Skip to main content

Respiratory and Nutritional Support in Amyotrophic Lateral Sclerosis

Current Treatment Options in Neurology volume 16, Article number: 270 (2014) Cite this article

Opinion statement

Amyotrophic lateral sclerosis (ALS) is an uncommon and almost invariably fatal neurodegenerative disease. There is no known cure for ALS, and only one disease-modifying therapy is currently approved. In the absence of robust pharmacologic treatment options, the value of nutritional and respiratory support in the management of the disease should not be underestimated. The primary causes of morbidity and mortality in ALS are complications from dysphagia, leading to malnutrition and respiratory insufficiency, and these require focused therapeutic attention. This article reviews the current evidence for nutritional and respiratory support in the management of ALS patients.

This is a preview of subscription content, access via your institution.

Access options

Buy single article

Instant access to the full article PDF.

43,34 €

Price includes VAT (Finland)
Tax calculation will be finalised during checkout.

Abbreviations

AAN:

American Academy of Neurology

ALS:

Amyotrophic lateral sclerosis

BMI:

Body mass index

DPS:

Diaphragmatic pacing stimulators

EN:

Enteral nutrition

FVC:

Forced vital capacity

HFCWO:

High-frequency chest wall oscillation

IPAP:

Inspiratory airway pressure

MIE:

Mechanical insufflation/exsufflation

MIP:

Maximal inspiratory pressure

MIF:

Maximal mouth-inspiratory force

NIPPV:

Noninvasive positive pressure ventilation

NIV:

Noninvasive ventilation

PCEF:

Peak cough expiratory flow

Pdi:

Transdiaphragmatic pressure

PEG:

Percutaneous endoscopic gastrostomy

PN:

Parenteral nutrition

RIG:

Radiologically inserted gastrostomy

SNIP:

Sniff nasal inspiratory pressure

SVC:

Slow vital capacity

References and Recommended Reading

  1. Rowland, Shneider N. Amyotrophic lateral sclerosis. N Engl J Med. 2001;344(22):1688–700.

    CAS  PubMed  Article  Google Scholar 

  2. Miller R, Mitchell J, Moore D. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database Syst Rev. 2012;3:CD001447.

  3. Miller R, Rosenberg J, Gelinas D, 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):1311–23.

    CAS  PubMed  Article  Google Scholar 

  4. Miller R, Jackson C, Kasarskis E, et al. 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):1218–26.

    CAS  PubMed  Article  Google Scholar 

  5. Corcia P, Meininger V. Management of amyotrophic lateral sclerosis. Drugs. 2008;68(8):1037–48.

    CAS  PubMed  Article  Google Scholar 

  6. Chiò A, Logroscino G, Hardiman O, et al. Prognostic factors in ALS: A critical review. Amyotroph Lateral Scler. 2009;10(5–6):310–23.

    PubMed Central  PubMed  Article  Google Scholar 

  7. Bergofsky E. Respiratory failure in disorders of the thoracic cage. Am Rev Respir Dis. 1979;119(4):643–69.

    CAS  PubMed  Google Scholar 

  8. De Troyer A, Borenstein S, Cordier R. Analysis of lung volume restriction in patients with respiratory muscle weakness. Thorax. 1980;35(8):603–10.

    PubMed  Article  Google Scholar 

  9. Bach J. Amyotrophic lateral sclerosis: prolongation of life by noninvasive respiratory AIDS. Chest. 2002;122(1):92–8.

    PubMed  Article  Google Scholar 

  10. Hadjikoutis S, Wiles C. Respiratory complications related to bulbar dysfunction in motor neuron disease. Acta Neurol Scand. 2001;103(4):207–13.

    CAS  PubMed  Article  Google Scholar 

  11. Mustfa N, Moxham J. Respiratory muscle assessment in motor neurone disease. QJM. 2001;94(9):497–502.

    CAS  PubMed  Article  Google Scholar 

  12. Bourke S, Tomlinson M, Williams T, 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):140–7.

    PubMed  Article  Google Scholar 

  13. 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):31–5.

