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Hypermobility |
Classification and external resources |
Hypermobile fingers and thumb
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ICD-10 |
M35.7 |
ICD-9 |
728.5 |
OMIM |
147900 |
DiseasesDB |
31101 |
MedlinePlus |
003295 |
MeSH |
D007593 |
Hypermobility describes joints that stretch farther than normal. For example, some hypermobile people can bend their thumbs backwards to their wrists, bend their knee joints backwards, put their leg behind the head or perform other contortionist "tricks". It can affect a single joint or multiple joints throughout the body.
Contents
- 1 Causes
- 1.1 Anxiety and joint hypermobility
- 2 Hypermobility syndrome
- 2.1 Causes
- 2.2 Symptoms
- 2.3 Diagnosis
- 2.3.1 Beighton's criteria
- 2.3.1.1 Major criteria
- 2.3.1.2 Minor criteria
- 2.3.2 Beighton score
- 3 Treatments
- 3.1 Physical therapy
- 3.2 Medication
- 3.3 Lifestyle modification
- 3.4 Other treatments
- 4 References
- 5 External links
Causes[edit source | edit]
Hypermobility generally results from one or more of the following:
- Misaligned joints
- Abnormally shaped ends of one or more bones at a joint
- A Type 1 collagen or other connective tissue defect (found in Ehlers-Danlos syndrome, Marfan syndrome) resulting in weakened ligaments/ligamentous laxity, muscles and tendons. This same defect also results in weakened bones, which may result in osteoporosis and fractures
- Abnormal joint proprioception (an impaired ability to determine where in space parts of the body are, and how stretched a joint is)
These abnormalities cause abnormal stress on a joint or many joints, meaning that the joints can wear out, leading to osteoarthritis.
The condition tends to run in families, suggesting that there may be a genetic basis for at least some forms of hypermobility. The term double jointed is often used to describe hypermobility; however, the name is a misnomer and should not to be taken literally, as an individual with hypermobility in a joint does not actually have two separate joints where others would have just one.
Most people have hypermobility with no other symptoms. In fact, approximately 5% of the healthy population have hypermobile joints. However, people with "joint hypermobility syndrome" may experience many difficulties. For example, their joints may be easily injured, be more prone to complete dislocation due to the weakly stabilized joint and they may develop problems from muscle fatigue (as muscles must work harder to compensate for the excessive weakness in the ligaments that support the joints). Hypermobility syndrome can also lead to chronic pain or even disability in severe cases. Instrumentalists with hypermobile fingers may have difficulties when fingers collapse into the finger locking position.
Hypermobility may also be symptomatic of a serious medical condition, such as Stickler Syndrome, Ehlers-Danlos syndrome,[1] Marfan syndrome,[1] rheumatoid arthritis, osteogenesis imperfecta,[1] lupus, polio, Down syndrome,[1] morquio syndrome, cleidocranial dysostosis or myotonia congenita.
In addition, hypermobility has been associated with chronic fatigue syndrome and fibromyalgia. Just as people with hypermobility have a higher risk for Osteoarthritis, they may also have a higher risk for Fibromyalgia. Some speculate that those with Fibromyalgia might have some form of Connective Tissue Disorder, such as the ones that cause hypermobility. Hypermobility causes physical trauma (In the form of Joint Dislocations, Joint Subluxations, Joint Instability, Sprains, etc), in turn causes emotional trauma; physical trauma, emotional trauma, and a few other factors either trigger and/or are possible causes for Fibromyalgia.[2]
During pregnancy certain hormones alter the physiology of ligaments making them able to stretch to accommodate the birthing process. For some women with hypermobility pregnancy-related pelvic girdle pain can be debilitating due to these two converging factors, and prohibits them from standing up or walking. Use of a bedpan and wheelchair may be necessary, or even permanent in some cases due to hip stretching. Also many people complain about joints or muscles hurting because of this.
Symptoms of hypermobility include a dull but intense pain around the knee and ankle joints and the soles of the feet. The condition affecting these parts can be alleviated by using insoles in the footwear that have been specially made for the individual after assessment by an orthopaedic surgeon and orthotist.
