Current Treatment Options in Neurology

, Volume 10, Issue 2, pp 77–85 | Cite as

Management of symptoms in amyotrophic lateral sclerosis

Article

Opinion statement

The mainstay of treatment of amyotrophic lateral sclerosis (ALS) is management of symptoms. Health care providers involved in the care of ALS patients should be armed with the most current knowledge about symptomatic management of these patients so that an aggressive approach to controlling symptoms can be undertaken at the most appropriate time. Among the important modalities is noninvasive positive pressure ventilation, which has been shown to improve not only quality of life but also survival. Similarly, clinicians should consider earlier intervention with enteral feeding. Palliative care should begin soon after ALS is diagnosed.

Keywords

Amyotrophic Lateral Sclerosis Palliative Care Percutaneous Endoscopic Gastrostomy Amyotrophic Lateral Sclerosis Patient Enteral Feeding 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References and Recommended Reading

  1. 1.
    Norris F, Shepherd R, Denys E, et al.: Onset, natural history and outcome in idiopathic adult motor neuron disease. J Neurol Sci 1993, 118:48–55.PubMedCrossRefGoogle Scholar
  2. 2.
    Brooks BR: Clinical epidemiology of amyotrophic lateral sclerosis. Neurol Clin 1996, 14:399–420.PubMedCrossRefGoogle Scholar
  3. 3.
    Tandan R, Bradley WG: Amyotrophic lateral sclerosis: part I. Ann Neurol 1985, 18:271–280.PubMedCrossRefGoogle Scholar
  4. 4.
    Meyrignac C, Poirer J, Degos JD: Amyotrophic lateral sclerosis presenting with respiratory insufficiency as the primary complaint. Eur Neurol 1985, 24:115–120.PubMedCrossRefGoogle Scholar
  5. 5.
    Culebras A: Sleep and neuromuscular disorders. Neurol Clin 2005, 23:1209–1233.PubMedCrossRefGoogle Scholar
  6. 6.
    Chokroverty S: Sleep dysfunction in neuromuscular disorders. Schweiz Arch Neurol Psychiatr 2003, 154:400–406.Google Scholar
  7. 7.
    Cameron A, Rosenfeld J: Nutritional issues and supplements in amyotrophic lateral sclerosis and other neurodegenerative disorder. Curr Opin Clin Metab Care 2002, 5:631–643.CrossRefGoogle Scholar
  8. 8.
    Desport JC, Preux PM, Truong CT, et al.: Nutritional status is a prognostic factor for survival in ALS patients. Neurology 1999, 53:1059–1065.PubMedGoogle Scholar
  9. 9.
    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, 7:16–21.PubMedGoogle Scholar
  10. 10.
    Charchaflie RJ, Fernandez LB, Perec CJ, et al.: Functional studies of the parotid and pancreas glands in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 1974, 37:863–867.PubMedGoogle Scholar
  11. 11.
    Gallagher JP: Pathologic laughter and crying in ALS: a search for their origin. Acta Neurol Scand 1989, 80:114–117.PubMedGoogle Scholar
  12. 12.
    McCullagh S, Moore M, Gawel M, et al.: Pathological laughing and crying in amyotrophic lateral sclerosis: an association with prefrontal cognitive dysfunction. J Neurol Sci 1999, 169:43–48.PubMedCrossRefGoogle Scholar
  13. 13.
    Denny-Brown D, Foley J: Myokymia and benign fasciculations of muscle cramps. Trans Assoc Phys 1948, 61:88–96.Google Scholar
  14. 14.
    Mitsumoto H, Rabkin JG: Palliative care for patients with amyotrophic lateral sclerosis: “prepare for the worst and hope for the best.“; JAMA 2007, 298:207–216.PubMedCrossRefGoogle Scholar
  15. 15.
    Borasio GD, Voltz R: Palliative care in amyotrophic lateral sclerosis. J Neurol 1997, 244(Suppl 4):S11–S17.PubMedCrossRefGoogle Scholar
  16. 16.
    Lyall RA, Donaldson N, Fleming T, et al.: A prospective study of quality of life in ALS patients treated with noninvasive ventilation. Neurology 2001, 57:153–156.PubMedGoogle Scholar
  17. 17.
    Miller RG, Rosenberg JA, Gelinas DF, et al.: Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review). Neurology 1999, 245(Suppl 2):S13–S19.Google Scholar
  18. 18.
