Current Psychiatry Reports

, Volume 12, Issue 3, pp 239–245 | Cite as

Psychiatric Aspects of Amyotrophic Lateral Sclerosis (ALS)



Amyotrophic lateral sclerosis (ALS) is a progressive disorder characterized by degeneration of motor neurons. Given the severe nature of ALS, many believed that patients would suffer from a high level of depression and a low quality of life. However, research into the psychological health of patients with ALS has shown that this is not the case. This article reviews the state of current knowledge as it pertains to the psychological health of ALS patients in four broad areas: quality of life, personality characteristics, emotional reactions, and end-of-life choices.


Amyotrophic lateral sclerosis Quality of life Psychiatry Psychological reactions Personality Major depressive disorder End of life 


Papers of particular interest, published recently, have been highlighted as: •• Of major importance

  1. 1.
    •• Averill AJ, Kasarskis EJ, Segerstrom SC: Psychological health in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2007, 8:243–254. In this recent review, the authors do a superb job of summarizing many of the current studies involving ALS and depression. In addition, they present a very informative summary table that discusses the merits of all 28 studies.CrossRefPubMedGoogle Scholar
  2. 2.
    Hillemacher T, Grassel E, Tigges S, et al.: Depression and bulbar involvement in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2004, 5:245–249.CrossRefPubMedGoogle Scholar
  3. 3.
    Lomen-Hoerth C, Anderson T, Miller B: The overlap of amyotrophic lateral sclerosis and frontotemporal dementia. Neurology 2002, 59:1077–1079.CrossRefPubMedGoogle Scholar
  4. 4.
    Vignola A, Guzzo A, Calvo A, et al.: Anxiety undermines quality of life in ALS patients and caregivers. Eur J Neurol 2008, 15:1231–1236.CrossRefPubMedGoogle Scholar
  5. 5.
    Murphy JM, Henry RG, Langmore S, et al.: Continuum of frontal lobe impairment in amyotrophic lateral sclerosis. Arch Neurol 2007, 64:530–534.CrossRefPubMedGoogle Scholar
  6. 6.
    Murphy J, Henry R, Lomen-Hoerth C: Establishing subtypes of the continuum of frontal lobe impairment in amyotrophic lateral sclerosis. Arch Neurol 2007, 64:330–334.CrossRefPubMedGoogle Scholar
  7. 7.
    Rabkin JG, Wagner GJ, Del Bene M: Resilience and distress among amyotrophic lateral sclerosis patients and caregivers. Psychosom Med 2000, 62:271–279.PubMedGoogle Scholar
  8. 8.
    Rabkin JG, Albert SM, Tider T, et al.: Predictors and course of elective long-term mechanical ventilation: a prospective study of ALS patients. Amyotroph Lateral Scler 2006, 7:86–95.CrossRefPubMedGoogle Scholar
  9. 9.
    Rabkin JG, Albert SM, Del Bene ML, et al.: Prevalence of depressive disorders and change over time in late-stage ALS. Neurology 2005, 65:62–67.CrossRefPubMedGoogle Scholar
  10. 10.
    McElhiney MC, Rabkin JG, Gordon PH, et al.: Prevalence of fatigue and depression in ALS patients and change over time. J Neurol Neurosurg Psychiatry 2009, 80:1146–1149.CrossRefPubMedGoogle Scholar
  11. 11.
    Kubler A, Winter S, Ludolph AC, et al.: Severity of depressive symptoms and quality of life in patients with amyotrophic lateral sclerosis. Neurorehabil Neural Repair 2005, 19:182–193.CrossRefPubMedGoogle Scholar
  12. 12.
    Lomen-Hoerth C: Characterization of amyotrophic lateral sclerosis and frontotemporal dementia. Dement Geriatr Cogn Disord 2004, 17:337–341.CrossRefPubMedGoogle Scholar
  13. 13.
    Lomen-Hoerth C, Murphy J, Langmore S, et al.: Are amyotrophic lateral sclerosis patients cognitively normal? Neurology 2003, 60:1094–1097.PubMedGoogle Scholar
  14. 14.
    Coons SJ, Rao S, Keininger DL, Hays RD: A comparative review of generic quality-of-life instruments. Pharmacoeconomics 2000, 17:13–35.CrossRefPubMedGoogle Scholar
  15. 15.
    Jenkinson C, Fitzpatrick R, Swash M, Peto V; ALS-HPS Steering Group: The ALS health profile study: quality of life of amyotrophic lateral sclerosis patients and carers in Europe. J Neurol 2000, 247:835–840.CrossRefPubMedGoogle Scholar
