{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T15:02:38Z","timestamp":1774364558354,"version":"3.50.1"},"reference-count":25,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2024,5,25]],"date-time":"2024-05-25T00:00:00Z","timestamp":1716595200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Amyotrophic lateral sclerosis (ALS) is a neuromuscular progressive disorder characterized by limb and bulbar muscle wasting and weakness. A total of 30% of patients present a bulbar onset, while 70% have a spinal outbreak. Respiratory involvement represents one of the worst prognostic factors, and its early identification is fundamental for the early starting of non-invasive ventilation and for the stratification of patients. Due to the lack of biomarkers of early respiratory impairment, we aimed to evaluate the role of chest dynamic MRI in ALS patients. Methods: We enrolled 15 ALS patients and 11 healthy controls. We assessed the revised ALS functional rating scale, spirometry, and chest dynamic MRI. Data were analyzed by using the Mann\u2013Whitney U test and Cox regression analysis. Results: We observed a statistically significant difference in both respiratory parameters and pulmonary measurements at MRI between ALS patients and healthy controls. Moreover, we found a close relationship between pulmonary measurements at MRI and respiratory parameters, which was statistically significant after multivariate analysis. A sub-group analysis including ALS patients without respiratory symptoms and with normal spirometry values revealed the superiority of chest dynamic MRI measurements in detecting signs of early respiratory impairment. Conclusions: Our data suggest the usefulness of chest dynamic MRI, a fast and economically affordable examination, in the evaluation of early respiratory impairment in ALS patients.<\/jats:p>","DOI":"10.3390\/jcm13113103","type":"journal-article","created":{"date-parts":[[2024,5,27]],"date-time":"2024-05-27T09:33:31Z","timestamp":1716802411000},"page":"3103","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Chest Dynamic MRI as Early Biomarker of Respiratory Impairment in Amyotrophic Lateral Sclerosis Patients: A Pilot Study"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4642-6827","authenticated-orcid":false,"given":"Francesco","family":"Barbato","sequence":"first","affiliation":[{"name":"Department of Emergency and Urgent Medicine, Stroke Unit, Santa Maria delle Grazie Hospital, 80078 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3961-6780","authenticated-orcid":false,"given":"Alessandro","family":"Bombaci","sequence":"additional","affiliation":[{"name":"PhD Program of Neuroscience, Department of Neuroscience \u201cRita Levi Montalcini\u201d, University of Turin, 10124 Turin, Italy"},{"name":"Neurology Unit, IRCSS Policlinico San Donato, 20097 San Donato Milanese, Italy"},{"name":"Department of Neurology, Vita-Salute San Raffaele University, 20132 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2253-069X","authenticated-orcid":false,"given":"Giovanni","family":"Colacicco","sequence":"additional","affiliation":[{"name":"NeuroMuscular Omnicentre (NEMO), Serena Onlus, 20162 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9388-9336","authenticated-orcid":false,"given":"Giorgia","family":"Bruno","sequence":"additional","affiliation":[{"name":"Division of Pediatric Neurology, Department of Neurosciences, \u201cSantobono-Pausilipon\u201d Children\u2019s Hospital, 80121 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Domenico","family":"Ippolito","sequence":"additional","affiliation":[{"name":"NeuroMuscular Omnicentre (NEMO), Serena Onlus, 20162 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9999-3388","authenticated-orcid":false,"given":"Vincenzo","family":"Pota","sequence":"additional","affiliation":[{"name":"Department of Women, Child, General and Specialistic Surgery, University of Campania \u201cLuigi Vanvitelli\u201d, 81100 Caserta, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Salvatore","family":"Dongiovanni","sequence":"additional","affiliation":[{"name":"NeuroMuscular Omnicentre (NEMO), Serena Onlus, 20162 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9518-9744","authenticated-orcid":false,"given":"Giacomo","family":"Sica","sequence":"additional","affiliation":[{"name":"Department of Radiology, Monaldi Hospital, Azienda Ospedaliera dei Colli, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giorgio","family":"Bocchini","sequence":"additional","affiliation":[{"name":"Department of Radiology, Monaldi Hospital, Azienda Ospedaliera dei Colli, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8799-0398","authenticated-orcid":false,"given":"Tullio","family":"Valente","sequence":"additional","affiliation":[{"name":"Department of Radiology, Monaldi Hospital, Azienda Ospedaliera dei Colli, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0910-8064","authenticated-orcid":false,"given":"Mariano","family":"Scaglione","sequence":"additional","affiliation":[{"name":"Department of Medicine, Surgery and Pharmacy, University of Sassari, 07100 Sassari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3592-808X","authenticated-orcid":false,"given":"Pier Paolo","family":"Mainenti","sequence":"additional","affiliation":[{"name":"Institute of Biostructures and Bioimaging of the National Council of Research (CNR), 80145 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Salvatore","family":"Guarino","sequence":"additional","affiliation":[{"name":"Department of Radiology, Monaldi Hospital, Azienda Ospedaliera dei Colli, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,5,25]]},"reference":[{"key":"ref_1","unstructured":"(2023, October 01). Available online: https:\/\/www.orpha.net\/consor\/cgi-bin\/OC_Exp.php?Lng=IT&Expert=803."