Optimizing the noninvasive ventilation pathway for patients with amyotrophic lateral sclerosis/motor neuron disease: a systematic review

References

• Bourke SC, Bullock RE, Williams TL, Shaw PJ, Gibson GJ. Noninvasive ventilation in ALS: indications and effect on quality of life. Neurology. 2003;61:171–7.
   PubMed Web of Science ®Google Scholar
• O'Neill CL, Williams TL, Peel ET, McDermott CJ, Faull C. Non-invasive ventilation in motor neuron disease: an update of current UK practice. J Neurol Neurosurg Psychiatry. 2012;83:371–6.
   PubMed Web of Science ®Google Scholar
• Khamankar N, Coan G, Weaver B, Mitchell CS. Associative increases in amyotrophic lateral sclerosis survival duration with non-invasive ventilation initiation and usage protocols. Front Neurol. 2018;9:578.
   PubMed Web of Science ®Google Scholar
• Gruis KL, Brown DL, Schoennemann A, Zebarah VA, Feldman EL. Predictors of noninvasive ventilation tolerance in patients with amyotrophic lateral sclerosis. Muscle Nerve. 2005;32:808–11.
   PubMed Web of Science ®Google Scholar
• Oliver D, Turner MR. Some difficult decisions in ALS/MND. Amyotroph Lateral Scler Frontotemporal Degener. 2010;11:339–43.
   PubMed Web of Science ®Google Scholar
• Chiò A, Calvo A, Moglia C, Gamna F, Mattei A, Mazzini L, et al. Non-invasive ventilation in amyotrophic lateral sclerosis: a 10 year population based study. J Neurol Neurosurg Psychiatry. 2012;83:377–81.
   PubMed Web of Science ®Google Scholar
• Radunovic A, Annane D, Rafiq MK, Mustfa N. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev. 2013;3:CD004427.
   Google Scholar
• Liberati A, Altman DG, Tetzlaff J, Mulrow C, Gøtzsche PC, Ioannidis JPA, et al. The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate health care interventions: explanation and elaboration. PLoS Med. 2009;6:e1000100.
   PubMed Web of Science ®Google Scholar
• Centre for Reviews and Dissemination. CRD's guidance for undertaking reviews in health care. CRD, University of York; 2009.
   Google Scholar
• Thomas J, Harden A, Oakley A, Oliver S, Sutcliffe K, Rees R, et al. Integrating qualitative research with trials in systematic reviews. Brit Med J. 2004;328:1010–2.
   PubMed Web of Science ®Google Scholar
• Yampolskaya S, Nesman TM, Hernandez M, Koch D. Using concept mapping to develop a logic model and articulate a program theory: a case example. Am J Eval. 2004;25:191–207.
   Web of Science ®Google Scholar
• Masterson-Algar P, Burton CR, Rycroft-Malone J. Process evaluations in neurological rehabilitation: a mixed-evidence systematic review and recommendations for future research. BMJ Open. 2016;6:11.
   Web of Science ®Google Scholar
• Andersen PM, Abrahams S, Borasio GD, de Carvalho M, Chio A, Van Damme P, et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS) - revised report of an EFNS task force. Eur J Neurol. 2012;19:360–75.
   PubMed Web of Science ®Google Scholar
• Andersen PM, Borasio GD, Dengler R, Hardiman O, Kollewe K, Leigh PN, et al. EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients and relatives: an evidence-based review with good practice points. Eur J Neurol. 2005;12:921–38.
   PubMed Web of Science ®Google Scholar
• Andersen PM, Borasio GD, Dengler R, Hardiman O, Kollewe K, Leigh PN, et al. Good practice in the management of amyotrophic lateral sclerosis: clinical guidelines. An evidence-based review with good practice points. EALSC Working Group. Amyotroph Lateral Scler Frontotemporal Degener. 2007;84:195–213.
   Google Scholar
• Vitacca M, Vianello A, Scientific Group on Respiratory Intensive Care of the Italian Association of Hospital Pulmonologists. Respiratory outcomes of patients with amyotrophic lateral sclerosis: an Italian nationwide survey. Respir Care. 2013;589:1433–41.
   Google Scholar
• Burden T, Davis C, Johnstone E, Spence J, Shrikrishna D. . Experience of a joint palliative and respiratory clinic on NIV treatment initiation in motor neurone disease. Thorax. 2016;71:A126.