  16. 16.
    Jenkinson C, Hobart J, Chandola T, et al.: Use of the Short Form Health Survey (SF-36) in patients with amyotrophic lateral sclerosis: tests of data quality, score reliability, response rate and scaling assumptions. J Neurol 2002, 249:178–183.CrossRefPubMedGoogle Scholar
  17. 17.
    Simmons Z, Bremer BA, Robbins RA, et al.: Quality of life in ALS depends on factors other than strength and physical function. Neurology 2000, 55:388–392.PubMedGoogle Scholar
  18. 18.
    Simmons Z, Felgoise SH, Bremer BA, et al.: The ALSSQOL: balancing physical and nonphysical factors in assessing quality of life in ALS. Neurology 2006, 67:1659–1664.CrossRefPubMedGoogle Scholar
  19. 19.
    Nelson ND, Trail M, Van JN, et al.: Quality of life in patients with amyotrophic lateral sclerosis: perceptions, coping resources, and illness characteristics. J Palliat Med 2003, 6:417–424.CrossRefPubMedGoogle Scholar
  20. 20.
    Bromberg MB: Assessing quality of life in ALS. J Clin Neuromuscul Dis 2007, 9:318–325.CrossRefPubMedGoogle Scholar
  21. 21.
    Epton J, Harris R, Jenkinson C: Quality of life in amyotrophic lateral sclerosis/motor neuron disease: a structured review. Amyotroph Lateral Scler 2009, 10:15–26.CrossRefPubMedGoogle Scholar
  22. 22.
    Chio A, Gauthier A, Montuschi A, et al.: A cross sectional study on determinants of quality of life in ALS. J Neurol Neurosurg Psychiatry 2004, 75:1597–1601.CrossRefPubMedGoogle Scholar
  23. 23.
    Neudert C, Wasner M, Borasio GD: Patients’ assessment of quality of life instruments: a randomised study of SIP, SF-36 and SEIQoL-DW in patients with amyotrophic lateral sclerosis. J Neurol Sci 2001, 191:103–109.CrossRefPubMedGoogle Scholar
  24. 24.
    •• Roach AR, Averill AJ, Segerstrom SC, Kasarskis EJ: The dynamics of quality of life in ALS patients and caregivers. Ann Behav Med 2009, 37:197–206. This was the first article to examine how QOL changes over time. To this point, QOL assessments were essentially snapshots. This article and others introduced the idea that QOL changes in ALS, and these changes must be taken into account.CrossRefPubMedGoogle Scholar
  25. 25.
    Koenig HG, George LK, Peterson BL, Pieper CF: Depression in medically ill hospitalized older adults: prevalence, characteristics, and course of symptoms according to six diagnostic schemes. Am J Psychiatry 1997, 154:1376–1383.PubMedGoogle Scholar
  26. 26.
    Clarke DM, Smith GC, Herrman HE: A comparative study of screening instruments for mental disorders in general hospital patients. Int J Psychiatry Med 1993, 23:323–337.PubMedGoogle Scholar
  27. 27.
    Clarke DM, Kissane DW, Trauer T, Smith GC: Demoralization, anhedonia and grief in patients with severe physical illness. World Psychiatry 2005, 4:96–105.PubMedGoogle Scholar
  28. 28.
    Clarke DM, Cook K, Smith GC, Piterman L: What do general practitioners think depression is? A taxonomy of distress and depression for general practice. Med J Aust 2008, 188(12 Suppl):S110–S113.PubMedGoogle Scholar
  29. 29.
    Lazarus RS: From psychological stress to the emotions: a history of changing outlooks. Annu Rev Psychol 1993, 44:1–21.CrossRefPubMedGoogle Scholar
  30. 30.
    Nichols SE: Psychosocial reactions of persons with the acquired immunodeficiency syndrome. Ann Intern Med 1985, 103:765–767.PubMedGoogle Scholar
  31. 31.
    Lou JS, Reeves A, Benice T, Sexton G: Fatigue and depression are associated with poor quality of life in ALS. Neurology 2003, 60:122–123.PubMedGoogle Scholar
  32. 32.
    Trail M, Nelson ND, Van JN, et al.: A study comparing patients with amyotrophic lateral sclerosis and their caregivers on measures of quality of life, depression, and their attitudes toward treatment options. J Neurol Sci 2003, 209:79–85.CrossRefPubMedGoogle Scholar
  33. 33.
    Wicks P, Abrahams S, Masi D, et al.: Prevalence of depression in a 12-month consecutive sample of patients with ALS. Eur J Neurol 2007, 14:993–1001. (Published erratum appears in Eur J Neurol 2008, 15:1009.)Google Scholar
