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Symptom-based management of amyotrophic lateral sclerosis

Nestor Galvez-Jimenez, MD, MSc, MHSA, FACP
Section Editors
Jeremy M Shefner, MD, PhD
Ira N Targoff, MD
R Sean Morrison, MD
Deputy Editor
John F Dashe, MD, PhD


Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that causes muscle weakness, disability, and eventually death, with a median survival of three to five years. Symptomatic management is the mainstay of treatment for ALS, and is reviewed in this topic. Other aspects of ALS are discussed separately. (See "Clinical features of amyotrophic lateral sclerosis and other forms of motor neuron disease" and "Diagnosis of amyotrophic lateral sclerosis and other forms of motor neuron disease" and "Disease modifying treatment of amyotrophic lateral sclerosis".)


ALS is a progressive neurodegenerative disorder. Most ALS patients die within three to five years of diagnosis. However, longer survival is not rare. About 30 percent of ALS patients are alive five years after diagnosis, and 10 to 20 percent survive for greater than 10 years. Long-term survival is associated with a younger age at symptom onset, male gender, and limb rather than bulbar symptom onset [1-3].


Informing the patient and family of the diagnosis of ALS is a daunting task for the clinician. The diagnosis should always be given face-to-face, with enough time to ensure no rushing [4,5]. It may be helpful to have the patient's support network present; European guidelines note a perceived advantage if the patient’s network outnumbers the medical staff present at the meeting [5]. It is important to give a warning that bad news is coming and to acknowledge and explore the patient's reaction. Allow for questions, give reassurance that the complications of ALS are treatable, and give reassurance that the patient will not be abandoned. Offer a second opinion if the patient wishes. It is often useful to schedule a return visit within a short time frame so that questions can be addressed and family members can attend if desired. (See "Communication of prognosis in palliative care".)

Many patients have a delay in diagnosis that is very distressing. For these patients, a diagnosis may be good news, in that uncertainty is reduced, and active therapy can be initiated. The discussion of diagnosis should always be accompanied by a discussion of the ways that symptoms can be treated, as well as by institution of disease modifying treatment, assuming cost is not prohibitive. (See "Disease modifying treatment of amyotrophic lateral sclerosis", section on 'Riluzole'.)


Multidisciplinary ALS clinics provide care from neurologists, physical therapists, occupational therapists, speech therapists, respiratory therapists, dietitians, social workers, and nursing care managers [5-9]. Thus, specialized clinics can guide the management of the complex issues related to ALS, which include respiratory symptoms, nutrition, dysarthria, dysphagia, functional decline, and psychosocial problems.


