{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T01:38:29Z","timestamp":1787017109928,"version":"build-2736575974"},"reference-count":24,"publisher":"BMJ","issue":"6","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Pract Neurol"],"accepted":{"date-parts":[[2023,7,25]]},"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:p>Patients with neuromuscular diseases (NMD) can present to the neurologist with symptoms and signs of respiratory failure, either acutely or as an insidious process in the outpatient setting. Since the advent of non-invasive ventilation, the outcomes of patients with ventilatory failure due to NMD have dramatically improved. However, the natural history of different NMDs requires a nuanced approach to respiratory investigation and management. Respiratory failure dictates the prognosis of many NMDs and timing the most appropriate investigation and referral to ventilation services is crucial in optimising care.<\/jats:p>","DOI":"10.1136\/pn-2023-003841","type":"journal-article","created":{"date-parts":[[2023,11,17]],"date-time":"2023-11-17T13:20:15Z","timestamp":1700227215000},"page":"464-475","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":4,"title":["Ventilatory failure in chronic neuromuscular disease"],"prefix":"10.1136","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9286-2887","authenticated-orcid":false,"given":"Gabriella Heloise","family":"Long","sequence":"first","affiliation":[{"name":"North West Ventilation Unit, Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Bentley","sequence":"additional","affiliation":[{"name":"North West Ventilation Unit, Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, UK"},{"name":"Manchester Academic Health Sciences Centre, University of Manchester, Manchester, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Roberts","sequence":"additional","affiliation":[{"name":"North West Ventilation Unit, Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, UK"},{"name":"Muscle Diseases Unit, Salford Royal Hospital Manchester Centre for Clinical Neurosciences, Salford, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9230-4137","authenticated-orcid":false,"given":"James B","family":"Lilleker","sequence":"additional","affiliation":[{"name":"Muscle Diseases Unit, Salford Royal Hospital Manchester Centre for Clinical Neurosciences, Salford, UK"},{"name":"Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Sciences, University of Manchester, Manchester, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2023,11,17]]},"reference":[{"key":"2025090409183825000_23.6.464.1","unstructured":"National Institute for Health and Care Excellence [NICE] . Motor neurone disease: assessment and management [NG42], . 2014 Available: https:\/\/www.nice.org.uk\/guidance\/ng42\/chapter\/recommendations [Accessed 21 Feb 2023]."},{"key":"2025090409183825000_23.6.464.2","doi-asserted-by":"crossref","first-page":"ii1","DOI":"10.1136\/thoraxjnl-2015-208209","article-title":"BTS\/ICS guideline for the ventilatory management of acute hypercapnic respiratory failure in adults","volume":"71 Suppl 2","author":"Davidson","year":"2016","journal-title":"Thorax"},{"key":"2025090409183825000_23.6.464.3","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.58.6.893"},{"key":"2025090409183825000_23.6.464.4","doi-asserted-by":"crossref","unstructured":"Konstantelou E , Pasparaki E , Tzilas V , et al . Neuromuscular diseases. In: Wuyts WA , Cottin V , Spagnolo P , eds. Pulmonary manifestations of systemic diseases (ERS Monograph). Sheffield: European Respiratory Society, 2019: 278\u201395. doi:10.1183\/2312508X.erm8619","DOI":"10.1183\/2312508X.erm8619"},{"key":"2025090409183825000_23.6.464.5","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201412-2224CI"},{"key":"2025090409183825000_23.6.464.6","doi-asserted-by":"crossref","unstructured":"Steier J , Kent B . \u2018Sleep-disordered breathing\u2019. In: Leschziner G , ed. Oxford handbook of sleep medicine, Oxford medical handbooks. Oxford, 2022.","DOI":"10.1093\/med\/9780192848253.003.0007"},{"key":"2025090409183825000_23.6.464.7","doi-asserted-by":"crossref","unstructured":"Walters J . Weakness in the intensive care unit. Pract Neurol 