Universität zu Köln

Henke, Carolin, Spiesshoefer, Jens ORCID: 0000-0001-8205-1749, Kabitz, Hans-Joachim, Herkenrath, Simon, Randerath, Winfried, Brix, Tobias, Goerlich, Dennis, Young, Peter and Boentert, Matthias ORCID: 0000-0001-6133-1397 (2020). Characteristics of respiratory muscle involvement in myotonic dystrophy type 1. Neuromusc. Disord., 30 (1). S. 17 - 28. OXFORD: PERGAMON-ELSEVIER SCIENCE LTD. ISSN 1873-2364

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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(2)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(2)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. (C) 2019 Elsevier B.V. All rights reserved.

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 UNSPECIFIED 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:38-351017
DOI:	10.1016/j.nmd.2019.10.011
Journal or Publication Title:	Neuromusc. Disord.
Volume:	30
Number:	1
Page Range:	S. 17 - 28
Date:	2020
Publisher:	PERGAMON-ELSEVIER SCIENCE LTD
Place of Publication:	OXFORD
ISSN:	1873-2364
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language MAGNETIC STIMULATION; PULMONARY-FUNCTION; INSUFFICIENCY; PRESSURES; MORTALITY; DISEASE; VALUES; AGE Multiple languages Clinical Neurology; Neurosciences Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/35101