JOINT HYPERMOBILITY SYNDROME

19 October 2015

assessments

Diagnose based on:

• Beighton score
• Brighton criteria
• Excluding other (HDCT) conditions

Joint Hypermobility Syndrome

Prevalence of Injury

Signs & symptoms

Beighton score & Brighton criteria

• Reduced physical fitness (48- 67%)
• Motor delay
• Flat feet
• Altered movement strategies.
• Fatigue

• Sprain P= 0,009
• Back pain P=0,032
• stress fractures P=0,012
• Dislocations P=0,013

• (Joint) pain (74%)
• Headache, migraine
• Poor balance
• Poor coordination (36%)
• Reduced joint proprioception

Adib N, Davies K, Grahame R, Woo P, Murray KJ. Joint hypermobility syndrome in childhood. A not so benign multisystem disorder? Rheumatology (Oxford, England). 2005;44(6):744.

Russek LN, Errico DM. Prevalence, injury rate and, symptom frequencyin generalized joint laxity and joint hypermobility syndrome in a 'healthy' college population. Clinical Rheumatology. 2015.

Statistic numbers

Different cut-off points (Delphi study) [a]

• 4/9 used by 46%
• 5/9 used by 46%
• 6/9 used by 8%

Reliability?

Adib N, Davies K, Grahame R, Woo P, Murray KJ. Joint hypermobility syndrome in childhood. A not so benign multisystem disorder? Rheumatology (Oxford, England). 2005;44(6):744.

Cronbach alpha: 0.44 [a]

Sensitivity: 84%

Specificity: 87% [b]

[a] Remvig L, Flycht L, Christensen KB, Juul-Kristensen B (2014) Lack of consensus on tests and criteria for generalized joint hyper- mobility, Ehlers-Danlos syndrome: hypermobile type and joint hypermobility syndrome. Am J Med Genet A 164a(3):591–596.

[b] Hakim AJ, Grahame R: A simple questionnaire to detect hypermobility: an adjunct to the assessment of patients with diffuse muscoskeletal pain. It J clin Pract 2003, 57: 163-166.

Russek LN, Errico DM. Prevalence, injury rate and, symptom frequencyin generalized joint laxity and joint hypermobility syndrome in a 'healthy' college population. Clinical Rheumatology. 2015.

Grahame R. Joint hypermobility syndrome pain. Current Pain and Headache Reports. 2009;13(6):427-33.

HDCT

Heritable disorder of the connective tissues

Samistra, Marfan syndrome, a life threathening rare genetic disorder, http://www.pinkjooz.com/index.php/marfan-syndrome-a-life-threatening-rare-genetic-disorder/

Treatment

• Osteogenesis imperfecta: bone structures and eyes
• Marfan syndrome: Skin, bone and heart
• Ehlers-Danlos syndrome: tendon, ligaments, skin, joints & bloodvessels
• JHS/EDS-HT: clinically indistinguishable

• Child
• Exercise program (30-40% pain reduction) [a]
• Postural control (core and postural muscles)
• Proprioception
• Stability, coordination, balance and strength
• Motor control (handwriting)
• Self management

Assessment period 1

Minor Pediatric physiotherapy

Avans

Colombi M, Dordoni C, Chiarelli N, Ritelli M. 2015. Differential diagnosis anddiagnostic flow chart of joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility typecompared to other heritable connective tissue disorders. Am J Med Genet Part C 169C:6–22.

[a] Adib N, Davies K, Grahame R, Woo P, Murray KJ. Joint hypermobility syndrome in childhood. A not so benign multisystem disorder? Rheumatology (Oxford, England). 2005;44(6):744.

Pacey V, Tofts L, Wesley A, Collins F, Singh‐Grewal D. Joint hypermobility syndrome: A review for clinicians. Journal of Paediatrics and Child Health. 2015;51(4):373-80.

Prevalence

Joint hypermobility syndrome

Prevalence among 267 students [Russek, 2015]:

• 19,5% (24,5% females, 13,7% males)

Prevalence of JHS varies due to

• Inconsistency in cutoff-point Beighton score
• Gender
• ethnicity

Russek LN, Errico DM. Prevalence, injury rate and, symptom frequency in generalized joint laxity and joint hypermobility syndrome in a “healthy” college population. Clinical Rheumatology. 2015.

• Generelized joint hypermobility (GHS): large ROM
• Joint hypermobility syndrome (JHS): impaired QOL.

'commonly overlooked, underdiagnosed, multifaceted and multi-systematic'

Conclusion

Pacey V, Tofts L, Wesley A, Collins F, Singh‐Grewal D. Joint hypermobility syndrome: A review for clinicians. Journal of Paediatrics and Child Health. 2015;51(4):373-80.

• JHS is not the same as GHS
• Underdiagnosed, overlooked and multisystematic
• Chronical condition
• More common in females
• Heritable disorder of the connective tissue (HDCT)
• Assessment: Beighton score & Brighton criteria
• Treatment: Hands-off!

It isn't that they can't see the solution, but that they can't see the problem (GK Chesterton)

Adib N, Davies K, Grahame R, Woo P, Murray KJ. Joint hypermobility syndrome in childhood. A not so benign multisystem disorder? Rheumatology (Oxford, England). 2005;44(6):744.

contents

• Joint hypermobility
• Prevalence
• Classification HDCT's
• Diagnose
• Treatment
• Conclusion
• Workshop

Workshop

how hypermobile are you?

Normal ROM?

Position?

Rachel van de Vorst