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Exercise and amyotrophic lateral sclerosis

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Abstract

Amyotrophic Lateral Sclerosis (ALS) is a progressive and fatal neurodegenerative disease in which much burden is geared towards end-of-life care. Particularly in the earlier stages of ALS, many people have found both physiological and psychological boosts from various types of physical exercise for disused muscles. Proper exercise is important for preventing atrophy of muscles from disuse—a key for remaining mobile for as long as possible—and as long as it is possible to exercise comfortably and safely, for preserving cardiovascular fitness. However, the typical neuromuscular patient features a great physical inactivity and disuse weakness, and for that reason many controversial authors have contested exercise in these patients during years, especially in ALS which is rapidly progressive. There is an urgent need for dissecting in detail the real risks or benefits of exercise in controlled clinical trials to demystify this ancient paradigm. Yet, recent research studies document significant benefits in terms of survival and quality of life in ALS, poor cooperation, small sample size, uncontrolled and short-duration trials, remain the main handicaps. Sedentary barriers such as early fatigue and inherent muscle misuse should be overcome, for instance with body-weight supporting systems or non-invasive ventilation, and exercise should be faced as a potential non-monotonous way for contributing to better health-related quality of life.

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Abbreviations

ALS:

Amyotrophic lateral sclerosis

ALSFRS:

Amyotrophic lateral sclerosis functional rate score

NIV:

Non-invasive ventilation

References

  1. Rowland LP (2001) How amyotrophic lateral sclerosis got its name: the clinical-pathologic genius of Jean-Martin Charcot. Arch Neurol 58:512–515

    Article  PubMed  CAS  Google Scholar 

  2. Rowland LP, Shneider NA (2001) Amyotrophic lateral sclerosis. N Engl J Med 344:1688–1700

    Article  PubMed  CAS  Google Scholar 

  3. Preux PM, Druet-Cabanac M, Couratier P, Debrock C, Truong T, Marcharia W, Vallat JM, Dumas M, Boutros-Toni F (2000) Estimation of the amyotrophic lateral sclerosis incidence by capture-recapture method in the Limousin region of France. J Clin Epidemiol 53:1025–1029

    Article  PubMed  CAS  Google Scholar 

  4. Kurtzke JF (1982) Epidemiology of amyotrophic lateral sclerosis. Adv Neurol 36:281–302

    PubMed  CAS  Google Scholar 

  5. Kurtzke JF, Beebe GW (1980) Epidemiology of amyotrophic lateral sclerosis: 1. A case-control comparison based on ALS deaths. Neurology 30:453–462

    PubMed  CAS  Google Scholar 

  6. Mandrioli J, Faglioni P, Merelli E, Sola P et al (2003) The epidemiology of ALS in Modena, Italy. Neurology 60:683–689

    PubMed  Google Scholar 

  7. Brooks BR (1996) Clinical epidemiology of amyotrophic lateral sclerosis. Neurol Clin 14:399–420

    Article  PubMed  CAS  Google Scholar 

  8. Boillee S, Vande Velde C, Cleveland DW (2006) ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron 52:39–59

    Article  PubMed  CAS  Google Scholar 

  9. Boillee S, Yamanaka K, Lobsiger CS, Copeland NG, Jenkins NA, Kassiotis G, Kollias G, Cleveland DW et al (2006) Onset and progression in inherited ALS determined by motor neurons and microglia. Science 312:1389–1392

    Article  PubMed  CAS  Google Scholar 

  10. Christensen PB, Hojer-Pedersen E, Jensen NB (1990) Survival of patients with amyotrophic lateral sclerosis in 2 Danish counties. Neurology 40:600–604

    PubMed  CAS  Google Scholar 

  11. Magnus T, Beck M, Giess R, Puls I, Naumann M, Toyka KV et al (2002) Disease progression in amyotrophic lateral sclerosis: predictors of survival. Muscle Nerve 25:709–714

    Article  PubMed  CAS  Google Scholar 

  12. Eisen A, Kim S, Pant B (1992) Amyotrophic lateral sclerosis (ALS): a phylogenetic disease of the corticomotoneuron? Muscle Nerve 15:219–224

