Hypermobility Syndrome: Difference between revisions

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<br>The primary symptom is excessive laxity of multiple joints. <ref name="p3" />  
<br>The primary symptom is excessive laxity of multiple joints. <ref name="p3" />  


<br>Hypermobility syndrome is different from localized joint hypermobility and other disorders that have generalized joint hypermobility, such as [[Ehlers-Danlos Syndrome]], [[Rheumatoid Arthritis]], [[Systemic Lupus Erythematosus]], and [[Marfan Syndrome]].<ref name="p3" /> HMS may occur also in chromosomal and genetic disorders such as Down syndrome and in metabolic disorders such as homocystinuria and hyperlysinemia.<ref name="p4">MAJ Michael, R.Simpson. Benign Joint Hypermobility Syndrome: Evaluation, Diagnosis, and Management. J Am Osteopath Assoc. 2006;106:531–536 (Level of Evidence: 2C)</ref> Laboratory tests are used to exclude these other systemic disorders when HMS is suspected.<ref name="p3" /><br><br>  
<br>Hypermobility syndrome is different from localized joint hypermobility and other disorders that have generalized joint hypermobility, such as [[Ehlers-Danlos Syndrome]], [[Rheumatoid Arthritis]], [[Systemic Lupus Erythematosus]], and [[Marfan Syndrome]].<ref name="p3" /> HMS may occur also in chromosomal and genetic disorders such as Down syndrome and in metabolic disorders such as homocystinuria and hyperlysinemia.<ref name="p4"/> Laboratory tests are used to exclude these other systemic disorders when HMS is suspected.<ref name="p3" /><br><br>  


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Joint mobility is highest at birth, there is a decrease in children around nine to twelve years old.<br>  
Joint mobility is highest at birth, there is a decrease in children around nine to twelve years old.<br>  


In adolescent girls there is a peak at the age of fifteen years, after this age the joint mobility decreases, as well in boys as in girls. <ref name="p6">Jansson A. Et al. General joint laxity in 1845 Swedish school children of different ages: age- and gender-specific distributions. Acta Paediatr. 2004;93(9):1202–1206. (Level of Evidence: 2C)</ref>
In adolescent girls there is a peak at the age of fifteen years, after this age the joint mobility decreases, as well in boys as in girls. <ref name="p6"/>


Hormonal changes that occur in puberty by adolescent girls, will influence the joint mobility.<ref name="p6">Jansson A. Et al. General joint laxity in 1845 Swedish school children of different ages: age- and gender-specific distributions. Acta Paediatr. 2004;93(9):1202–1206. (Level of Evidence: 2C)</ref><br>  
Hormonal changes that occur in puberty by adolescent girls, will influence the joint mobility.<ref name="p6">Jansson A. Et al. General joint laxity in 1845 Swedish school children of different ages: age- and gender-specific distributions. Acta Paediatr. 2004;93(9):1202–1206. (Level of Evidence: 2C)</ref><br>  


In general, hypermobility is more common in children than adults, is more common in girls than in boys <ref name="p7">Clinch J. Et al. Epidemiology of Generalized Joint Laxity (Hypermobility) in Fourteen-Year-Old Children From the UK:A Population-Based Evaluation.2011(Level of Evidence: 2B)</ref>, more common in Asian, African and middle eastern people. <ref name="p4">MAJ Michael, R.Simpson. Benign Joint Hypermobility Syndrome: Evaluation, Diagnosis, and Management. J Am Osteopath Assoc. 2006;106:531–536 (Level of Evidence: 2C)</ref><br>
In general, hypermobility is more common in children than adults, is more common in girls than in boys <ref name="p7">Clinch J. Et al. Epidemiology of Generalized Joint Laxity (Hypermobility) in Fourteen-Year-Old Children From the UK:A Population-Based Evaluation.2011(Level of Evidence: 2B)</ref>, more common in Asian, African and middle eastern people. <ref name="p4"/><br>


== Characteristics/Clinical Presentation  ==
== Characteristics/Clinical Presentation  ==


