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The initial NIV settings are simple, but progression of the disease, ventilator dependence and upper airway involvement sometimes make long\u2010term adjustment of NIV more difficult, with a major impact on survival. Unique data concerning the long\u2010term adjustment of NIV in ALS show that correction of leaks, management of obstructive apnoea and adaptation to the patient's degree of ventilator dependence improve the prognosis. Non\u2010ventilatory factors also impact the efficacy of NIV and various solutions have been described and must be applied, including cough assist techniques, control of excess salivation and renutrition. NIV in ALS has been considerably improved as a result of application of all of these measures, avoiding the need for tracheostomy in the very great majority of cases. More advanced use of NIV also requires pulmonologists to master the associated end\u2010of\u2010life palliative care, as well as the modalities of discontinuing ventilation when it becomes unreasonable.<\/jats:p>","DOI":"10.1111\/resp.13525","type":"journal-article","created":{"date-parts":[[2019,3,26]],"date-time":"2019-03-26T00:27:39Z","timestamp":1553560059000},"page":"521-530","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":84,"title":["NIV in amyotrophic lateral sclerosis: The \u2018when\u2019 and \u2018how\u2019 of the matter"],"prefix":"10.1111","volume":"24","author":[{"given":"Capucine","family":"Morelot\u2010Panzini","sequence":"first","affiliation":[{"name":"INSERM, UMRS1158 Neurophysiologie Respiratoire Exp\u00e9rimentale et Clinique Sorbonne Universit\u00e9  Paris France"},{"name":"Service de Pneumologie et R\u00e9animation M\u00e9dicale du D\u00e9partement R3S AP\u2010HP, Groupe Hospitalier Piti\u00e9\u2010Salp\u00eatri\u00e8re Charles Foix  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ga\u00eblle","family":"Bruneteau","sequence":"additional","affiliation":[{"name":"Institut du Cerveau et de la Moelle \u00e9pini\u00e8re, ICM, Inserm U 1127, CNRS UMR 7225 Sorbonne Universit\u00e9  Paris France"},{"name":"D\u00e9partement de Neurologie, Centre R\u00e9f\u00e9rent SLA APHP, Groupe Hospitalier Piti\u00e9\u2010Salp\u00eatri\u00e8re Charles Foix  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2395-4694","authenticated-orcid":false,"given":"Jesus","family":"Gonzalez\u2010Bermejo","sequence":"additional","affiliation":[{"name":"INSERM, UMRS1158 Neurophysiologie Respiratoire Exp\u00e9rimentale et Clinique Sorbonne Universit\u00e9  Paris France"},{"name":"Service de Pneumologie et R\u00e9animation M\u00e9dicale du D\u00e9partement R3S AP\u2010HP, Groupe Hospitalier Piti\u00e9\u2010Salp\u00eatri\u00e8re Charles Foix  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,3,25]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1159\/000351153"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2010.224501"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(14)70219-4"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.161.3.9805008"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.2174\/092986710791163939"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(96)91680-3"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1080\/21678421.2018.1425455"},{"key":"e_1_2_12_9_1","first-page":"S175","article-title":"Edaravone in the treatment of amyotrophic lateral sclerosis: efficacy and access to therapy \u2013 a roundtable discussion","volume":"24","author":"Brooks BR","year":"2018","journal-title":"Am. 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