    Article  PubMed  CAS  Google Scholar 

  13. Eisen A, Nakajima M, Weber M (1999) Electrophysiological studies of the corticomotoneuron in ALS. Rinsho Shinkeigaku 39:99

    PubMed  CAS  Google Scholar 

  14. Andreadou E, Kapaki E, Kokotis P, Paraskevas GP, Katsaros N, Libitaki G, Petropoulou O, Zis V, Sfagos C, Vassilopoulos D et al (2008) Plasma glutamate and glycine levels in patients with amyotrophic lateral sclerosis. In Vivo 22:137–141

    PubMed  CAS  Google Scholar 

  15. Bogaert E, d’Ydewalle C, Van Den Bosch L (2010) Amyotrophic lateral sclerosis and excitotoxicity: from pathological mechanism to therapeutic target. CNS Neurol Disord Drug Targets 9:297–304

    Google Scholar 

  16. Brighina L, Sala G, Ceresa C, Tremolizzo L, Ferrarese C et al (2001) Recent advances in the therapy of amyotrophic lateral sclerosis: focus on excitotoxicity. Funct Neurol 16:189–202

    PubMed  CAS  Google Scholar 

  17. Cluskey S, Ramsden DB (2001) Mechanisms of neurodegeneration in amyotrophic lateral sclerosis. Mol Pathol 54:386–392

    PubMed  CAS  Google Scholar 

  18. Goodall EF, Morrison KE (2006) Amyotrophic lateral sclerosis (motor neuron disease): proposed mechanisms and pathways to treatment. Expert Rev Mol Med 8:1–22

    Article  PubMed  Google Scholar 

  19. (1997) Practice advisory on the treatment of amyotrophic lateral sclerosis with riluzole: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 49:657–659

  20. (1997) Riluzole for amyotrophic lateral sclerosis. Drug Ther Bull 35:11–12

  21. Bensimon G, Lacomblez L, Meininger V et al (1994) A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N Engl J Med 330:585–591

    Article  PubMed  CAS  Google Scholar 

  22. Takahashi H, Oyanagi K, Ikuta F, Tanaka M, Yuasa T, Miyatake T et al (1993) Widespread multiple system degeneration in a patient with familial amyotrophic lateral sclerosis. J Neurol Sci 120:15–21

    Article  PubMed  CAS  Google Scholar 

  23. Takahashi H, Snow BJ, Bhatt MH, Peppard R, Eisen A, Calne DB et al (1993) Evidence for a dopaminergic deficit in sporadic amyotrophic lateral sclerosis on positron emission scanning. Lancet 342:1016–1018

    Article  PubMed  CAS  Google Scholar 

  24. Cleveland DW, Rothstein JD (2001) From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Nat Rev Neurosci 2:806–819

    Article  PubMed  CAS  Google Scholar 

  25. Siddique T (1996) ALS: molecular clues to the jigsaw puzzle of neuronal degeneration. Cold Spring Harb Symp Quant Biol 61:699–708

    Article  PubMed  CAS  Google Scholar 

  26. Siddique T, Deng HX (1996) Genetics of amyotrophic lateral sclerosis. Hum Mol Genet 5 Spec No:1465–1470

    PubMed  CAS  Google Scholar 

  27. Menzies FM, Grierson AJ, Cookson MR, Heath PR, Tomkins J, Figlewicz DA, Ince PG, Shaw PJ et al (2002) Selective loss of neurofilament expression in Cu/Zn superoxide dismutase (SOD1) linked amyotrophic lateral sclerosis. J Neurochem 82:1118–1128

    Article  PubMed  CAS  Google Scholar 

  28. Menzies FM, Ince PG, Shaw PJ (2002) Mitochondrial involvement in amyotrophic lateral sclerosis. Neurochem Int 40:543–551

    Article  PubMed  CAS  Google Scholar 

  29. Lambrechts D, Storkebaum E, Morimoto M, Del-Favero J, Desmet F, Marklund SL, Wyns S, Thijs V, Andersson J, van Marion I, Al-Chalabi A, Bornes S, Musson R, Hansen V, Beckman L, Adolfsson R, Pall HS, Prats H, Vermeire S, Rutgeerts P, Katayama S, Awata T, Leigh N, Lang-Lazdunski L, Dewerchin M, Shaw C, Moons L, Vlietinck R, Morrison KE, Robberecht W, Van Broeckhoven C, Collen D, Andersen PM, Carmeliet P et al (2003) VEGF is a modifier of amyotrophic lateral sclerosis in mice and humans and protects motoneurons against ischemic death. Nat Genet 34:383–394