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Literature review current through: Sep 2016. | This topic last updated: Jun 2, 2016.
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  1. Eisen A, Schulzer M, MacNeil M, et al. Duration of amyotrophic lateral sclerosis is age dependent. Muscle Nerve 1993; 16:27.
  2. Jablecki CK, Berry C, Leach J. Survival prediction in amyotrophic lateral sclerosis. Muscle Nerve 1989; 12:833.
  3. Strong MJ, Hudson AJ, Alvord WG. Familial amyotrophic lateral sclerosis, 1850-1989: a statistical analysis of the world literature. Can J Neurol Sci 1991; 18:45.
  4. 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; 52:1311.
  5. EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis:, Andersen PM, Abrahams S, 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.
  6. Miller RG, Jackson CE, Kasarskis EJ, et al. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2009; 73:1227.
  7. Van den Berg JP, Kalmijn S, Lindeman E, et al. Multidisciplinary ALS care improves quality of life in patients with ALS. Neurology 2005; 65:1264.
  8. 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.
  9. Rooney J, Byrne S, Heverin M, et al. A multidisciplinary clinic approach improves survival in ALS: a comparative study of ALS in Ireland and Northern Ireland. J Neurol Neurosurg Psychiatry 2015; 86:496.
  10. Chiò A, Bottacchi E, Buffa C, et al. Positive effects of tertiary centres for amyotrophic lateral sclerosis on outcome and use of hospital facilities. J Neurol Neurosurg Psychiatry 2006; 77:948.
  11. Traynor BJ, Alexander M, Corr B, et al. Effect of a multidisciplinary amyotrophic lateral sclerosis (ALS) clinic on ALS survival: a population based study, 1996-2000. J Neurol Neurosurg Psychiatry 2003; 74:1258.
  12. Zoccolella S, Beghi E, Palagano G, et al. ALS multidisciplinary clinic and survival. Results from a population-based study in Southern Italy. J Neurol 2007; 254:1107.
  13. Miller RG, Jackson CE, Kasarskis EJ, 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; 73:1218.
  14. Gruis KL, Lechtzin N. Respiratory therapies for amyotrophic lateral sclerosis: a primer. Muscle Nerve 2012; 46:313.
  15. Borasio GD, Voltz R, Miller RG. Palliative care in amyotrophic lateral sclerosis. Neurol Clin 2001; 19:829.
  16. Borasio GD, Miller RG. Clinical characteristics and management of ALS. Semin Neurol 2001; 21:155.
  17. Rabinstein AA, Wijdicks EF. Warning signs of imminent respiratory failure in neurological patients. Semin Neurol 2003; 23:97.
  18. Fitting JW, Paillex R, Hirt L, et al. Sniff nasal pressure: a sensitive respiratory test to assess progression of amyotrophic lateral sclerosis. Ann Neurol 1999; 46:887.
  19. Lyall RA, Donaldson N, Polkey MI, et al. Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis. Brain 2001; 124:2000.
  20. Stefanutti D, Benoist MR, Scheinmann P, et al. Usefulness of sniff nasal pressure in patients with neuromuscular or skeletal disorders. Am J Respir Crit Care Med 2000; 162:1507.
  21. 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.
  22. Karam CY, Paganoni S, Joyce N, et al. Palliative Care Issues in Amyotrophic Lateral Sclerosis: An Evidenced-Based Review. Am J Hosp Palliat Care 2016; 33:84.
  23. Mustfa N, Walsh E, Bryant V, et al. The effect of noninvasive ventilation on ALS patients and their caregivers. Neurology 2006; 66:1211.
  24. Lo Coco D, Marchese S, Pesco MC, et al. Noninvasive positive-pressure ventilation in ALS: predictors of tolerance and survival. Neurology 2006; 67:761.
  25. 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; 5:140.
  26. Radunovic A, Annane D, Rafiq MK, Mustfa N. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev 2013; :CD004427.
  27. Berlowitz DJ, Howard ME, Fiore JF Jr, et al. Identifying who will benefit from non-invasive ventilation in amyotrophic lateral sclerosis/motor neurone disease in a clinical cohort. J Neurol Neurosurg Psychiatry 2016; 87:280.
  28. Faull C, Oliver D. Withdrawal of ventilation at the request of a patient with motor neurone disease: guidance for professionals. BMJ Support Palliat Care 2016; 6:144.
  29. 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; 53:1123.
  30. Mazzini L, Corrà T, Zaccala M, et al. Percutaneous endoscopic gastrostomy and enteral nutrition in amyotrophic lateral sclerosis. J Neurol 1995; 242:695.
  31. 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; 169:118.
  32. Wills AM, Hubbard J, Macklin EA, et al. Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled phase 2 trial. Lancet 2014; 383:2065.
  33. Andersen PM, Borasio GD, 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; 8:195.
  34. Körner S, Sieniawski M, Kollewe K, et al. Speech therapy and communication device: impact on quality of life and mood in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2013; 14:20.
  35. Londral A, Pinto A, Pinto S, et al. Quality of life in amyotrophic lateral sclerosis patients and caregivers: Impact of assistive communication from early stages. Muscle Nerve 2015; 52:933.
  36. Rabkin JG, Gordon PH, McElhiney M, et al. Modafinil treatment of fatigue in patients with ALS: a placebo-controlled study. Muscle Nerve 2009; 39:297.
  37. FDA advances effort against marketed unapproved drugs. FDA orders unapproved quinine drugs from the market and cautions consumers about "off-label" use of quinine to treat leg cramps. U. S. Food and Drug Administration www.fda.gov/bbs/topics/NEWS/2006/NEW01521.html (Accessed on March 23, 2009).
