Seminars in Arthritis and Rheumatism
Volume 40, Issue 3 , Pages 267-274 , December 2010

Fatigue Is a Frequent and Clinically Relevant Problem in Ehlers-Danlos Syndrome

  • Nicol C. Voermans, MD

      Affiliations

    • Neuromuscular Centre Nijmegen, Department of Neurology, Nijmegen, The Netherlands
    • Corresponding Author InformationAddress reprint requests to: N.C. Voermans, MD, Department of Neurology, 935, Donders Institute of Brain, Cognition and Behaviour, Radboud University Nijmegen Medical Centre, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands
    • ,
  • Hans Knoop, PhD

      Affiliations

    • Clinical psychologist, Expert Centre Chronic Fatigue, Nijmegen, The Netherlands
    • ,
  • Nicole van de Kamp, MSc

      Affiliations

    • Neuromuscular Centre Nijmegen, Department of Neurology, Nijmegen, The Netherlands
    • ,
  • Ben C. Hamel, MD, PhD

      Affiliations

    • Department of Human Genetics, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    • ,
  • Gijs Bleijenberg, PhD

      Affiliations

    • Clinical psychologist, Expert Centre Chronic Fatigue, Nijmegen, The Netherlands
    • ,
  • Baziel G. van Engelen, MD, PhD

      Affiliations

    • Neuromuscular Centre Nijmegen, Department of Neurology, Nijmegen, The Netherlands