   PubMed Web of Science ®Google Scholar
• Nixon I, Popkiewicz F, Banerjee S, Vincent-Smith L, Oliver D. Using a joint approach to non-invasive ventilation in motor neurone disease. Eur J Palliat Care. 2015;22:182–4.
   Google Scholar
• Oliver D, Banerjee S, Vincent-Smith L. The development of a multidisciplinary clinic to improve respiratory care of people with MND/ALS in Medway area. Amyotroph Lateral Scler Frontotemporal Degener. 2015;16:124–5.
   Google Scholar
• Kareus SA, Fewell DF, Kagebein S, Rudnicki SA. Effect of respiratory therapist on noninvasive ventilation use in patients with amyotrophic lateral sclerosis. Neurology. 2006;66:A231–A.
   Web of Science ®Google Scholar
• Kareus SA, Kagebein S, Rudnicki SA. The importance of a respiratory therapist in the ALS clinic. Amyotroph Lateral Scler Frontotemporal Degener. 2008;9:173–6.
   PubMed Web of Science ®Google Scholar
• Pinto A, de Carvalho M, Evangelista T, Lopes A, Sales‐Luís L. Nocturnal pulse oximetry: a new approach to establish the appropriate time for non-invasive ventilation in ALS patients. Amyotroph Lateral Scler Frontotemporal Degener. 2003;4:31–5.
   PubMed Web of Science ®Google Scholar
• Cedarbaum JM, Stambler N. Disease status and use of ventilatory support by ALS patients. BDNF Study Group. Amyotroph Lateral Scler Frontotemporal Degener. 2001;2:19–22.
   PubMed Web of Science ®Google Scholar
• Terzano C, Romani S. Early use of noninvasive ventilation in patients with amyotrophic lateral sclerosis: what benefits? Eur Rev Med Pharmacol Sci. 2015;19:4304–13.
   PubMed Web of Science ®Google Scholar
• National Institute for Health and Care Excellence. Motor Neurone Disease Assessment and Management NICE Guideline NG42. London: National Institute for Health and Care Excellence; 2016.
   Google Scholar
• Elman LB, Siderowf AD, McCluskey LF. Nocturnal oximetry: utility in the respiratory management of amyotrophic lateral sclerosis. Am J Phys Med Rehabil. 2003;82:866–70.
   PubMed Web of Science ®Google Scholar
• Pinto S, de Carvalho M. Correlation between forced vital capacity and slow vital capacity for the assessment of respiratory involvement in amyotrophic lateral sclerosis: a prospective study. Amyotroph Lateral Scler Frontotemporal Degener. 2017;18:86–91.
   PubMed Web of Science ®Google Scholar
• Yamauchi R, Imai T, Tsuda E, Hozuki T, Yamamoto D, Shimohama S. Respiratory insufficiency with preserved diaphragmatic function in amyotrophic lateral sclerosis. Intern Med. 2014;53:1325–31.
   PubMed Web of Science ®Google Scholar
• Yamauchi R, Imai T, Tsuda E, Hozuki T, Yamamoto D, Shimohama S, et al. Respiratory impairment with preserved diaphragmatic function in ALS. Clin Neurophysiol. 2013;124:e36.
   Google Scholar
• Yamauchi R, Imai T, Tsuda E, Yamamoto D, Hozuki T, Shimohama S. Respiratory assistive care applied to hypoventilation without significant diaphragmatic dysfunction in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2013;14.
   Google Scholar
• Carratu P, D'Alba G, Marra L, Dragonieri S, Cassano G, Capozzolo A, et al. Sniff nasal inspiratory pressure test is an early marker of diurnal hypercapnia in amyotrophic lateral sclerosis patients. Preliminary results. Eur Respir J. 2016;48:PA2165.
   Web of Science ®Google Scholar
• Chaudri MB, Liu C, Watson L, Jefferson D, Kinnear WJ. Sniff nasal inspiratory pressure as a marker of respiratory function in motor neuron disease. Eur Respir J. 2000;15:539–42.
   PubMed Web of Science ®Google Scholar
• Jackson CE, Rosenfeld J, Moore DH, Bryan WW, Barohn RJ, Wrench M, et al. A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of hypoventilation in ALS/MND patients. J Neurol Sci. 2001;191:75–8.
   PubMed Web of Science ®Google Scholar
• Chechyk N, Rushkevich Y, Likhachev S. Sleep-disordered breathing in various onsets of amyotrophic lateral sclerosis. J Neurol Sci. 2017;381:293.
