Clinical Features of Hypermobility: Locomotor System and Extra-articular

  • Peter Beighton
  • Rodney Grahame
  • Howard Bird


The majority of people with lax ligaments and loose joints probably suffer no articular problems. For example, symptoms were no more prevalent amongst a group of 31 healthy hypermobile blood donors (average age 28 years) than in age- and sex-matched controls (Jessee et al. 1980). For most hypermobile subjects the impression is that it is a positive attribute, which enables enhanced participation in a wide variety of physical activities (see Chapter 8). However, not all are so fortunate, and some do experience locomotor and other problems as a direct effect of their laxity. Joint hypermobility is a feature common to most of the heritable disorders of connective tissue (HDCT), which include the Ehlers-Danlos and Marfan syndromes (EDS; MFS) and osteogenesis imperfecta (01) (see Chapter 9). It is seen in its grossest form in the EDS. Generally speaking the likelihood of developing symptoms, notably arthralgia and joint instability, is directly proportional to the degree of hypermobility, although exceptions to this rule occur. When symptoms occur in seemingly otherwise healthy individuals the term hypermobility syndrome (HMS) is applied (Kirk et al. 1967). In recent years, however, its usage has been increasingly replaced by the preferred term “benign joint hypermobility syndrome” (BJHS) because of the favourable prognosis, at least, as far as threat to life is concerned. Some authorities refer to BJHS as the familial articular hypermobilty; others to Ehlers-Danlos Syndrome Type III (EDS III), although this remains a contentious issue.


Mitral Valve Prolapse Joint Laxity Joint Hypermobility Ligamentous Laxity Locomotor System 
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  1. Acasuso-Diaz M, Collantes-Estevez E (1998) Joint hypermobility in patients with fibromyalgia syndrome. Arthritis Care & Res 11(1): 39–42CrossRefGoogle Scholar
  2. Adair SM, Hecht C (1993) Association of generalized joint hypermobility with history, signs, and symptoms of temporomandibular joint dysfunction in children. Paediatr Dent 15(5): 323–326Google Scholar
  3. Al-Rawi Z, Nessan AH (1997) Joint hypermobility in patients with chondromalacia patellae. Brit J Rheumatol 36(12): 1324–1327CrossRefGoogle Scholar
  4. Al-Rawi ZS, Al-Rawi ZT (1982) Joint hypermobility in women with genital prolapse. Lancet i:1439–1441CrossRefGoogle Scholar
  5. Al-Rawi ZS, Al-Aszawi AJ, Al-Chalabi T (1985) Joint mobility among university students in Iraq. Brit J Rheumatol 24: 326–331.CrossRefGoogle Scholar
  6. Ansell BM (1972) Hypermobility of joints. Mod Trends Orthop 6: 419–425Google Scholar
  7. Barabas AP (1966) Ehlers-Danlos syndrome: associated with prematurity premature rupture of foetal membranes; possible increase in incidence. Brit Med J 5515: 682–684.CrossRefGoogle Scholar
  8. Beighton P, Horan F (1969) Orthopaedic aspects of the Ehlers-Danlos syndrome. J Bone Joint Surg (Br) 51: 414–449Google Scholar
  9. Beighton P, Solomon L, Soskolne CL (1973) Articular mobility in an African population. Ann Rheum Dis 32: 413–418PubMedCrossRefGoogle Scholar
  10. Binns M (1988) Joint laxity in idiopathic adolescent scoliosis. J Bone Joint Surg (Br) 70: 420–422Google Scholar
  11. Bird HA, Wright V (1978) Joint hypermobility mimicking periarticular juvenile chronic arthritis. Br Med J 3: 4023–4024Google Scholar
  12. Bird HA, Calguneri M, Wright V (1981) Changes in joint laxity occurring during pregnancy. Ann Rheum Dis 40: 209–214CrossRefGoogle Scholar
  13. Biro F, Gewanter L, Baum L (1983) The hypermobility syndrome. Pediatrics 72: 701–706PubMedGoogle Scholar