    PubMed  Article  Google Scholar 

  14. Bourke S, Bullock R, Williams T, et al. Noninvasive ventilation in ALS: indications and effect on quality of life. Neurology. 2003;61(2):171–7.

    CAS  PubMed  Article  Google Scholar 

  15. Carratù P, Spicuzza L, Cassano A, et al. Early treatment with noninvasive positive pressure ventilation prolongs survival in Amyotrophic Lateral Sclerosis patients with nocturnal respiratory insufficiency. Orphanet J Rare Dis. 2009;4:10.

    PubMed Central  PubMed  Article  Google Scholar 

  16. Kleopa K, 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):82–8.

    CAS  PubMed  Article  Google Scholar 

  17. McKim D, Road J, Avendano M, et al. Home mechanical ventilation: a Canadian Thoracic Society clinical practice guideline. Can Respir J. 2011;18(4):197–215.

    PubMed Central  PubMed  Google Scholar 

  18. Melo J, Homma A, Iturriaga E, et al. Pulmonary evaluation and prevalence of non-invasive ventilation in patients with amyotrophic lateral sclerosis: a multicenter survey and proposal of a pulmonary protocol. J Neurol Sci. 1999;169(1–2):114–7.

    CAS  PubMed  Article  Google Scholar 

  19. Czaplinski A, Yen A, Appel S. Forced vital capacity (FVC) as an indicator of survival and disease progression in an ALS clinic population. J Neurol Neurosurg Psychiatry. 2006;77(3):390–2.

    CAS  PubMed  Article  Google Scholar 

  20. Hardiman O. Management of respiratory symptoms in ALS. J Neurol. 2011;258(3):359–65.

    PubMed  Article  Google Scholar 

  21. Varrato J, Siderowf A, Damiano P, et al. Postural change of forced vital capacity predicts some respiratory symptoms in ALS. Neurology. 2001;57(2):357–9.

    CAS  PubMed  Article  Google Scholar 

  22. Baumann F, Henderson R, Morrison S, et al. Use of respiratory function tests to predict survival in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2010;11(1–2):194–202.

    PubMed  Article  Google Scholar 

  23. Lechtzin N, Wiener C, Shade D, et al. Spirometry in the supine position improves the detection of diaphragmatic weakness in patients with amyotrophic lateral sclerosis. Chest. 2002;121(2):436–42.

    PubMed  Article  Google Scholar 

  24. Fitting J, Paillex R, Hirt L, et al. Sniff nasal pressure: a sensitive respiratory test to assess progression of amyotrophic lateral sclerosis. Ann Neurol. 1999;46(6):887–93.

    CAS  PubMed  Article  Google Scholar 

  25. Morgan R, 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):269–74.

    PubMed  Article  Google Scholar 

  26. Lyall R, Donaldson N, Polkey M, et al. Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis. Brain. 2001;124(Pt 10):2000–13.

    CAS  PubMed  Article  Google Scholar 

  27. Stefanutti D, Benoist M, Scheinmann P, et al. Usefulness of sniff nasal pressure in patients with neuromuscular or skeletal disorders. Am J Respir Crit Care Med. 2000;162(4 Pt 1):1507–11.

    CAS  PubMed  Article  Google Scholar 

  28. Jackson C, Rosenfeld J, Moore D, 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):75–8.

    CAS  PubMed  Article  Google Scholar 

  29. Rafiq M, Bradburn M, Proctor A, 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. 2012;13(6):528–32.

    CAS  PubMed  Article  Google Scholar 

  30. Chaudri M, Liu C, Hubbard R, et al. Relationship between supramaximal flow during cough and mortality in motor neurone disease. Eur Respir J. 2002;19(3):434–8.

    CAS  PubMed  Article  Google Scholar 

  31. Mustfa N, Walsh E, Bryant V, et al. The effect of noninvasive ventilation on ALS patients and their caregivers. Neurology. 2006;66(8):1211–7.

    CAS  PubMed  Article  Google Scholar 

  32. Andersen P, Borasio G, Dengler R, 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. 2007;8(4):195–213.

    Google Scholar 

  33. Radunovic A, Annane D, Rafiq M, et al. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev. 2013;3:CD004427.

    Google Scholar 

  34. Gruis K, Lechtzin N. Respiratory therapies for amyotrophic lateral sclerosis: a primer. Muscle Nerve. 2012;46(3):313–31.

