Clinical Rheumatology

, Volume 29, Issue 3, pp 289–295 | Cite as

Joint position sense and vibratory perception sense in patients with Ehlers–Danlos syndrome type III (hypermobility type)

  • Lies Rombaut
  • Anne De Paepe
  • Fransiska Malfait
  • Ann Cools
  • Patrick Calders
Original Article

Abstract

Neurophysiological deficits could make patients with Ehlers–Danlos syndrome (EDS) type III (hypermobility type) more vulnerable to musculoskeletal problems, particularly to joint instability. The purpose of this study was to investigate whether joint position sense (JPS) and vibratory perception sense (VPS) in EDS type III patients in the knee and shoulder joints are impaired. Thirty-two female EDS type III patients as defined by the Villefranche criteria and 32 individually gender- and age-matched healthy control subjects were included in the study. Range of motion was determined using a goniometer, passive and active JPS were assessed with an isokinetic dynamometer system, and the VPS was measured by a biothesiometer. Daily physical activity was evaluated by the Baecke questionnaire. The EDS type III group showed significantly larger ranges of movement (P < 0.05) and lower levels of sport physical activity (SPA) compared to the control group (P = 0.023). Considering SPA as covariate, the EDS type III group demonstrated a significant impairment in knee joint reposition compared to the control group (P = 0.018). No significant differences were found for shoulder JPS. The VPS was not significantly different in the EDS type III group compared to the control group. In addition, no significant correlation was found between JPS and VPS, neither at the knee nor at the shoulder joint. This is the first study examining proprioception deficits in EDS type III patients as defined by the Villefranche criteria. Further research on the neurophysiological dysfunctions and mechanisms in this pathologic entity is needed.

Keywords

Connective tissue disorder Ehlers–Danlos syndrome Joint hypermobility Joint position sense Proprioception Vibratory perception sense 