   Web of Science ®Google Scholar
• Sugie M, Ando N, Ueno S. Usefulness of polysomnography at an early stage of amyotrophic lateral sclerosis. Rinsho Shinkeigaku. 2006;46:297–300.
   PubMedGoogle Scholar
• Vrijsen B, Buyse B, Belge C, Vanpee G, Van Damme P, Testelmans D, et al. Randomized cross-over trial of ventilator modes during non-invasive ventilation titration in amyotrophic lateral sclerosis. Respirology. 2017;22:1212–8.
   PubMed Web of Science ®Google Scholar
• Prell T, Ringer TM, Wullenkord K, Garrison P, Gunkel A, Stubendorff B, et al. Assessment of pulmonary function in amyotrophic lateral sclerosis: when can polygraphy help evaluate the need for non-invasive ventilation? J Neurol Neurosurg Psychiatry. 2016;87:1022–6.
   PubMed Web of Science ®Google Scholar
• Loewen AHS, Korngut L, Rimmer K, Damji O, Turin TC, Hanly PJ, et al. Limitations of split-night polysomnography for the diagnosis of nocturnal hypoventilation and titration of non-invasive positive pressure ventilation in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2014;15:494.
   PubMed Web of Science ®Google Scholar
• Desai U, Langford V, Bravver E, Brooks B. Timely intervention of non-invasive ventilation (NIV) to treat sleep-ordered breathing (SDB) causing respiratory insufficiency (RI) in amyotrophic lateral sclerosis (ALS) patients is enhanced by implementation of home-based unattended sleep study (HBUSS): Results of the first quarterly audit at the Carolinas neuromuscular/ALS-MDA center. Neurology. 2012;78:P01.104-P01.104
   Web of Science ®Google Scholar
• Desai U, Langford V, Bravver E, Brooks BR. Home based unattended sleep study as an integral component of multidisciplinary ALS clinic. Amyotroph Lateral Scler Frontotemporal Degener. 2011;12 (Suppl 1): 60–68.
   Google Scholar
• Desai U, Langford VL, Bravver E, Sanjak M, et al. Home based unattended sleep study accelerates non-invasive ventilation (NIV) deployment for amyotrophic lateral sclerosis (ALS) patients: audit results at Carolinas neuromuscular/ALSMDA center. Sleep. 2012;35:A287.
   PubMed Web of Science ®Google Scholar
• Johnson O, Rodger J, Robertson A. Home initiation of non-invasive ventilation for motor neurone disease. Amyotroph Lateral Scler Frontotemporal Degener. 2009;10:59–60.
   Google Scholar
• Rafiq MK, Bradburn M, Proctor AR, Billings C, Bianchi S, McDermott CJ, et al. Using transcutaneous carbon dioxide monitor (TOSCA 500) to detect respiratory failure in patients with amyotrophic lateral sclerosis: a validation study. Amyotroph Lateral Scler Frontotemporal Degener. 2012;13:528–32.
   PubMed Web of Science ®Google Scholar
• Onders R, Elmo M, Kaplan C, Katirji B, Katirji B, Schilz R. Diaphragm functional analysis at the upper and lower spectrum of forced vital capacity (FVC) in ALS/MND: FVC inadequately assesses diaphragm function or upper motor neuron involvement for stimulatability. Amyotroph Lateral Scler Frontotemporal Degener. 2013;14 (Suppl 2): 1–63.
   Google Scholar
• Fantini R, Mandrioli J, Zona S, Antenora F, Iattoni A, Monelli M, et al. Ultrasound assessment of diaphragmatic function in patients with amyotrophic lateral sclerosis. Respirology. 2016;21:932–8.
   PubMed Web of Science ®Google Scholar
• Karwa K, Malek A, Stickler D. Diaphragm ultrasound: a potential outcome measure in amyotrophic lateral sclerosis. Neurology. 2015;84 (Suppl 14) 4.
   Google Scholar
• Stewart H, Eisen A, Road J, Mezei M, Weber M. Electromyography of respiratory muscles in amyotrophic lateral sclerosis. J Neurol Sci. 2001;191:67–73.
   PubMed Web of Science ®Google Scholar
• Jenkins L, Sakamuri S, Miller RG, Forshew D, Guion L, Moore D, et al. Does bulbar dysfunction render respiratory assessment by standard pulmonary function tests meaningless? Quantifying the impact of bulbar dysfunction on measures of respiratory insufficiency in 100 patients with ALS. Amyotroph Lateral Scler Frontotemporal Degener. 2014;15:98–9.