2022:pn-2022-003422. doi:10.1136\/pn-2022-003422","DOI":"10.1136\/pn-2022-003422"},{"key":"2025090409183825000_23.6.464.8","doi-asserted-by":"crossref","first-page":"394","DOI":"10.1016\/j.chest.2023.03.011","article-title":"Respiratory management of patients with neuromuscular weakness: an American college of chest physicians clinical practice guideline and expert panel report","volume":"164","author":"Khan","year":"2023","journal-title":"Chest"},{"key":"2025090409183825000_23.6.464.9","doi-asserted-by":"crossref","first-page":"224","DOI":"10.5935\/1984-0063.20200119","article-title":"Oxygen desaturation index as alternative parameter in screening patients with severe obstructive sleep apnea","volume":"15","author":"Varghese","year":"2022","journal-title":"Sleep Sci"},{"key":"2025090409183825000_23.6.464.10","unstructured":"National Institute for Health and Care Excellence [NICE] . Obstructive sleep apnoea\/hypopnoea syndrome and obesity hypoventilation syndrome in over 16s [NG202], . 2021 Available: https:\/\/www.nice.org.uk\/guidance\/ng202\/chapter\/terms-used-in-this-guideline#severity-of-osahs [Accessed 21 Feb 2023]."},{"key":"2025090409183825000_23.6.464.11","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2021-217205"},{"key":"2025090409183825000_23.6.464.12","doi-asserted-by":"publisher","DOI":"10.1002\/mus.24929"},{"key":"2025090409183825000_23.6.464.13","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm\/138.4.862"},{"key":"2025090409183825000_23.6.464.14","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200403-314OC"},{"key":"2025090409183825000_23.6.464.15","doi-asserted-by":"publisher","DOI":"10.1183\/20734735.0021711"},{"key":"2025090409183825000_23.6.464.16","doi-asserted-by":"crossref","unstructured":"Janssens J-P , Michel F , Schwarz EI , et al . Long-term mechanical ventilation: recommendations of the Swiss society of pulmonology. Respiration 2020:1\u201336. doi:10.1159\/000510086","DOI":"10.1159\/000510086"},{"key":"2025090409183825000_23.6.464.17","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1513\/AnnalsATS.202002-169OC","article-title":"Noninvasive ventilation use is associated with better survival in amyotrophic lateral sclerosis","volume":"18","author":"Ackrivo","year":"2021","journal-title":"Ann Am Thorac Soc"},{"key":"2025090409183825000_23.6.464.18","article-title":"Mechanical ventilation for amyotrophic lateral sclerosis\/motor neuron disease","volume":"10","author":"Radunovic","year":"2017","journal-title":"Cochrane Database Syst Rev"},{"key":"2025090409183825000_23.6.464.19","doi-asserted-by":"crossref","first-page":"702","DOI":"10.1016\/S1474-4422(15)00104-0","article-title":"Gastrostomy in patients with amyotrophic lateral sclerosis (ProGas): a prospective cohort study","volume":"14","year":"2015","journal-title":"Lancet Neurol"},{"key":"2025090409183825000_23.6.464.20","doi-asserted-by":"publisher","DOI":"10.1136\/practneurol-2018-002109"},{"key":"2025090409183825000_23.6.464.21","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2021-218110"},{"key":"2025090409183825000_23.6.464.22","doi-asserted-by":"publisher","DOI":"10.1097\/ALN.0000000000001877"},{"key":"2025090409183825000_23.6.464.23","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(98)03078-5"},{"key":"2025090409183825000_23.6.464.24","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1111\/anae.15317","article-title":"Malignant hyperthermia 2020: guideline from the association of anaesthetists","volume":"76","author":"Hopkins","year":"2021","journal-title":"Anaesthesia"}],"container-title":["Practical Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/pn-2023-003841","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,4]],"date-time":"2025-09-04T16:18:49Z","timestamp":1757002729000},"score":1,"resource":{"primary":{"URL":"https:\/\/pn.bmj.com\/lookup\/doi\/10.1136\/pn-2023-003841"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11,17]]},"references-count":24,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2023,11,23]]},"published-print":{"date-parts":[[2023,12]]}},"alternative-id":["10.1136\/pn-2023-003841"],"URL":"https:\/\/doi.org\/10.1136\/pn-2023-003841","relation":{},"ISSN":["1474-7758","1474-7766"],"issn-type":[{"value":"1474-7758","type":"print"},{"value":"1474-7766","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,11,17]]}}}