  38. Bedlack RS, Pastula DM, Hawes J, Heydt D. Open-label pilot trial of levetiracetam for cramps and spasticity in patients with motor neuron disease. Amyotroph Lateral Scler 2009; 10:210.
  39. Ashworth NL, Satkunam LE, Deforge D. Treatment for spasticity in amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev 2012; :CD004156.
  40. Chou R, Peterson K, Helfand M. Comparative efficacy and safety of skeletal muscle relaxants for spasticity and musculoskeletal conditions: a systematic review. J Pain Symptom Manage 2004; 28:140.
  41. Dal Bello-Haas V, Florence JM. Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst Rev 2013; :CD005229.
  42. Giess R, Naumann M, Werner E, et al. Injections of botulinum toxin A into the salivary glands improve sialorrhoea in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 2000; 69:121.
  43. Lipp A, Trottenberg T, Schink T, et al. A randomized trial of botulinum toxin A for treatment of drooling. Neurology 2003; 61:1279.
  44. Verma A, Steele J. Botulinum toxin improves sialorrhea and quality of living in bulbar amyotrophic lateral sclerosis. Muscle Nerve 2006; 34:235.
  45. Stone CA, O'Leary N. Systematic review of the effectiveness of botulinum toxin or radiotherapy for sialorrhea in patients with amyotrophic lateral sclerosis. J Pain Symptom Manage 2009; 37:246.
  46. Jackson CE, Gronseth G, Rosenfeld J, et al. Randomized double-blind study of botulinum toxin type B for sialorrhea in ALS patients. Muscle Nerve 2009; 39:137.
  47. Young CA, Ellis C, Johnson J, et al. Treatment for sialorrhea (excessive saliva) in people with motor neuron disease/amyotrophic lateral sclerosis. Cochrane Database Syst Rev 2011; :CD006981.
  48. Tuite PJ, Lang AE. Severe and prolonged dysphagia complicating botulinum toxin A injections for dystonia in Machado-Joseph disease. Neurology 1996; 46:846.
  49. Harriman M, Morrison M, Hay J, et al. Use of radiotherapy for control of sialorrhea in patients with amyotrophic lateral sclerosis. J Otolaryngol 2001; 30:242.
  50. Andersen PM, Grönberg H, Franzen L, Funegård U. External radiation of the parotid glands significantly reduces drooling in patients with motor neurone disease with bulbar paresis. J Neurol Sci 2001; 191:111.
  51. Neppelberg E, Haugen DF, Thorsen L, Tysnes OB. Radiotherapy reduces sialorrhea in amyotrophic lateral sclerosis. Eur J Neurol 2007; 14:1373.
  52. Newrick PG, Langton-Hewer R. Pain in motor neuron disease. J Neurol Neurosurg Psychiatry 1985; 48:838.
  53. Rosen HJ, Cummings J. A real reason for patients with pseudobulbar affect to smile. Ann Neurol 2007; 61:92.
  54. Gallagher JP. Pathologic laughter and crying in ALS: a search for their origin. Acta Neurol Scand 1989; 80:114.
  55. Ahmed A, Simmons Z. Pseudobulbar affect: prevalence and management. Ther Clin Risk Manag 2013; 9:483.
  56. Schiffer RB, Herndon RM, Rudick RA. Treatment of pathologic laughing and weeping with amitriptyline. N Engl J Med 1985; 312:1480.
  57. Iannaccone S, Ferini-Strambi L. Pharmacologic treatment of emotional lability. Clin Neuropharmacol 1996; 19:532.
  58. Brooks BR, Thisted RA, Appel SH, et al. Treatment of pseudobulbar affect in ALS with dextromethorphan/quinidine: a randomized trial. Neurology 2004; 63:1364.
  59. Pioro EP, Brooks BR, Cummings J, et al. Dextromethorphan plus ultra low-dose quinidine reduces pseudobulbar affect. Ann Neurol 2010; 68:693.
  60. Klein M, Musacchio JM. High affinity dextromethorphan binding sites in guinea pig brain. Effect of sigma ligands and other agents. J Pharmacol Exp Ther 1989; 251:207.
  61. Tortella FC, Pellicano M, Bowery NG. Dextromethorphan and neuromodulation: old drug coughs up new activities. Trends Pharmacol Sci 1989; 10:501.
  62. Hildebrand M, Seifert W, Reichenberger A. Determination of dextromethorphan metabolizer phenotype in healthy volunteers. Eur J Clin Pharmacol 1989; 36:315.
  63. Schadel M, Wu D, Otton SV, et al. Pharmacokinetics of dextromethorphan and metabolites in humans: influence of the CYP2D6 phenotype and quinidine inhibition. J Clin Psychopharmacol 1995; 15:263.
  64. Zhang Y, Britto MR, Valderhaug KL, et al. Dextromethorphan: enhancing its systemic availability by way of low-dose quinidine-mediated inhibition of cytochrome P4502D6. Clin Pharmacol Ther 1992; 51:647.
  65. Pioro EP. Current concepts in the pharmacotherapy of pseudobulbar affect. Drugs 2011; 71:1193.
  66. Hackett ML, Yang M, Anderson CS, et al. Pharmaceutical interventions for emotionalism after stroke. Cochrane Database Syst Rev 2010; :CD003690.
  67. Simmons Z, Bremer BA, Robbins RA, et al. Quality of life in ALS depends on factors other than strength and physical function. Neurology 2000; 55:388.
  68. Goldstein LH, Atkins L, Landau S, et al. Longitudinal predictors of psychological distress and self-esteem in people with ALS. Neurology 2006; 67:1652.
  69. Thakore NJ, Pioro EP. Depression in ALS in a large self-reporting cohort. Neurology 2016; 86:1031.
  70. Averill AJ, Kasarskis EJ, Segerstrom SC. Psychological health in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2007; 8:243.
  71. Reeves WC, Strine TW, Pratt LA, et al. Mental illness surveillance among adults in the United States. MMWR Suppl 2011; 60:1.
  72. Albert SM, Rabkin JG, Del Bene ML, et al. Wish to die in end-stage ALS. Neurology 2005; 65:68.
  73. McDonald ER, Wiedenfeld SA, Hillel A, et al. Survival in amyotrophic lateral sclerosis. The role of psychological factors. Arch Neurol 1994; 51:17.
  74. Vrijsen B, Buyse B, Belge C, et al. Noninvasive ventilation improves sleep in amyotrophic lateral sclerosis: a prospective polysomnographic study. J Clin Sleep Med 2015; 11:559.