    PubMed  Article  Google Scholar 

  35. Gruis K, Brown D, Schoennemann A, et al. Predictors of noninvasive ventilation tolerance in patients with amyotrophic lateral sclerosis. Muscle Nerve. 2005;32(6):808–11.

    CAS  PubMed  Article  Google Scholar 

  36. Olney R, Murphy J, Forshew D, et al. The effects of executive and behavioral dysfunction on the course of ALS. Neurology. 2005;65(11):1774–7.

    CAS  PubMed  Article  Google Scholar 

  37. Jaye J, Chatwin M, Dayer M, et al. Autotitrating versus standard noninvasive ventilation: a randomised crossover trial. Eur Respir J. 2009;33(3):566–71.

    CAS  PubMed  Article  Google Scholar 

  38. Onders R, Carlin A, Elmo M, et al. Amyotrophic lateral sclerosis: the Midwestern surgical experience with the diaphragm pacing stimulation system shows that general anesthesia can be safely performed. Am J Surg. 2009;197(3):386–90.

    PubMed  Article  Google Scholar 

  39. Moss A, Casey P, Stocking C, et al. Home ventilation for amyotrophic lateral sclerosis patients: outcomes, costs, and patient, family, and physician attitudes. Neurology. 1993;43(2):438–43.

    CAS  PubMed  Article  Google Scholar 

  40. Borasio G, Gelinas D, Yanagisawa N. Mechanical ventilation in amyotrophic lateral sclerosis: a cross-cultural perspective. J Neurol. 1998;245 Suppl 2:S7–12.

    PubMed  Article  Google Scholar 

  41. 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):

  42. Kaub-Wittemer D, Steinbüchel N, 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):890–6.

    PubMed  Article  Google Scholar 

  43. Bach J, Bianchi C, Aufiero E. Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis. Chest. 2004;126(5):1502–7.

    PubMed  Article  Google Scholar 

  44. Bradley W, Anderson F, Gowda N, et al. Changes in the management of ALS since the publication of the AAN ALS practice parameter 1999. Amyotroph Lateral Scler Other Motor Neuron Disord. 2004;5(4):240–4.

    CAS  PubMed  Google Scholar 

  45. Chiò A, Calvo A, Ghiglione P, et al. Tracheostomy in amyotrophic lateral sclerosis: a 10-year population-based study in Italy. J Neurol Neurosurg Psychiatry. 2010;81(10):1141–3.

    PubMed  Article  Google Scholar 

  46. 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):64–9.

    PubMed  Article  Google Scholar 

  47. Hayashi H, Oppenheimer E. ALS patients on TPPV: totally locked-in state, neurologic findings and ethical implications. Neurology. 2003;61(1):135–7.

    PubMed  Article  Google Scholar 

  48. Rabkin J, Albert S, 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.

    PubMed  Article  Google Scholar 

  49. Sancho J, Servera E, Díaz J, et al. Efficacy of mechanical insufflation-exsufflation in medically stable patients with amyotrophic lateral sclerosis. Chest. 2004;125(4):1400–5.

    PubMed  Article  Google Scholar 

  50. Arens R, Gozal D, Omlin K, et al. Comparison of high frequency chest compression and conventional chest physiotherapy in hospitalized patients with cystic fibrosis. Am J Respir Crit Care Med. 1994;150(4):1154–7.

    CAS  PubMed  Article  Google Scholar 

  51. Warwick W, Hansen L. The long-term effect of high-frequency chest compression therapy on pulmonary complications of cystic fibrosis. Pediatr Pulmonol. 1991;11(3):265–71.

    CAS  PubMed  Article  Google Scholar 

  52. Chaisson K, Walsh S, Simmons Z, et al. A clinical pilot study: high frequency chest wall oscillation airway clearance in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2006;7(2):107–11.

    PubMed  Article  Google Scholar 

  53. Piquet MA. [Nutritional approach for patients with amyotrophic lateral sclerosis]. Rev Neurol (Paris). 2006;162(Spec No 2):4S177-4S187.