References

  1. 1.
    Beighton P, De Paepe A, Danks D, Finidori G, Gedde-Dahl T, Goodman R et al (1988) International nosology of heritable disorders of connective tissue, Berlin, 1986. Am J Med Genet 29:581–594CrossRefPubMedGoogle Scholar
  2. 2.
    Ainsworth SR, Aulicino PL (1993) A survey of patients with Ehlers–Danlos syndrome. Clin Orthop Relat Res 286:250–256PubMedGoogle Scholar
  3. 3.
    Steinmann B, Royce PM, Superti-Furga A (1993) The Ehlers–Danlos syndrome. In: Royce P, Steinmann B (eds) Connective tissue and its heritable disorders: molecular, genetic and medical aspects. Wiley-Liss, New York, pp 351–407Google Scholar
  4. 4.
    Grahame R (2000) Heritable disorders of connective tissue. Best Pract Res Clin Rheumatol 14:345–361CrossRefGoogle Scholar
  5. 5.
    Beighton P (1970) Ehlers–Danlos syndrome. Ann Rheum Dis 29:332–333CrossRefPubMedGoogle Scholar
  6. 6.
    Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup RJ (1998) Ehlers–Danlos syndromes: revised nosology, Villefranche. Am J Med Genet 77:31–37CrossRefPubMedGoogle Scholar
  7. 7.
    Beighton P, De Paepe A, Hall JG, Hollister DW, Pope FM, Pyeritz RE et al (1992) Molecular nosology of heritable disorders of connective tissue. Am J Med Genet 42:431–448CrossRefPubMedGoogle Scholar
  8. 8.
    Stanitski DF, Nadjarian R, Stanitski CL, Bawle E, Tsipouras P (2000) Orthopaedic manifestations of Ehlers–Danlos syndrome. Clin Orthop Relat Res 376:213–221CrossRefPubMedGoogle Scholar
  9. 9.
    Riemann BL, Lephart SM (2002) The sensorimotor system, part I: the physiologic basis of functional joint stability. J Athl Train 37:71–79PubMedGoogle Scholar
  10. 10.
    Riemann BL, Lephart SM (2002) The sensorimotor system, part II: the role of proprioception in motor control and functional joint stability. J Athl Train 37:80–84PubMedGoogle Scholar
  11. 11.
    Lephart SM, Riemann BL, Fu FH (2000) Introduction to the sensorimotor system. In: Lephart SM, Fu FH (eds) Proprioception and neuromuscular control in joint stability. Human Kinetics, Champaign, pp 67–51Google Scholar
  12. 12.
    Mallik AK, Ferrell WR, McDonald AG, Sturrock RD (1994) Impaired proprioceptive acuity at the proximal interphalangeal joint in patients with the hypermobility syndrome. Br J Rheumatol 33:631–637CrossRefPubMedGoogle Scholar
  13. 13.
    Hall MG, Ferrell WR, Sturrock RD, Hamblen DL, Baxendale RH (1995) The effect of the hypermobility syndrome on knee joint proprioception. Br J Rheumatol 34:121–125CrossRefPubMedGoogle Scholar
  14. 14.
    Sahin N, Baskent A, Cakmak A, Salli A, Ugurlu H, Berker E (2008) Evaluation of knee proprioception and effects of proprioception exercise in patients with benign joint hypermobility syndrome. Rheumatol Int 28:995–1000CrossRefPubMedGoogle Scholar
  15. 15.
    Grahame R (1999) Joint hypermobility and genetic collagen disorders: are they related? Arch Dis Child 80:188–191CrossRefPubMedGoogle Scholar
  16. 16.
    Gilman S (2002) Joint position sense and vibration sense: anatomical organisation and assessment. J Neurol Neurosurg Psychiatry 73:473–477CrossRefPubMedGoogle Scholar
  17. 17.
    Rowin J, Meriggioli MN (2007) Propioception, touch, and vibratory sensation. In: Goetz CG (ed) Textbook of clinical neurology, part I. Saunders, PhiladelphiaGoogle Scholar
  18. 18.
    Johansson H, Sjolander P (1993) The neurophysiology of joints. In: Wright V, Radin EL (eds) Mechanics of joints: physiology, pathophysiology and treatment. Dekker, New York, pp 243–290Google Scholar
  19. 19.
    Dover G, Powers ME (2003) Reliability of joint position sense and force-reproduction measures during internal and external rotation of the shoulder. J Athl Train 38:304–310PubMedGoogle Scholar
  20. 20.
    Brindle TJ, Mizelle JC, Lebiedowska MK, Miller JL, Stanhope SJ (2009) Visual and proprioceptive feedback improves knee joint position sense. Knee Surg Sports Traumatol 17:40–47CrossRefGoogle Scholar
  21. 21.
    Baecke JA, Burema J, Frijters JE (1982) A short questionnaire for the measurement of habitual physical activity in epidemiological studies. Am J Clin Nutr 36:936–942PubMedGoogle Scholar