    Article  PubMed  CAS  Google Scholar 

  30. Pinto A, De Carvalho M, Evangelista T, Lopes A, Sales-Luis L et al (2003) 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 4:31–35

    Article  PubMed  Google Scholar 

  31. Lechtzin N, Scott Y, Busse AM, Clawson LL, Kimball R, Wiener CM et al (2007) Early use of non-invasive ventilation prolongs survival in subjects with ALS. Amyotroph Lateral Scler 8:185–188

    Article  PubMed  Google Scholar 

  32. Jackson CE, Rosenfeld J, Moore DH, Bryan WW, Barohn RJ, Wrench M, Myers D, Heberlin L, King R, Smith J, Gelinas D, Miller RG et al (2001) A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of hypoventilation in ALS/MND patients. J Neurol Sci 191:75–78

    Article  PubMed  CAS  Google Scholar 

  33. Lechtzin N 2006 Respiratory effects of amyotrophic lateral sclerosis: problems and solutions. Respir Care 51:871–881; (discussion 881–884)

    Google Scholar 

  34. Bach JR (2002) Amyotrophic lateral sclerosis: prolongation of life by noninvasive respiratory AIDS. Chest 122:92–98

    Article  PubMed  Google Scholar 

  35. Bach JR, Bianchi C, Aufiero E (2004) Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis. Chest 126:1502–1507

    Article  PubMed  Google Scholar 

  36. Pinto AC, Alves M, Nogueira A, Evangelista T, Carvalho J, Coelho A, de Carvalho M, Sales-Luis ML et al (1999) Can amyotrophic lateral sclerosis patients with respiratory insufficiency exercise? J Neurol Sci 169:69–75

    Article  PubMed  CAS  Google Scholar 

  37. Pinto AC, Evangelista T, de Carvalho M, Paiva T, de Lurdes Sales-Luis M et al (1999) Respiratory disorders in ALS: sleep and exercise studies. J Neurol Sci 169:61–68

    Article  PubMed  CAS  Google Scholar 

  38. Wyatt J, Bellis F (2002) British Thoracic Society guidelines on non-invasive ventilation. Emerg Med J 19:435

    Article  Google Scholar 

  39. Jackson CE, Lovitt S, Gowda N, Anderson F, Miller RG et al (2006) Factors correlated with NPPV use in ALS. Amyotroph Lateral Scler 7:80–85

    Article  PubMed  Google Scholar 

  40. Babu KS, Chauhan AJ (2003) Non-invasive ventilation in chronic obstructive pulmonary disease. Bmj 326:177–178

    Article  PubMed  Google Scholar 

  41. Baydur A, Layne E, Aral H, Krishnareddy N, Topacio R, Frederick G, Bodden W et al (2000) Long term non-invasive ventilation in the community for patients with musculoskeletal disorders: 46 year experience and review. Thorax 55:4–11

    Article  PubMed  CAS  Google Scholar 

  42. Aghai ZH, Saslow JG, Nakhla T, Milcarek B, Hart J, Lawrysh-Plunkett R, Stahl G, Habib RH, Pyon KH et al (2006) Synchronized nasal intermittent positive pressure ventilation (SNIPPV) decreases work of breathing (WOB) in premature infants with respiratory distress syndrome (RDS) compared to nasal continuous positive airway pressure (NCPAP). Pediatr Pulmonol 41:875–881

    Article  PubMed  Google Scholar 

  43. Achour L, Letellier C, Cuvelier A, Verin E, Muir JF et al (2007) Asynchrony and cyclic variability in pressure support noninvasive ventilation. Comput Biol Med 37:1308–1320

    Article  PubMed  Google Scholar 

  44. Lujan M, Moreno A, Veigas C, Monton C, Pomares X, Domingo C et al (2007) Non-invasive home mechanical ventilation: effectiveness and efficiency of an outpatient initiation protocol compared with the standard in-hospital model. Respir Med 101:1177–1182