    Google Scholar 

  54. Traynor B, Codd M, Corr B, et al. Incidence and prevalence of ALS in Ireland, 1995-1997: a population-based study. Neurology. 1999;52(3):504–9.

    CAS  PubMed  Article  Google Scholar 

  55. Borasio G, Shaw P, Hardiman O, et al. Standards of palliative care for patients with amyotrophic lateral sclerosis: results of a European survey. Amyotroph Lateral Scler Other Motor Neuron Disord. 2001;2(3):159–64.

    CAS  PubMed  Article  Google Scholar 

  56. Desport J, Preux P, Truong C, et al. Nutritional assessment and survival in ALS patients. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000;1(2):91–6.

    CAS  PubMed  Article  Google Scholar 

  57. Desport J, Preux P, Truong T, et al. Nutritional status is a prognostic factor for survival in ALS patients. Neurology. 1999;53(5):1059–63.

    CAS  PubMed  Article  Google Scholar 

  58. Heffernan C, Jenkinson C, Holmes T, et al. Nutritional management in MND/ALS patients: an evidence based review. Amyotroph Lateral Scler Other Motor Neuron Disord. 2004;5(2):72–83.

    PubMed  Article  Google Scholar 

  59. Kasarskis E, Neville H. Management of ALS: nutritional care. Neurology. 1996;47(4 Suppl 2):S118–20.

    CAS  PubMed  Article  Google Scholar 

  60. Atsuta N, Watanabe H, Ito M, et al. Age at onset influences on wide-ranged clinical features of sporadic amyotrophic lateral sclerosis. J Neurol Sci. 2009;276(1–2):163–9.

    PubMed  Article  Google Scholar 

  61. Mitsumoto H, Rabkin J. Palliative care for patients with amyotrophic lateral sclerosis: "prepare for the worst and hope for the best". JAMA. 2007;298(2):207–16.

    CAS  PubMed  Article  Google Scholar 

  62. Park J, Kang S-W. Percutaneous radiologic gastrostomy in patients with amyotrophic lateral sclerosis on noninvasive ventilation. Arch Phys Med Rehabil. 2009;90(6):1026–9.

    PubMed  Article  Google Scholar 

  63. Mattson M, Cutler R, Camandola S. Energy intake and amyotrophic lateral sclerosis. Neuromolecular Med. 2007;9(1):17–20.

    CAS  PubMed  Article  Google Scholar 

  64. Dupuis L, Oudart H, René F, et al. Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model. Proc Natl Acad Sci U S A. 2004;101(30):11159–64.

    CAS  PubMed Central  PubMed  Article  Google Scholar 

  65. Hamadeh M, Rodriguez M, Kaczor J, et al. Caloric restriction transiently improves motor performance but hastens clinical onset of disease in the Cu/Zn-superoxide dismutase mutant G93A mouse. Muscle Nerve. 2005;31(2):214–20.

    CAS  PubMed  Article  Google Scholar 

  66. Langmore S. Issues in the management of dysphagia. Folia Phoniatr Logop. 1999;51(4–5):220–30.

    CAS  PubMed  Article  Google Scholar 

  67. Perry A, Anderson K, Lean R, et al. Elevation of the soft palate in speech and swallowing in normal female participants and females with motor neuron disease: an innovative procedure for measuring palatal elevation. Int J Lang Commun Disord. 2002;37(2):197–214.

    PubMed  Article  Google Scholar 

  68. Cameron A, Rosenfeld J. Nutritional issues and supplements in amyotrophic lateral sclerosis and other neurodegenerative disorders. Curr Opin Clin Nutr Metab Care. 2002;5(6):631–43.

    CAS  PubMed  Article  Google Scholar 

  69. Katzberg H, Benatar M. Enteral tube feeding for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev. 2011;1:CD004030.

  70. Allen J, 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):308–14.

    PubMed  Article  Google Scholar 

  71. Desport J-C, 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.

    PubMed  Article  Google Scholar 

  72. McLoughlin R, Gibney R. Fluoroscopically guided percutaneous gastrostomy: tube function and malfunction. Abdom Imaging. 1994;19(3):195–200.