Anxiety and joint hypermobility[edit source | edit]
In 1998, a study was done concerning the link between panic disorder with joint hypermobility. The prevalence of joint hypermobility syndrome among patients with panic disorder was 67.7% compared to the control psychiatric group (10.1%). Women and subjects who were younger were found to be over 20 times more likely to have the hypermobile joints than their counterparts in the control group. In the study, there was also a higher prevalence for mitral valve prolapse (8%). The study does not determine whether hypermobility is associated in any way with psychiatric dysfunction or dysautonomia; many factors could explain the relation, and hypermobility's unwelcome gift of chronic pain may very well cause many people anxiety or depression. These people feel alone in that nobody understands their disorder and they find it difficult to explain to skeptics because their body looks perfectly normal. Often accused of not getting enough rest these patients become agitated that their previous life activities become increasingly difficult as their condition worsens. Lashing out, anxiety, depression, and sadness are all common [3]
Hypermobility syndrome[edit source | edit]
Hypermobile metacarpo-phalangeal joints
Hypermobility syndrome is generally considered to comprise hypermobility together with other symptoms, such as myalgia and arthralgia. It is relatively common among children and affects more females than males.
Causes[edit source | edit]
Current thinking suggests that there are four factors: These four factors affect different people to varying degrees.
- The shape of the ends of the bones: Some joints normally have a large range of movement, like the shoulder and hip. Both these joints are ball in sockets. If a shallow rather than a deep socket is inherited, a relatively large range of movement will be possible at the affected joints. If the hip socket is particularly shallow, then the hip may dislocate easily.
- Weak or stretched ligaments caused by protein or hormone problems: ligaments are made up of several types of protein fibre. These proteins include elastin, which gives elasticity, which may be altered in some people. Also, the female sex hormones alter the collagen proteins. Women are generally more supple just before a period, and even more so in the latter stages of pregnancy, because of the effect of a hormone called relaxin. This hormone allows the pelvis to expand so the head of the baby can pass through. Different races have differences in their joint mobility, which may reflect differences in the structure of the collagen proteins. People from the Indian sub-continent, for example, often have much more supple hands than Europeans.[4]
- The tone of the muscles: The tone (or stiffness) of the muscles is controlled by the nervous system, and influences the range of movement in the joints. Some people use special techniques to change their muscle tone and increase their flexibility. Yoga, for example, can help to relax the muscles and make the joints more supple. Gymnasts and athletes can sometimes acquire hypermobility in at least some of their joints through the exercises they do in training.
- The sense of joint movement (proprioception): if it is difficult to detect the exact position of the joints with closed eyes, then it is possible to develop hypermobile joints because of over-stretching.[5]
Hypermobility can also be caused by Connective Tissue Disorders, such as Ehlers-Danlos Syndrome and Marfan syndrome. Joint Hypermobility is a common symptom for both of these. Ehlers Danlos Syndrome has numerous types; however, most types have hypermobility in some degree. When hypermobility is the main symptom, to where it overshadows the rest, then it most likely is Ehlers Danlos Syndrome - The Hypermobility Type (EDS-HT). People with EDS-HT suffer frequent joint dislocations and subluxations (partial/incomplete dislocations), with or without trauma, and sometimes spontaneously. Commonly, the hypermobility is assumed by medical professionals to be nonpainful, mild, and dismissed in general. Consequently, they receive little to no treatment and left to suffer debilitating pain by many health professionals. They are labeled hypochondriacs, lazy, and/or as if the pain were a figment of their imagination.[6]
Symptoms[edit source | edit]
People with hypermobility syndrome may develop other conditions caused by their unstable joints. These conditions include:
- Joint instability causing frequent sprains, tendinitis, or bursitis when doing activities that would not affect the normal individual
- Joint Pain
- Early-onset osteoarthritis as early as their teen years; depending on the severity of the joint hypermobility and how many joints that are affected. It also depends on how active they were. For example, if they lifted weights for several years and their joints dislocate frequently and easily (less than 1 pound can dislocate their fingers) then they might have Osteoarthritis before they hit their 20s.
- Subluxations or dislocations, especially in the shoulder (According to the Social Security Administration (SSA), if this severely limits the person's ability to push, pull, grasp, finger, reach, etc then it's a disability in the SSA's "Blue Book," a list of impairments that they use to automatically grant disability according to 1.02 Major dysfunction of a joint(s) (due to any cause (http://www.ssa.gov/disability/professionals/bluebook/1.00-Musculoskeletal-Adult.htm#1_02)).
- Knee pain
- Back pain, prolapsed discs or spondylolisthesis
- Joints that make clicking noises (Sometimes, this can be a symptom of Osteoarthritis.)