  34. 34.
    Gauthier A, Vignola A, Calvo A, et al.: A longitudinal study on quality of life and depression in ALS patient-caregiver couples. Neurology 2007, 68:923–926.CrossRefPubMedGoogle Scholar
  35. 35.
    Passik SD, Roth AJ: Anxiety symptoms and panic attacks preceding pancreatic cancer diagnosis. Psychooncology 1999, 8:268–272.CrossRefPubMedGoogle Scholar
  36. 36.
    Cloninger CR: The psychobiological theory of temperament and character: comment on Farmer and Goldberg (2008). Psychol Assess 2008, 20:292–299; discussion 300–304.Google Scholar
  37. 37.
    Cloninger CR: How does personality influence mortality in the elderly? Psychosom Med 2005, 67:839–840.CrossRefPubMedGoogle Scholar
  38. 38.
    Cloninger CR, Svrakic DM, Przybeck TR: Can personality assessment predict future depression? A twelve-month follow-up of 631 subjects. J Affect Disord 2006, 92:35–44.CrossRefPubMedGoogle Scholar
  39. 39.
    Grossman AB, Levin BE, Bradley WG: Premorbid personality characteristics of patients with ALS. Amyotroph Lateral Scler 2006, 7:27–31.CrossRefPubMedGoogle Scholar
  40. 40.
    Krampe H, Bartels C, Victorson D, et al.: The influence of personality factors on disease progression and health-related quality of life in people with ALS. Amyotroph Lateral Scler 2008, 9:99–107.CrossRefPubMedGoogle Scholar
  41. 41.
    Beck AT, Weissman A, Lester D, Trexler L: The measurement of pessimism: the Hopelessness Scale. J Consult Clin Psychol 1974, 42:861–865.CrossRefPubMedGoogle Scholar
  42. 42.
    •• Miglioretti M, Mazzini L, Oggioni GD, et al.: Illness perceptions, mood and health-related quality of life in patients with amyotrophic lateral sclerosis. J Psychosom Res 2008, 65:603–609. This article took a critical look at illness perceptions in ALS. In many ways, it was bringing together the observations of others, such as Plathua and Lazarus. Of note, this article also identified standing variables with which patients assess their current state.CrossRefPubMedGoogle Scholar
  43. 43.
    Browne GB, Byrne C, Roberts J, et al.: The Meaning of Illness Questionnaire: reliability and validity. Nurs Res 1988, 37:368–373.CrossRefPubMedGoogle Scholar
  44. 44.
    Folkman S, Lazarus RS: The relationship between coping and emotion: implications for theory and research. Soc Sci Med 1988, 26:309–317.CrossRefPubMedGoogle Scholar
  45. 45.
    Folkman S, Lazarus RS, Gruen RJ, DeLongis A: Appraisal, coping, health status, and psychological symptoms. J Pers Soc Psychol 1986, 50:571–579.CrossRefPubMedGoogle Scholar
  46. 46.
    Plahuta JM, McCulloch BJ, Kasarskis EJ, et al.: Amyotrophic lateral sclerosis and hopelessness: psychosocial factors. Soc Sci Med 2002, 55:2131–2140.CrossRefPubMedGoogle Scholar
  47. 47.
    Fanos JH, Gelinas DF, Foster RS, et al.: Hope in palliative care: from narcissism to self-transcendence in amyotrophic lateral sclerosis. J Palliat Med 2008, 11:470–475.CrossRefPubMedGoogle Scholar
  48. 48.
    Goldstein LH, Atkins L, Landau S, et al.: Longitudinal predictors of psychological distress and self-esteem in people with ALS. Neurology 2006, 67:1652–1658.CrossRefPubMedGoogle Scholar
  49. 49.
    Centers LC: Beyond denial and despair: ALS and our heroic potential for hope. J Palliat Care 2001, 17:259–264.PubMedGoogle Scholar
  50. 50.
    •• Maessen M, Veldink JH, Onwuteaka-Philipsen BD, et al.: Trends and determinants of end-of-life practices in ALS in the Netherlands. Neurology 2009, 73:954–961. This is a very important article that gives insight into the end-of-life choices in those with ALS. It basically shows that those with ALS who opt for PAS are for the most part not depressed. This along with the work of Rabkin et al. further argues against the idea of MDD being an inevitable psychosocial outcome of ALS.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  1. 1.Department of Psychiatry and Behavioral ScienceGeorge Washington UniversityWashington DCUSA

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