    CAS  PubMed  Article  Google Scholar 

  73. 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):1123–5.

    PubMed  Article  Google Scholar 

  74. Desport J-C, Torny F, Lacoste M, et al. Hypermetabolism in ALS: correlations with clinical and paraclinical parameters. Neurodegener Dis. 2005;2(3–4):202–7.

    PubMed  Article  Google Scholar 

  75. Mazzini L, Corrà T, Zaccala M, et al. Percutaneous endoscopic gastrostomy and enteral nutrition in amyotrophic lateral sclerosis. J Neurol. 1995;242(10):695–8.

    CAS  PubMed  Article  Google Scholar 

  76. Mitsumoto H, Davidson M, Moore D, et al. Percutaneous endoscopic gastrostomy (PEG) in patients with ALS and bulbar dysfunction. Amyotroph Lateral Scler Other Motor Neuron Disord. 2003;4(3):177–85.

    CAS  PubMed  Article  Google Scholar 

  77. Shaw A, Ampong M-A, 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.

    PubMed  Article  Google Scholar 

  78. Verschueren A, Monnier A, Attarian S, et al. Enteral and parenteral nutrition in the later stages of ALS: an observational study. Amyotroph Lateral Scler. 2009;10(1):42–6.

    PubMed  Article  Google Scholar 

  79. Rosenfeld J, Ellis A. Nutrition and dietary supplements in motor neuron disease. Phys Med Rehabil Clin N Am. 2008;19(3):573.

    PubMed Central  PubMed  Article  Google Scholar 

  80. 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.

    PubMed Central  PubMed  Article  Google Scholar 

  81. Groeneveld G, Veldink J, van der Tweel I, et al. A randomized sequential trial of creatine in amyotrophic lateral sclerosis. Ann Neurol. 2003;53(4):437–45.

    CAS  PubMed  Article  Google Scholar 

  82. Shefner J, Cudkowicz M, Schoenfeld D, et al. A clinical trial of creatine in ALS. Neurology. 2004;63(9):1656–61.

    CAS  PubMed  Article  Google Scholar 

  83. Desnuelle C, Dib M, Garrel C, et al. A double-blind, placebo-controlled randomized clinical trial of alpha-tocopherol (vitamin E) in the treatment of amyotrophic lateral sclerosis. ALS riluzole-tocopherol Study Group Amyotroph Lateral Scler Other Motor Neuron Disord. 2001;2(1):9–18.

    CAS  Article  Google Scholar 

  84. Graf M, Ecker D, Horowski R, et al. High dose vitamin E therapy in amyotrophic lateral sclerosis as add-on therapy to riluzole: results of a placebo-controlled double-blind study. J Neural Transm. 2005;112(5):649–60.

    CAS  PubMed  Article  Google Scholar 

  85. Orrell R. Understanding the causes of amyotrophic lateral sclerosis. N Engl J Med. 2007;357(8):822–3.

    CAS  PubMed  Article  Google Scholar 

Download references

Compliance with Ethics Guidelines

Conflict of Interest

Namita A. Goyal and Tahseen Mozaffar declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Author information

Affiliations

  1. Department of Neurology, University of California, Irvine, CA, USA

    Namita A. Goyal MD & Tahseen Mozaffar MD

Authors
  1. Namita A. Goyal MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Tahseen Mozaffar MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Namita A. Goyal MD.

Additional information

This article is part of the Topical Collection on Neuromuscular Disorders

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Goyal, N.A., Mozaffar, T. Respiratory and Nutritional Support in Amyotrophic Lateral Sclerosis. Curr Treat Options Neurol 16, 270 (2014). https://doi.org/10.1007/s11940-013-0270-5

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11940-013-0270-5

Keywords

  • Amyotrophic lateral sclerosis
  • ALS
  • Respiratory support
  • Nutritional support
  • Respiratory insufficiency
  • Noninvasive ventilation
  • Percutaneous endoscopic gastrostomy
  • Enteral nutrition
  • Diaphragmatic pacing