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"17071","DOI":"10.1038\/nrdp.2017.71","article-title":"Amyotrophic lateral sclerosis","volume":"3","author":"Hardiman","year":"2017","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2211","DOI":"10.1007\/s10072-021-05188-0","article-title":"Cognitive dysfunction in amyotrophic lateral sclerosis: Can we predict it?","volume":"42","author":"SIgN","year":"2021","journal-title":"Neurol. Sci."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1136\/jnnp-2019-320624","article-title":"Plasma pNfH levels differentiate SBMA from ALS","volume":"91","author":"Lombardi","year":"2019","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1111\/cge.12117","article-title":"Clinical and genetic heterogeneity of amyotrophic lateral sclerosis","volume":"83","author":"Sabatelli","year":"2013","journal-title":"Clin. Genet."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"3457","DOI":"10.1007\/s10072-022-05889-0","article-title":"Neuromuscular diseases in the pandemic age: 6 months of experience of a newborn clinical center","volume":"43","author":"Barbato","year":"2022","journal-title":"Neurol. Sci."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"e2022470","DOI":"10.1590\/1516-3180.2022.0470.r1.100423","article-title":"Noninvasive mechanical ventilation assistance in amyotrophic lateral sclerosis: A systematic review","volume":"142","author":"Gama","year":"2024","journal-title":"Sao Paulo Med. J."},{"key":"ref_8","doi-asserted-by":"crossref","unstructured":"American Thoracic Society\/European Respiratory Society (2002). ATS\/ERS Statement on Respiratory Muscle Testing. Am. J. Respir. Crit. Care Med., 166, 518\u2013624.","DOI":"10.1164\/rccm.166.4.518"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.nmd.2021.11.001","article-title":"Diaphragmatic dysfunction in neuromuscular disease, an MRI study","volume":"32","author":"Harlaar","year":"2021","journal-title":"Neuromuscul. Disord."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"2752","DOI":"10.1007\/s00415-019-09481-z","article-title":"Imaging respiratory muscle quality and function in Duchenne muscular dystrophy","volume":"266","author":"Barnard","year":"2019","journal-title":"J. Neurol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1016\/j.chest.2021.08.078","article-title":"Characterizing Expiratory Respiratory Muscle Degeneration in Duchenne Muscular Dystrophy Using MRI","volume":"161","author":"Barnard","year":"2021","journal-title":"Chest"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"852","DOI":"10.1016\/j.nmd.2015.09.003","article-title":"Clinical and pathophysiological clues of respiratory dysfunction in late-onset Pompe disease: New insights from a comparative study by MRI and respiratory function assessment","volume":"25","author":"Gaeta","year":"2015","journal-title":"Neuromuscul. Disord."},{"key":"ref_13","doi-asserted-by":"crossref","unstructured":"Wens, S.C., Ciet, P., Perez-Rovira, A., Logie, K., Salamon, E., Wielopolski, P., de Bruijne, M., E Kruijshaar, M., Tiddens, H.A., and A van Doorn, P. (2015). Lung MRI and impairment of diaphragmatic function in Pompe disease. BMC Pulm. Med., 15.","DOI":"10.1186\/s12890-015-0058-3"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1186\/s13023-020-01627-x","article-title":"Chest MRI to diagnose early diaphragmatic weakness in Pompe disease","volume":"16","author":"Harlaar","year":"2021","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1002\/acn3.440","article-title":"Respiratory magnetic resonance imaging biomarkers in Duchenne muscular dystrophy","volume":"4","author":"Mankodi","year":"2017","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"9","DOI":"10.3389\/fneur.2018.00009","article-title":"Semi-Automated Analysis of Diaphragmatic Motion with Dynamic Magnetic Resonance Imaging in Healthy Controls and Non-Ambulant Subjects with Duchenne Muscular Dystrophy","volume":"9","author":"Bishop","year":"2018","journal-title":"Front. Neurol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1002\/jmri.26864","article-title":"Diaphragm Involvement in Duchenne Muscular Dystrophy (DMD): An MRI Study","volume":"51","author":"Pennati","year":"2019","journal-title":"J. Magn. Reson. Imaging"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"19006","DOI":"10.1038\/s41598-019-54314-8","article-title":"Dynamic respiratory muscle function in late-onset Pompe disease","volume":"9","author":"Smith","year":"2019","journal-title":"Sci. Rep."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1093\/brain\/115.2.495","article-title":"Primary lateral sclerosis: Clinical features, neuropathology and diagnostic criteria","volume":"115","author":"Pringle","year":"1992","journal-title":"Brain"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3233\/JND-170276","article-title":"Diaphragm: Pathophysiology and Ultrasound Imaging in Neuromuscular Disorders","volume":"5","author":"Fayssoil","year":"2018","journal-title":"J. Neuromuscul. Dis."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1159\/000446518","article-title":"A Review of the Ultrasound Assessment of Diaphragmatic Function in Clinical Practice","volume":"91","author":"Papa","year":"2016","journal-title":"Respiration"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1040","DOI":"10.1016\/j.chest.2017.08.1157","article-title":"Diaphragm Dysfunction in Critical Illness","volume":"153","author":"Supinski","year":"2018","journal-title":"Chest"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/j.ccm.2018.01.013","article-title":"Assessing Diaphragm Function in Chest Wall and Neuromuscular Diseases","volume":"39","author":"Minami","year":"2018","journal-title":"Clin. Chest Med."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1002\/mus.23671","article-title":"Neuromuscular ultrasound for evaluation of the diaphragm","volume":"47","author":"Sarwal","year":"2013","journal-title":"Muscle Nerve"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1756286419857040","DOI":"10.1177\/1756286419857040","article-title":"Non-invasive ventilation in amyotrophic lateral sclerosis","volume":"12","author":"Dorst","year":"2019","journal-title":"Ther. Adv. Neurol. 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