Advanced search

Vol 54, No 5 (2020)
Invited Review Article
Published online: 2020-10-15

open access

View PDF Download PDF file
Supp./Additional Files
Page views 1764
Article views/downloads 1671
Get Citation

Connect on Social Media

Connect on Social Media

Gastrostomy and mechanical ventilation in amyotrophic lateral sclerosis: how best to support the decision-making process?

Krzysztof Barć1, Magdalena Kuźma-Kozakiewicz123
DOI: 10.5603/PJNNS.a2020.0081
Pubmed: 33058114
Neurol Neurochir Pol 2020;54(5):366-377.

Abstract

The unfavourable outcome of amyotrophic lateral sclerosis (ALS) confronts patients with challenging decisions regarding life-sustaining measures. The decision-making process is usually triggered by medical consultations and patient-dependent factors. This may largely depend on the physician’s depth of knowledge and professional experience. This paper presents an overview of the life-sustaining methods used in ALS and their effects on disease progression, survival and quality of life of patients and their caregivers. It is intended to aid physicians in their discussions with patients. We interrogate all the positive and negative facets of life-sustaining measures that may allow for optimisation of the decision-making process and care provision.

Keywords: amyotrophic lateral sclerosistherapeutic strategiesgastrostomymechanical ventilationquality of lifesurvival