  14. Birrell FN, Adebajo AO, Hazleman BL, Silman AJ (1994) High prevalence of joint laxity in West Africans. Brit J Rheum 33: 56–59CrossRefGoogle Scholar
  15. Bluestone R, Bywaters EG, Hartog M, Holt PJL, Hide S (1971) Acromegalic arthropathy. Ann Rheum Dis 30: 243–258PubMedCrossRefGoogle Scholar
  16. Brandt KD, Summer RD, Ryan TG, Cohen AS (1975) Herniation of mitral valve leaflet in the Ehlers-Danlos syndrome. Am J Cardiol 36: 524–528PubMedCrossRefGoogle Scholar
  17. Brear SG, Beton D, Slaven YM, Honeybourne D (1984). Spontaneous pneumothoraces are associated with mitral valve prolapse. Thorax 39: 219Google Scholar
  18. Bridges AJ, Smith E, Reid J (1992) Joint hypermobility in adults referred to rheumatology clinics. Ann Rheum Dis 51:793–796PubMedCrossRefGoogle Scholar
  19. Brown OR, Demots H, Kloster JE, Roberts A, Menasche VD, Beals RK (1975) Aortic root dilatation and mitral valve prolapse in Marfan’s syndrome. Circulation 52: 651–657PubMedCrossRefGoogle Scholar
  20. Buckingham RB, Braun T, Harinstein DA et al. (1992) Temporomandibular joint dysfunction syndrome: a close association with systemic joint laxity (the hypermobile joint syndrome). Oral Surg Oral Med Oral Path 72(5): 514–519CrossRefGoogle Scholar
  21. Bulbena A, Duro JC, Porta M, Martin-Santos R, Mateo A, Molina et al.(1993) Anxiety disorders in the joint hypermobility syndrome. Psychiatr Res 46(1):59–68CrossRefGoogle Scholar
  22. Cabeen WR, Reza MF, Kovick RB, Stern MS (1977) Mitral valve prolapse and conduction defects in Ehlers-Danlos syndrome. Arch Intern Med 137: 1227–1231PubMedCrossRefGoogle Scholar
  23. Callegarini U (1957) Clinical study on the hyperextensibility of fingers in rheumatoid children. Bull St Francis Hosp NY 14: 32–36Google Scholar
  24. Carlsson C, Rundgren A (1980) Hypermobility of the joints in woman alcoholics, J Stud Alcohol 41: 78–81PubMedGoogle Scholar
  25. Carter C, Wilkinson L (1964) Persistent joint laxity and congenital dislocation of the hip. J Bone Joint Surg(Br) 46: 40–45Google Scholar
  26. Child AH (1986) Joint hypermobility syndrome: inherited disorder of collagen synthesis. J Rheumatol 13:239–242PubMedGoogle Scholar
  27. Davidovitch M, Tirosh E, Tal Y (1994) The relationship between joint hypermobility and neurodevel-opmental attributes in elementary school children. J Child Neurol 9(4): 417–419PubMedCrossRefGoogle Scholar
  28. Diaz MA, Estevez EC, Sanchez Guijo P (1993) Joint hyperlaxity and musculoligamentous lesions: study of a population of homogeneous age, sex and physical exertion. Br J Rheumatol 32(2):120–122CrossRefGoogle Scholar
  29. Dijkstra PU, de Bont LG, de Leeuw R, Stregenga B, Boering G (1993) Temporomandibular joint osteoarthrosis and temporomandibular joint hypermobility. Cranio 11(4): 268–275PubMedGoogle Scholar
  30. Dolan L, Arden N, Spector R, Grahame R. Assessment of bone in Ehlers-Danlos syndrome by ultrasound and densitometry. Ann Rheum Dis (1998) 57(10): 630–633PubMedCrossRefGoogle Scholar
  31. Dubs L, Gschwend N (1988) General joint laxity. Quantification and clinical relevance. Arch Orthop Trauma Surg 107: 65–72PubMedCrossRefGoogle Scholar
  32. Fairbank JCT, Pynsent PB, Van Poortvliet JA, Phillips H (1984) Influence of anthropometric factors and joint laxity in the incidence of adolescent back pain. Spine 9: 461–464PubMedCrossRefGoogle Scholar
  33. Floyd A, Phillips P, Khan MRH, Webb JN, Mclnnes A, Hughes SPF (1987) Recurrent dislocation of the patella histochemical and electromyographical evidence of primary muscle pathology. J Bone Joint Surg (Br) 69: 790–793Google Scholar
  34. Forleo LHA. Hilário MOE, Peixoto AL, Naspitz CK, Goldenberg J (1994) Articular hypermobility in school children in Sao Paulo, Brazil. J Rheumatol 20: 916Google Scholar