Possible Neuromusculoskeletal Signs for Individuals With Hypermobile Joint Syndrome:<br>• Acute or Traumatic sprains: <br>- recurrent ankle sprains<br>• Meniscus tears<br>• Joint instability<ref name="p3">Castori M. Et al. Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. American Journal of Medical Genetics part A. 2013. (Level of Evidence: 2C)</ref>: Acute or recurrent dislocations or subluxations<ref name="p6">Bravo JF. Et al. Clinical study of hereditary disorders of connective tissues in a Chilean population: joint hypermobility syndrome and vascular Ehlers-Danlos syndrome. Arthritis Rheum. 2006;54:515-23. (Level of Evidence: 4)</ref> of the:<br>- shoulder<br>- patella<br>- meta-carpo-phalangeal joint<br>- temporo-mandibular joint<br>• Traumatic arthritis<ref name="p5">Castori M. Ehlers-Danlos syndrome, Hypermobility type: An underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012. (Level of Evidence: 2C)</ref><br>• Bruising<br>• Fractures: chronic or non-traumatic<br>• Soft tissue rheumatism<br>- tendinitis<ref name="p6">Bravo JF. Et al. Clinical study of hereditary disorders of connective tissues in a Chilean population: joint hypermobility syndrome and vascular Ehlers-Danlos syndrome. Arthritis Rheum. 2006;54:515-23. (Level of Evidence: 4)</ref><br>- epicondylitis<br>- rotator cuff syndrome<br>- synovitis<br>- juvenile episodic synovitis<br>- bursitis<br>• Chondromalacia<br>• Scoliosis<br>• Fibromyalgia<ref name="p5">Castori M. Ehlers-Danlos syndrome, Hypermobility type: An underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012. (Level of Evidence: 2C)</ref><br>• Chronic fatigue syndrome<ref name="p3">Castori M. Et al. Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. American Journal of Medical Genetics part A. 2013. (Level of Evidence: 2C)</ref>,<ref name="p5">Castori M. Ehlers-Danlos syndrome, Hypermobility type: An underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012. (Level of Evidence: 2C)</ref><br>• Nerve compression disorders<br>- carpal tunnel syndrome<br>- tarsal Tunnel Syndrome<br>- Acroparesthesia<br>- thoracic outlet syndrome<br>• Raynaud syndrome<br>• Flat feet and sequelae<br>• Unspecified arthralgia or effusion of affected joint(s)<br>(foot, ankle, knee, hip, back, neck, shoulder, elbow,<br>wrist, finger)<ref name="p6">Bravo JF. Et al. Clinical study of hereditary disorders of connective tissues in a Chilean population: joint hypermobility syndrome and vascular Ehlers-Danlos syndrome. Arthritis Rheum. 2006;54:515-23. (Level of Evidence: 4)</ref><br>• Back pain<ref name="p2">Kemp S. Et al. A randomized comparative trial of generalized vs targeted physiotherapy in the management of childhood hypermobility.2009.(Level of Evidence: 1B)</ref>,<ref name="p6">Bravo JF. Et al. Clinical study of hereditary disorders of connective tissues in a Chilean population: joint hypermobility syndrome and vascular Ehlers-Danlos syndrome. Arthritis Rheum. 2006;54:515-23. (Level of Evidence: 4)</ref><br>• Osteoarthritis<br>• Delayed motor development <br>• Congenital hip dislocation<br>• Exercise-related/post-exercise-related pains<ref name="p2">Kemp S. Et al. A randomized comparative trial of generalized vs targeted physiotherapy in the management of childhood hypermobility.2009.(Level of Evidence: 1B)</ref><br>• Nocturnal leg pains<ref name="p2">Kemp S. Et al. A randomized comparative trial of generalized vs targeted physiotherapy in the management of childhood hypermobility.2009.(Level of Evidence: 1B)</ref><br>• Low nocturnal sleep quality<ref name="p9">Celletti C. Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type. (Level of Evidence: 2C)</ref><br>• Joint swelling<ref name="p2">Kemp S. Et al. A randomized comparative trial of generalized vs targeted physiotherapy in the management of childhood hypermobility.2009.(Level of Evidence: 1B)</ref><br>• Clumsiness<ref name="p2">Kemp S. Et al. A randomized comparative trial of generalized vs targeted physiotherapy in the management of childhood hypermobility.2009.(Level of Evidence: 1B)</ref><br>• Enhanced flexibility<ref name="p3">Castori M. Et al. Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. American Journal of Medical Genetics part A. 2013. (Level of Evidence: 2C)</ref><br>• Chronic pain<ref name="p3">Castori M. Et al. Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. American Journal of Medical Genetics part A. 2013. (Level of Evidence: 2C)</ref><br>• Little changes of the skin<ref name="p9">Celletti C. Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type. (Level of Evidence: 2C)</ref>,<ref name="p3">Castori M. Et al. Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. American Journal of Medical Genetics part A. 2013. (Level of Evidence: 2C)</ref><br>• Greater risk in failures in tendon, ligament, bone, skin and cartilage<ref name="p4">Beighton P. Et al. Hypermobility of Joints. 2012. pp 65-99</ref><br>• Functional gastro-intestinal disorders<ref name="p5">Castori M. Ehlers-Danlos syndrome, Hypermobility type: An underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012. (Level of Evidence: 2C)</ref><br>• Chronic headache<ref name="p3">Castori M. Et al. Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. American Journal of Medical Genetics part A. 2013. (Level of Evidence: 2C)</ref>,<ref name="p5">Castori M. Ehlers-Danlos syndrome, Hypermobility type: An underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012. (Level of Evidence: 2C)</ref><br>• Immune system dysregulations<ref name="p5">Castori M. Ehlers-Danlos syndrome, Hypermobility type: An underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012. (Level of Evidence: 2C)</ref><br>• Pelvic dysfunction<ref name="p5">Castori M. Ehlers-Danlos syndrome, Hypermobility type: An underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012. (Level of Evidence: 2C)</ref><br>• Cardio-vascular dysautonomia<ref name="p5">Castori M. Ehlers-Danlos syndrome, Hypermobility type: An underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012. (Level of Evidence: 2C)</ref><br>• Exocrine glands dysfunction<ref name="p5">Castori M. Ehlers-Danlos syndrome, Hypermobility type: An underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012. (Level of Evidence: 2C)</ref><br>• Ankylosing spondylitis<ref name="p5">Castori M. Ehlers-Danlos syndrome, Hypermobility type: An underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012. (Level of Evidence: 2C)</ref><br><br><br>  
Possible Neuromusculoskeletal Signs for Individuals With Hypermobile Joint Syndrome:<br>• Acute or Traumatic sprains: <br>- recurrent ankle sprains<br>• Meniscus tears<br>• Joint instability<ref name="p3"/>: Acute or recurrent dislocations or subluxations<ref name="p6"/> of the:<br>- shoulder<br>- patella<br>- meta-carpo-phalangeal joint<br>- temporo-mandibular joint<br>• Traumatic arthritis<ref name="p5"/><br>• Bruising<br>• Fractures: chronic or non-traumatic<br>• Soft tissue rheumatism<br>- tendinitis<ref name="p6"/><br>- epicondylitis<br>- rotator cuff syndrome<br>- synovitis<br>- juvenile episodic synovitis<br>- bursitis<br>• Chondromalacia<br>• Scoliosis<br>• Fibromyalgia<ref name="p5"/><br>• Chronic fatigue syndrome<ref name="p3"/>,<ref name="p5"/><br>• Nerve compression disorders<br>- carpal tunnel syndrome<br>- tarsal Tunnel Syndrome<br>- Acroparesthesia<br>- thoracic outlet syndrome<br>• Raynaud syndrome<br>• Flat feet and sequelae<br>• Unspecified arthralgia or effusion of affected joint(s)<br>(foot, ankle, knee, hip, back, neck, shoulder, elbow,<br>wrist, finger)<ref name="p6"/><br>• Back pain<ref name="p2"/>,<ref name="p6"/><br>• Osteoarthritis<br>• Delayed motor development <br>• Congenital hip dislocation<br>• Exercise-related/post-exercise-related pains<ref name="p2"/><br>• Nocturnal leg pains<ref name="p2"/><br>• Low nocturnal sleep quality<ref name="p9">Celletti C. Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type. (Level of Evidence: 2C)</ref><br>• Joint swelling<ref name="p2"/><br>• Clumsiness<ref name="p2"/><br>• Enhanced flexibility<ref name="p3"/><br>• Chronic pain<ref name="p3"/><br>• Little changes of the skin<ref name="p9"/>,<ref name="p3"/><br>• Greater risk in failures in tendon, ligament, bone, skin and cartilage<ref name="p4"/><br>• Functional gastro-intestinal disorders<ref name="p5"/><br>• Chronic headache<ref name="p3">Castori M. Et al. Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. American Journal of Medical Genetics part A. 2013. (Level of Evidence: 2C)</ref>,<ref name="p5"/><br>• Immune system dysregulations<ref name="p5"/><br>• Pelvic dysfunction<ref name="p5"/><br>• Cardio-vascular dysautonomia<ref name="p5"/><br>• Exocrine glands dysfunction<ref name="p5"/><br>• Ankylosing spondylitis<ref name="p5"/><br><br><br>  