Article available in PDF format

View PDF Download PDF file

References

  1. Wijesekera LC, Leigh PN. Amyotrophic lateral sclerosis. Orphanet J Rare Dis. 2009; 4: 3.
    CrossRefPubMed
  2. Kuźma-Kozakiewicz M. Edaravone in the treatment of amyotrophic lateral sclerosis. Neurol Neurochir Pol. 2018; 52(2): 124–128.
    CrossRefPubMed
  3. Lavernhe S, Antoine JC, Court-Fortune I, et al. Home care organization impacts patient management and survival in ALS. Amyotroph Lateral Scler Frontotemporal Degener. 2017; 18(7-8): 562–568.
    CrossRefPubMed
  4. Andersen PM, Kuzma-Kozakiewicz M, Keller J, et al. Therapeutic decisions in ALS patients: cross-cultural differences and clinical implications. J Neurol. 2018; 265(7): 1600–1606.
    CrossRefPubMed
  5. Lulé D, Nonnenmacher S, Sorg S, et al. Live and let die: existential decision processes in a fatal disease. J Neurol. 2014; 261(3): 518–525.
    CrossRefPubMed
  6. McGeachan AJ, Hobson EV, Al-Chalabi A, et al. Supportive and symptomatic management of amyotrophic lateral sclerosis. Nat Rev Neurol. 2016; 12(9): 526–538.
    CrossRefPubMed
  7. World Health Organization. The World Health Organization Quality of Life Assessment. Field Trial Version for Adults. Administration Manual. Geneva: World Health Organization. ; 1995.
  8. World Health Organization. WHOQoL: measuring quality of life. Geneva: World Health Organization. ; 1997.
  9. Goldstein LH, Atkins L, Leigh PN. Correlates of Quality of Life in people with motor neuron disease (MND). Amyotroph Lateral Scler Other Motor Neuron Disord. 2002; 3(3): 123–129.
    CrossRefPubMed
  10. Simmons Z, Bremer BA, Robbins RA, et al. Quality of life in ALS depends on factors other than strength and physical function. Neurology. 2000; 55(3): 388–392.
    CrossRefPubMed
  11. Robbins RA, Simmons Z, Bremer BA, et al. Quality of life in ALS is maintained as physical function declines. Neurology. 2001; 56(4): 442–444.
    CrossRefPubMed
  12. Neudert C, Wasner M, Borasio GD. Patients' assessment of quality of life instruments: a randomised study of SIP, SF-36 and SEIQoL-DW in patients with amyotrophic lateral sclerosis. J Neurol Sci. 2001; 191(1-2): 103–109.
    CrossRefPubMed
  13. Ganzini L, Johnston WS, Hoffman WF. Correlates of suffering in amyotrophic lateral sclerosis. Neurology. 1999; 52(7): 1434–1440.
    CrossRefPubMed
  14. Clarke S, Hickey A, O'Boyle C, et al. Assessing individual quality of life in amyotrophic lateral sclerosis. Qual Life Res. 2001; 10(2): 149–158.
    CrossRefPubMed
  15. Lulé D, Zickler C, Häcker S, et al. Life can be worth living in locked-in syndrome. Prog Brain Res. 2009; 177: 339–351.
    CrossRefPubMed
  16. Kuzma-Kozakiewicz M, Andersen PM, Ciecwierska K, et al. An observational study on quality of life and preferences to sustain life in locked-in state. Neurology. 2019; 93(10): e938–e945.
    CrossRefPubMed
  17. Körner S, Hendricks M, Kollewe K, et al. Weight loss, dysphagia and supplement intake in patients with amyotrophic lateral sclerosis (ALS): impact on quality of life and therapeutic options. BMC Neurol. 2013; 13: 84.
    CrossRefPubMed
  18. Desport JC, Preux PM, Truong CT, et al. Nutritional status is a prognostic factor for survival in ALS patients. Neurology. 1999; 53(5): 1059–1063.
    CrossRefPubMed
  19. Desport JC, Preux PM, Truong CT, et al. Nutritional status is a prognostic factor for survival in ALS patients. Neurology. 1999; 53(5): 1059–1063.
    CrossRefPubMed
  20. Bouteloup C, Desport JC, Clavelou P, et al. Hypermetabolism in ALS patients: an early and persistent phenomenon. J Neurol. 2009; 256(8): 1236–1242.
    CrossRefPubMed
  21. Vaisman N, Lusaus M, Nefussy B, et al. Do patients with amyotrophic lateral sclerosis (ALS) have increased energy needs? J Neurol Sci. 2009; 279(1-2): 26–29.
    CrossRefPubMed
  22. Desport JC, Torny F, Lacoste M, et al. Hypermetabolism in ALS: correlations with clinical and paraclinical parameters. Neurodegener Dis. 2005; 2(3-4): 202–207.
    CrossRefPubMed
  23. Jeejeebhoy KN. Muscle function and nutrition. Gut. 1986; 27 Suppl 1: 25–39.