  35. Francis H, March LM, Terenty T, Webb J (1987) Benign joint hypermobility with neuropathies: (II) documentation and mechanism of tarsal tunnel syndrome. J Rheumatol 14: 577–581PubMedGoogle Scholar
  36. Gage JP (1985) Collagen biosynthesis related to temporomandibular joint clicking in childhood. J Prosthet Dent 53: 714–717PubMedCrossRefGoogle Scholar
  37. Gedalia A, Press J (1991) Articular symptoms in hypermobile schoolchildren: a prospective study. J Paediatr 119: 944–946CrossRefGoogle Scholar
  38. Gedalia A, Person DA, Brewer EJ, Giannini EH (1985) Juvenile episodic arthralgia and hypermobility. J Pediatr 107: 873–876PubMedCrossRefGoogle Scholar
  39. Gedalia A, Press J, Klein M, Buskila D (1993) Joint hypermobility and fibromyalgia in schoolchildren. Ann Rheum Dis. 52(7): 494–496PubMedCrossRefGoogle Scholar
  40. Goodman JA. (1991) Hypermobility and deconditioning: important links to fibromyalgia/fibrositis. South Med J 84(10): 1192–1196CrossRefGoogle Scholar
  41. Grahame R (1971) Joint hypermobility — clinical aspects. Proc Roy Soc Med 64: 692–694PubMedGoogle Scholar
  42. Grahame R (1986) Clinical manifestations of the joint hypermobility syndrome. Revmatologia (USSR) 2: 2024Google Scholar
  43. Grahame R. (1996) Hypermobility syndrome in the elderly. In Rovensky J (ed) Proceedings of the 1st Central European Congress of Rheumatology. Piestany, SlovakiaGoogle Scholar
  44. Grahame R. Hypermobility and genetic collagen disorders — are they related? Arch Dis Child 1999: 80(2): 189–191CrossRefGoogle Scholar
  45. Grahame R, Harvey W (1974) Defect of collagen in growth hormone disorders? Lancet ii 1332Google Scholar
  46. Grahame R, Pyeritz R. (1995) Marfan Syndrome: joint and skin manifestations are prevalent and correlated. Br J Rheum 34(2): 126–131CrossRefGoogle Scholar
  47. Grahame R, Mitchell ABS, Scott JT (1970) Chronic post-rheumatic fever (Jaccoud’s) arthropathy. Ann Rheum Dis 29: 622–625PubMedCrossRefGoogle Scholar
  48. Grahame R, Edwards JC, Pitcher D, Gabell A, Harvey W (1981) A clinical and echocardiographic study of patients with the hypermobility syndrome. Ann Rheum Dis 40: 541–546PubMedCrossRefGoogle Scholar
  49. Hagroo GA, Zaki HM, Choudhary MT, Hussain A (1995) Pulled elbow — not the effect of hypermobility of joints. Injury 26(10): 687–690PubMedCrossRefGoogle Scholar
  50. Handler CE, Child A, Light ND, Dorance DE (1985) Mitral valve prolapse, aortic compliance and skin collagen in joint hypermobility syndrome. Br Heart J 54: 501–508PubMedCrossRefGoogle Scholar
  51. Harinstein D, Buckingham RB, Braun T, Oral K, Bauman DH, Killian PJ, Bidula LP (1988) Systemic joint laxity (the hypermobile joint syndrome) is associated with temporomandibular joint dysfunction. Arthritis Rheum 31: 1259–1264PubMedCrossRefGoogle Scholar
  52. Hordon LD, Bird HA (1988) Joint laxity and ankylosing spondylitis. Br J Rheumatol 27: 241–242PubMedCrossRefGoogle Scholar
  53. Howes RJ, Isdale IC (1971). The loose back: an unrecognised syndrome. Rheumatol Phys Med 11: 72–77PubMedCrossRefGoogle Scholar
  54. Hudson N, Starr MR, Esdaile JM, Fitzcharles M-A (1995) Diagnostic associations with hypermobility in rheumatology patients. Br J Rheumatol 34(12): 1157–1161PubMedCrossRefGoogle Scholar
  55. Jessee EF, Owen DS, Sagar KB (1980) The benign hypermobile joint syndrome. Arthritis Rheum 23: 1053–1056PubMedCrossRefGoogle Scholar
  56. Jonsson H, Valtysdottir ST, Kjartansson O, Brekkan A (1996) Hypermobility associated with osteoarthritis of the thumb base: a clinical and radiological subset of hand osteoarthritis. Ann Rheum Dis 55(8): 540–543PubMedCrossRefGoogle Scholar
  57. Jonsson H, Valtysdottir ST (1995) Hypermobility features in patients with hand osteoarthritis. Osteoarthritis Cartilage 3(1): 1–5PubMedCrossRefGoogle Scholar
