Universität zu Köln

Spiesshoefer, Jens ORCID: 0000-0001-8205-1749, Henke, Carolin, Kabitz, Hans Joachim, Brix, Tobias, Goerlich, Dennis, Herkenrath, Simon, Randerath, Winfried, Young, Peter and Boentert, Matthias (2019). The nature of respiratory muscle weakness in patients with late-onset Pompe disease. Neuromusc. Disord., 29 (8). S. 618 - 628. OXFORD: PERGAMON-ELSEVIER SCIENCE LTD. ISSN 1873-2364

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Abstract

Late-onset Pompe disease (LOPD) causes myopathy of skeletal and respiratory muscles, and phrenic nerve pathology putatively contributes to diaphragm weakness. The aim of this study was to investigate neural contributions to diaphragm dysfunction, usefulness of diaphragm ultrasound, and involvement of expiratory abdominal muscles in LOPD. Thirteen patients with LOPD (7 male, 51 +/- 17 years) and 13 age- and gender-matched controls underwent respiratory muscle strength testing, ultrasound evaluation of diaphragm excursion and thickness, cortical and cervical magnetic stimulation (MS) of the diaphragm with simultaneous recording of surface electromyogram and twitch transdiaphragmatic pressure (twPdi; n = 6), and MS of the abdominal muscles with recording of twitch gastric pressure (twPgas; n = 6). The following parameters were significantly reduced in LOPD patients versus controls: forced vital capacity (p<0.01), maximum inspiratory and expiratory pressure (both p<0.001), diaphragm excursion velocity (p<0.05), diaphragm thickening ratio (1.8 +/- 0.4 vs. 2.6 +/- 0.6, p<0.01), twPdi following cervical MS (12.0 +/- 6.2 vs. 19.4 +/- 4.8 cmH(2)O, p<0.05), and twPgas following abdominal muscle stimulation (8.8 +/- 8.1 vs. 34.6 +/- 17.1 cmH(2)O, p<0.01). Diaphragm motor evoked potentials and compound muscle action potentials showed no between-group differences. In conclusion, phrenic nerve involvement in LOPD could not be electrophysiologically confirmed. Ultrasound supports assessment of diaphragm function. Abdominal expiratory muscles are functionally involved in LOPD. (C) 2019 Elsevier B.V. All rights reserved.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Spiesshoefer, Jens UNSPECIFIED orcid.org/0000-0001-8205-1749 UNSPECIFIED Henke, Carolin UNSPECIFIED UNSPECIFIED UNSPECIFIED Kabitz, Hans Joachim UNSPECIFIED UNSPECIFIED UNSPECIFIED Brix, Tobias UNSPECIFIED UNSPECIFIED UNSPECIFIED Goerlich, Dennis UNSPECIFIED UNSPECIFIED UNSPECIFIED Herkenrath, Simon UNSPECIFIED UNSPECIFIED UNSPECIFIED Randerath, Winfried UNSPECIFIED UNSPECIFIED UNSPECIFIED Young, Peter UNSPECIFIED UNSPECIFIED UNSPECIFIED Boentert, Matthias UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-145066
DOI:	10.1016/j.nmd.2019.06.011
Journal or Publication Title:	Neuromusc. Disord.
Volume:	29
Number:	8
Page Range:	S. 618 - 628
Date:	2019
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 CERVICAL MAGNETIC STIMULATION; ENZYME REPLACEMENT THERAPY; PULMONARY-FUNCTION; CLINICAL METHODS; HUMAN DIAPHRAGM; MRI; DYSFUNCTION; PROGRESSION; STRENGTH; CHILDREN Multiple languages Clinical Neurology; Neurosciences Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/14506