Carolin Henke1, Jens Spiesshoefer1, Hans-Joachim Kabitz2, Simon Herkenrath3,4, Winfried Randerath3,4, Tobias Brix5, Dennis Görlich6, Peter Young7, Matthias Boentert1. 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.
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.
Authors: Anna Christina Meyer; Jens Spiesshoefer; Nina Christina Siebers; Anna Heidbreder; Christian Thiedemann; Hartmut Schneider; Andrew T Braun; Winfried Randerath; Peter Young; Michael Dreher; Matthias Boentert Journal: Sleep Breath Date: 2020-12-02 Impact factor: 2.816
Authors: Sjan Teeselink; Sanne C C Vincenten; Nicol C Voermans; Jan T Groothuis; Jonne Doorduin; Peter J Wijkstra; Corinne G C Horlings; Baziel G M van Engelen; Karlien Mul Journal: J Neurol Date: 2022-02-11 Impact factor: 4.849