Journal of Neurology

, Volume 259, Issue 2, pp 334–341 | Cite as

Emotional adjustment in amyotrophic lateral sclerosis (ALS)

  • Dorothée LuléEmail author
  • Sandra Pauli
  • Ertan Altintas
  • Ulrike Singer
  • Thomas Merk
  • Ingo Uttner
  • Niels Birbaumer
  • Albert C. Ludolph
Original Communication


Despite the devastating motor impairment, a significant number of patients with amyotrophic lateral sclerosis (ALS) maintain a good psychosocial adjustment. Here we investigated whether this is specific for ALS or a more general characteristic of terminal disease. Psychosocial adjustment was investigated in 30 ALS patients, 29 cancer patients in palliative treatment and 29 age-, gender- and level of education-matched healthy controls. Subjective quality of life (sQoL), degree of depressive symptoms and coping were evaluated as measures of psychosocial adjustment. Personality factors were described. ALS and cancer patients showed a good psychosocial adjustment. Subjective QoL and depression did not differ significantly. Both patient groups presented a good sQoL. The level of mild depressive symptoms in both patient groups was similar and none showed clinically relevant depression. ALS patients expressed fewer active coping strategies than cancer patients which were explained by gender differences. Both patient groups showed comparable psychosocial adjustment to their disease. Overall, in terminally ill patients the psychological response to the prognosis is not associated with neurobiological changes (e.g., associated with subclinical deficits in ALS) or with physical decline.


ALS Cancer Quality of life Coping Emotion 



Supported by the Deutsche Forschungsgemeinschaft (DFG), Ministry of Education and Research (BMBF), Bernstein Center for Computational Neuroscience, European Community grants (EU) and European Research Council (ERC). We would like to thank the physicians of the outpatient clinic of the department of Neurology, in specific Corinna Hendrich and Anne Sperfeld, and the physicians of the outpatient clinic of the department of Oncology at the University of Ulm for supporting us with the recruitment of patients, and the organizing committee for senior education at the University of Ulm (Zentrum für angewandte wissenschaftliche Weiterbildung, ZAWIW) for the recruitment of healthy controls. We are greatly indebted to all patients and participants who participated in this study.

