{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T21:21:25Z","timestamp":1787952085115,"version":"build-2784847793"},"reference-count":20,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2019,10,23]],"date-time":"2019-10-23T00:00:00Z","timestamp":1571788800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2019,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>The purpose of this study was to comprehensively evaluate respiratory muscle function in adults with facioscapulohumeral muscular dystrophy (FSHD).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Fourteen patients with FSHD (9 men, 53\u2009\u00b1\u200916\u2009years 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).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>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\u2009\u00b1\u20095 vs 20\u2009\u00b1\u20096 cmH<jats:sub>2<\/jats:sub>O) and twPgas (7\u2009\u00b1\u20093 vs 25\u2009\u00b1\u200920 cmH<jats:sub>2<\/jats:sub>O). Diaphragm CMAP showed no group differences. FVC correlated inversely with the clinical severity scale score (<jats:italic>r<\/jats:italic> = \u22120.63, <jats:italic>P<\/jats:italic> = .02).<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>In FSHD, respiratory muscle weakness involves both the diaphragm and the expiratory abdominal muscles.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.26717","type":"journal-article","created":{"date-parts":[[2019,10,1]],"date-time":"2019-10-01T05:00:08Z","timestamp":1569906008000},"page":"679-686","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":44,"title":["Respiratory muscle weakness in facioscapulohumeral muscular dystrophy"],"prefix":"10.1002","volume":"60","author":[{"given":"Carolin","family":"Henke","sequence":"first","affiliation":[{"name":"Respiratory Physiology Laboratory, Department of Neurology with Institute of Translational Neurology University of M\u00fcnster  M\u00fcnster Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens","family":"Spiesshoefer","sequence":"additional","affiliation":[{"name":"Respiratory Physiology Laboratory, Department of Neurology with Institute of Translational Neurology University of M\u00fcnster  M\u00fcnster Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans\u2010Joachim","family":"Kabitz","sequence":"additional","affiliation":[{"name":"Department of Pneumology, Cardiology and Intensive Care Medicine Klinikum Konstanz  Konstanz Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Herkenrath","sequence":"additional","affiliation":[{"name":"Bethanien Hospital gGmbH Solingen  Solingen Germany"},{"name":"Institute for Pneumology University of Cologne  Solingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Winfried","family":"Randerath","sequence":"additional","affiliation":[{"name":"Bethanien Hospital gGmbH Solingen  Solingen Germany"},{"name":"Institute for Pneumology University of Cologne  Solingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias","family":"Brix","sequence":"additional","affiliation":[{"name":"Institute of Medical Informatics University of M\u00fcnster  M\u00fcnster Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dennis","family":"G\u00f6rlich","sequence":"additional","affiliation":[{"name":"Institute for Biostatistics and Clinical Research University Hospital  M\u00fcnster Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Young","sequence":"additional","affiliation":[{"name":"Medical Park Klinik Reithofpark  Bad Feilnbach Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6133-1397","authenticated-orcid":false,"given":"Matthias","family":"Boentert","sequence":"additional","affiliation":[{"name":"Respiratory Physiology Laboratory, Department of Neurology with Institute of Translational Neurology University of M\u00fcnster  M\u00fcnster Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,10,23]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1159\/000369343"},{"key":"e_1_2_9_3_1","first-page":"659","article-title":"Facioscapulohumeral muscular dystrophy","volume":"24","author":"Lemmers RJ","year":"2014","journal-title":"Gene Rev."},{"key":"e_1_2_9_4_1","first-page":"520","article-title":"Facioscapulohumeral muscular dystrophy","volume":"49","author":"Statland J","year":"2014","journal-title":"Neurol 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