    CrossRefPubMed
  24. Lopes J, Russell DM, Whitwell J, et al. Skeletal muscle function in malnutrition. Am J Clin Nutr. 1982; 36(4): 602–610.
    CrossRefPubMed
  25. Andersen PM, Abrahams S, Borasio GD, et al. EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis:. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)--revised report of an EFNS task force. Eur J Neurol. 2012; 19(3): 360–375.
    CrossRefPubMed
  26. Scott AG, Austin HE. Nasogastric feeding in the management of severe dysphagia in motor neurone disease. Palliat Med. 1994; 8(1): 45–49.
    CrossRefPubMed
  27. Gastrostomy in patients with amyotrophic lateral sclerosis (ProGas): a prospective cohort study. The Lancet Neurology. 2015; 14(7): 702–709.
    CrossRef
  28. Mazzini L, Corrà T, Zaccala M, et al. Percutaneous endoscopic gastrostomy and enteral nutrition in amyotrophic lateral sclerosis. J Neurol. 1995; 242(10): 695–698.
    CrossRefPubMed
  29. Bokuda K, Shimizu T, Imamura K, et al. Predictive factors for prognosis following unsedated percutaneous endoscopic gastrostomy in ALS patients. Muscle Nerve. 2016; 54(2): 277–283.
    CrossRefPubMed
  30. Kasarskis EJ, Scarlata D, Hill R, et al. A retrospective study of percutaneous endoscopic gastrostomy in ALS patients during the BDNF and CNTF trials. J Neurol Sci. 1999; 169(1-2): 118–125.
    CrossRefPubMed
  31. Allen JA, Chen R, Ajroud-Driss S, et al. Gastrostomy tube placement by endoscopy versus radiologic methods in patients with ALS: a retrospective study of complications and outcome. Amyotroph Lateral Scler Frontotemporal Degener. 2013; 14(4): 308–314.
    CrossRefPubMed
  32. Blondet A, Lebigot J, Nicolas G, et al. Radiologic versus endoscopic placement of percutaneous gastrostomy in amyotrophic lateral sclerosis: multivariate analysis of tolerance, efficacy, and survival. J Vasc Interv Radiol. 2010; 21(4): 527–533.
    CrossRefPubMed
  33. Thornton FJ, Fotheringham T, Haslam PJ, et al. Amyotrophic lateral sclerosis: enteral nutrition provision--endoscopic or radiologic gastrostomy? Radiology. 2002; 224(3): 713–717.
    CrossRefPubMed
  34. Chiò A, Galletti R, Finocchiaro C, et al. Percutaneous radiological gastrostomy: a safe and effective method of nutritional tube placement in advanced ALS. J Neurol Neurosurg Psychiatry. 2004; 75(4): 645–647.
    CrossRefPubMed
  35. Shaw AS, Ampong MA, Rio A, et al. Survival of patients with ALS following institution of enteral feeding is related to pre-procedure oximetry: a retrospective review of 98 patients in a single centre. Amyotroph Lateral Scler. 2006; 7(1): 16–21.
    CrossRefPubMed
  36. Yang B, Shi X. Percutaneous endoscopic gastrostomy versus fluoroscopic gastrostomy in amyotrophic lateral sclerosis (ALS) sufferers with nutritional impairment: A meta-analysis of current studies. Oncotarget. 2017; 8(60): 102244–102253.
    CrossRefPubMed
  37. Sancho J, Servera E, Chiner E, et al. Noninvasive respiratory muscle aids during PEG placement in ALS patients with severe ventilatory impairment. J Neurol Sci. 2010; 297(1-2): 55–59.
    CrossRefPubMed
  38. Czell D, Bauer M, Binek J, et al. Outcomes of percutaneous endoscopic gastrostomy tube insertion in respiratory impaired amyotrophic lateral sclerosis patients under noninvasive ventilation. Respir Care. 2013; 58(5): 838–844.
    CrossRefPubMed
  39. Desport JC, Mabrouk T, Bouillet P, et al. Complications and survival following radiologically and endoscopically-guided gastrostomy in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2005; 6(2): 88–93.
    CrossRefPubMed
  40. Barć K, Weber C, Maksymowicz-Śliwińska A, et al. Healthcare provision in amyotrophic lateral sclerosis: procedures, queries and pitfalls in Germany and Poland. Amyotroph Lateral Scler Frontotemporal Degener. 2020; 21(3-4): 193–202.
    CrossRefPubMed
  41. Albert SM, Murphy PL, Del Bene M, et al. Incidence and predictors of PEG placement in ALS/MND. J Neurol Sci. 2001; 191(1-2): 115–119.
    CrossRefPubMed
  42. Zhang L, Sanders L, Fraser RJL. Nutritional support teams increase percutaneous endoscopic gastrostomy uptake in motor neuron disease. World J Gastroenterol. 2012; 18(44): 6461–7; discussion p.6466.