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== Differential Diagnosis  ==
== Differential Diagnosis  ==


The signs and symptoms of hypermobility syndrome are variable. Most commonly, the initial complaint in a hypermobile patient is joint pain, which may affect one or multiple joints and may be generalized or symmetric. Primary care physicians can use the five simple questions to aid in recognizing hypermobility.<ref name="p4">MAJ Michael, R.Simpson. Benign Joint Hypermobility Syndrome: Evaluation, Diagnosis, and Management. J Am Osteopath Assoc. 2006;106:531–536 (Level of Evidence: 2C)</ref>
The signs and symptoms of hypermobility syndrome are variable. Most commonly, the initial complaint in a hypermobile patient is joint pain, which may affect one or multiple joints and may be generalized or symmetric. Primary care physicians can use the five simple questions to aid in recognizing hypermobility.<ref name="p4"/>


Other complaints are muscle cramps<ref name="p4">Beighton P. Et al. Hypermobility of Joints. 2012. pp 65-99</ref>( after physical activity) and joint stiffness. People with HS can suffer from subluxations and dislocations. <ref name="p0">Howard P Levy. Ehlers-Danlos Syndrome, Hypermobility Type. 1993-2013, University of Washington, Seattle</ref><br><br>  
Other complaints are muscle cramps<ref name="p4"/>( after physical activity) and joint stiffness. People with HS can suffer from subluxations and dislocations. <ref name="p0">Howard P Levy. Ehlers-Danlos Syndrome, Hypermobility Type. 1993-2013, University of Washington, Seattle</ref><br><br>  


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*2 minor criteria and unequivocally affected first-degree relative in family history<br><br>
*2 minor criteria and unequivocally affected first-degree relative in family history<br><br>


Another tests that the physiotherapist can do is: '[[Beighton score|The Beighton score]]' <ref name="p1">Alter M. Science of Flexibility. Sheridan books. 2004. page 89</ref><br>  
Another tests that the physiotherapist can do is: '[[Beighton score|The Beighton score]]' <ref name="p1"/><br>  


[[Image:Beighton Score.png]]  
[[Image:Beighton Score.png]]  
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#Flexion of the trunk, whit knees straight and the palms flat on the ground ( 1 point)<br>
#Flexion of the trunk, whit knees straight and the palms flat on the ground ( 1 point)<br>


According to the Beighton and Horan criteria, generalized joint laxity is present when four or more of five tests are positive, including contralateral knee hyperextension.<ref name="p7">Clinch J. Et al. Epidemiology of Generalized Joint Laxity (Hypermobility) in Fourteen-Year-Old Children From the UK:A Population-Based Evaluation.2011(Level of Evidence: 2B)</ref> In fact there is no universal agreement on a threshold for BJHMS, some researchers use a Beighton scale score of 5/9, other researchers use a score of 6/9 and still others use a modified score of 3/9.  
According to the Beighton and Horan criteria, generalized joint laxity is present when four or more of five tests are positive, including contralateral knee hyperextension.<ref name="p7"/> In fact there is no universal agreement on a threshold for BJHMS, some researchers use a Beighton scale score of 5/9, other researchers use a score of 6/9 and still others use a modified score of 3/9.  


