Quality of Life Research

, Volume 23, Issue 5, pp 1479–1488 | Cite as

Fatigue in patients with spinal muscular atrophy type II and congenital myopathies: evaluation of the fatigue severity scale

  • Ulla Werlauff
  • A. Højberg
  • R. Firla-Holme
  • B. F. Steffensen
  • J. Vissing



The aim of this study was to evaluate whether the fatigue severity scale (FSS) is an appropriate instrument to assess fatigue in patients with spinal muscular atrophy type II (SMA II) and congenital myopathies (CM).


FSS and visual analog scale (VAS) were administered to 33 SMA II- and 72 CM patients. The psychometric properties of the FSS were evaluated by means of classical test theories for each of the disease groups. If abnormal fatigue was present in the disease group, the construct of fatigue was evaluated by means of focus group interviews.


Fatigue was rare in SMA II patients, but very frequent in patients with CM. The cut-off score designating abnormal fatigue (FSS score ≥ 4) was exceeded by 10 % of the SMA II patients in contrast to 76 % of the CM patients, of whom 52 % suffered from severe fatigue (FSS score ≥ 5). Focus group interviews demonstrated that fatigue had an adverse effect on motor function, level of energy, social relations, and identity, four themes that could be captured by the FSS. The FSS and VAS were strongly correlated in SMA II patients, but only moderately in CM patients. The psychometric properties indicated that the original FSS with nine items measures more than one construct of fatigue, eliminating the first two items improved scale properties.


This study demonstrates that fatigue is characteristic in patients with CM, but not in patients with SMA II, in whom fatigue does not seem to impact daily life. While fatigue in CM and SMA II can be captured by FSS, omitting the first two items of the scale will improve its properties and content validity, along with comprehension of the scale itself.


Neuromuscular diseases Fatigue Focus groups Qualitative research Reliability Validity 



We thank all the patients in the studies for participating and sharing their knowledge.


