{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,8]],"date-time":"2026-05-08T08:18:31Z","timestamp":1778228311869,"version":"3.51.4"},"reference-count":32,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2023,7,19]],"date-time":"2023-07-19T00:00:00Z","timestamp":1689724800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100005614","name":"Biogen","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005614","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>The leading clinical feature of 5q\u2010associated spinal muscular atrophy (SMA) is symmetric, proximal muscle weakness. Muscles involved in ventilation exhibit a specific pattern of denervation: intercostal muscles are severely atrophic, whereas the diaphragm muscle is less affected. The aim of this study was to investigate the involvement of diaphragmatic function by ultrasound imaging in adult patients with SMA and to quantify dynamics of diaphragmatic function during nusinersen treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Diaphragmatic thickness, thickening, and excursion during quiet breathing were assessed in 24 adult patients with SMA type 2 and 3 by diaphragm ultrasound imaging before and during nusinersen treatment and were correlated with spirometric parameters.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Diaphragm thickness was not reduced, but increased in a remarkable proportion of patients, whereas diaphragm thickening and excursion were reduced in about 20% to 30% of nusinersen\u2010naive, adult patients with SMA types 2 and 3. During 26\u2009months of nusinersen treatment, diaphragm thickening fraction and excursion improved.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Diaphragm ultrasound imaging can provide disease\u2010 and treatment\u2010relevant information that is not identified during routine clinical assessments and may therefore be a valuable complementary outcome measure.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27938","type":"journal-article","created":{"date-parts":[[2023,7,19]],"date-time":"2023-07-19T11:55:02Z","timestamp":1689767702000},"page":"278-285","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Impaired diaphragmatic motility in treatment\u2010naive adult patients with spinal muscular atrophy improved during nusinersen treatment"],"prefix":"10.1002","volume":"68","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0941-6075","authenticated-orcid":false,"given":"Maren","family":"Freigang","sequence":"first","affiliation":[{"name":"Department of Neurology, University Hospital Carl Gustav Carus Technische Universit\u00e4t Dresden  Dresden Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simona","family":"Langner","sequence":"additional","affiliation":[{"name":"Division of Pulmonology, Medical Department I, University Hospital Carl Gustav Carus Technische Universit\u00e4t Dresden  Dresden Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Hermann","sequence":"additional","affiliation":[{"name":"Translational Neurodegeneration Section \u201cAlbrecht\u2010Kossel,\u201d Department of Neurology, University Medical Center Rostock University of Rostock  Rostock Germany"},{"name":"Center for Transdisciplinary Neurosciences Rostock, University Medical Center Rostock University of Rostock  Rostock Germany"},{"name":"Deutsches Zentrum f\u00fcr Neurodegenerative Erkrankungen Rostock\/Greifswald  Rostock Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ren\u00e9","family":"G\u00fcnther","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital Carl Gustav Carus Technische Universit\u00e4t Dresden  Dresden Germany"},{"name":"Deutsches Zentrum f\u00fcr Neurodegenerative Erkrankungen Dresden  Dresden 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