<br>Kinesiophobia: Tampa Scale for Kinesiophobia (TSK-I) is a questionnaire to measure pain and pain-related fear of movement. It can be used for patients with musculoskeletal complaints. This questionnaire has two sub-scales: “evaluating activity avoidance” ( a belief that activities who cause pain should be avoided) and “harm” (a belief that pain is a sign of damage of the body).<ref name="p9">Celletti C. Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type. (Level of Evidence: 2C)</ref>
<br>Kinesiophobia: Tampa Scale for Kinesiophobia (TSK-I) is a questionnaire to measure pain and pain-related fear of movement. It can be used for patients with musculoskeletal complaints. This questionnaire has two sub-scales: “evaluating activity avoidance” ( a belief that activities who cause pain should be avoided) and “harm” (a belief that pain is a sign of damage of the body).<ref name="p9"/>


Fatigue: Fatique Severity Scale (FSS) is a scale that quantifies the fatique intensity. This scale has a hight internal consistency and validity. FSS excist out 9 items with a 7-point response format that indicates the degree of agreement with each item.<ref name="p9">Celletti C. Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type. (Level of Evidence: 2C)</ref>
Fatigue: Fatique Severity Scale (FSS) is a scale that quantifies the fatique intensity. This scale has a hight internal consistency and validity. FSS excist out 9 items with a 7-point response format that indicates the degree of agreement with each item.<ref name="p9"/>


Pain: Numeric Rating Scale (NRS) is a 11-point numeric scale to roughly measure any kind of pain.<ref name="p9">Celletti C. Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type. (Level of Evidence: 2C)</ref>
Pain: Numeric Rating Scale (NRS) is a 11-point numeric scale to roughly measure any kind of pain.<ref name="p9"/>


Quality of Life: The Medical Outcome Study 36-item Short Form Health Survey (SF-36) is a survey to evaluate aspects of health that are most closely related to quality of life. It consist of 36 questions that measure 8 domains: body pain, physical functioning, physical limitations, general health, vitality, social functioning, emotional limitations and mental health.<ref name="p9">Celletti C. Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type. (Level of Evidence: 2C)</ref><br><br>
Quality of Life: The Medical Outcome Study 36-item Short Form Health Survey (SF-36) is a survey to evaluate aspects of health that are most closely related to quality of life. It consist of 36 questions that measure 8 domains: body pain, physical functioning, physical limitations, general health, vitality, social functioning, emotional limitations and mental health.<ref name="p9"/><br><br>


== Outcome Measures  ==
== Outcome Measures  ==
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*joint integrity and mobility<ref name="p3" />
*joint integrity and mobility<ref name="p3" />
*muscle performance<ref name="p3" />
*muscle performance<ref name="p3" />
*function and muscle strength<ref name="p2">Kemp S. Et al. A randomized comparative trial of generalized vs targeted physiotherapy in the management of childhood hypermobility.2009.(Level of Evidence: 1B)</ref>
*function and muscle strength<ref name="p2"/>
*visual analog scale was used to assess musculoskeletal pain.<ref name="p9">Ferrell WR. Et al. Amelioration of symptoms by enhancement of proprioception in patients with joint hypermobility syndrome. Artritis Rheum. 2004;50(10):3323-8 (Level of Evidence: 4)</ref>
*visual analog scale was used to assess musculoskeletal pain.<ref name="p9"/>
*quality of life was evaluated by a Short Form 36 questionnaire (SF-36).<ref name="p9">Ferrell WR. Et al. Amelioration of symptoms by enhancement of proprioception in patients with joint hypermobility syndrome. Artritis Rheum. 2004;50(10):3323-8 (Level of Evidence: 4)</ref><br><br>
*quality of life was evaluated by a Short Form 36 questionnaire (SF-36).<ref name="p9"/><br><br>


add links to outcome measures here (also see [[Outcome Measures|Outcome Measures Database]])
add links to outcome measures here (also see [[Outcome Measures|Outcome Measures Database]])
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== Examination  ==
== Examination  ==


*ROM: Norkin and White<ref name="p3">Russek LN. Examination and Treatment of a Patient with Hypermobility Syndrome. Physical Therapy. 2000;80(4):386-398. (Level of Evidence:  4)</ref>  
*ROM: Norkin and White<ref name="p3"/>  
*End feel<ref name="p3">Russek LN. Examination and Treatment of a Patient with Hypermobility Syndrome. Physical Therapy. 2000;80(4):386-398. (Level of Evidence:  4)</ref>  
*End feel<ref name="p3"/>  
*Manual testing<ref name="p3">Russek LN. Examination and Treatment of a Patient with Hypermobility Syndrome. Physical Therapy. 2000;80(4):386-398. (Level of Evidence:  4)</ref><br><br>
*Manual testing<ref name="p3"/><br><br>


add text here related to physical examination and assessment<br>
add text here related to physical examination and assessment<br>
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== Medical Management <br>  ==
== Medical Management <br>  ==


For HMS, there is no medical management. To control pain you can use NSAIDs (Non-steroidal anti-inflammatory drugs) or acetaminophen.<ref name="p4">MAJ Michael, R.Simpson. Benign Joint Hypermobility Syndrome: Evaluation, Diagnosis, and Management. J Am Osteopath Assoc. 2006;106:531–536 (Level of Evidence: 2C)</ref><br>
For HMS, there is no medical management. To control pain you can use NSAIDs (Non-steroidal anti-inflammatory drugs) or acetaminophen.<ref name="p4"/><br>