    Article  PubMed  Google Scholar 

  45. Work group recommendations (2003) 2002 Exercise and Physical Activity Conference, St. Louis, Missouri. Session V: evidence of benefit of exercise and physical activity in arthritis. Arthritis Rheum 49:453–454

  46. McCarthy WJ, Kuo T (2009) Support for benefit of physical activity on satiety, weight control, and diabetes risk. Arch Intern Med 169: 634–635; author reply 635

    Google Scholar 

  47. Press V, Freestone I, George CF (2003) Physical activity: the evidence of benefit in the prevention of coronary heart disease. QJM 96:245–251

    Article  PubMed  CAS  Google Scholar 

  48. Fiatarone MA, O’Neill EF, Ryan ND, Clements KM, Solares GR, Nelson ME, Roberts SB, Kehayias JJ, Lipsitz LA, Evans WJ et al (1994) Exercise training and nutritional supplementation for physical frailty in very elderly people. N Engl J Med 330:1769–1775

    Article  PubMed  CAS  Google Scholar 

  49. DeLateur BJ, Lehmann JF, Giaconi R (1976) Mechanical work and fatigue: their roles in the development of muscle work capacity. Arch Phys Med Rehabil 57:319–324

    PubMed  CAS  Google Scholar 

  50. Aboussouan LS (2009) Mechanisms of exercise limitation and pulmonary rehabilitation for patients with neuromuscular disease. Chron Respir Dis 6:231–249

    PubMed  CAS  Google Scholar 

  51. Abresch RT, Han JJ, Carter GT (2009) Rehabilitation management of neuromuscular disease: the role of exercise training. J Clin Neuromuscul Dis 11:7–21

    Article  PubMed  Google Scholar 

  52. Adornato BT (1979) Exercise and neuromuscular disease. West J Med 131:334–335

    PubMed  CAS  Google Scholar 

  53. Florence JM, Hagberg JM (1984) Effect of training on the exercise responses of neuromuscular disease patients. Med Sci Sports Exerc 16:460–465

    Article  PubMed  CAS  Google Scholar 

  54. Kilmer DD (1998) The role of exercise in neuromuscular disease. Phys Med Rehabil Clin N Am 9:115–125, vi

    Google Scholar 

  55. Krivickas LS (2003) Exercise in neuromuscular disease. J Clin Neuromuscul Dis 5:29–39

    Article  PubMed  Google Scholar 

  56. Chen A, Montes J, Mitsumoto H (2008) The role of exercise in amyotrophic lateral sclerosis. Phys Med Rehabil Clin N Am 19: 545–557, ix–x

    Google Scholar 

  57. de Carvalho M, Nogueira A, Pinto A, Miguens J, Sales Luis ML (1999) Reflex sympathetic dystrophy associated with amyotrophic lateral sclerosis. J Neurol Sci 169:80–83

    Article  PubMed  Google Scholar 

  58. Sanjak M, Paulson D, Sufit R, Reddan W, Beaulieu D, Erickson L, Shug A, Brooks BR et al (1987) Physiologic and metabolic response to progressive and prolonged exercise in amyotrophic lateral sclerosis. Neurology 37:1217–1220

    PubMed  CAS  Google Scholar 

  59. Drory VE, Goltsman E, Reznik JG, Mosek A, Korczyn AD et al (2001) The value of muscle exercise in patients with amyotrophic lateral sclerosis. J Neurol Sci 191:133–137

    Article  PubMed  CAS  Google Scholar 

  60. Hakkinen A, Hakkinen K, Hannonen P et al (1994) Effects of strength training on neuromuscular function and disease activity in patients with recent-onset inflammatory arthritis. Scand J Rheumatol 23:237–242

    Article  PubMed  CAS  Google Scholar 

  61. Aitkens SG, McCrory MA, Kilmer DD, Bernauer EM et al (1993) Moderate resistance exercise program: its effect in slowly progressive neuromuscular disease. Arch Phys Med Rehabil 74:711–715

    Article  PubMed  CAS  Google Scholar 

  62. Gozal D, Thiriet P (1999) Respiratory muscle training in neuromuscular disease: long-term effects on strength and load perception. Med Sci Sports Exerc 31:1522–1527