   PubMedGoogle Scholar
• Parker J, Robinson GV, McGown AD, Davies C. Predictors of successful domiciliary non-invasive ventilation for motor neurone disease in a district general hospital. Thorax. 2009;64:A75.
   PubMed Web of Science ®Google Scholar
• Chio A, Silani V, Italian ALS, Study G. Amyotrophic lateral sclerosis care in Italy: a nationwide study in neurological centers. J Neurol Sci. 2001;191:145–50.
   PubMed Web of Science ®Google Scholar
• Kartas S, Morelo-Panzini C, Golmard JL, Dorsett J, Meininger V, Similowski T, et al. How does the "real-life" practice of non-invasive ventilation in amyotrophic lateral sclerosis at a large reference center compare with consensus expert recommendations? Amyotroph Lateral Scler Frontotemporal Degener. 2011;12 (Suppl 1): 60–68.
   Google Scholar
• Georges M, Golmard J-L, Llontop C, Shoukri A, Salachas F, Similowski T, et al. Initiation of non-invasive ventilation in amyotrophic lateral sclerosis and clinical practice guidelines: single-centre, retrospective, descriptive study in a national reference centre. Amyotroph Lateral Scler Frontotemporal Degener. 2017;18:46–52.
   PubMed Web of Science ®Google Scholar
• Cazzolli P, Brooks BR, Lewarski J, McKim D, Nakayama Y, Chatburn R. Independent factors associated with failed use of noninvasive ventilation (NIV) in patients with ALS/MND. Amyotroph Lateral Scler Frontotemporal Degener. 2014;15:38–9.
   Google Scholar
• Juntas-Morales R, Pageot N, Alphandery S, Camu W. Worsening of respiratory function is predictive of prognosis of ALS even at a very early stage. Amyotroph Lateral Scler Frontotemporal Degener. 2015;16 (Suppl 1): 124–135.
   Google Scholar
• Ruffell TO, Martin NH, Janssen A, Ampong MA, Knights C, Burman R, et al. Healthcare professionals' views about the provision of gastrostomy and non-invasive ventilation in amyotrophic lateral sclerosis (ALS). Amyotroph Lateral Scler Frontotemporal Degener. 2012;13 (Suppl 1) 124–135.
   Google Scholar
• Ruffell TO, Martin NH, Janssen A, Wijesekera L, Knights C, Burman R, et al. Healthcare professionals' views on the provision of gastrostomy and noninvasive ventilation to amyotrophic lateral sclerosis patients in England, Wales, and Northern Ireland. J Palliat Care. 2013;29:225–31.
   PubMed Web of Science ®Google Scholar
• Kewin P, Tsim S, McGlone J, Clarke A, Banham S, Davidson S. NIV for MND in the West of Scotland assisted ventilation service (WoSAVS). Eur Respir J. 2011;38:p2101.
   Google Scholar
• Oliver D, Campbell C, Sykes N, Tallon C, Edwards A. Decision-making for gastrostomy and ventilatory support for people with motor neurone disease: variations across UK hospices. J Palliat Care. 2011;27:198–201.
   PubMed Web of Science ®Google Scholar
• Martin NH, Lawrence V, Murray J, Janssen A, Higginson I, Lyall R, et al. Decision making about gastrostomy and noninvasive ventilation in amyotrophic lateral sclerosis. Qual Health Res. 2016;26:1366–81.
   PubMed Web of Science ®Google Scholar
• Ando H, Williams C, Angus R, Thornton E, Chakrabarti B, Piggin L, et al. The lived experience of patients with motor neuron disease who declined or did not tolerate non-invasive ventilation. Amyotroph Lateral Scler Frontotemporal Degener. 2010;11 (Suppl 1): 135–142.
   Google Scholar
• Piggin LH, Thornton EW, Angus RM, Chakrabarti B, Young CA. Non-invasive ventilation in motor neuron disease: qualitative accounts of initiation and impact. Thorax. 2009;64:A31.
   Web of Science ®Google Scholar
• Ando H, Williams C, Angus RM, Thornton EW, Chakrabarti B, Cousins R, et al. Why don't they accept non-invasive ventilation?: insight into the interpersonal perspectives of patients with motor neurone disease. Br J Health Psychol. 2015;20:341–59.
