Universität zu Köln

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

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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
Creators:	Creators Email ORCID ORCID Put Code Henke, Carolin UNSPECIFIED UNSPECIFIED UNSPECIFIED Spiesshoefer, Jens UNSPECIFIED orcid.org/0000-0001-8205-1749 UNSPECIFIED Kabitz, Hans-Joachim UNSPECIFIED orcid.org/0000-0002-0080-7125 UNSPECIFIED Herkenrath, Simon UNSPECIFIED UNSPECIFIED UNSPECIFIED Randerath, Winfried UNSPECIFIED UNSPECIFIED UNSPECIFIED Brix, Tobias UNSPECIFIED UNSPECIFIED UNSPECIFIED Goerlich, Dennis UNSPECIFIED UNSPECIFIED UNSPECIFIED Young, Peter UNSPECIFIED UNSPECIFIED UNSPECIFIED Boentert, Matthias UNSPECIFIED orcid.org/0000-0001-6133-1397 UNSPECIFIED
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
Uncontrolled Keywords:	Keywords Language INVOLVEMENT Multiple languages Clinical Neurology; Neurosciences Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/13053