  1. 1.
    Lou, J. S., Weiss, M. D., & Carter, G. T. (2010). Assessment and management of fatigue in neuromuscular disease. American Journal of Hospice and Palliative Medicine, 27(2), 145–157.PubMedCrossRefGoogle Scholar
  2. 2.
    World Health organization. (2001). International classification of functioning, disability and health: ICF. Geneva: WHO.Google Scholar
  3. 3.
    de Vries, J. M., Hagemans, M. L., Bussmann, J. B., van der Ploeg, A. T., & van Doorn, P. A. (2010). Fatigue in neuromuscular disorders: Focus on Guillain–Barré syndrome and Pompe disease. Cellular and Molecular Life Sciences, 67(5), 701–713.PubMedCentralPubMedCrossRefGoogle Scholar
  4. 4.
    Wokke, J. H. (2007). Fatigue is part of the burden of neuromuscular diseases. Journal of Neurology, 254(7), 948–949.PubMedCentralPubMedCrossRefGoogle Scholar
  5. 5.
    Bigland-Ritchie, B., Jones, D. A., Hosking, G. P., & Edwards, R. H. (1978). Central and peripheral fatigue in sustained maximum voluntary contractions of human quadriceps muscle. Clinical Science and Molecular Medicine, 54(6), 609–614.PubMedGoogle Scholar
  6. 6.
    Chaudhuri, A., & Behan, P. O. (2004). Fatigue in neurological disorders. Lancet, 363(9413), 978–988.PubMedCrossRefGoogle Scholar
  7. 7.
    Krupp, L. B., LaRocca, N. G., Muir-Nash, J., & Steinberg, A. D. (1989). The fatigue severity scale. Application to patients with multiple sclerosis and systemic lupus erythematosus. Archives of Neurology, 46, 1121–1123.PubMedCrossRefGoogle Scholar
  8. 8.
  9. 9.
    Pettersson, S., Möller, S., Svenungsson, E., Gunnarsson, I., & Welin Henriksson, E. (2010). Women’s experience of SLE-related fatigue: A focus group interview study. Rheumatology (Oxford), 49(10), 1935–1942.CrossRefGoogle Scholar
  10. 10.
    Kalkmann, J. S., Schillings, M. L., van der Werf, S. P., Padberg, G. W., Zwarts, M. J., van Engelen, B. G., et al. (2005). Experienced fatigue in facioscapulohumeral dystrophy, myotonic dystrophy, and HMSN-I. Journal of Neurology, Neurosurgery and Psychiatry, 76(10), 1406–1409.CrossRefGoogle Scholar
  11. 11.
    Féasson, L., Camdessanché, J. P., El Mandhi, L., Calmels, P., & Millet, G. Y. (2006). Fatigue and neuromuscular diseases. Annales de Readaptation et de Medecine Physique, 49(6), 289–300.PubMedCrossRefGoogle Scholar
  12. 12.
    de Groot, I. J., & de Witte, L. P. (2005). Physical complaints in ageing persons with spinal muscular atrophy. Journal of Rehabilitation Medicine, 37(4), 8–62.CrossRefGoogle Scholar
  13. 13.
    North, K. (2008). What’s new in congenital myopathies? Neuromuscular Disorders, 18(6), 433–442.PubMedCrossRefGoogle Scholar
  14. 14.
    Hagemans, M. L., van Schie, S. P., Janssens, A. C., van Doorn, P. A., Reuser, A. J., & van der Ploeg, A. T. (2007). Fatigue: An important feature of late-onset Pompe disease. Journal of Neurology, 254(7), 941–945.PubMedCentralPubMedCrossRefGoogle Scholar
  15. 15.
    Merkies, I. S., Schmitz, P. I., Samijn, J. P., van der Meché, F. G., & van Doorn, P. A. (1999). Fatigue in immune-mediated polyneuropathies. European Inflammatory Neuropathy Cause and Treatment (INCAT) Group. Neurology, 53(8), 1648–1654.PubMedCrossRefGoogle Scholar
  16. 16.
    Kleinman, L., Zodet, M. W., Hakim, Z., Aledort, J., Barker, C., Chan, K., et al. (2000). Psychometric evaluation of the fatigue severity scale for use in chronic hepatitis C. Quality of Life Research, 9(5), 499–508.PubMedCrossRefGoogle Scholar
  17. 17.
    Ferentinos, P., Kontaxakis, V., Havaki-Kontaxaki, B., Dikeos, D., & Lykouras, L. (2011). Psychometric evaluation of the Fatigue Severity Scale in patients with major depression. Quality of Life Research, 20(3), 457–465.PubMedCrossRefGoogle Scholar
  18. 18.
    Valko, P. O., Bassetti, C. L., Bloch, K. E., Held, U., & Baumann, C. R. (2008). Validation of the Fatigue Severity Scale in a Swiss cohort. Sleep, 31(11), 1601–1607.PubMedCentralPubMedGoogle Scholar
  19. 19.
    Lerdal, A., Wahl, A., Rustøen, T., Hanestad, B. R., & Moum, T. (2005). Fatigue in the general population: A translation and test of the psychometric properties of the Norwegian version of the fatigue severity scale. Scandinavian Journal of Public Health, 33(2), 123–130.PubMedCrossRefGoogle Scholar
  20. 20.
    Mills, R. J., Young, C. A., Nicholas, R. S., Pallant, J. F., & Tennant, A. (2009). Rasch analysis of the Fatigue Severity Scale in multiple sclerosis. Multiple sclerosis, 15(1), 81–87.PubMedCrossRefGoogle Scholar
  21. 21.