- Susceptibility to whiplash
- Temporomandibular Joint Syndrome also known as TMJ
- Increased nerve compression disorders (e.g. carpal tunnel syndrome)
- The ability of Finger Locking
- Do not respond well to normal doses of anaesthetic or pain medication
- "Growing pains" as described in children in late afternoon or night
Diagnosis[edit source | edit]
Joint hypermobility syndrome needs to be distinguished from other disorders that share many common features, such as Marfan syndrome, Ehlers-Danlos Syndrome, and osteogenesis imperfecta. In the last couple of years, collaborations of experts in connective tissue disorders formally agreed that there was little distinction between severe forms of Hypermobility Syndrome and mild forms Ehlers-Danlos Syndrome Hypermobility Type, and that they are in fact the same disorder. Information on this can be found in an article titled "The lack of clinical distinction between the hypermobility type of Ehlers–Danlos syndrome and the joint hypermobility syndrome (a.k.a. hypermobility syndrome)"[7]
Generalized hypermobility is a common feature in all these hereditary connective tissue disorders and many features overlap, but often distinguishing features are present that enable differentiating these disorders.[8] The inheritance pattern of Ehlers-Danlos syndrome varies by type. The arthrochalasia, classic, hypermobility, and vascular forms of the disorder usually have an autosomal dominant pattern of inheritance. Autosomal dominant inheritance means that one copy of the altered gene in each cell is sufficient to cause the disorder. In some cases, an affected person inherits the mutation from one affected parent. Other cases result from new (sporadic) gene mutations. These cases occur in people with no history of the disorder in their family. The dermatosparaxis and kyphoscoliosis types of Ehlers-Danlos syndrome, and some cases of the classic and hypermobility forms, are inherited in an autosomal recessive pattern. In autosomal recessive inheritance, two copies of the gene in each cell are altered. Most often, the parents of an individual with an autosomal recessive disorder are carriers of one copy of the altered gene but do not show signs and symptoms of the disorder[10].
Beighton's criteria[edit source | edit]
As of July 2000, hypermobility is diagnosed using the Beighton criteria.[9] The Beighton criteria do not replace the Beighton score but instead use the previous criteria in conjunction with other symptoms. HMS is diagnosed in the presence of either two major criteria, one major and two minor criteria, or four minor criteria. The criteria are as follows:
Major criteria[edit source | edit]
- A Beighton score of 4/9 or more (either current or historic)
- Arthralgia for more than three months in four or more joints
Minor criteria[edit source | edit]
- A Beighton score of 1, 2 or 3/9 (0, 1, 2 or 3 if aged 50+)
- Arthralgia (> 3 months) in one to three joints or back pain (> 3 months), spondylosis, spondylolysis/spondylolisthesis.
- Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.
- Soft tissue rheumatism. > 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis).
- Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactyly (positive Steinberg thumb / Walker wrist signs).
- Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring.
- Eye signs: drooping eyelids, myopia or antimongoloid slant (Palpebral slant)
- Varicose veins or hernia or uterine/rectal prolapse.
Beighton score[edit source | edit]
The Beighton score is an edited version of the Carter & Wilkinson scoring system which has been used for many years as an indicator of widespread hyper-mobility. However medical professionals varied in their interpretations of the results; some accepting as low as 1/9 and some 4/9 as a diagnosis of HMS. Therefore it was incorporated, with clearer guidelines, into the Beighton Criteria. The Beighton score is measured by adding 1 point for each of the following:
- Placing flat hands on the floor with straight legs
- Left knee bending backward
- Right knee bending backward
- Left elbow bending backward
- Right elbow bending backward
- Left thumb touching the forearm
- Right thumb touching the forearm
- Left little finger bending backward past 90 degrees
- Right little finger bending backward past 90 degrees
Treatments[edit source | edit]
Physical therapy[edit source | edit]
It is important that the individual with hypermobility remain extremely fit - even more so than the average individual - to prevent recurrent injuries. Regular exercise and physical therapy or hydrotherapy can reduce symptoms of hypermobility because strong muscles increase joint stability. These treatments can also help by stretching tight, overused muscles and ensuring the person uses joints within the ideal ranges of motion, avoiding hyperextension or hyperflexion. Low-impact exercise such as Pilates or T'ai chi is usually recommended for hypermobile people as it is less likely to cause injury than high-impact exercise or contact sports.
