No cure is available for ALS. However, riluzole is believed to reduce the damage to motor neurons by decreasing the release of the excitatory neurotransmitter glutamate. Another drug, edaravone, is a free radical scavenger that is thought to decrease oxidative stress on neurons in ALS. Both drugs claim to slow the progression of ALS, and studies reveal modest results.34,35

Continue Reading

Symptom management and palliative care

A neurologist and a multidisciplinary team are required for the management of ALS. Whenever possible, primary care clinicians should refer the patient to a specialized ALS clinic. Treatments to manage the various symptoms can improve the quality of life (Table 2). Medications to reduce fatigue, relieve muscle cramps, control spasticity, and reduce excessive oral secretions can be beneficial. Gentle, low-impact aerobic activity and range-of-motion and stretching exercises can help to prevent painful muscle spasticity and contractures. When respiratory muscles weaken, intermittent positive pressure ventilation may be used to ease the work of breathing during sleep. Most patients remain asymptomatic until the forced vital capacity (FVC) is less than 50% of the predicted level as a result of respiratory muscle weakness. The patient’s preferences regarding life-sustaining treatment and approaches to palliative care should be discussed soon after the diagnosis of ALS, and any decisions should be reconsidered on several occasions before respiratory failure occurs. 

Click to enlarge

Related Articles


The primary care clinician may be involved early in the ALS disease process. Often, the diagnosis is delayed because the clinical presentation is unclear at an early stage. The primary care clinician may be involved in managing the symptoms of a patient with ALS and serve as a source of support for the caregiver(s). Patients require multidisciplinary care and rehabilitative interventions; maximizing independent function and quality of life are the goals. Family members, particularly spouses, commonly care for patients with ALS. The caregiver role frequently causes strain, which generally increases as the disease process progresses. Continued support and education from the primary care provider are essential for caregivers. Currently, numerous investigations into the genetic basis of ALS and the development of drugs to diminish the progressive degeneration associated with this disease are under way. 

Teri Capriotti, DO, MSN, CRNP, is a clinical associate professor, Erin Donnelly is an honors student, and Simone Brissenden is an honors student at Villanova University, PA.


