{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,10]],"date-time":"2026-01-10T03:14:41Z","timestamp":1768014881927,"version":"3.49.0"},"reference-count":23,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2023,3,22]],"date-time":"2023-03-22T00:00:00Z","timestamp":1679443200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2023,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Respiratory insufficiency and its complications are the main cause of death in amyotrophic lateral sclerosis (ALS). Respiratory symptoms are scored in questions Q10 (dyspnoea) and Q11 (orthopnoea) of the Amyotrophic Lateral Sclerosis Functional Rating Scale\u2013Revised (ALSFRS\u2010R). The association of respiratory test alterations with respiratory symptoms is unclear.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with ALS and progressive muscular atrophy were included. We retrospectively recorded demographic data, ALSFRS\u2010R, forced vital capacity (FVC), maximal inspiratory (MIP) and expiratory (MEP) pressures, mouth occlusion pressure at 100\u2009ms, nocturnal oximetry (SpO<jats:sub>2<\/jats:sub>mean), arterial blood gases, and phrenic nerve amplitude (PhrenAmpl). Three groups were categorized: G1, normal Q10 and Q11; G2, abnormal Q10; and G3, abnormal Q10 and Q11 or only abnormal Q11. A binary logistic regression model explored independent predictors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We included 276 patients (153 men, onset age\u00a0=\u00a062.6\u00a0\u00b1\u200911.0\u2009years, disease duration\u00a0=\u00a013.0\u00a0\u00b1\u20099.6\u2009months, spinal onset in 182) with mean survival of 40.1\u00a0\u00b1\u200926.0\u2009months. Gender, onset region, and disease duration were similar in G1 (<jats:italic>n<\/jats:italic>\u00a0=\u2009149), G2 (<jats:italic>n<\/jats:italic>\u00a0=\u200978), and G3 (<jats:italic>n<\/jats:italic>\u00a0=\u200949). Time to noninvasive ventilation (NIV) was shorter in G3 (<jats:italic>p<\/jats:italic>\u00a0&lt;\u20090.001), but survival was similar. ALSFRS\u2010R subscores were significantly different (G1\u2009&gt;\u2009G2\u2009&gt;\u2009G3, <jats:italic>p<\/jats:italic>\u00a0&lt;\u20090.001), except for lower limb subscore (<jats:italic>p<\/jats:italic>\u00a0=\u20090.077). G2 and G3 patients were older than G1 (<jats:italic>p<\/jats:italic>\u00a0&lt;\u20090.001), and had lower FVC, MIP, MEP, PhrenAmpl, and SpO<jats:sub>2<\/jats:sub>mean. Independent predictors for G2 were MIP and SpO<jats:sub>2<\/jats:sub>mean; for G3, the only independent predictor was PhrenAmpl.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These three distinct ALS phenotypic respiratory categories represent progressive stages of ventilatory dysfunction, supporting ALSFRS\u2010R clinical relevance. Orthopnoea is a severe symptom that should prompt NIV, phrenic nerve response being an independent predictor. Early NIV promotes similar survival for G2 and G3.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.15765","type":"journal-article","created":{"date-parts":[[2023,3,6]],"date-time":"2023-03-06T04:59:15Z","timestamp":1678078755000},"page":"1594-1599","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Respiratory phenotypes in amyotrophic lateral sclerosis as determined by respiratory questions on the Amyotrophic Lateral Sclerosis Functional Rating Scale\u2013Revised and their relation to respiratory tests"],"prefix":"10.1111","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0727-5897","authenticated-orcid":false,"given":"Susana","family":"Pinto","sequence":"first","affiliation":[{"name":"Institute of Physiology, Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes, Centro de Estudos Egas Moniz, Faculdade de Medicina Universidade de Lisboa  Lisbon Portugal"},{"name":"Rehabiliteringsmedicin, Instituten f\u00f6r Medicinska Vetenskaper Uppsala Universitet\u2014Akademiska Sjukhuset  Uppsala Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8290-0410","authenticated-orcid":false,"given":"Miguel","family":"Oliveira\u00a0Santos","sequence":"additional","affiliation":[{"name":"Institute of Physiology, Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes, Centro de Estudos Egas Moniz, Faculdade de Medicina Universidade de Lisboa  Lisbon Portugal"},{"name":"Department of Neurosciences and Mental Health, Hospital de Santa Maria Centro Hospitalar Universit\u00e1rio de Lisboa\u2010Norte  Lisbon Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2111-4579","authenticated-orcid":false,"given":"Marta","family":"Gromicho","sequence":"additional","affiliation":[{"name":"Institute of Physiology, Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes, Centro de Estudos Egas Moniz, Faculdade de Medicina Universidade de Lisboa  Lisbon Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Swash","sequence":"additional","affiliation":[{"name":"Institute of Physiology, Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes, Centro de Estudos Egas Moniz, Faculdade de Medicina Universidade de Lisboa  Lisbon Portugal"},{"name":"Departments of Neurology and Neuroscience, Barts and London School of Medicine Queen Mary University of London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7556-0158","authenticated-orcid":false,"given":"Mamede","family":"de Carvalho","sequence":"additional","affiliation":[{"name":"Institute of Physiology, Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes, Centro de Estudos Egas Moniz, Faculdade de Medicina Universidade de Lisboa  Lisbon Portugal"},{"name":"Department of Neurosciences and Mental Health, Hospital de Santa Maria Centro Hospitalar 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