  22. 22.
    Beighton P, Solomon L, Soskolne CL (1973) Articular mobility in an African population. Ann Rheum Dis 32:413–418CrossRefPubMedGoogle Scholar
  23. 23.
    Watkins MA, Riddle DL, Lamb RL, Personius WJ (1991) Reliability of goniometric measurements and visual estimates of knee range of motion obtained in a clinical setting. Phys Ther 71:90–96PubMedGoogle Scholar
  24. 24.
    MacDermid JC, Chesworth BM, Patterson S, Roth JH (1999) Intratester and intertester reliability of goniometric measurement of passive lateral shoulder rotation. J Hand Ther 12:187–192PubMedGoogle Scholar
  25. 25.
    Van Meeteren J, Roebroeck ME, Stam HJ (2002) Test–retest reliability in isokinetic muscle strength measurements of the shoulder. J Rehabil Med 34:91–95CrossRefPubMedGoogle Scholar
  26. 26.
    Drouin JM, Valovich-McLeod TC, Shultz SJ, Gansneder BM, Perrin DH (2004) Reliability and validity of the Biodex System® 3 pro isokinetic dynamometer velocity, torque and position measurements. Eur J Appl Physiol 91:22–29CrossRefPubMedGoogle Scholar
  27. 27.
    Frenette B, Mergler D, Ferraris J (1990) Measurement precision of a portable instrument to assess vibrotactile perception threshold. Euro J App Physiol Occup Physiol 61:386–391CrossRefGoogle Scholar
  28. 28.
    Shakoor N, Agrawal A, Block JA (2008) Reduced lower extremity vibratory perception in osteoarthritis of the knee. Arthritis Rheum 59:117–121CrossRefPubMedGoogle Scholar
  29. 29.
    Philippaerts RM, Lefevre J (1998) Reliability and validity of three physical activity questionnaires in Flemish males. Am J Epidemiol 147:982–990PubMedGoogle Scholar
  30. 30.
    Ferrell WR, Tennant N, Sturrock RD, Ashton L, Creed G, Brydson G et al (2004) Amelioration of symptoms by enhancement of proprioception in patients with joint hypermobility syndrome. Arthritis Rheum 50:3323–3328CrossRefPubMedGoogle Scholar
  31. 31.
    Petrella RJ, Lattanzio PJ, Nelson MG (1997) Effect of age and activity on knee joint proprioception. Am J Phys Med Rehabil 76:235–241CrossRefPubMedGoogle Scholar
  32. 32.
    Lumley MA, Jordan M, Rubenstein R, Tsipouras P, Evans MI (1994) Psychosocial functioning in the Ehlers–Danlos syndrome. Am J Med Genet 53:149–152CrossRefPubMedGoogle Scholar
  33. 33.
    Sacheti A, Szemere J, Bernstein B, Tafas T, Schechter N, Tsipouras P (1997) Chronic pain is a manifestation of the Ehlers–Danlos syndrome. J Pain Symptom Manage 14:88–93CrossRefPubMedGoogle Scholar
  34. 34.
    Janwantanakul P, Magarey ME, Jones MA, Dansie BR (2002) Variation in shoulder position sense at mid and extreme range of motion. Arch Phys Med Rehabil 82:840–844CrossRefGoogle Scholar
  35. 35.
    Smith RJ, Brunolli J (1989) Shoulder kinaesthesia after anterior glenohumeral joint dislocation. Phys Ther 69:106–112PubMedGoogle Scholar
  36. 36.
    Blasier RB, Carpenter JE, Huston LJ (1994) Shoulder proprioception. Effect of joint laxity, joint position, and direction of motion. Orthop Rev 23:45–51PubMedGoogle Scholar
  37. 37.
    Fríden T, Roberts D, Zatterstrom R, Lindstrand A, Moritz U (1996) Proprioception in the nearly extended knee: measurements of position and movement in healthy individuals and in symptomatic anterior cruciate ligament injured patients. Knee Surg Sports Traumatol Artrosc 4:217–224CrossRefGoogle Scholar
  38. 38.
    Berglund B, Nordstrom G (2001) Symptoms and functional health status of individuals with Ehlers–Danlos syndrome (EDS). J Clin Rheumatol 7:308–314CrossRefPubMedGoogle Scholar

Copyright information

© Clinical Rheumatology 2009

Authors and Affiliations

  • Lies Rombaut
    • 1
  • Anne De Paepe
    • 2
  • Fransiska Malfait
    • 2
  • Ann Cools
    • 3
  • Patrick Calders
    • 1
  1. 1.Department of Rehabilitation Sciences and Physiotherapy, Artevelde University CollegeGhent UniversityGhentBelgium
  2. 2.Centre for Medical GeneticsGhent University HospitalGhentBelgium
  3. 3.Department of Rehabilitation Sciences and Physiotherapy, Faculty of Medicine and Health SciencesGhent UniversityGhentBelgium

Personalised recommendations