    Bourke SC, Tomlinson M, Williams TL, et al.: Effects of noninvasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomized controlled trial. Lancet Neurol 2006, 5:140–147.PubMedCrossRefGoogle Scholar
  19. 19.
    Bach JR: ALS communication status and survival with ventilator support. Am J Med Rehab 1993, 72:343–349.Google Scholar
  20. 20.
    Bach JR: Amyotrophic lateral sclerosis: prolongation of life by noninvasive respiratory aids. Chest 2002, 122:92–98.PubMedCrossRefGoogle Scholar
  21. 21.
    Borasio GD, Shaw PJ, 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, 2:159–164.PubMedCrossRefGoogle Scholar
  22. 22.
    Bradley WG, Anderson F, Bromberg M, et al.: Current management of ALS: comparison of the ALS CARE database and the AAN Practice Parameter. The American Academy of Neurology. Neurology 2001, 57:500–504.PubMedGoogle Scholar
  23. 23.
    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, 164:82–88.PubMedCrossRefGoogle Scholar
  24. 24.
    Aboussouan LS, Khan SU, Banerjee M, et al.: Objective measures of the efficacy of noninvasive ventilation in amyotrophic lateral sclerosis. Muscle Nerve 2001, 24:403–409.PubMedCrossRefGoogle Scholar
  25. 25.
    Practice advisory on the treatment of amyotrophic lateral sclerosis with riluzole: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 1997, 49:657–659.Google Scholar
  26. 26.
    Miller RG, Mitchell JD, Lyon M, Moore DH: Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database Syst Rev 2007, 1:CD001447.Google Scholar
  27. 27.
    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, 191:75–78.PubMedCrossRefGoogle Scholar
  28. 28.
    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, 171:269–274.PubMedCrossRefGoogle Scholar
  29. 29.
    Farrero E, Prats E, Povedano M, et al.: Survival in amyotrophic lateral sclerosis with home mechanical ventilation. Chest 2005, 127:2132–2138.PubMedCrossRefGoogle Scholar
  30. 30.
    Respiratory assist devices. Available at http://www.tricenturion.com/content/Doc_View.cfm?type=LCDArch&File=respiratory%20assist%20devices1%2Ehtm. Accessed December 10, 2007.
  31. 31.
    Sancho J, Servera E, Diaz J, et al.: Efficacy of mechanical insufflation-exsufflation in medically stable patients with amyotrophic lateral sclerosis. Chest 2004, 125:1400–1405.PubMedCrossRefGoogle Scholar
  32. 32.
    Gelinas DF, O’Connor P, Miller RG: Quality of life for ventilator-dependent ALS patients and their caregivers. J Neurol Sci 1998, 160:S134–S136.PubMedCrossRefGoogle Scholar
  33. 33.
    Sonies BC: Patterns of care for dysphagic patients with degenerative neurological diseases. Semin Speech Lang 2000, 21:333–344.PubMedCrossRefGoogle Scholar
  34. 34.
    Borasio GD, Miller RG: Clinical characteristics and management of ALS. Semin Neurol 2001, 21:155–166.PubMedCrossRefGoogle Scholar
  35. 35.
    Park RH, Allison MC, Lang J, et al.: Randomised comparison of percutaneous endoscopic gastrostomy and nasogastric tube feeding in patients with persisting neurological dysphagia. BMJ 1992, 304:1406–1409.PubMedCrossRefGoogle Scholar
  36. 36.
    Silani V, Kasarskis EJ, Yanagisawa N: Nutritional management in amyotrophic lateral sclerosis: a worldwide perspective. J Neurol 1998, 245(Suppl 2):S13–S19.PubMedCrossRefGoogle Scholar
  37. 37.
    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, 6:88–93.PubMedCrossRefGoogle Scholar
  38. 38.
    Andersen PM, Borasio GD, Dengler R, et al.: EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients and relatives. Eur J Neurol 2005, 12:921–938.PubMedCrossRefGoogle Scholar
  39. 39.
    Willig TN, Bach JR, Venance V, et al.: Nutritional rehabilitation in neuromuscular disorders. Semin Neurol 1995, 15:18–23.PubMedGoogle Scholar
  40. 40.
    Evans J, Shaw PJ: Motor neurone disease: (2) management of the condition. Pharm J 2001, 267:714–717.Google Scholar
  41. 41.