  58. Kaplinsky C, Kenet G, Seligsohn U, Rechavi G (1998) Association between hyperflexibility of the thumb and an unexpected bleeding tendency: is it a rule of thumb? Br J Haematol 101(2): 260–263PubMedCrossRefGoogle Scholar
  59. Kirk JA, Ansell BM, Bywaters FGL (1967) The hypermobility syndrome. Ann Rheum Dis 26: 419–425PubMedCrossRefGoogle Scholar
  60. Labowitz R, Schumacher HR (1971) Articular manifestations of systemic lupus erythematosus. Ann Intern Med 74: 911–921PubMedGoogle Scholar
  61. Larsson L-G, Baum J, Mudholkar GS (1987) Hypermobility: features and differential incidence between the sexes. Arthritis Rheum. 30:1426–1430PubMedCrossRefGoogle Scholar
  62. Lewkonia RM (1986) Does generalised articular hypermobility predispose to generalised osteoarthritis? Clin Exp Rheumatol 4: 115–119PubMedGoogle Scholar
  63. MacLennan AH (1991) The role of the hormone relaxin in human reproduction and pelvic girdle relaxation. Scand J Rheumatol (Suppl) 88: 7–15Google Scholar
  64. March L, Silman A (1993) Joint hyperlaxity: is there a case for screening? Br J Rheumatol 32(2): 91–92PubMedCrossRefGoogle Scholar
  65. March LM, Francis H, Webb J (1988) Benign joint hypermobility with neuropathies: documentation and mechanism of median, sciatic, and common peroneal nerve compression. Clin Rheumatol 7(1): 35–40PubMedCrossRefGoogle Scholar
  66. Marks JS, Sharp J, Brear SG, Edwards JD (1983) Normal joint mobility in mitral valve prolapse. Ann Rheum Dis 42: 54–55PubMedCrossRefGoogle Scholar
  67. Marshman D, Percy J, Fielding I, Delbridge L (1987).Rectal prolapse: relationship with joint mobility. Aust NZ J Surg 57: 827–829CrossRefGoogle Scholar
  68. McClennan AH, Nicolson R, Green RC, Bath MC (1986) Serum relaxin and pelvic pain of pregnancy. Lancet ii: 243–245CrossRefGoogle Scholar
  69. Mcintosh LJ, Mallett VT, Frahm JD, Richardson DA, Evans ML (1995) Gynaecologic disorders in women with Ehlers-Danlos syndrome. J Soc Gynecol Invest. 2(3): 559–564CrossRefGoogle Scholar
  70. Mikkelsson M, Salminen JJ, Kautiainen H (1996) Joint hypermobility is not a contributing factor to musculoskeletal pain in pre-adolescents. J Rheumatol 23(11): 1963–1967PubMedGoogle Scholar
  71. Mishra MB, Ryan P, Atkinson P et al. (1996) Extra-articular features of the benign joint hypermobility syndrome. Br J Rheumatol 35: 861–866PubMedCrossRefGoogle Scholar
  72. Morgan AW, Gibbon W, Bird HA (1996a) A controlled study of spinal laxity in subjects with joint hyperlaxity and Ehlers-Danlos syndrome. Br J Rheumatol 36 Abstract supplement 1:58Google Scholar
  73. Morgan AW, Pearson SB, Bird HA. (1996b) Respiratory symptoms in Ehlers-Danlos syndrome and the benign joint hypermobility syndrome. Arth Rheum 39(9): S136Google Scholar
  74. Nemec V, Svadlenkova D (1996) Hypermobile syndrome in childhood. Prakticky Lekar 76(12): 593–595Google Scholar
  75. Norton PA, Baker JE, Sharp HC, Warenski JC, Norton PA (1995) Genitourinary prolapse and joint hypermobility in women. Obs Gynecol 85(2): 225–228CrossRefGoogle Scholar
  76. Ondrasik M, Rybar I, Rus V, Bosak V (1988) Joint hypermobility in mitral valve prolapse patients. Clin Rheumatol 7: 69–73PubMedCrossRefGoogle Scholar
  77. Perrini F, Tallenets RH, Katzberg RX, Ribeiro RF, Kirkanides S, Moss ME (1997) Generalised joint laxity and temporomandibular disorders. J Orofacial Pain 11(3): 215–221PubMedGoogle Scholar
  78. Persellin RH, Rutstein RE (1979) In McCarty DJ (ed) Rheumatic aspects of endocrinopathies in arthritis and allied conditions, 9th edn. Lea and Febiger, Philadelphia, p 1326Google Scholar
  79. Pitcher D, Grahame R (1982) Mitral valve prolapse and joint hypermobility: evidence of systemic connective tissue abnormality. Ann Rheum Dis 41: 352–354PubMedCrossRefGoogle Scholar