   PubMed Web of Science ®Google Scholar
• Saunders N, Salmon K, Magnussen C, Bertone D, Vitale A, Genge A. Attitudes towards life-prolonging measures and use of interventions in a multidisciplinary ALS clinic. Amyotroph Lateral Scler Frontotemporal Degener. 2016;17:316–7.
   Google Scholar
• Greenaway LP, Martin NH, Lawrence V, Janssen A, Al-Chalabi A, Leigh PN, et al. Accepting or declining non-invasive ventilation or gastrostomy in amyotrophic lateral sclerosis: patients' perspectives. J Neurol. 2015;262:1002–13.
   PubMed Web of Science ®Google Scholar
• Lemoignan J, Ells C. Amyotrophic lateral sclerosis and assisted ventilation: how patients decide. Pall Supp Care. 2010;8:207–13.
   PubMed Web of Science ®Google Scholar
• Chum MC, Gofton T, Shoesmith C. Non-invasive ventilation in patients with amyotrophic lateral sclerosis: practice patterns amongst Canadian care providers. Can J Neurol Sci. 2016;43:S18.
   Google Scholar
• Vandenberghe N, Vallet A-E, Petitjean T, Le Cam P, Peysson S, Guerin C, et al. Absence of airway secretion accumulation predicts tolerance of noninvasive ventilation in subjects with amyotrophic lateral sclerosis. Respir Care. 2013;58:1424–32.
   PubMed Web of Science ®Google Scholar
• Farrero E, Prats E, Povedano M, Martinez-Matos JA, Manresa F, Escarrabill J, et al. Survival in amyotrophic lateral sclerosis with home mechanical ventilation: the impact of systematic respiratory assessment and bulbar involvement. Chest. 2005;127:2132–8.
   PubMed Web of Science ®Google Scholar
• Sancho J, Servera E, Morelot-Panzini C, Salachas F, Similowski T, Gonzalez-Bermejo J, et al. Non-invasive ventilation effectiveness and the effect of ventilatory mode on survival in ALS patients. Amyotroph Lateral Scler Frontotemporal Degener. 2014;15:55–61.
   PubMed Web of Science ®Google Scholar
• Proctor A, Walsh T, Billings C, McDermott C, Angus R, Young C. Non-invasive ventilation in motor neurone disease: one centre's experience. Amyotroph Lateral Scler Frontotemporal Degener. 2013;14 (Supl 2) 84–94.
   Google Scholar
• Cousins R, Ando H, Thornton E, Chakrabarti B, Angus R, Young C, et al. Determinants of accepting non-invasive ventilation treatment in motor neurone disease: a quantitative analysis at point of need. Health Psychol Behav Med. 2013;1:47–58.
   PubMedGoogle Scholar
• Cousins R, Ando H, Young C. Carer influence on non-invasive ventilation treatment in motor neurone disease. Amyotroph Lateral Scler Frontotemporal Degener. 2011;12:53–4.
   Google Scholar
• Cousins R, Ando H, Young C, Chakrabarti B, Angus R. Uptake of NIV treatment in MND is dependent upon caregiver variables. Eur Respir J. 2012;40:P2070.
   Web of Science ®Google Scholar
• Martinez D, Sancho J, Servera E, Marin J. Tolerance of volume control noninvasive ventilation in subjects with amyotrophic lateral sclerosis. Respir Care. 2015;60:1765–71.
   PubMed Web of Science ®Google Scholar
• Peysson S, Vandenberghe N, Philit F, Vial C, Petitjean T, Bouhour F, et al. Factors predicting survival following noninvasive ventilation in amyotrophic lateral sclerosis. Eur Neurol. 2008;59:164–71.
   PubMed Web of Science ®Google Scholar
• Garabelli B, De Mattia E, Iatomasi M, Roma E, Falcier E, Lunetta C, et al. ALS and NIV: a four-year experience in the "NEMO" centre in Italy. A retrospective study. Eur Respir J. 2013;42 (Suppl 57).
   Web of Science ®Google Scholar
• Gonzalez Calzada N, Prats Soro E, Mateu Gomez L, Giro Bulta E, Cordoba Izquierdo A, Povedano Panades M, et al. Factors predicting survival in amyotrophic lateral sclerosis patients on non-invasive ventilation. Amyotroph Lateral Scler Frontotemporal Degener. 2016;17:337–42.
   PubMed Web of Science ®Google Scholar
• Foley G, Hynes G. Decision-making among patients and their family in ALS care: a review. Amyotroph Lateral Scler Frontotemporal Degener. 2018;19:173–93.