    CrossRefPubMed
  43. Mitsumoto H, Davidson M, Moore D, et al. ALS CARE Study Group. Percutaneous endoscopic gastrostomy (PEG) in patients with ALS and bulbar dysfunction. Amyotroph Lateral Scler Other Motor Neuron Disord. 2003; 4(3): 177–185.
    CrossRefPubMed
  44. Dupuis L, Pradat PF, Ludolph A, et al. Energy metabolism in amyotrophic lateral sclerosis. The Lancet Neurology. 2011; 10(1): 75–82.
    CrossRef
  45. Rosenfeld J, Ellis A. Nutrition and dietary supplements in motor neuron disease. Phys Med Rehabil Clin N Am. 2008; 19(3): 573–89, x.
    CrossRefPubMed
  46. Paganoni S, Deng J, Jaffa M, et al. Body mass index, not dyslipidemia, is an independent predictor of survival in amyotrophic lateral sclerosis. Muscle Nerve. 2011; 44(1): 20–24.
    CrossRefPubMed
  47. Chiò A, Finocchiaro E, Meineri P, et al. Safety and factors related to survival after percutaneous endoscopic gastrostomy in ALS. ALS Percutaneous Endoscopic Gastrostomy Study Group. Neurology. 1999; 53(5): 1123–1125.
    CrossRefPubMed
  48. Dorst J, Dupuis L, Petri S, et al. Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: a prospective observational study. J Neurol. 2015; 262(4): 849–858.
    CrossRefPubMed
  49. Del Pi, Occhipinti P, Orsello M, et al. Percutaneous endoscopic gastrostomy (PEG) reduces complications and improves survival in amyotrophic lateral sclerosis (ALS). Gastrointest Endosc. 1999; 49: AB192.
  50. Reich-Slotky R, Andrews J, Cheng B, et al. Body mass index (BMI) as predictor of ALSFRS-R score decline in ALS patients. Amyotroph Lateral Scler Frontotemporal Degener. 2013; 14(3): 212–216.
    CrossRefPubMed
  51. Ngo ST, Steyn FJ, McCombe PA. Body mass index and dietary intervention: implications for prognosis of amyotrophic lateral sclerosis. J Neurol Sci. 2014; 340(1-2): 5–12.
    CrossRefPubMed
  52. Mathus-Vliegen LM, Louwerse LS, Merkus MP, et al. Percutaneous endoscopic gastrostomy in patients with amyotrophic lateral sclerosis and impaired pulmonary function. Gastrointest Endosc. 1994; 40(4): 463–469.
    CrossRefPubMed
  53. Salachas F, Fourmestraux AR, Le Fo, et al. Gastrostomy in patients with amyotrophic lateral sclerosis: a retrospective study in 110 patients. in: 7th International Symposium on ALS/MND, Chicago. : IL1996.
  54. Forbes RB, Colville S, Swingler RJ, et al. Scottish Motor Neurone Disease Research Group. Frequency, timing and outcome of gastrostomy tubes for amyotrophic lateral sclerosis/motor neurone disease--a record linkage study from the Scottish Motor Neurone Disease Register. J Neurol. 2004; 251(7): 813–817.
    CrossRefPubMed
  55. Miller RG, Jackson CE, Kasarskis EJ, et al. Quality Standards Subcommittee of the American Academy of Neurology. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009; 73(15): 1218–1226.
    CrossRefPubMed
  56. Finocchiaro E, Point MCDa, Chid A, et al. P.28 Percutaneous endoscopic gastrostomy inpatients with amyotrophic lateral sclerosis. Clinical Nutrition. 1998; 17: 37.
    CrossRef
  57. Chiò A, Ilardi A, Cammarosano S, et al. Neurobehavioral dysfunction in ALS has a negative effect on outcome and use of PEG and NIV. Neurology. 2012; 78(14): 1085–1089.
    CrossRefPubMed
  58. Strong MJ, Rowe A, Rankin RN. Percutaneous gastrojejunostomy in amyotrophic lateral sclerosis. J Neurol Sci. 1999; 169(1-2): 128–132.
    CrossRefPubMed
  59. Mitchell SL, Tetroe JM. Survival after percutaneous endoscopic gastrostomy placement in older persons. J Gerontol A Biol Sci Med Sci. 2000; 55(12): M735–M739.
    CrossRefPubMed
  60. Spataro R, Ficano L, Piccoli F, et al. Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: effect on survival. J Neurol Sci. 2011; 304(1-2): 44–48.
    CrossRefPubMed
  61. Cui F, Sun L, Xiong J, et al. Therapeutic effects of percutaneous endoscopic gastrostomy on survival in patients with amyotrophic lateral sclerosis: A meta-analysis. PLoS One. 2018; 13(2): e0192243.
    CrossRefPubMed