== Physical Therapy Management <br>  ==
== Physical Therapy Management <br>  ==


The aim of physical therapy in hypermobility syndrome, is to approach the muscle inhibition, atrophy and the reduced joint control caused by the joint pain. Another important step of treating hypermobility syndrome is education. Without this education patients will continue to go over the normal joint range and their extreme joint range can cause a more unstable joint. Fatigue, anxiety and depression are sometimes associated with HMS, and we must try to ameliorate their quality of life. It is necessarily to encourage an active lifestyle, so give for example a schedule with exercises to fulfill at least 3 times a week.&nbsp;<ref name="p7">Palmera S. Et al. The effectiveness of therapeutic exercise for joint hypermobility syndrome: a systematic review. (Level of Evidence: 2A)</ref> (Level of Evidence: 2A)<br> Treatment overview:<br>  
The aim of physical therapy in hypermobility syndrome, is to approach the muscle inhibition, atrophy and the reduced joint control caused by the joint pain. Another important step of treating hypermobility syndrome is education. Without this education patients will continue to go over the normal joint range and their extreme joint range can cause a more unstable joint. Fatigue, anxiety and depression are sometimes associated with HMS, and we must try to ameliorate their quality of life. It is necessarily to encourage an active lifestyle, so give for example a schedule with exercises to fulfill at least 3 times a week.&nbsp;<ref name="p7"/> (Level of Evidence: 2A)<br> Treatment overview:<br>  


*Education of hypermobility syndrome  
*Education of hypermobility syndrome  
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*Streching affected joint  
*Streching affected joint  
*strengthening excercises for affected joint  
*strengthening excercises for affected joint  
*osteopathic manipulative treatment<ref name="p4">MAJ Michael, R.Simpson. Benign Joint Hypermobility Syndrome: Evaluation, Diagnosis, and Management. J Am Osteopath Assoc. 2006;106:531–536 (Level of Evidence: 2C)</ref>
*osteopathic manipulative treatment<ref name="p4"/>


<u>Active mobilisation exercises:</u> shoulder rolls, arm circles, neck rotations, neck lateral flexions, wrist circles, side flexions of spine, thoracic rotations in sitting.<ref name="p7">Palmera S. Et al. The effectiveness of therapeutic exercise for joint hypermobility syndrome: a systematic review. (Level of Evidence: 2A)</ref>(Level of Evidence: 2A)  
<u>Active mobilisation exercises:</u> shoulder rolls, arm circles, neck rotations, neck lateral flexions, wrist circles, side flexions of spine, thoracic rotations in sitting.<ref name="p7"/>(Level of Evidence: 2A)  


Closed chain exercises are good exercises in many regards: it may reduce strain on injured ligaments, augment proprioceptive feedback, and optimise muscle action. In the studies by Sahin et al. <ref name="p8">Sahin N. Et al. Evaluation of knee proprioception and effects of proprioception exercise in patients with benign joint hypermobility. Rheumatology. 2012;49:315-25 (Level of Evidence: 1B)</ref>(Level of Evidence: 1B) and Ferrell et al. <ref name="p9">Ferrell WR. Et al. Amelioration of symptoms by enhancement of proprioception in patients with joint hypermobility syndrome. Artritis Rheum. 2004;50(10):3323-8 (Level of Evidence: 4)</ref>(Level of Evidence: 4) they trained specific with the knee joint, whilst the other two studies incorporated whole body exercise interventions to treat joint hypermobility syndrome.  
Closed chain exercises are good exercises in many regards: it may reduce strain on injured ligaments, augment proprioceptive feedback, and optimise muscle action. In the studies by Sahin et al. <ref name="p8"/>(Level of Evidence: 1B) and Ferrell et al. <ref name="p9"/>(Level of Evidence: 4) they trained specific with the knee joint, whilst the other two studies incorporated whole body exercise interventions to treat joint hypermobility syndrome.  


<br> <u>Strengthening exercises:</u> stabilizing muscles around hypermobile joints can be effective for joint support during movement or can reduce pain. These included shuttle-runs, bunny-hops, squat-thrusts, sitting-to-standing, step-ups and star-jumps. (30seconds or a predetermined number of repetitions) <ref name="p7">Palmera S. Et al. The effectiveness of therapeutic exercise for joint hypermobility syndrome: a systematic review. (Level of Evidence: 2A)</ref>(Level of Evidence: 2A)  
<br> <u>Strengthening exercises:</u> stabilizing muscles around hypermobile joints can be effective for joint support during movement or can reduce pain. These included shuttle-runs, bunny-hops, squat-thrusts, sitting-to-standing, step-ups and star-jumps. (30seconds or a predetermined number of repetitions) <ref name="p7"/>(Level of Evidence: 2A)  


<br>  
<br>  


<u>Proprioception exercises:</u> a decreased joint position sense (is there proof of will make patient more vulnerable for damage. Decreased sensory feedback may lead to biomechanically unsound limb positions being adopted, leading to abnormal postures. <ref name="p0">Divya S. Et al. Evaluation of posture and pain in persons with benign joint hypermobility syndrome. 2011 (Level of Evidence: 2C )</ref>(Level of Evidence: 2C)<br> Coordination and balance exercises may improve proprioception. Exercise on board balance wood (2 to 3 minutes), mini-trampoline jumping (30 reps), walking with eyes closed, single leg balance, single leg ball rolling, forward/backward bends on one leg (eyes open or closed)  
<u>Proprioception exercises:</u> a decreased joint position sense (is there proof of will make patient more vulnerable for damage. Decreased sensory feedback may lead to biomechanically unsound limb positions being adopted, leading to abnormal postures. <ref name="p0"/>(Level of Evidence: 2C)<br> Coordination and balance exercises may improve proprioception. Exercise on board balance wood (2 to 3 minutes), mini-trampoline jumping (30 reps), walking with eyes closed, single leg balance, single leg ball rolling, forward/backward bends on one leg (eyes open or closed)  