  1. Mehta P, Kaye W, Bryan L, et al. Prevalence of amyotrophic lateral sclerosis–United States 2012-2013. MMWR Surveill Summ. 2016;65(No. SS-8):1-12. Accessed April 24, 2018.
  2. Centers for Disease Control and Prevention. National amyotrophic lateral sclerosis registry. Updated February 8, 2018. Accessed April 24, 2018.
  3. Beard JD, Kamel F. Military service, deployments, and exposures in relation to amyotrophic lateral sclerosis etiology and survival. Epidemiol Rev. 2015;37:55-70. 
  4. Peters TL, Kamel F, Lundholm C, et al. Occupational exposures and the risk of amyotrophic lateral sclerosis. Occup Environ Med. 2017;74:87-92.
  5. Wang MD, Little J, Gomes J, Cashman NR, Krewski D. Identification of risk factors associated with onset and progression of amyotrophic lateral sclerosis using systematic review and meta-analysis. Neurotoxicology. 2017;61:101-130. 
  6. Al-Chalabi A, Hardiman O. The epidemiology of ALS: a conspiracy of genes, environment and time. Nat Rev Neurol. 2013;9:617-628. 
  7. Strong MJ, Abrahams S, Goldstein LH, et al. Amyotrophic lateral sclerosis – frontotemporal spectrum disorder (ALS-FTSD): revised diagnostic criteria. Amyotroph Lateral Scler Frontotemporal Degener. 2017;18(3-4):153-174. 
  8. Brown RH, Al-Chalabi A. Amyotrophic lateral sclerosis. N Engl J Med. 2017;377:162-172. 
  9. Byrne S, Elamin M, Bede P, et al. Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study. Lancet Neurol. 2012;11:232-240.
  10. King AE, Woodhouse A, Kirkcaldie MT, Vickers JC. Excitotoxicity in ALS: overstimulation, or overreaction? Exp Neurol. 2016;275 Pt 1:162-171.
  11. Smith EF, Shaw PJ, De Vos KJ. The role of mitochondria in amyotrophic lateral sclerosis. Neurosci Lett. 2017 Jun 30. doi: 10.1016/j.neulet.2017.06.052 [Epub ahead of print]
  12. Webster CP, Smith EF, Shaw PJ, De Vos KJ. Protein homeostasis in amyotrophic lateral sclerosis: therapeutic opportunities? Front Mol Neurosci. 2017;10:123. doi: 10.3389/fnmol.2017.00123. eCollection 2017.
  13. Keifer OP Jr, O’Connor DM, Boulis NM. Gene and protein therapies utilizing VEGF for ALS. Pharmacol Ther. 2014;141:261-271.
  14. Cox PA, Richer R, Metcalf JS, Banack SA, Codd GA, Bradley WG. Cyanobacteria and BMAA exposure from desert dust: a possible link to sporadic ALS among Gulf War veterans. Amyotroph Lateral Scler. 2009;10 Suppl 2:109-117. 
  15. Xu Z, Henderson RD, David M, McCombe PA. Neurofilaments as biomarkers for amyotrophic lateral sclerosis: a systematic review and meta-analysis. PLoS One 2016;11: e0164625. doi: 10.1371/journal.pone.0164625. eCollection 2016.
  16. Menke RA, Agosta F, Grosskreutz J, Filippi M, Turner MR. Neuroimaging endpoints in amyotrophic lateral sclerosis. Neurotherapeutics. 2017;14:11-23.
  17. Philips T, Rothstein JD. Glial cells in amyotrophic lateral sclerosis. Exp Neurol. 2014;262 Pt B:111-120. 
  18. Connolly S, Galvin M, Hardiman O. End-of-life management in patients with amyotrophic lateral sclerosis. Lancet Neurol. 2015;14:435-442. 
  19. Couratier P, Corcia P, Lautrette G, Nicol M, Marin B. ALS and frontotemporal dementia belong to a common disease spectrum. Rev Neurol (Paris). 2017;173:273-279.
  20. Liscic RM. Als and Ftd: insights into the disease mechanisms and therapeutic targets. Eur J Pharmacol. 2017 Oct 12. doi: 10.1016/j.ejphar.2017.10.012 [Epub ahead of print]
  21. Trojsi F, Siciliano M, Femiano C, et al. Comorbidity of dementia with amyotrophic lateral sclerosis (ALS): insights from a large multicenter Italian cohort. J Neurol. 2017;264:2224-2231. 
  22. van Es MA, Hardiman O, Chio A, et al. Amyotrophic lateral sclerosis. Lancet. 2017 Nov 4;390(10107):2084-2098. doi: 10.1016/S0140-6736(17)31287-4 [Epub ahead of print]
  23. Thakore NJ, Pioro EP. Laughter, crying and sadness in ALS. J Neurol Neurosurg Psychiatry. 2017;88:825-831.
  24. Vucic S. Hypermetabolism appears to be an adverse prognostic biomarker in ALS. A potential for therapeutic intervention? Eur J Neurol. 2017 Sep 20. doi: 10.1111/ene.13470 [Epub ahead of print]
  25. Gillingham SM, Yunusova Y, Ganda A, et al. Assessing cognitive functioning in ALS: a focus on frontal lobe processes. Amyotroph Lateral Scler Frontotemporal Degener. 2017;18:182-192. 
  26. Hobson EV, McDermott CJ. Supportive and symptomatic management of amyotrophic lateral sclerosis. Nat Rev Neurol. 2016;12:526-538.
  27. Phukan J, Pender NP, Hardiman O. Cognitive impairment in amyotrophic lateral sclerosis. Lancet Neurol. 2007;6:994-1003.
  28. Salameh JS, Brown RH Jr, Berry JD. Amyotrophic lateral sclerosis: review. Semin Neurol. 2015;35:469-476. 
  29. Jésus P, Fayemendy P, Nicol M, et al. Hypermetabolism is a deleterious prognostic factor in patients with amyotrophic lateral sclerosis. Eur J Neurol. 2018;25:97-104.
  30. Al-Chalabi A, Hardiman O, Kiernan MC, Chiò A, Rix-Brooks B, van den Berg LH. Amyotrophic lateral sclerosis: moving towards a new classification system. Lancet Neurol. 2016;5:1182-1194.
  31. Costa J, Swash M, de Carvalho M. Awaji criteria for the diagnosis of amyotrophic lateral sclerosis: a systematic review. Arch Neurol. 2012;69:1410-1416.
  32. Geevasinga N, Menon P, Scherman DB, et al. Diagnostic criteria in amyotrophic lateral sclerosis: a multicenter prospective study. Neurology. 2016;87:684-690. 
  33. Li DW, Liu M, Cui B, et al. The Awaji criteria increases the diagnostic sensitivity of the revised El Escorial criteria for amyotrophic lateral sclerosis diagnosis in a Chinese population. PLoS One. 2017;12:e0171522. doi: 10.1371/journal.pone.0171522. eCollection 2017.
  34. Miller RG, Appel SH. Introduction to supplement: the current status of treatment for ALS. Amyotroph Lateral Scler Frontotemporal Degener. 2017;18(sup1):1-4.
  35. Sawada H. Clinical efficacy of edaravone for the treatment of amyotrophic lateral sclerosis. Expert Opin Pharmacother. 2017;18:735-738.
  36. Hardiman O, van den Berg LH, Kiernan MC. Clinical diagnosis and management of amyotrophic lateral sclerosis. Nat Rev Neurol. 2011;7:639-649.
  37. Amtmann D, Weydt P, Johnson KL, Jensen MP, Carter GT. Survey of cannabis use in patients with amyotrophic lateral sclerosis. Am J Hosp Palliat Care. 2004;21:95-104.
  38. Hardiman O, Al-Chalabi A, Chio A, et al. Amyotrophic lateral sclerosis. Nat Rev Dis Primers. 2017;3:17071.