  62. Zamietra K, Lehman EB, Felgoise SH, et al. Non-invasive ventilation and gastrostomy may not impact overall quality of life in patients with ALS. Amyotroph Lateral Scler. 2012; 13(1): 55–58.
    CrossRefPubMed
  63. Trail M, Nelson ND, Van JN, et al. A study comparing patients with amyotrophic lateral sclerosis and their caregivers on measures of quality of life, depression, and their attitudes toward treatment options. J Neurol Sci. 2003; 209(1-2): 79–85.
    CrossRefPubMed
  64. Lo Coco D, Marchese S, Corrao S, et al. Development of chronic hypoventilation in amyotrophic lateral sclerosis patients. Respir Med. 2006; 100(6): 1028–1036.
    CrossRefPubMed
  65. Kaplan LM, Hollander D. Respiratory dysfunction in amyotrophic lateral sclerosis. Clin Chest Med. 1994; 15(4): 675–681.
    PubMed
  66. Miller RG, Rosenberg JA, Gelinas DF, et al. Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology: ALS Practice Parameters Task Force. Neurology. 1999; 52(7): 1311–1323.
    CrossRefPubMed
  67. Stambler N, Charatan M, Cedarbaum JM. Prognostic indicators of survival in ALS. ALS CNTF Treatment Study Group. Neurology. 1998; 50(1): 66–72.
    CrossRefPubMed
  68. Kleopa KA, Sherman M, Neal B, et al. Bipap improves survival and rate of pulmonary function decline in patients with ALS. J Neurol Sci. 1999; 164(1): 82–88.
    CrossRefPubMed
  69. Morgan RK, McNally S, Alexander M, et al. Use of Sniff nasal-inspiratory force to predict survival in amyotrophic lateral sclerosis. Am J Respir Crit Care Med. 2005; 171(3): 269–274.
    CrossRefPubMed
  70. Clinical indications for noninvasive positive pressure ventilation in chronic respiratory failure due to restrictive lung disease, COPD, and nocturnal hypoventilation--a consensus conference report. Chest. 1999; 116(2): 521–534.
    CrossRefPubMed
  71. Jackson CE, Rosenfeld J, Moore DH, 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(1-2): 75–78.
    CrossRefPubMed
  72. Allen SM, Hunt B, Green M. Fall in vital capacity with posture. Br J Dis Chest. 1985; 79(3): 267–271.
    PubMed
  73. Fallat RJ, Jewitt B, Bass M, et al. Spirometry in amyotrophic lateral sclerosis. Arch Neurol. 1979; 36(2): 74–80.
    CrossRefPubMed
  74. Nickol AH, Hart N, Hopkinson NS, et al. Mechanisms of improvement of respiratory failure in patients with restrictive thoracic disease treated with non-invasive ventilation. Thorax. 2005; 60(9): 754–760.
    CrossRefPubMed
  75. Gonzalez-Bermejo J, Morelot-Panzini C, Arnol N, 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(5-6): 373–379.
    CrossRefPubMed
  76. Aboussouan LS, Khan SU, Banerjee M, et al. Objective measures of the efficacy of noninvasive positive-pressure ventilation in amyotrophic lateral sclerosis. Muscle Nerve. 2001; 24(3): 403–409, doi: 10.1002/1097-4598(200103)24:3<403::aid-mus1013>3.0.co;2-3.
    PubMed
  77. Gruis KL, Brown DL, Schoennemann A, et al. Predictors of noninvasive ventilation tolerance in patients with amyotrophic lateral sclerosis. Muscle Nerve. 2005; 32(6): 808–811.
    CrossRefPubMed
  78. Sheers N, Berlowitz DJ, Rautela L, et al. Improved survival with an ambulatory model of non-invasive ventilation implementation in motor neuron disease. Amyotroph Lateral Scler Frontotemporal Degener. 2014; 15(3-4): 180–184.
    CrossRefPubMed
  79. Bertella E, Banfi P, Paneroni M, et al. Early initiation of night-time NIV in an outpatient setting: a randomized non-inferiority study in ALS patients. Eur J Phys Rehabil Med. 2017; 53(6): 892–899.
    CrossRefPubMed
  80. Bourke SC, Tomlinson M, Williams TL, et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006; 5(2): 140–147.
    CrossRefPubMed
  81. Pinto AC, Evangelista T, Carvalho M, et al. Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: Survival rates in a controlled trial. Journal of the Neurological Sciences. 1995; 129: 19–26.
    CrossRef
  82. Burkhardt C, Neuwirth C, Sommacal A, et al. Is survival improved by the use of NIV and PEG in amyotrophic lateral sclerosis (ALS)? A post-mortem study of 80 ALS patients. PLoS One. 2017; 12(5): e0177555.
    CrossRefPubMed