    Article  PubMed  CAS  Google Scholar 

  63. Kilmer DD (2002) Response to resistive strengthening exercise training in humans with neuromuscular disease. Am J Phys Med Rehabil 81:S121–S126

    Article  PubMed  Google Scholar 

  64. Kilmer DD, McCrory MA, Wright NC, Aitkens SG, Bernauer EM et al (1994) The effect of a high resistance exercise program in slowly progressive neuromuscular disease. Arch Phys Med Rehabil 75:560–563

    PubMed  CAS  Google Scholar 

  65. Sanjak M, Reddan W, Brooks BR (1987) Role of muscular exercise in amyotrophic lateral sclerosis. Neurol Clin 5:251–268, vi

    Google Scholar 

  66. Longstreth WT, Nelson LM, Koepsell TD, Van Belle G et al (1991) Hypotheses to explain the association between vigorous physical activity and amyotrophic lateral sclerosis. Med Hypotheses 34:144–148

    Article  PubMed  CAS  Google Scholar 

  67. Brooks BR, Miller RG, Swash M, Munsat TL et al (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1:293–299

    Article  PubMed  CAS  Google Scholar 

  68. Mitchell JD (2000) Guidelines in motor neurone disease (MND)/amyotrophic lateral sclerosis (ALS)—from diagnosis to patient care. J Neurol 247:7–12

    Article  PubMed  CAS  Google Scholar 

  69. Sinaki M, Mulder DW (1978) Rehabilitation techniques for patients with amyotrophic lateral sclerosis. Mayo Clin Proc 53:173–178

    PubMed  CAS  Google Scholar 

  70. DeLisa JA, Mikulic MA, Miller RM, Melnick RR et al (1979) Amyotrophic lateral sclerosis: comprehensive management. Am Fam Physician 19:137–142

    PubMed  CAS  Google Scholar 

  71. Bohannon RW (1983) Results of resistance exercise on a patient with amyotrophic lateral sclerosis. A case report. Phys Ther 63:965–968

    PubMed  CAS  Google Scholar 

  72. Kirkinezos IG, Hernandez D, Bradley WG, Moraes CT et al (2003) Regular exercise is beneficial to a mouse model of amyotrophic lateral sclerosis. Ann Neurol 53:804–807

    Article  PubMed  Google Scholar 

  73. Dalbello-Haas V, Florence JM, Krivickas LS (2008) Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst Rev CD005229

  74. Cheah BC, Boland RA, Brodaty NE, Zoing MC, Jeffery SE, McKenzie DK, Kiernan MC (2009) INSPIRATIonAL—INSPIRAtory muscle training in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 10(5–6):384–392

    Google Scholar 

  75. Deforges S, Branchu J, Biondi O, Grondard C, Pariset C, Lecolle S, Lopes P, Vidal PP, Chanoine C, Charbonnier F et al (2009) Motoneuron survival is promoted by specific exercise in a mouse model of amyotrophic lateral sclerosis. J Physiol 587:3561–3572

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Physical Medicine and Rehabilitation, University of Lisbon Medical School, Santa Maria Hospital, Lisbon, Portugal

    J. P. Lopes de Almeida & A. C. Pinto

  2. Neuromuscular Unit, Institute of Molecular Medicine, University of Lisbon Medical School, Lisbon, Portugal

    J. P. Lopes de Almeida, A. C. Pinto & M. de Carvalho

  3. Faculty of Physical Education and Sport, Universidade Lusófona de Humanidades e Tecnologias, Lisbon, Portugal

    R. Silvestre

  4. Department of Neurosciences, University of Lisbon Medical School, Santa Maria Hospital, Lisbon, Portugal

    M. de Carvalho

  5. Clínica Universitária de Medicina Física e de Reabilitação, Faculdade de Medicina de Lisboa, Hospital de Santa Maria, Av. Prof. Egas Moniz, 1649-028, Lisboa, Portugal

    J. P. Lopes de Almeida

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  1. J. P. Lopes de Almeida
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  2. R. Silvestre
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  3. A. C. Pinto
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Correspondence to J. P. Lopes de Almeida.

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de Almeida, J.P.L., Silvestre, R., Pinto, A.C. et al. Exercise and amyotrophic lateral sclerosis. Neurol Sci 33, 9–15 (2012). https://doi.org/10.1007/s10072-011-0921-9

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