   PubMed Web of Science ®Google Scholar
• Martin NH, Landau S, Janssen A, Lyall R, Higginson I, Burman R, et al. Psychological as well as illness factors influence acceptance of non-invasive ventilation (NIV) and gastrostomy in amyotrophic lateral sclerosis (ALS): a prospective population study. Amyotroph Lateral Scler Frontotemporal Degener. 2014;15:376–87.
   PubMed Web of Science ®Google Scholar
• Sundling IM, Ekman SL, Weinberg J, Klefbeck B. Patients' with ALS and caregivers' experiences of non-invasive home ventilation. Adv Physiother. 2009;11:114–20.
   Google Scholar
• Oliver D, Campbell C, O'Brien T, Sloan R, Sykes N, Tallon C. Decision making for gastrostomy and respiratory support - variations across UK hospices. Amyotroph Lateral Scler Frontotemporal Degener. 2009;10 (Supl 1): 180–190.
   Google Scholar
• Oliver D, Phelps K, Regen E, McDermott C, Faull C. The management symptoms during the withdrawal of assisted ventilation in MND/ALS. Palliat Med. 2016;30:239.
   Google Scholar
• Volanti P, Cibella F, Sarvà M, De Cicco D, Spanevello A, Mora G, et al. Predictors of non-invasive ventilation tolerance in amyotrophic lateral sclerosis. J Neurol Sci. 2011;303:114–8.
   PubMed Web of Science ®Google Scholar
• Mckim DA, King J, Walker K, Leblanc C, Timpson D, Wilson KG, et al. Formal ventilation patient education for ALS predicts real-life choices. Amyotroph Lateral Scler Frontotemporal Degener. 2012;13:59–65.
   PubMed Web of Science ®Google Scholar
• Cooper-Knock J, Ahmedzai SH, Shaw P. The use of subcutaneous glycopyrrolate in the management of sialorrhoea and facilitating the use of non-invasive ventilation in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 2011;12:464–5.
   PubMed Web of Science ®Google Scholar
• De Vito EL, Suarez AA, Monteiro SG. The use of full-setting non-invasive ventilation in the home care of people with amyotrophic lateral sclerosis-motor neuron disease with end-stage respiratory muscle failure: a case series. J Med Case Rep. 2012;6:42.
   PubMedGoogle Scholar
• Palmer J, Hughes P. A 7 year retrospective evaluation of initiation of long term non-invasive ventilatory support for motor neurone disease. Thorax. 2011;66:A178–9.
   Web of Science ®Google Scholar
• Bertella E, Gile S, Fiorenza D, Paneroni M, Bianchi L, Banfi P, et al. Is outpatient initiation of non invasive ventilatory support not inferior to inpatient initiation in ALS patients?. Eur Respir J. 2014;44:P4740.
   Google Scholar
• Howard M, Berlowitz D, Batchelder I, Smith C. Day implementation model for non-invasive ventilation in motor neurone disease. Am J Respir Crit Care Med. 2010;181:A3059.
   Web of Science ®Google Scholar
• Sheers N, Berlowitz DJ, Rautela L, Batchelder I, Hopkinson K, Howard ME, et al. Improved survival with an ambulatory model of non-invasive ventilation implementation in motor neuron disease. Amyotroph Lateral Scler Frontotemporal Degener. 2014;15:180–4.
   PubMed Web of Science ®Google Scholar
• Sheers N, Berlowitz DJ, Smith C, Rautela L, Hopkinson K, Howard ME. An ambulatory model of non-invasive ventilation implementation improves survival in motor neurone disease. Amyotroph Lateral Scler Frontotemporal Degener. 2013;14 (Supl 1): 180–190.
   Google Scholar
• Gonzalez-Bermejo J, Morelot-Panzini C, Arnol N, Meininger V, Kraoua S, Salachas F, et al. Prognostic value of efficiently correcting nocturnal desaturations after one month of non-invasive ventilation in amyotrophic lateral sclerosis: a retrospective monocentre observational cohort study. Amyotroph Lateral Scler Frontotemporal Degener. 2013;14:373–9.
   PubMed Web of Science ®Google Scholar
• Jackson C, George A, Sherman M, Verma A, Shefner J, Scelsa, S, et al. Early treatment with NIPPV: factors affecting compliance over time. Amyotroph Lateral Scler Frontotemporal Degener. 2016;17:45–6.