  83. Lechtzin N, Scott Y, Busse AM, et al. Early use of non-invasive ventilation prolongs survival in subjects with ALS. Amyotroph Lateral Scler. 2007; 8(3): 185–188.
    CrossRefPubMed
  84. Pinto A, de Carvalho M, Evangelista T, et al. Nocturnal pulse oximetry: a new approach to establish the appropriate time for non-invasive ventilation in ALS patients. Amyotroph Lateral Scler Other Motor Neuron Disord. 2003; 4(1): 31–35.
    CrossRefPubMed
  85. Mustfa N, Walsh E, Bryant V, et al. The effect of noninvasive ventilation on ALS patients and their caregivers. Neurology. 2006; 66(8): 1211–1217.
    CrossRefPubMed
  86. Bourke SC, Shaw PJ, Gibson GJ. Respiratory function vs sleep-disordered breathing as predictors of QOL in ALS. Neurology. 2001; 57(11): 2040–2044.
    CrossRefPubMed
  87. Newsom-Davis IC, Lyall RA, Leigh PN, et al. The effect of non-invasive positive pressure ventilation (NIPPV) on cognitive function in amyotrophic lateral sclerosis (ALS): a prospective study. J Neurol Neurosurg Psychiatry. 2001; 71(4): 482–487.
    CrossRefPubMed
  88. Bourke SC, Bullock RE, Williams TL, et al. Noninvasive ventilation in ALS: indications and effect on quality of life. Neurology. 2003; 61(2): 171–177.
    CrossRefPubMed
  89. Lo Coco D, Marchese S, Pesco MC, et al. Noninvasive positive-pressure ventilation in ALS: predictors of tolerance and survival. Neurology. 2006; 67(5): 761–765.
    CrossRefPubMed
  90. Mahajan KR, Bach JR, Saporito L, et al. Diaphragm pacing and noninvasive respiratory management of amyotrophic lateral sclerosis/motor neuron disease. Muscle Nerve. 2012; 46(6): 851–855.
    CrossRefPubMed
  91. Schiffman P, Belsh J. Pulmonary Function at Diagnosis of Amyotrophic Lateral Sclerosis. Chest. 1993; 103(2): 508–513.
    CrossRef
  92. Radunovic A, Annane D, Rafiq MK, et al. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev. 2017; 10: CD004427.
    CrossRefPubMed
  93. Mizutani T, Aki M, Shiozawa R, et al. Development of ophthalmoplegia in amyotrophic lateral sclerosis during long-term use of respirators. J Neurol Sci. 1990; 99(2-3): 311–319.
    CrossRefPubMed
  94. Kawata A, Mizoguchi K, Hayashi H. [A nationwide survey of ALS patients on trachoestomy positive pressure ventilation (TPPV) who developed a totally locked-in state (TLS) in Japan]. Rinsho Shinkeigaku. 2008; 48(7): 476–480.
    CrossRefPubMed
  95. Moss AH, Oppenheimer EA, Casey P, et al. Patients with amyotrophic lateral sclerosis receiving long-term mechanical ventilation. Advance care planning and outcomes. Chest. 1996; 110(1): 249–255.
    CrossRefPubMed
  96. Cazzolli PA, Oppenheimer EA. Home mechanical ventilation for amyotrophic lateral sclerosis: nasal compared to tracheostomy-intermittent positive pressure ventilation. J Neurol Sci. 1996; 139 Suppl: 123–128.
    CrossRefPubMed
  97. Kaub-Wittemer D, Steinbüchel Nv, Wasner M, et al. Quality of life and psychosocial issues in ventilated patients with amyotrophic lateral sclerosis and their caregivers. J Pain Symptom Manage. 2003; 26(4): 890–896.
    CrossRefPubMed
  98. Hirano YM, Yamazaki Y, Shimizu J, et al. Ventilator dependence and expressions of need: a study of patients with amyotrophic lateral sclerosis in Japan. Soc Sci Med. 2006; 62(6): 1403–1413.
    CrossRefPubMed
  99. Sancho J, Servera E, Bañuls P, et al. Prolonging survival in amyotrophic lateral sclerosis: efficacy of noninvasive ventilation and uncuffed tracheostomy tubes. Am J Phys Med Rehabil. 2010; 89(5): 407–411.
    CrossRefPubMed
  100. Chiò A, Calvo A, Ghiglione P, et al. PARALS. Tracheostomy in amyotrophic lateral sclerosis: a 10-year population-based study in Italy. J Neurol Neurosurg Psychiatry. 2010; 81(10): 1141–1143.
    CrossRefPubMed
  101. Vianello A, Arcaro G, Palmieri A, et al. Survival and quality of life after tracheostomy for acute respiratory failure in patients with amyotrophic lateral sclerosis. J Crit Care. 2011; 26(3): 329.e7–329.14.
    CrossRefPubMed
  102. Sancho J, Servera E, Díaz JL, et al. Home tracheotomy mechanical ventilation in patients with amyotrophic lateral sclerosis: causes, complications and 1-year survival. Thorax. 2011; 66(11): 948–952.
    CrossRefPubMed