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Ludolph AC, Langen KJ, Regard M, Herzog H, Kemper B, Kuwert T, Böttger IG, Feinendegen L (1992) Frontal lobe function in amyotrophic lateral sclerosis: a neuropsychologic and positron emission tomography study. Acta Neurol Scand 85(2):81–89PubMedCrossRefGoogle Scholar
  2. 2.
    Lulé D, Diekmann V, Müller HP, Kassubek J, Ludolph AC, Birbaumer N (2010) Neuroimaging of multimodal sensory stimulation in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 81(8):899–906PubMedCrossRefGoogle Scholar
  3. 3.
    Lulé D, Diekmann V, Anders S, Kassubek J, Kübler A, Ludolph AC, Birbaumer N (2007) Brain responses to emotional stimuli in patients with amyotrophic lateral sclerosis (ALS). J Neurol 254(4):519–527PubMedCrossRefGoogle Scholar
  4. 4.
    Papps B, Abrahams S, Wicks P, Leigh PN, Goldstein LH (2005) Changes in memory for emotional material in amyotrophic lateral sclerosis (ALS). Neuropsychologia 43(8):1107–1114PubMedCrossRefGoogle Scholar
  5. 5.
    Phukan J, Pender NP, Hardiman O (2007) Cognitive impairment in amyotrophic lateral sclerosis. Lancet Neurol 6(11):994–1003PubMedCrossRefGoogle Scholar
  6. 6.
    Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz PE (2005) Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology 65(4):586–590PubMedCrossRefGoogle Scholar
  7. 7.
    Schreiber H, Gaigalat T, Wiedemuth-Catrinescu U, Graf M, Uttner I, Muche R, Ludolph AC (2005) Cognitive function in bulbar- and spinal-onset amyotrophic lateral sclerosis. A longitudinal study in 52 patients. J Neurol 252(7):772–781PubMedCrossRefGoogle Scholar
  8. 8.
    Murphy J, Henry R, Lomen-Hoerth C (2007) Establishing subtypes of the continuum of frontal lobe impairment in amyotrophic lateral sclerosis. Arch Neurol 64(3):330–334PubMedCrossRefGoogle Scholar
  9. 9.
    Röttig D, Leplow B, Eger K, Ludolph AC, Graf M, Zierz S (2006) Only subtle cognitive deficits in non-bulbar amyotrophic lateral sclerosis patients. J Neurol 253(3):333–339PubMedCrossRefGoogle Scholar
  10. 10.
    Livneh H, Antonak RF (2005) Psychosocial adaptation to chronic illness and disability: a primer for counselors. J Couns Dev 83:12–20CrossRefGoogle Scholar
  11. 11.
    Lazarus RS, Folkman S (1984) Stress, appraisal and coping. Springer, New YorkGoogle Scholar
  12. 12.
    Anderson NB (2004) Encyclopedia of health and behavior. Sage, CaliforniaGoogle Scholar
  13. 13.
    Matuz T, Birbaumer N, Hautzinger M, Kübler A (2010) Coping with amyotrophic lateral sclerosis: an integrative view. J Neurol Neurosurg Psychiatry 81(8):893–898PubMedCrossRefGoogle Scholar
  14. 14.
    Kurt A, Nijboer F, Matuz T, Kübler A (2007) Depression and anxiety in individuals with amyotrophic lateral sclerosis: epidemiology and management. CNS Drugs 21(4):279–291PubMedCrossRefGoogle Scholar
  15. 15.
    Lulé D, Häcker S, Ludolph A, Birbaumer N, Kübler A (2008) Depression and quality of life in patients with amyotrophic lateral sclerosis. Dtsch Arztebl Int 105(23):397–403PubMedGoogle Scholar
  16. 16.
    Albrecht GL, Devlieger PJ (1999) The disability paradox: high quality of life against all odds. Soc Sci Med 48(8):977–988PubMedCrossRefGoogle Scholar
  17. 17.
    Kubler A, Winter S, Ludolph AC, Hautzinger M, Birbaumer N (2005) Severity of depressive symptoms and quality of life in patients with amyotrophic lateral sclerosis. Neurorehabil Neural Repair 19:182–193PubMedCrossRefGoogle Scholar
  18. 18.
    Lulé D, Zickler C, Häcker S, Bruno MA, Demertzi A, Pellas F, Laureys S, Kübler A (2009) Life can be worth living in locked-in syndrome. Prog Brain Res 177:339–351PubMedCrossRefGoogle Scholar
  19. 19.
    Brown WA, Mueller PS (1970) Psychological function in individuals with amyotrophic lateral sclerosis (ALS). Psychosom Med 32:141–152PubMedGoogle Scholar
  20. 20.
    Lule D, Ludolph AC, Ludolph AG (2008) Neurodevelopmental and neurodegenerative diseases—is there a pathophysiological link? Attention-deficit/hyperactivity disorder and amyotrophic lateral sclerosis as examples. Med Hypotheses 70(6):1133–1138PubMedCrossRefGoogle Scholar
  21. 21.
    Neudert C, Wasner M, Borasio GD (2001) Patients’ assessment of quality of life instruments: a randomized study of SIP, SF-36 and SEIQoL-DW in patients with amyotrophic lateral sclerosis. J Neurol Sci 191:103–109PubMedCrossRefGoogle Scholar