References 

  1. Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup RJ. Ehlers-Danlos syndromes: revised nosology. Villefranche, 1997. Ehlers-Danlos National Foundation (USA) and Ehlers-Danlos Support Group (UK). Am J Med Genet. 1998;77:31–37
  2. Schalkwijk J, Zweers MC, Steijlen PM, Dean WB, Taylor G, van Vlijmen I, et al. A recessive form of the Ehlers-Danlos syndrome caused by tenascin-X deficiency. N Engl J Med. 2001;345:1167–1175
  3. Burch GH, Gong Y, Liu W, Dettman RW, Curry CJ, Smith L, et al. Tenascin-X deficiency is associated with Ehlers-Danlos syndrome. Nat Genet. 1997;17:104–108
  4. Steinmann B, Royce PM, Superti-Furga A. The Ehlers-Danlos syndromes. In:  Steinmann B,  Royce PM editor. Connective Tissue and Its Heritable Disorders. Wilmington (DE): Wiley-Liss Inc; 2002;p. 431–523
  5. Gawthrop F, Mould R, Sperritt A, Neale F. Ehlers-Danlos syndrome. BMJ. 2007;335:448–450
  6. Vercoulen JH, Swanink CM, Fennis JF, Galama JM, van der Meer JW, Bleijenberg G. Dimensional assessment of chronic fatigue syndrome. J Psychosom Res. 1994;38:383–392
  7. Kalkman JS, Schillings ML, van der Werf SP, Padberg GW, Zwarts MJ, van Engelen BG, et al. Experienced fatigue in facioscapulohumeral dystrophy, myotonic dystrophy, and HMSN-I. J Neurol Neurosurg Psychiatry. 2005;76:1406–1409
  8. Schwid SR, Covington M, Segal BM, Goodman AD. Fatigue in multiple sclerosis: current understanding and future directions. J Rehabil Res Dev. 2002;39:211–224
  9. Friedman J, Friedman H. Fatigue in Parkinson's disease. Neurology. 1993;43:2016–2018
  10. van der Werf SP, van den Broek HL, Anten HW, Bleijenberg G. Experience of severe fatigue long after stroke and its relation to depressive symptoms and disease characteristics. Eur Neurol. 2001;45:28–33
  11. Repping-Wuts H, Fransen J, van Achterberg T, Bleijenberg G, van Riel P. Persistent severe fatigue in patients with rheumatoid arthritis. J Clin Nurs. 2007;16:377–383
  12. Servaes P, Verhagen CA, Bleijenberg G. Relations between fatigue, neuropsychological functioning, and physical activity after treatment for breast carcinoma: daily self-report and objective behavior. Cancer. 2002;95:2017–2026
  13. Kalkman JS, Schillings ML, Zwarts MJ, van Engelen BG, Bleijenberg G. The development of a model of fatigue in neuromuscular disorders: a longitudinal study. J Psychosom Res. 2007;62:571–579
  14. Voermans NC, van Alfen N, Pillen S, Lammens M, Schalkwijk J, Zwarts MJ, et al. Neuromuscular involvement in various types of Ehlers-Danlos syndrome. Ann Neurol. 2009;65:687–697
  15. Scheeres K, Wensing M, Bleijenberg G, Severens JL. Implementing cognitive behavior therapy for chronic fatigue syndrome in mental health care: a costs and outcomes analysis. BMC Health Serv Res. 2008;8:175
  16. Vercoulen JH, Hommes OR, Swanink CM, Jongen PJ, Fennis JF, Galama JM, et al. The measurement of fatigue in patients with multiple sclerosis (A multidimensional comparison with patients with chronic fatigue syndrome and healthy subjects). Arch Neurol. 1996;53:642–649
  17. Berger F, Brahler E, Kunkel R, Stephanos S. [Verbal behavior and communication experience of psychosomatic patients in the first psychoanalytic interview in connection with the concept of “pensee operatoire”]. Z Psychosom Med Psychoanal. 1981;27:45–58
  18. Jacobs HM, Luttik A, Touw-Otten FW, de Melker RA. [The sickness impact profile; results of an evaluation study of the Dutch version]. Ned Tijdschr Geneeskd. 1990;134:1950–1954
  19. Pfeiffer G, Wicklein EM, Ratusinski T, Schmitt L, Kunze K. Disability and quality of life in Charcot-Marie-Tooth disease type 1. J Neurol Neurosurg Psychiatry. 2001;70:548–550
  20. Ware JE, Snow KK, Kosinski M. SF-36 Health Survey manual and interpretation guide. Boston (MA): New England Medical Center, The Health Institute; 2008;
  21. Stewart JW, Quitkin FM, McGrath PJ, Rabkin JG, Markowitz JS, Tricamo E, et al. Social functioning in chronic depression: effect of 6 weeks of antidepressant treatment. Psychiatry Res. 1988;25:213–222
  22. Beck AT, Guth D, Steer RA, Ball R. Screening for major depression disorders in medical inpatients with the Beck Depression Inventory for Primary Care. Behav Res Ther. 1997;35:785–791
  23. Arrindell WA, Ettema JHM. SCL-90-R: Handleiding bij een multidimensionele psychopathologie-indicator. Lisse, Swets & Zeitlinger; 1-1-1986;
  24. Prins JB, Bleijenberg G, Bazelmans E, Elving LD, de Boo TM, Severens JL, et al. Cognitive behaviour therapy for chronic fatigue syndrome: a multicentre randomised controlled trial. Lancet. 2001;357:841–847
  25. Melzack R. The McGill Pain Questionnaire: major properties and scoring methods. Pain. 1975;1:277–299
  26. Vercoulen JH, Alberts M, Bleijenberg G. De Checklist Individual Strength (CIS). Gedragstherapie [behaviour therapy]. 1999;32:131–136
  27. Sacheti A, Szemere J, Bernstein B, Tafas T, Schechter N, Tsipouras P. Chronic pain is a manifestation of the Ehlers-Danlos syndrome. J Pain Symptom Manage. 1997;14:88–93
  28. Naughton F, Ashworth P, Skevington SM. Does sleep quality predict pain-related disability in chronic pain patients? (The mediating roles of depression and pain severity). Pain. 2007;127:243–252
  29. Verbraecken J, Declerck A, van de Heyning P, de Backer W, Wouters EF. Evaluation for sleep apnea in patients with Ehlers-Danlos syndrome and Marfan: a questionnaire study. Clin Genet. 2001;60:360–365
  30. Prins JB, Bos E, Huibers MJ, Servaes P, van der Werf SP, van der Meer JW, et al. Social support and the persistence of complaints in chronic fatigue syndrome. Psychother Psychosom. 2004;73:174–182
  31. Prins JB, van der Meer JW, Bleijenberg G. Chronic fatigue syndrome. Lancet. 2006;367:346–355
  32. Gielissen MF, Verhagen S, Witjes F, Bleijenberg G. Effects of cognitive behavior therapy in severely fatigued disease-free cancer patients compared with patients waiting for cognitive behavior therapy: a randomized controlled trial. J Clin Oncol. 2006;24:4882–4887
  33. Milne HM, Wallman KE, Gordon S, Courneya KS. Effects of a combined aerobic and resistance exercise program in breast cancer survivors: a randomized controlled trial. Breast Cancer Res Treat. 2008;108:279–288

 N.C. Voermans was supported by a research grant of the NWO (Netherlands Organization for Scientific Research). The NWO had no influence on the design or outcome of the study; hence, N.C. Voermans was independent of this sponsor. None of the authors have any conflicts of interest to disclose. All authors of the article had access to the original data, have fulfilled the criteria for authorship, and have read and approved the final version of the article.

PII: S0049-0172(09)00098-5

doi: 10.1016/j.semarthrit.2009.08.003

Seminars in Arthritis and Rheumatism
Volume 40, Issue 3 , Pages 267-274 , December 2010

Article Tools