Moist hot packs can relieve the pain of aching joints and muscles. For some patients, ice packs also help to relieve pain.
Medication[edit source | edit]
Medications frequently used to reduce pain and inflammation caused by hypermobility include analgesics, anti-inflammatory drugs (though these have been linked with an increase in pain and joint instability for some sufferers), and tricyclic antidepressants. Some people with hypermobility may benefit from other medications such as steroid injections or gabapentin, a drug originally used for treating epilepsy. Europe is far ahead of the United States in terms of HMS recognition and its knowledge of how to treat it{{www.hypermobility.org}}. Tramadol, a non-narcotic yet opioid pain reliever that is nearly as effective as narcotics, has been used in England to treat HMS joint pain, and it is available either by prescription from a doctor in the United States or from Mexico. Benzodiazapines are also used in HMS sufferers who experience painful muscles spasms around loose joints.
Lifestyle modification[edit source | edit]
For some people with hypermobility, lifestyle changes decrease the severity of symptoms. For example:
- If writing is painful, people may be able to reduce the pain by typing.
- If typing is painful, they may try voice control software for their computer or a more ergonomic keyboard.
- If standing is painful, keep a bend in the knees or use a wheelchair.
- Avoid activities that can bring on symptoms. These include standing, stretching the joints (such as in some forms of yoga), and lifting heavy objects or weights.
- A decrease in heavy exercise, such as lifting heavy weights or running with wrist weights, which can be jarring to many joints, can be replaced with a more gentle run on an elliptical machine. Swimming fans could try using a kickboard to exercise in a pool (careful not to hyperextend knees while kicking the water and keep a bend in your elbows if you swim; if you have weak shoulders then avoid "arms-only" "pull" exercises in the water and try to focus on kicking without hyperextension while keeping a relaxed pace with your arms.)
- Posture should be closely monitored. Weakened ligaments and muscles contribute to poor posture, which may result in numerous other medical conditions. Isometric exercises and attention to where joints are (avoid crossing legs for example if you have hip problems) are helpful to keep posture from leading to more pain.
- Remember to ask for help when needed, as to not risk excessive strain when extra assistance can be acquired.
Other treatments[edit source | edit]
- Bracing to support weak joints may be helpful when joints are injured or painful, but caution must be used not to weaken the joints further.
- Prolotherapy injections have allegedly been reported to work for some, according to testimonies. Though not a typical route to take, it may be preferable to steroid injections for some, as it can strengthen degenerate tendons and ligaments, as opposed to masking the symptoms of joint injury through means of corticosteroid injection. Prolotherapy works by injecting an irritant solution, usually between 12.5% to 25% dextrose or P2G (2% Phenol, 25% Glucose, 25% Glycerin), into the superior and inferior ends of a ligament. This creates a localized inflammatory response in the ligament, causing an increase in growth factors which assists collagen formation in the injected structures, thus repairing and stengthening them. This allows the muscles to then relax as they are no longer tightening up due to overcompensating for the injured connective tissue of the affected joint. It is generally suggested that patients with HMS take longer to respond to prolotherapy compared to the average patient, and full recovery can potentially take months to years depending on the methods through which prolotherapy is given.
References[edit source | edit]
- ^ a b c d Simpson, MR (September 2006). "Benign joint hypermobility syndrome: evaluation, diagnosis, and mabᎬᏁᏁᎽ ᎪᏁᎠ mᎪᎠᎠᎥᎬnagement.". The Journal of the American Osteopathic Association 106 (9): 531–536. PMID 17079522.
- ^ WebMD (2013). Found by http://www.webmd.com/fibromyalgia/guide/fibromyalgia-causes
- ^ Martin-Santos R, Bulbena A, Porta M, Gago J, Molina L, Duró JC (November 1998). "Association Between Joint Hypermobility Syndrome and Panic Disorder". American Journal of Psychiatry 155 (11): 1578–1583. PMID 9812121.
- ^ Keer, Rosemary; Rodney Grahame (2003). Hypermobility syndrome : recognition and management for physiotherapists. Edinburgh: Butterworth-Heinemann. p. 71. ISBN 0-7506-5390-6. "Asian Indians were found by Wordsworth et al. (1987) to be significantly more mobile than English Caucasians."
- ^ "Joint hypermobility". Arthritis Research UK.