    Leigh PN, Abrahams S, Al-Chalabi A, et al.: The management of motor neuron disease. J Neurol Neurosurg Psychiatry 2003, 70(suppl IV):iv32–iv47.Google Scholar
  42. 42.
    Lucas V, Amass C: Use of enteral glycopyrrolate in the management of drooling. Palliative Med 1998, 12:207–208.CrossRefGoogle Scholar
  43. 43.
    Mier RJ, Bachrach SJ, Lakin RC, et al.: Treatment of sialorrhea with glycopyrrolate: a double-blind dose-ranging study. Arch Pediatr Adolesc Med 2000, 154:1214–1218.PubMedGoogle Scholar
  44. 44.
    Camp-Bruno JA, Winsberg BG, Green-Parsons AR, Abrams JP: Efficacy of benztropine therapy for drooling. Dev Med Child Neurol 1989, 31:309–319.PubMedCrossRefGoogle Scholar
  45. 45.
    Lewis DW, Fontana C, Mehallick LK, et al.: Transdermal scopolamine for drooling in developmentally delayed children. Dev Med Child Neurol 1994, 36:484–486.PubMedCrossRefGoogle Scholar
  46. 46.
    Newell AR, Orser R, Hunt M: The control of oral secretions in bulbar ALS/MND. J Neurol Sci 1996, 139(suppl):43–44.CrossRefGoogle Scholar
  47. 47.
    Lipp A, Trottenberg T, Schink T, et al.: A randomized trial of botulinum toxin A for treatment of drooling. Neurology 2003, 61:1279–1281.PubMedGoogle Scholar
  48. 48.
    Andersen PM, Gronberg H, Franzen L, et al.: External radiation of the parotid glands significantly reduces drooling in patients with motor neurone disease with bulbar paresis. J Neurol Sci 2001, 191:111–114.PubMedCrossRefGoogle Scholar
  49. 49.
    Schiffer RB, Herndon RM, Rudick RA: Treatment of pathologic laughing and weeping with amitriptyline. N Engl J Med 1985, 312:1480–1482.PubMedCrossRefGoogle Scholar
  50. 50.
    Iannaconne S, Ferini-Strambi L: Pharmacologic treatment of emotional lability. Clin Neuropharmacol 1996, 19:532–535.Google Scholar
  51. 51.
    Anderson G, Vestergaard K, Riis JO: Citalopram for poststroke pathological crying. Lancet 1993, 342:837–839.CrossRefGoogle Scholar
  52. 52.
    Brooks BR, Thisted RA, Appel SH, et al.: Treatment of pseudobulbar affect in ALS with dextromethorphan/quinidine: a randomized trial. Neurology 2004, 63:1364–1370.PubMedGoogle Scholar
  53. 53.
    ALS Association: Clinical trial of Neurodex for pseudobulbar affect in people with amyotrophic lateral sclerosis. Available at http://www.alsa.org/patient/drug.cfm/id=46.
  54. 54.
    Department of Health and Human Services (Food and Drug Administration): Drug products containing quinine: enforcement action dates. Federal Register 2006, 71:75557–75560.Google Scholar
  55. 55.
    Miller TM, Layzer RB: Muscle cramps. Muscle Nerve 2005, 32:431–442.PubMedCrossRefGoogle Scholar
  56. 56.
    Serrao M, Rossi P, Cardinali P, et al.: Gabapentin treatment for muscle cramps: an open-label trial. Clin Neuropharmacol 2000, 23:45–49.PubMedCrossRefGoogle Scholar
  57. 57.
    Bertolasi L, Priori A, Tomelleri G, et al.: Botulinum toxin treatment of muscle cramps: a clinical and neurophysiological study. Ann Neurol 1997, 41:181–186.PubMedCrossRefGoogle Scholar
  58. 58.
    Riley JD, Anthony SJ: Leg cramps: differential diagnosis and management. Am Fam Physician 1995, 52:1794–1798.PubMedGoogle Scholar
  59. 59.
    Daniell HW: Simple cure for nocturnal leg cramps. N Engl J Med 1979, 301:216.PubMedGoogle Scholar
  60. 60.
    Dahle LO, Berg G, Hammar M, et al.: The effect of oral magnesium substitution on pregnancy-induced leg cramps. Am J Obstet Gynecol 1995, 173:175–180.PubMedCrossRefGoogle Scholar
  61. 61.
    Newrick PG, Langton-Hewer R: Pain in motor neuron disease. J Neurol Neurosurg Psychiatry 1985, 48:838–840.PubMedGoogle Scholar
  62. 62.
    O’Brien T, Kelly M, Saunders G: Motor neurone disease. A hospice perspective. BMJ 1992, 304:471–473.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  1. 1.Department of NeurologyLouisiana State University Health Sciences CenterNew OrleansUSA

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