  103. Lo Coco D, Marchese S, La Bella V, et al. The amyotrophic lateral sclerosis functional rating scale predicts survival time in amyotrophic lateral sclerosis patients on invasive mechanical ventilation. Chest. 2007; 132(1): 64–69.
    CrossRefPubMed
  104. Spataro R, Bono V, Marchese S, et al. Tracheostomy mechanical ventilation in patients with amyotrophic lateral sclerosis: clinical features and survival analysis. J Neurol Sci. 2012; 323(1-2): 66–70.
    CrossRefPubMed
  105. Laub M, Midgren B. Survival of patients on home mechanical ventilation: a nationwide prospective study. Respir Med. 2007; 101(6): 1074–1078.
    CrossRefPubMed
  106. Rabkin JG, Albert SM, Tider T, et al. Predictors and course of elective long-term mechanical ventilation: A prospective study of ALS patients. Amyotroph Lateral Scler. 2006; 7(2): 86–95.
    CrossRefPubMed
  107. Albert SM, Whitaker A, Rabkin JG, et al. Medical and supportive care among people with ALS in the months before death or tracheostomy. J Pain Symptom Manage. 2009; 38(4): 546–553.
    CrossRefPubMed
  108. Rabkin JG, Albert SM, Rowland LP, et al. How common is depression among ALS caregivers? A longitudinal study. Amyotroph Lateral Scler. 2009; 10(5-6): 448–455.
    CrossRefPubMed
  109. Tagami M, Kimura F, Nakajima H, et al. Tracheostomy and invasive ventilation in Japanese ALS patients: decision-making and survival analysis: 1990-2010. J Neurol Sci. 2014; 344(1-2): 158–164.
    CrossRefPubMed
  110. Chiò A, Calvo A, Moglia C, et al. PARALS. Non-invasive ventilation in amyotrophic lateral sclerosis: a 10 year population based study. J Neurol Neurosurg Psychiatry. 2012; 83(4): 377–381.
    CrossRefPubMed
  111. Young JM, Marshall CL, Anderson EJ. Amyotrophic lateral sclerosis patients' perspectives on use of mechanical ventilation. Health Soc Work. 1994; 19(4): 253–260.
    CrossRefPubMed
  112. Lyall R, Gelinas D. Control of symtoms: dysnoea and resiratory symtoms. In: Oliver DB, GD; Walsh, D, editor. Palliative care in amyotrohic lateral sclerosis From diagnosis to bereavement. Oxford: Oxford University Press; 2006. p. : 63–93.
  113. Borasio GD, Gelinas DF, Yanagisawa N. Mechanical ventilation in amyotrophic lateral sclerosis: a cross-cultural perspective. J Neurol. 1998; 245 Suppl 2: S7–12; discussion S29.
    CrossRefPubMed
  114. Benjaminsen E, Alstadhaug KB, Gulsvik M, et al. Amyotrophic lateral sclerosis in Nordland county, Norway, 2000-2015: prevalence, incidence, and clinical features. Amyotroph Lateral Scler Frontotemporal Degener. 2018; 19(7-8): 522–527.
    CrossRefPubMed
  115. O'Toole O, Traynor BJ, Brennan P, et al. Epidemiology and clinical features of amyotrophic lateral sclerosis in Ireland between 1995 and 2004. J Neurol Neurosurg Psychiatry. 2008; 79(1): 30–32.
    CrossRefPubMed
  116. Atsuta N, Watanabe H, Ito M, et al. Research Committee on the Neurodegenerative Diseases of Japan. Age at onset influences on wide-ranged clinical features of sporadic amyotrophic lateral sclerosis. J Neurol Sci. 2009; 276(1-2): 163–169.
    CrossRefPubMed
  117. Stoller JK, Xu M, Mascha E, et al. Long-term outcomes for patients discharged from a long-term hospital-based weaning unit. Chest. 2003; 124(5): 1892–1899.
    CrossRefPubMed
  118. Bradley MD, Orrell RW, Clarke J, et al. Outcome of ventilatory support for acute respiratory failure in motor neurone disease. J Neurol Neurosurg Psychiatry. 2002; 72(6): 752–756.
    CrossRefPubMed
  119. Nakayama Y, Shimizu T, Matsuda C, et al. Body weight variation predicts disease progression after invasive ventilation in amyotrophic lateral sclerosis. Sci Rep. 2019; 9(1): 12262.
    CrossRefPubMed
  120. Rousseau MC, Pietra S, Blaya J, et al. Quality of life of ALS and LIS patients with and without invasive mechanical ventilation. J Neurol. 2011; 258(10): 1801–1804.
    CrossRefPubMed
  121. Linse K, Rüger W, Joos M, et al. Eye-tracking-based assessment suggests preserved well-being in locked-in patients. Ann Neurol. 2017; 81(2): 310–315.
    CrossRefPubMed