   Google Scholar
• Belchior I, Goncalves MR, Winck JC. Continuous noninvasive ventilation delivered by a novel total face mask: a case series report. Respir Care. 2012;57:449–53.
   PubMed Web of Science ®Google Scholar
• Agrafiotis M, Renessis V, Kousta A, Athanassiadou A, Tryfon S, Chloros D. Noninvasive ventilation via mouthpiece in a patient with amyotrophic lateral sclerosis: a method to avoid tracheostomy and improve cough efficacy. Pneumonology. 2017;30:97–101.
   Web of Science ®Google Scholar
• Bedard M, McKim DA. Daytime mouthpiece for continuous noninvasive ventilation in individuals with amyotrophic lateral sclerosis. Respir Care. 2016;61:1341–8.
   PubMed Web of Science ®Google Scholar
• Gruis KL, Brown DL, Lisabeth LD, Zebarah VA, Chervin RD, Feldman EL, et al. Longitudinal assessment of noninvasive positive pressure ventilation adjustments in ALS patients. J Neurol Sci. 2006;247:59–63.
   PubMed Web of Science ®Google Scholar
• Restopo R, Walsh B. Humidification during invasive and non invasive mechanical ventilation: 2012. Respir Care. 2012;57:782–8.
   PubMed Web of Science ®Google Scholar
• Georges M, Attali V, Golmard JL, Morélot-Panzini C, Crevier-Buchman L, Collet J-M, et al. Reduced survival in patients with ALS with upper airway obstructive events on non-invasive ventilation. J Neurol Neurosurg Psychiatry. 2016;87:1045–50.
   PubMed Web of Science ®Google Scholar
• Hannan LM, Rautela L, Wilson DL, Berlowitz DJ, Howard ME. Altering ventilator inspiratory time can reduce autocycling during sleep. Sleep Med. 2015;16:1301–3.
   PubMed Web of Science ®Google Scholar
• Gonzalez-Bermejo J, Morelot-Panzini C, Dorsett J, Servera E, Salachas F, Similowski T, et al. The survival of amyotrophic lateral sclerosis patients placed under non invasive ventilation (NIV) does not depend on the ventilatory mode used (volume vs. pressure NIV). Amyotroph Lateral Scler Frontotemporal Degener. 2011;12:63–4.
   Google Scholar
• Katz J, Guion L. Average volume assured pressure support (AVAPS) in ALS patients with pure respiratory involvement: a randomized double-blind crossover trial Amyotroph Lateral Scler Frontotemporal Degener. 2015;16:126–7.
   Google Scholar
• Park D. Application of different ventilator modes in patients with amyotrophic lateral sclerosis according to certain clinical situations: a case report. Medicine (Baltimore). 2017;96:e7899.
   PubMed Web of Science ®Google Scholar
• Baxter SK, Baird WO, Thompson S, Bianchi SM, Walters SJ, Lee E, et al. The initiation of non-invasive ventilation for patients with motor neuron disease: patient and carer perceptions of obstacles and outcomes. Amyotroph Lateral Scler Frontotemporal Degener. 2013;14:105–10.
   PubMed Web of Science ®Google Scholar
• Jackson C, Heiman-Patterson T, Sherman M, Feldman S, Mitchell M, Verma A, et al. Factors associated with non-invasive positive pressure ventilation compliance in ALS/MND patients. Amyotroph Lateral Scler Frontotemporal Degener. 2009;10:184–5.
   Google Scholar
• Braga AC, Pinto A, Almeida JP, De Carvalho M. Can NIV parameters settings and changes overtime predict functional and survival outcome in ALS patients? Amyotroph Lateral Scler Frontotemporal Degener. 2013;14:P3842.
   Google Scholar
• Braga AC, Pinto AC, De Carvalho M. Parameters settings of NIV: can they predict functional and survival outcome in ALS patients? J Neurol Sci. 2013;333:e445.
   Google Scholar
• Braga ACM PS, Pinto A. Can non-invasive ventilation settings predicts functional and survival outcome in ALS patients? J Community Med Health Educ. 2017;7:521.
   Google Scholar
• Atkeson AD, Persaud R, RoyChoudhury A, Harrington-Moroney G, Mitsumoto H, Basner RC. Patient-ventilator asynchrony in amyotrophic lateral sclerosis (ALS) patients beginning nocturnal non invasive ventilation (NNIV) for FVC. Am J Respir Crit Care Med. 2011;183:A5276.