<br><u>Control neutral joint position:</u> identifying abnormal resting position of symptomatic joints, re-training postural muscles to facilitate optimal joint alignment (avoid hyperextension of knee when standing)  
<br><u>Control neutral joint position:</u> identifying abnormal resting position of symptomatic joints, re-training postural muscles to facilitate optimal joint alignment (avoid hyperextension of knee when standing)  
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<br><u>Re-train dynamic control:</u> once a ‘neutral’ resting position is achieved, re-training of specific muscles to maintain joint position while moving adjacent joints (hip flexion while maintaining spinal neutral) Dynamic control will be exercised with daily activities or sports.  
<br><u>Re-train dynamic control:</u> once a ‘neutral’ resting position is achieved, re-training of specific muscles to maintain joint position while moving adjacent joints (hip flexion while maintaining spinal neutral) Dynamic control will be exercised with daily activities or sports.  


<br> <u>Motion control:</u> improving the ability of specific muscles to control the joint through its entire range, both concentrically and eccentrically, static and posture ( on sitting to- standing quadriceps or working concentrically on standing up and eccentrically on sitting down) <ref name="p2">Kemp S. Et al. A randomized comparative trial of generalized vs targeted physiotherapy in the management of childhood hypermobility.2009.(Level of Evidence: 1B)</ref>(Level of Evidence: 1B)<br><br>
<br> <u>Motion control:</u> improving the ability of specific muscles to control the joint through its entire range, both concentrically and eccentrically, static and posture ( on sitting to- standing quadriceps or working concentrically on standing up and eccentrically on sitting down) <ref name="p2"/>(Level of Evidence: 1B)<br><br>


== Key Research  ==
== Key Research  ==

Revision as of 15:26, 6 June 2017

Welcome to Vrije Universiteit Brussel's Evidence-based Practice project. This space was created by and for the students in the Rehabilitation Sciences and Physiotherapy program of the Vrije Universiteit Brussel, Brussels, Belgium. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!

Search Strategy[edit | edit source]

Search on Pubmed and Pedro with keywords: “hypermobility”, “hypermobility syndrome” , “hypermobility ”and “therapy”. Search in libraries books about sport injuries, sports medicine, sport anatomy.

Definition/Description
[edit | edit source]

The hypermobility syndrome(HMS) was first described in 1967 by Kirk et al as the occurrence of musculoskeletal symptoms in hypermobile in healthy persons.[1] Meanwhile, other names are given to HMS, such as joint hypermobility syndrome and benign hypermobility joint syndrome. HMS is a dominant inherited connective tissue disorder described as “generalized articular hypermobility, with or without subluxation or dislocation.[2]


The primary symptom is excessive laxity of multiple joints. [3]


Hypermobility syndrome is different from localized joint hypermobility and other disorders that have generalized joint hypermobility, such as Ehlers-Danlos Syndrome, Rheumatoid Arthritis, Systemic Lupus Erythematosus, and Marfan Syndrome.[3] HMS may occur also in chromosomal and genetic disorders such as Down syndrome and in metabolic disorders such as homocystinuria and hyperlysinemia.[4] Laboratory tests are used to exclude these other systemic disorders when HMS is suspected.[3]



Clinically Relevant Anatomy[edit | edit source]

The pathophysiology in Hypermobility Syndrome is not yet fully understood, it appears to be a systemic collagen abnormality. The abnormality in collagen ratios is related with joint hypermobility and laxity of other tissues. The ratio of collagen (type I,II and III) is decreased in skin.


The diagnostic criteria for HMS includes joint abnormalities but it also affects cardiac tissue, smooth muscle in the female genital system and the gastrointestinal system. HMS also affects the joint position sense.[3]

Epidemiology /Etiology[edit | edit source]

Joint hypermobility happen most often in children and reduce with age.[5]

Joint mobility is highest at birth, there is a decrease in children around nine to twelve years old.

In adolescent girls there is a peak at the age of fifteen years, after this age the joint mobility decreases, as well in boys as in girls. [6]

Hormonal changes that occur in puberty by adolescent girls, will influence the joint mobility.[6]

In general, hypermobility is more common in children than adults, is more common in girls than in boys [7], more common in Asian, African and middle eastern people. [4]

Characteristics/Clinical Presentation[edit | edit source]