    Lerdal, A., Johansson, S., Kottorp, A., & von Koch, L. (2010). Psychometric properties of the Fatigue Severity Scale: Rasch analyses of responses in a Norwegian and Swedish MS cohort. Multiple sclerosis, 16(6), 733–741.PubMedCrossRefGoogle Scholar
  22. 22.
    Lerdal, A., & Kottorp, A. (2011). Psychometric properties of the Fatigue Severity Scale—Rasch analyses of individual responses in a Norwegian stroke cohort. International Journal of Nursing Studies, 48(10), 1258–1265.PubMedCrossRefGoogle Scholar
  23. 23.
    Amtmann, D., Bamer, A. M., Noonan, V., Lang, N., Kim, J., & Cook, K. F. (2012). Comparison of the psychometric properties of two fatigue scales in multiple sclerosis. Rehabilitation Psychology, 57(2), 159–166.PubMedCentralPubMedCrossRefGoogle Scholar
  24. 24.
    Takasaki, H., & Treleaven, J. (2012). Construct validity and test–retest reliability of the Fatigue Severity Scale in people with chronic neck pain. Archives of Physical Medicine and Rehabilitation, 94(7), 1328–1334.Google Scholar
  25. 25.
    Rothmann, M., Burke, L., Erickson, P., Leidy, N. K., Patrick, D. L., & Petrie, C. D. (2009). Use of existing patient-reported outcome (PRO) instruments and their modification: The ISPOR good research practice for evaluating and documenting content validity for the use of existing instruments and their modification PRO task report. Value in Health, 12(8), 1075–1083.CrossRefGoogle Scholar
  26. 26.
    Patrick, D. L., Burke, L., Erickson, P., Gwaltney, C. J., Leidy, N. K., Martin, M. L., et al. (2011). Content validity—Establishing and reporting the evidence in newly developed patient-reported outcome (PRO) instruments for medical product evaluation: ISPOR PRO good research practices task force report: Part I—Eliciting concepts for a new PRO instrument. Value in Health, 14(8), 967–977.PubMedCrossRefGoogle Scholar
  27. 27.
    Patrick, D. L., Burke, L., Gwaltney, C. J., Leidy, N. K., Martin, M. L., Molsen, E., et al. (2011). Content validity—Establishing and reporting the evidence in newly developed patient-reported outcome (PRO) instruments for medical product evaluation: ISPOR PRO good research practices task force report: Part 2—Assessing respondent understanding. Value in Health, 14(8), 978–988.PubMedCrossRefGoogle Scholar
  28. 28.
    Tashakkori, A., & Teddlie, C. (1998). Mixed methodology. Combining quantitative and qualitative approaches. Thousand Oaks, CA: Sage.Google Scholar
  29. 29.
    Morgan, D. L. (1997). Focus groups as qualitative research (2nd ed.). London: Sage.Google Scholar
  30. 30.
    Quimet, J. A., Bunnage, J. B., Carini, R. M., Kuh, G. D., & Kennedy, J. (2004). Using focus groups to establish the validity and reliability of a college student survey. Research in Higher Education, 45, 233–250.CrossRefGoogle Scholar
  31. 31.
    Fayers, P. M., & Machin, D. (2009). Quality of life. The assessment analysis and interpretation of patient-reported outcomes. New York: Wiley.Google Scholar
  32. 32.
    Kaiser, H. F. (1960). The application of electronic computers to factor analysis. Educational and Psychological Measurement, 20, 141–151.CrossRefGoogle Scholar
  33. 33.
    Krueger, R. A. (1998). Moderating focus groups. Thousand Oaks, CA: Sage.Google Scholar
  34. 34.
    Hsieh, H., & Shannon, S. E. (2005). Three approaches to qualitative content analysis. Qualitative Health Research, 15(9), 1277–1288.PubMedCrossRefGoogle Scholar
  35. 35.
    Coffey, A., & Atkinson, P. (1996). Making sense of qualitative data. Complemtary research strategies. London: Sage.Google Scholar
  36. 36.
    Pawlikowska, T., Chalder, T., Hirsch, S. R., Wallace, P., Wright, D. J., & Wessely, S. C. (1994). Population based study of fatigue and psychological distress. BMJ, 19, 763–766.CrossRefGoogle Scholar
  37. 37.
    Flachenecker, P., Kümpfel, T., Kallmann, B., Gottschalk, M., Grauer, O., Rieckmann, P., et al. (2002). Fatigue in multiple sclerosis: A comparison of different rating scales and correlation to clinical parameters. Multiple sclerosis, 8(6), 523–526.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Dordrecht 2013

Authors and Affiliations

  • Ulla Werlauff
    • 1
  • A. Højberg
    • 1
  • R. Firla-Holme
    • 2
  • B. F. Steffensen
    • 1
  • J. Vissing
    • 3
  1. 1.The Danish National Rehabilitation Center for Neuromuscular DiseasesÅrhusDenmark
  2. 2.GentofteDenmark
  3. 3.Neuromuscular Clinic and Research Unit, Department of Neurology, RigshospitaletUniversity of CopenhagenCopenhagenDenmark

Personalised recommendations