   Google Scholar
• Atkeson AD, RoyChoudhury A, Harrington-Moroney G, Shah B, Mitsumoto H, Basner RC, et al. Patient-ventilator asynchrony with nocturnal noninvasive ventilation in ALS. Neurology. 2011;77:549–55.
   PubMed Web of Science ®Google Scholar
• Ando H, Ashcroft H, Chakrabarti B, Angus R, Hallhead R, Cousins R. Telemonitoring to optimise care in motor neurone disease: a pilot study. Eur Respir J. 2016;48:PA3055.
   Web of Science ®Google Scholar
• Porcu A, Balestracci S, Sposito R, Moretti M. Web-based home telemonitoring of nocturnal pulse oximetry: feasibility and clinical implications for amyotrophic lateral sclerosis patients. Eur Respir J. 2013;42:P4914.
   Web of Science ®Google Scholar
• Lopes De Almeida JP, Cardoso Pinto A, Pinto S, De Carvalho M. Cost-effectiveness of home telemonitoring non-invasive ventilation in ALS patients. Amyotroph Lateral Scler Frontotemporal Degener. 2009;10 (Suppl 1): 180–190.
   Google Scholar
• Pinto A, Almeida JP, Pinto S, Pereira J, Oliveira AG, de Carvalho M, et al. Home telemonitoring of non-invasive ventilation decreases healthcare utilisation in a prospective controlled trial of patients with amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 2010;81:1238–42.
   PubMed Web of Science ®Google Scholar
• Lopes de Almeida JP, Pinto A, Pinto S, Ohana B, de Carvalho M. Economic cost of home-telemonitoring care for BiPAP-assisted ALS individuals. Amyotroph Lateral Scler Frontotemporal Degener. 2012;13:533–7.
   PubMed Web of Science ®Google Scholar
• Motor Neurone Disease Association. Withdrawal of ventilation with MND. London: Motor Neurone Disease Association; 2017.
   Google Scholar
• Faull C, Rowe Haynes C, Oliver D. Issues for palliative medicine doctors surrounding the withdrawal of non-invasive ventilation at the request of a patient with motor neurone disease: a scoping study. BMJ Support Palliat Care. 2014;4:43–9.
   PubMedGoogle Scholar
• Baxter SK, Baird WO, Thompson S, Bianchi SM, Walters SJ, Lee E, et al. The use of non-invasive ventilation at end of life in patients with motor neurone disease: a qualitative exploration of family carer and health professional experiences. Palliat Med. 2013;27:516–23.
   PubMed Web of Science ®Google Scholar
• Phelps K, Regen E, Oliver D, McDermott C, Faull C. Withdrawal of ventilation at the patient's request in MND: a retrospective exploration of the ethical and legal issues that have arisen for doctors in the UK. BMJ Support Palliat Care. 2017;7:189–96.
   PubMed Web of Science ®Google Scholar
• Kettemann D, Funke A, Maier A, Spittel S, Meyer Charite T. Emotional experience in patients with amyotrophic lateral sclerosis withdrawing from long-term ventilation. Amyotroph Lateral Scler Frontotemporal Degener. 2016;17 (Suppl S1): 311–322.
   Google Scholar
• Andersen PM, Kuzma-Kozakiewicz M, Keller J, Aho-Oezhan H, Ciecwierska K, Szejko N, et al. Therapeutic decisions in ALS patients: cross-cultural differences and clinical implications. J Neurol. 2018;265:1600–6.
   PubMed Web of Science ®Google Scholar
• Kuzma-Kozakiewicz M, Andersen PM, Keller J, Aho-Ozhan H, Ciecwierska K, Szejko, N, et al. Determinants of therapeutic decision making in ALS in Germany, Sweden and Poland. Amyotroph Lateral Scler Frontotemporal Degener. 2016;17:7–8.
   Google Scholar
• Rowe-Haynes C, Faull C, Oliver D. Exploring issues for doctors surrounding the withdrawal of NIV at the request of patients with MND. BMJ Support Palliat Care. 2012;2:A34.
   Google Scholar
• Foot C, Gilburt H, Dunn P, Jabbal J, Seale B, Goodrich J, et al. People in control of their own health and care: the state of involvement. London: Kings Fund; 2014.
   Google Scholar
• Sancho J, Martínez D, Bures E, Díaz JL, Ponz A, Servera E. Bulbar impairment score and survival of stable amyotrophic lateral sclerosis patients after noninvasive ventilation initiation. ERJ Open Res. 2018;4:00159–2017.
   PubMedGoogle Scholar