  122. Lulé D, Ehlich B, Lang D, et al. Quality of life in fatal disease: the flawed judgement of the social environment. J Neurol. 2013; 260(11): 2836–2843.
    CrossRefPubMed
  123. Aho-Özhan HEA, Böhm S, Keller J, et al. Experience matters: neurologists' perspectives on ALS patients' well-being. J Neurol. 2017; 264(4): 639–646.
    CrossRefPubMed
  124. Maessen M, Veldink JH, Onwuteaka-Philipsen BD, et al. Trends and determinants of end-of-life practices in ALS in the Netherlands. Neurology. 2009; 73(12): 954–961.
    CrossRefPubMed
  125. Hecht M, Hillemacher T, Gräsel E, et al. Subjective experience and coping in ALS. Amyotroph Lateral Scler Other Motor Neuron Disord. 2002; 3(4): 225–231.
    CrossRefPubMed
  126. Kühnlein P, Kübler A, Raubold S, et al. Palliative care and circumstances of dying in German ALS patients using non-invasive ventilation. Amyotroph Lateral Scler. 2008; 9(2): 91–98.
    CrossRefPubMed
  127. Stutzki R, Schneider U, Reiter-Theil S, et al. Attitudes Toward Assisted Suicide and Life-Prolonging Measures in Swiss ALS Patients and Their Caregivers. Front Psychol. 2012; 3: 443.
    CrossRefPubMed
  128. Maessen M, Veldink JH, Onwuteaka-Philipsen BD, et al. Euthanasia and physician-assisted suicide among patients with amyotrophic lateral sclerosis in the Netherlands. N Engl J Med. 2002; 346(21): 1638–1644.
    CrossRefPubMed
  129. Leigh PN, Abrahams S, Al-Chalabi A, et al. King's MND Care and Research Team. The management of motor neurone disease. J Neurol Neurosurg Psychiatry. 2003; 74 Suppl 4: iv32–iv47.
    CrossRefPubMed
  130. Aboussouan LS, Khan SU, Meeker DP, et al. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Ann Intern Med. 1997; 127(6): 450–453.
    CrossRefPubMed
  131. Elman LB, Siderowf AD, McCluskey LF. Nocturnal oximetry: utility in the respiratory management of amyotrophic lateral sclerosis. Am J Phys Med Rehabil. 2003; 82(11): 866–870.
    CrossRefPubMed
  132. Sivak ED, Shefner JM, Mitsumoto H, et al. The use of non-invasive positive pressure ventilation (NIPPV) in ALS patients. A need for improved determination of intervention timing. Amyotroph Lateral Scler Other Motor Neuron Disord. 2001; 2(3): 139–145.
    CrossRefPubMed
  133. Morgan RK, McNally S, Alexander M, et al. Use of Sniff nasal-inspiratory force to predict survival in amyotrophic lateral sclerosis. Am J Respir Crit Care Med. 2005; 171(3): 269–274.
    CrossRefPubMed
  134. Chiò A, Mora G, Leone M, et al. Piemonte and Valle d'Aosta Register for ALS (PARALS). Early symptom progression rate is related to ALS outcome: a prospective population-based study. Neurology. 2002; 59(1): 99–103.
    CrossRefPubMed
  135. Lyall RA, Donaldson N, Fleming T, et al. A prospective study of quality of life in ALS patients treated with noninvasive ventilation. Neurology. 2001; 57(1): 153–156.
    CrossRefPubMed
  136. Bourke SC, Bullock RE, Williams TL, et al. Noninvasive ventilation in ALS: indications and effect on quality of life. Neurology. 2003; 61(2): 171–177.
    CrossRefPubMed
  137. Hein H, Schucher B, Magnussen H. Intermittierende Selbstbeatmung bei neuromuskulären Erkrankungen: Verlauf und Lebensqualität [Intermittent assisted ventilation in neuromuscular diseases: course and quality of life]. Pneumologie. 1999; 53(Suppl 2): S89–S90.

About cookies

Necessary cookies

Allways active

These cookies are necessary for the proper display and operation of the website. Blocking them may cause the website to malfunction.

Analytical cookies

As part of our website, we use analytical tools, such as Google Analytics, that allow us to verify website traffic. These tools may require the use of cookies.

Community cookies

These are cookies associated with the social networks we use. Accepting them will allow us to associate your visit to the site with our social media profiles.

Marketing cookies

These are cookies responsible for carrying out advertising and marketing activities - consenting to their use will allow us to target you with advertisements based on your previous activities within our website.

Copyright © 2023-2024 Via Medica Regulations Cookie policy Privacy Statement Legal Note