  22. 22.
    Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B, Nakanishi A (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci 169(1–2):13–21PubMedCrossRefGoogle Scholar
  23. 23.
    Folstein MF, Folstein SE, McHugh PR (1975) “Mini-Mental State”: a practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 12:189–198PubMedCrossRefGoogle Scholar
  24. 24.
    Hautzinger M, Bailer M, Worall H, Keller F (1995) Beck-Depressions-Inventar (BDI), 2nd edn. Hans-Huber-Verlag, BernGoogle Scholar
  25. 25.
    Jerusalem M, Schwarzer R (1998) Anxiety and self-concept as antecendents of stress and coping. Pers Individ Dif 10:785–792CrossRefGoogle Scholar
  26. 26.
    Fahrenberg J, Hampel R, Selg H (1994) Das Freiburger Persönlichkeitsinventar FPI, revidierte Fassung FPI-R. Hogrefe-Verlag für Psychologie, GöttingenGoogle Scholar
  27. 27.
    Borkenau P, Ostendorf F (1993) NEO-Fünf-Faktoren Inventar (NEO-FFI) nach Costa und McCrae. Hogrefe, GöttingenGoogle Scholar
  28. 28.
    Hecht M, Hillemacher T, Gräsel E, Tigges S, Winterholler M, Heuss D, Hilz MJ, Neundörfer B (2002) Subjective experience and coping in ALS. Amyotroph Lateral Scler Other Motor Neuron Disord 3(4):225–231PubMedGoogle Scholar
  29. 29.
    Taylor L, Wicks P, Leigh PN, Goldstein LH (2010) Prevalence of depression in amyotrophic lateral sclerosis and other motor disorders. Eur J Neurol 17(8):1047–1053PubMedCrossRefGoogle Scholar
  30. 30.
    Johansson CM, Axelsson B, Danielson E (2006) Living with incurable cancer at the end of life–patients perceptions on quality of life. Cancer Nurs 29:391–399PubMedCrossRefGoogle Scholar
  31. 31.
    Sharpe L, Butow P, Smith C, McConnell D, Clarke S (2005) Changes in quality of life in patients with advanced cancer: evidence of response shift and response restriction. J Psychosom Res 58:497–504PubMedCrossRefGoogle Scholar
  32. 32.
    Bungener C, Piquard A, Pradat PF, Salachas F, Meininger V, Lacomblez L (2005) Psychopathology in amyotrophic lateral sclerosis: a preliminary study with 27 ALS patients. Amyotroph Lateral Scler Other Motor Neuron Disord 6(4):221–225PubMedCrossRefGoogle Scholar
  33. 33.
    Krampe H, Bartels C, Victorson D, Enders CK, Beaumont J, Cella D, Ehrenreich H (2008) The influence of personality factors on disease progression and health-related quality of life in people with ALS. Amyotroph Lateral Scler 9(2):99–107PubMedCrossRefGoogle Scholar
  34. 34.
    Grossman AB, Levin BE, Bradley WG (2006) Premorbid personality characteristics of patients with ALS. Amyotroph Lateral Scler 7(1):27–31PubMedCrossRefGoogle Scholar
  35. 35.
    Houpt JL, Gould BS, Norris FH Jr (1977) Psychological characteristics of patients with amyotrophic lateral sclerosis. Psychosom Med 39:299–303PubMedGoogle Scholar
  36. 36.
    Peters PK, Swenson WM, Mulder DW (1978) Is there a characteristic personality profile in amyotrophic lateral sclerosis. Arch Neurol 35:321–322PubMedCrossRefGoogle Scholar
  37. 37.
    Carr AJ, Gibson B, Robinson PG (2001) Measuring quality of life: is quality of life determined by expectations or experience? BMJ 322(7296):1240–1243PubMedCrossRefGoogle Scholar
  38. 38.
    Felgoise SH, Stewart JL, Bremer BA, Walsh SM, Bromberg MB, Simmons Z (2009) The SEIQoL-DW for assessing quality of life in ALS: strengths and limitations. Amyotroph Lateral Scler 10(5–6):456–462PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2011

Authors and Affiliations

  • Dorothée Lulé
    • 1
    • 2
    • 3
    Email author
  • Sandra Pauli
    • 3
  • Ertan Altintas
    • 3
  • Ulrike Singer
    • 3
  • Thomas Merk
    • 4
  • Ingo Uttner
    • 3
  • Niels Birbaumer
    • 2
    • 5
  • Albert C. Ludolph
    • 3
  1. 1.Section of NeurophysiologyUniversity of UlmUlmGermany
  2. 2.Institute of Medical Psychology and Behavioral NeurobiologyEberhard-Karls-University of TübingenTübingenGermany
  3. 3.Department of NeurologyUniversity of UlmUlmGermany
  4. 4.Klinik für Pneumologie, Beatmungsmedizin, und Allgemeine Innere MedizinKrankenhaus vom Roten KreuzStuttgart/Bad-CannstadtGermany
  5. 5.Ospedale San Camillo, IRCCSVeneziaItaly

Personalised recommendations