Possible Neuromusculoskeletal Signs for Individuals With Hypermobile Joint Syndrome:
• Acute or Traumatic sprains:
- recurrent ankle sprains
• Meniscus tears
• Joint instability[3]: Acute or recurrent dislocations or subluxations[6] of the:
- shoulder
- patella
- meta-carpo-phalangeal joint
- temporo-mandibular joint
• Traumatic arthritis[5]
• Bruising
• Fractures: chronic or non-traumatic
• Soft tissue rheumatism
- tendinitis[6]
- epicondylitis
- rotator cuff syndrome
- synovitis
- juvenile episodic synovitis
- bursitis
• Chondromalacia
• Scoliosis
• Fibromyalgia[5]
• Chronic fatigue syndrome[3],[5]
• Nerve compression disorders
- carpal tunnel syndrome
- tarsal Tunnel Syndrome
- Acroparesthesia
- thoracic outlet syndrome
• Raynaud syndrome
• Flat feet and sequelae
• Unspecified arthralgia or effusion of affected joint(s)
(foot, ankle, knee, hip, back, neck, shoulder, elbow,
wrist, finger)[6]
• Back pain[2],[6]
• Osteoarthritis
• Delayed motor development
• Congenital hip dislocation
• Exercise-related/post-exercise-related pains[2]
• Nocturnal leg pains[2]
• Low nocturnal sleep quality[8]
• Joint swelling[2]
• Clumsiness[2]
• Enhanced flexibility[3]
• Chronic pain[3]
• Little changes of the skin[8],[3]
• Greater risk in failures in tendon, ligament, bone, skin and cartilage[4]
• Functional gastro-intestinal disorders[5]
• Chronic headache[3],[5]
• Immune system dysregulations[5]
• Pelvic dysfunction[5]
• Cardio-vascular dysautonomia[5]
• Exocrine glands dysfunction[5]
• Ankylosing spondylitis[5]



Differential Diagnosis[edit | edit source]

The signs and symptoms of hypermobility syndrome are variable. Most commonly, the initial complaint in a hypermobile patient is joint pain, which may affect one or multiple joints and may be generalized or symmetric. Primary care physicians can use the five simple questions to aid in recognizing hypermobility.[4]

Other complaints are muscle cramps[4]( after physical activity) and joint stiffness. People with HS can suffer from subluxations and dislocations. [9]


Diagnostic Procedures[edit | edit source]

- Brighton criteria:

Major Criteria:

  1. Beighton Score of ≥ 4/9
  2. Arthralgia for > 3 months in > 4 joints

Minor Criteria:

  1. Beighton score of 1–3
  2. Arthralgia in 1–3 joints
  3. History of joint dislocation
  4. Soft tissue lesions >3
  5. Marfan-like habitus
  6. Skin striae, hyperextensibility or scarring
  7. Eye signs, lid laxity
  8. History of varicose veins, hernia, visceral prolapse

For a diagnosis to be made either both of the the major criteria must be present or 1 major and 2 minor or 4 minor.[10]

Requirement for diagnosis of hypermobility syndrome:

  • 2 major criteria
  • 1 major criteria + 2 minor criteria
  • 4 minor criteria
  • 2 minor criteria and unequivocally affected first-degree relative in family history

Another tests that the physiotherapist can do is: 'The Beighton score' [1]

Beighton Score.png

  1. A passive dorsiflexion of the little finger above 90degrees (1 point for each hand).
  2. Bringing the thumb passive against the ventral side of the forearm (1 point for each thumb).
  3. Hyperextension of the elbow above 10 degrees ( 1 point for each elbow)
  4. Hyperextension of the knee above 10 degrees (1 point for each knee)
  5. Flexion of the trunk, whit knees straight and the palms flat on the ground ( 1 point)

According to the Beighton and Horan criteria, generalized joint laxity is present when four or more of five tests are positive, including contralateral knee hyperextension.[7] In fact there is no universal agreement on a threshold for BJHMS, some researchers use a Beighton scale score of 5/9, other researchers use a score of 6/9 and still others use a modified score of 3/9.


Kinesiophobia: Tampa Scale for Kinesiophobia (TSK-I) is a questionnaire to measure pain and pain-related fear of movement. It can be used for patients with musculoskeletal complaints. This questionnaire has two sub-scales: “evaluating activity avoidance” ( a belief that activities who cause pain should be avoided) and “harm” (a belief that pain is a sign of damage of the body).[8]

Fatigue: Fatique Severity Scale (FSS) is a scale that quantifies the fatique intensity. This scale has a hight internal consistency and validity. FSS excist out 9 items with a 7-point response format that indicates the degree of agreement with each item.[8]

Pain: Numeric Rating Scale (NRS) is a 11-point numeric scale to roughly measure any kind of pain.[8]

Quality of Life: The Medical Outcome Study 36-item Short Form Health Survey (SF-36) is a survey to evaluate aspects of health that are most closely related to quality of life. It consist of 36 questions that measure 8 domains: body pain, physical functioning, physical limitations, general health, vitality, social functioning, emotional limitations and mental health.[8]

Outcome Measures[edit | edit source]

  • Range of motion [3]
  • joint integrity and mobility[3]
  • muscle performance[3]
  • function and muscle strength[2]
  • visual analog scale was used to assess musculoskeletal pain.[8]
  • quality of life was evaluated by a Short Form 36 questionnaire (SF-36).[8]

add links to outcome measures here (also see Outcome Measures Database)

Examination[edit | edit source]

  • ROM: Norkin and White[3]
  • End feel[3]
  • Manual testing[3]

add text here related to physical examination and assessment

Medical Management
[edit | edit source]

For HMS, there is no medical management. To control pain you can use NSAIDs (Non-steroidal anti-inflammatory drugs) or acetaminophen.[4]

Physical Therapy Management
[edit | edit source]

