{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T10:57:09Z","timestamp":1779879429852,"version":"3.53.1"},"reference-count":7,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,1,13]],"date-time":"2025-01-13T00:00:00Z","timestamp":1736726400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Literature on the role of gastrostomy and noninvasive ventilation (NIV) in primary lateral sclerosis (PLS) is limited. We aim to investigate whether PLS patients develop dysphagia requiring feeding tubes or respiratory failure necessitating NIV.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted a retrospective study of PLS patients with a definite diagnosis followed at our center (1994\u20132024). Patients with marked dysphagia (score &lt;\u20093 on Question 3 of the ALSFRS\u2010R) received a recommendation for gastrostomy and were divided into two groups: G1\/G2 (accepted\/declined gastrostomy). We investigated NIV indications due to respiratory failure and compared these patients (G3) to those without respiratory impairment (G4). Demographic, clinical, and neurophysiological data were collected and compared.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010eight patients had a definite diagnosis of PLS. Gastrostomy was recommended to 18 (37.5%), yet only 7 patients (38.9%\u2010G1) consented. The median time to gastrostomy was 77\u2009months. Total survival and survival post\u2010gastrostomy recommendation were not different between G1 and G2. Six PLS patients (12.5%\u2010G3) developed respiratory failure and initiated NIV (median of 63\u2009months). At 63\u2009months, G3 had significantly lower median forced vital capacity (65% vs. 99%; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001) and phrenic nerve amplitude (0.43 vs. 0.75\u2009mV; <jats:italic>p<\/jats:italic>\u2009=\u20090.039), but a greater ALSFRS\u2010R slope (0.34 vs. 0.14; <jats:italic>p<\/jats:italic>\u2009=\u20090.046) and shorter survival (35 vs. 94.9\u2009months; <jats:italic>p<\/jats:italic>\u2009=\u20090.009) compared to G4.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Dysphagia requiring gastrostomy was common in our PLS cohort, but survival after gastrostomy recommendation did not differ between groups. Patients who developed respiratory impairment may represent a distinct group with faster disease progression and shorter survival. Our findings may contribute to a deeper understanding and improved management of PLS.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.28346","type":"journal-article","created":{"date-parts":[[2025,1,13]],"date-time":"2025-01-13T09:11:48Z","timestamp":1736759508000},"page":"450-456","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["The Role of Gastrostomy and Noninvasive Ventilation in Primary Lateral Sclerosis"],"prefix":"10.1002","volume":"71","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8290-0410","authenticated-orcid":false,"given":"Miguel","family":"Oliveira Santos","sequence":"first","affiliation":[{"name":"Department of Neurosciences and Mental Health ULS Hospital de Santa Maria  Lisbon Portugal"},{"name":"Institute of Physiology and Centro de Estudos Egas Moniz. Faculty of Medicine University of Lisbon  Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Domingues","sequence":"additional","affiliation":[{"name":"Institute of Physiology and Centro de Estudos Egas Moniz. Faculty of Medicine University of Lisbon  Lisbon Portugal"},{"name":"Department of Physical Medicine and Rehabilitation ULS Hospital de Santa Maria  Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Sim\u00e3o","sequence":"additional","affiliation":[{"name":"Institute of Physiology and Centro de Estudos Egas Moniz. Faculty of Medicine University of Lisbon  Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Gromicho","sequence":"additional","affiliation":[{"name":"Institute of Physiology and Centro de Estudos Egas Moniz. Faculty of Medicine University of Lisbon  Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"In\u00eas","family":"Alves","sequence":"additional","affiliation":[{"name":"Institute of Physiology and Centro de Estudos Egas Moniz. Faculty of Medicine University of Lisbon  Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7556-0158","authenticated-orcid":false,"given":"Mamede","family":"de Carvalho","sequence":"additional","affiliation":[{"name":"Department of Neurosciences and Mental Health ULS Hospital de Santa Maria  Lisbon Portugal"},{"name":"Institute of Physiology and Centro de Estudos Egas Moniz. Faculty of Medicine University of Lisbon  Lisbon Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,1,13]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1080\/14660820510012031"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1080\/17482960802382305"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2019-322541"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000200962.94777.71"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.3109\/17482968.2012.719237"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.2217\/nmt-2017-0051"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1080\/21678421.2020.1804591"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.28346","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,21]],"date-time":"2025-04-21T14:26:10Z","timestamp":1745245570000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.28346"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1,13]]},"references-count":7,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025,3]]}},"alternative-id":["10.1002\/mus.28346"],"URL":"https:\/\/doi.org\/10.1002\/mus.28346","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1,13]]},"assertion":[{"value":"2024-09-12","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-01-06","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-01-13","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}