- ^ Levy, Howard (2004). “The Ehlers Danlos Syndrome, Hypermobility Type.” University of Washington: NIH. Retrieved from http://newtons-online.net/documents/EDS%20b.pdf
- ^ "Rare illnesses and disabilities".
- ^ Zweers MC, Kucharekova M, Schalkwijk J (March 2005). "Tenascin-X: a candidate gene for benign joint hypermobility syndrome and hypermobility type Ehlers-Danlos syndrome?". Ann. Rheum. Dis. 64 (3): 504–5. doi:10.1136/ard.2004.026559. PMC 1755395. PMID 15708907.
- ^ Grahame R. The revised (Beighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol. 2000;27:1777–1779
Grahame R. The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol. 2000;27:1777–1779
[10] Genetics Home Reference - [1]
External links[edit source | edit]
- Gurley-Green S (May 2001). "Living with the hypermobility syndrome". Rheumatology (Oxford) 40 (5): 487–9. doi:10.1093/rheumatology/40.5.487. PMID 11371654.
- Hypermobile - A blog about Ehlers Danlos Syndrome The lack of clinical distinction between the hypermobility type of Ehlers–Danlos syndrome and the joint hypermobility syndrome (a.k.a. hypermobility syndrome)
- "Joint hypermobility syndrome". Indian Journal of Rheumatology 5 (4): 150.
- The Disability Handbook on Arthritis
- Martín-Santos R, Bulbena A, Porta M, Gago J, Molina L, Duró JC (November 1998). "Association between joint hypermobility syndrome and panic disorder". Am J Psychiatry 155 (11): 1578–83. PMID 9812121.
- The Hypermobility Syndrome Association (HMSA) is a charity run by and for people diagnosed with the Hypermobility Syndrome (HMS)
Systemic Connective tissue disorders (M32–M36, 710)
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General |
Systemic lupus erythematosus: Drug-induced SLE · Libman-Sacks endocarditis
Inflammatory myopathy/Myositis: Dermatopolymyositis (Dermatomyositis/Juvenile dermatomyositis, Polymyositis) · Inclusion body myositis
Scleroderma: Systemic scleroderma (Progressive systemic sclerosis, CREST syndrome)
Overlap syndrome / Mixed connective tissue disease
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Other hypersensitivity/autoimmune |
Sjögren's syndrome
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Other |
Behçet's disease · Polymyalgia rheumatica · Eosinophilic fasciitis · Eosinophilia–myalgia syndrome · fibrillin (Marfan syndrome, Congenital contractural arachnodactyly)
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anat (h/n, u, t/d, a/p, l)/phys/devp/hist
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noco (m, s, c)/cong (d)/tumr, sysi/epon, injr
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Soft tissue disorders / Rheumatism / Connective tissue arthropathy (M65–M79, 725–728)
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Capsular joint |
Synoviopathy
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Synovitis/Tenosynovitis (Calcific tendinitis, Stenosing tenosynovitis, Trigger finger, DeQuervain's syndrome) · Transient synovitis · Ganglion cyst
osteochondromatosis (Synovial osteochondromatosis) · Plica syndrome
villonodular synovitis (Giant cell tumor of the tendon sheath)
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Bursopathy
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Bursitis (Olecranon, Prepatellar, Trochanteric, Subacromial, Achilles, Retrocalcaneal, Ischial, Iliopsoas) · Synovial cyst (Baker's cyst) · Calcific bursitis
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Noncapsular joint |
Ligamentopathy
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Ligamentous laxity · Hypermobility
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Enthesopathy/Enthesitis
(and general tendinopathy)
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upper limb (Adhesive capsulitis of shoulder, Impingement syndrome Rotator cuff tear, Golfer's elbow, Tennis elbow)
lower limb (Iliotibial band syndrome, Patellar tendinitis, Achilles tendinitis, Calcaneal spur, Metatarsalgia) · Bone spur
other/general: Tendinitis · Tendinosis
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Nonjoint |
Fasciopathy
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Fasciitis: Plantar · Nodular · Necrotizing · Eosinophilic
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Fibromatosis/contracture
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Dupuytren's contracture · Plantar fibromatosis · Aggressive fibromatosis · Knuckle pads
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noco (arth/defr/back/soft)/cong, sysi/epon, injr
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anat (h/n, u, t/d, a/p, l)/phys/devp/hist
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noco (m, s, c)/cong (d)/tumr, sysi/epon, injr
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