The aim of physical therapy in hypermobility syndrome, is to approach the muscle inhibition, atrophy and the reduced joint control caused by the joint pain. Another important step of treating hypermobility syndrome is education. Without this education patients will continue to go over the normal joint range and their extreme joint range can cause a more unstable joint. Fatigue, anxiety and depression are sometimes associated with HMS, and we must try to ameliorate their quality of life. It is necessarily to encourage an active lifestyle, so give for example a schedule with exercises to fulfill at least 3 times a week. [7] (Level of Evidence: 2A)
Treatment overview:

  • Education of hypermobility syndrome
  • Activity modification
  • Streching affected joint
  • strengthening excercises for affected joint
  • osteopathic manipulative treatment[4]

Active mobilisation exercises: shoulder rolls, arm circles, neck rotations, neck lateral flexions, wrist circles, side flexions of spine, thoracic rotations in sitting.[7](Level of Evidence: 2A)

Closed chain exercises are good exercises in many regards: it may reduce strain on injured ligaments, augment proprioceptive feedback, and optimise muscle action. In the studies by Sahin et al. [10](Level of Evidence: 1B) and Ferrell et al. [8](Level of Evidence: 4) they trained specific with the knee joint, whilst the other two studies incorporated whole body exercise interventions to treat joint hypermobility syndrome.


Strengthening exercises: stabilizing muscles around hypermobile joints can be effective for joint support during movement or can reduce pain. These included shuttle-runs, bunny-hops, squat-thrusts, sitting-to-standing, step-ups and star-jumps. (30seconds or a predetermined number of repetitions) [7](Level of Evidence: 2A)


Proprioception exercises: a decreased joint position sense (is there proof of will make patient more vulnerable for damage. Decreased sensory feedback may lead to biomechanically unsound limb positions being adopted, leading to abnormal postures. [9](Level of Evidence: 2C)
Coordination and balance exercises may improve proprioception. Exercise on board balance wood (2 to 3 minutes), mini-trampoline jumping (30 reps), walking with eyes closed, single leg balance, single leg ball rolling, forward/backward bends on one leg (eyes open or closed)


Control neutral joint position: identifying abnormal resting position of symptomatic joints, re-training postural muscles to facilitate optimal joint alignment (avoid hyperextension of knee when standing)


Re-train dynamic control: once a ‘neutral’ resting position is achieved, re-training of specific muscles to maintain joint position while moving adjacent joints (hip flexion while maintaining spinal neutral) Dynamic control will be exercised with daily activities or sports.


Motion control: improving the ability of specific muscles to control the joint through its entire range, both concentrically and eccentrically, static and posture ( on sitting to- standing quadriceps or working concentrically on standing up and eccentrically on sitting down) [2](Level of Evidence: 1B)

Key Research[edit | edit source]

add links and reviews of high quality evidence here (case studies should be added on new pages using the case study template)

Resources [edit | edit source]

Ehlers-Danlos_Syndrome

Rheumatoid_Arthritis

Systemic_Lupus_Erythematosus

Marfan_Syndrome

Beighton_score

Clinical Bottom Line[edit | edit source]

Because hypermobility syndrome can excist with other symptoms and complaints, it is important that physiotherapists can diagnose hypermobility syndrome.
According to several articles physical therapy for HS patients includes: education of hypermobility syndrome, activity modification, stretching and strengthening exercises for affected joints and osteopathic manipulative treatment.[4]

Recent Related Research (from Pubmed)[edit | edit source]

see tutorial on Adding PubMed Feed

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References[edit | edit source]

  1. 1.0 1.1 Kirk JA. Et al. The hypermobility syndrome: musculoskeletal complaints look for more recent sources associated with generalized joint hypermobility. Ann Rheum Dis. 1967; 26: 419–25. (Level of Evidence: 4)
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Russek LN. Hypermobility Syndrome. Physical therapy . 1999 (Level of Evidence: 2C)
  3. 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 3.15 Castori M. Et al. Re-writing the natural history of pain and related symptoms in the joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. American Journal of Medical Genetics part A. 2013. (Level of Evidence: 2C)
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 MAJ Michael, R.Simpson. Benign Joint Hypermobility Syndrome: Evaluation, Diagnosis, and Management. J Am Osteopath Assoc. 2006;106:531–536 (Level of Evidence: 2C)
  5. 5.00 5.01 5.02 5.03 5.04 5.05 5.06 5.07 5.08 5.09 5.10 Seçkin U. Et al. The prevalence of joint hypermobility among high school students. Rheumatol Int. 2005;25 (4):260–263. (Level of Evidence: 2C)
  6. 6.0 6.1 6.2 6.3 6.4 6.5 Jansson A. Et al. General joint laxity in 1845 Swedish school children of different ages: age- and gender-specific distributions. Acta Paediatr. 2004;93(9):1202–1206. (Level of Evidence: 2C)
  7. 7.0 7.1 7.2 7.3 7.4 Clinch J. Et al. Epidemiology of Generalized Joint Laxity (Hypermobility) in Fourteen-Year-Old Children From the UK:A Population-Based Evaluation.2011(Level of Evidence: 2B)
  8. 8.0 8.1 8.2 8.3 8.4 8.5 8.6 8.7 8.8 Celletti C. Evaluation of Kinesiophobia and Its Correlations with Pain and Fatigue in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type. (Level of Evidence: 2C)
  9. 9.0 9.1 Howard P Levy. Ehlers-Danlos Syndrome, Hypermobility Type. 1993-2013, University of Washington, Seattle
  10. 10.0 10.1 Tofts Louise J. The differential diagnosis of children with joint hypermobility: a review of the literature. Pediatric Rheumatology. 2009 (Level of Evidence: 2C)