  80. Preston FN, Adicoff A (1962) Hyperparathyroidism with avulsion of 3 major tendons. New Engl J Med 266: 968–971PubMedCrossRefGoogle Scholar
  81. Propock WA, Barlow JB (1971) Aetiology and electrocardiographic features of the following posterior mitral leaflet syndrome: analysis of a further 130 patients with a later systolic murmur or non-ejection systolic click. Am J Med 51: 73–78Google Scholar
  82. Rillo OL, Babini SM, Basnak A, Wainer E, Balbachan E, Cocco JAM (1991) Tendinous and ligamentous hyperlaxity in patients receiving long-term hemodialysis. J Rheumatol 18(8): 1227–1231PubMedGoogle Scholar
  83. Runow A (1983) The dislocating patella. Etiology and prognosis in relation to joint laxity and anatomy of patella articulation. Acta Orthop Scand Suppl 202: 1–53Google Scholar
  84. Scharf Y, Nahir AM (1982) Hypermobility syndrome mimicking juvenile chronic arthritis. Rheumatol Rehabil 21:78–0PubMedCrossRefGoogle Scholar
  85. Schauberger CW, Rooney BL, Goldsmith L, Shenton D, Silva PD, Schaper A (1996) Peripheral joint laxity increases in pregnancy but does not correlate with serum relaxin levels. Am J Obstet Gynecol 174(2): 667–671PubMedCrossRefGoogle Scholar
  86. Scott D, Bird HA, Wright V (1979) Joint laxity leading to osteoarthrosis. Rheumatol Rehabil 18:167–169PubMedCrossRefGoogle Scholar
  87. Shore A, Macauley D, Ansell BM (1981) Idiopathic protrusio acetabulae in juveniles. Rheumatol Rehabil 20:1–10PubMedCrossRefGoogle Scholar
  88. Southwood TR, Sills JA (1993) Non-arthritic locomotor disorders in childhood. Practical problems No 24. Reports on Rheumatic Diseases (Series 2) Arthritis and Rheumatism Council, Chesterfield.Google Scholar
  89. Stern BD, Gincher, MJ, Mechanic GL (1965) Studies of collagen degradation during bone resorption in tissue culture. Proc Soc Exp Biol Med 119: 577–583PubMedGoogle Scholar
  90. Thornton JG, Hill J, Bird HA (1988) Complications of pregnancy and benign familial joint hyperlaxity. Ann Rheum Dis 47: 228–231PubMedCrossRefGoogle Scholar
  91. Tirosh E, Jaffe M, Marmur R, Taub Y, Rosenberg Z (1991) Prognosis of motor development and joint hypermobility. Arch Dis Child 66(8): 931–933PubMedCrossRefGoogle Scholar
  92. Walker HL, Schreck RC (1978) Relationship of hyperextended gait pattern to chondromalacia patellae. Physiotherapy 1: 8–9Google Scholar
  93. Westling L, Mattiason A (1992) General joint hypermobility and temporomandibular joint derangement in adolescents. Ann Rheum Dis 51: 87–90PubMedCrossRefGoogle Scholar
  94. Westling L (1992) Temporomandibular joint dysfunction and systemic joint laxity. Swedish Dent J Supplement 81, GoteburgGoogle Scholar
  95. Wood PHN (1971) Is hypermobility a discrete entity? Proc Roy Soc Med 1971; 64: 690–692PubMedGoogle Scholar
  96. Woods SJ, Themes J, Brambridge NV (1973) Mitral valve disease and open heart surgery in osteogenesis imperfecta tarda. Br Heart J 35: 103–106CrossRefGoogle Scholar
  97. Woodward EG, Morris MT (1990) Joint hypermobility in keratoconus. Ophthal Physiol Opt. 10: 360–362CrossRefGoogle Scholar
  98. Wynne-Davies R (1971) Familial joint laxity. Proc R Soc Med 64: 689–690PubMedGoogle Scholar
  99. Zarrow M, Holstrom EG, Salhanick HA (1955) The concentration of relaxin in the blood serum and other tissues of women during pregnancy. J Clin Endocrinol 15: 22–27CrossRefGoogle Scholar

Copyright information

© Springer-Verlag London 1999

Authors and Affiliations

  • Peter Beighton
    • 1
  • Rodney Grahame
    • 2
  • Howard Bird
    • 3
  1. 1.Department of Human GeneticsUniversity of Cape Town Medical SchoolCape TownSouth Africa
  2. 2.Hypermobility ClinicUCL HospitalsLondonUK
  3. 3.Clinical Pharmacology UnitUniversity of Leeds, Chapel Allerton HospitalLeeds, West YorkshireUK

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