 - 687.
HOBOKEN:
WILEY.
ISSN 1097-4598
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 cmH(2)O) and twPgas (7 +/- 3 vs 25 +/- 20 cmH(2)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.
Item Type: | Journal Article | ||||||||||||||||||||||||||||||||||||||||
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URN: | urn:nbn:de:hbz:38-130531 | ||||||||||||||||||||||||||||||||||||||||
DOI: | 10.1002/mus.26717 | ||||||||||||||||||||||||||||||||||||||||
Journal or Publication Title: | Muscle Nerve | ||||||||||||||||||||||||||||||||||||||||
Volume: | 60 | ||||||||||||||||||||||||||||||||||||||||
Number: | 6 | ||||||||||||||||||||||||||||||||||||||||
Page Range: | S. 679 - 687 | ||||||||||||||||||||||||||||||||||||||||
Date: | 2019 | ||||||||||||||||||||||||||||||||||||||||
Publisher: | WILEY | ||||||||||||||||||||||||||||||||||||||||
Place of Publication: | HOBOKEN | ||||||||||||||||||||||||||||||||||||||||
ISSN: | 1097-4598 | ||||||||||||||||||||||||||||||||||||||||
Language: | English | ||||||||||||||||||||||||||||||||||||||||
Faculty: | Unspecified | ||||||||||||||||||||||||||||||||||||||||
Divisions: | Unspecified | ||||||||||||||||||||||||||||||||||||||||
Subjects: | no entry | ||||||||||||||||||||||||||||||||||||||||
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Refereed: | Yes | ||||||||||||||||||||||||||||||||||||||||
URI: | http://kups.ub.uni-koeln.de/id/eprint/13053 |
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