Hypermobility

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Hypermobility
Classification and external resources
Hypermobile fingers and thumb.jpg
Hypermobile fingers and thumb
ICD-10M35.7
ICD-9728.5
OMIM147900
DiseasesDB31101
MedlinePlus003295
MeSHD007593
 
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Hypermobility
Classification and external resources
Hypermobile fingers and thumb.jpg
Hypermobile fingers and thumb
ICD-10M35.7
ICD-9728.5
OMIM147900
DiseasesDB31101
MedlinePlus003295
MeSHD007593

Hypermobility describes joints that stretch further 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 one or more joints throughout the body.

Causes[edit]

Hypermobility generally results from one or more of the following:

These abnormalities cause abnormal joint stress, meaning that the joints can wear out, leading to osteoarthritis.

The condition tends to run in families, suggesting that 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 be taken literally, as hypermobile joints are not doubled/extra in any sense.

Most people have hypermobility with no other symptoms. Approximately 5% of the healthy population have one or more hypermobile joints. However, people with "joint hypermobility syndrome" are subject to 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 weakness in the ligaments that support the joints). Hypermobility syndrome can lead to chronic pain or even disability in severe cases. Musical instrumentalists with hypermobile fingers may have difficulties when fingers collapse into the finger locking position or may display superior abilities due to their increased range of motion for fingering - such as in playing a violin or cello.

Hypermobility may be symptomatic of a serious medical condition, such as Stickler Syndrome, Ehlers-Danlos syndrome,[1] Marfan syndrome,[1] Loeys-Dietz syndrome, rheumatoid arthritis, osteogenesis imperfecta,[1] lupus, polio, Down syndrome,[1] morquio syndrome, cleidocranial dysostosis or myotonia congenita.

Hypermobility has been associated with chronic fatigue syndrome and fibromyalgia. Hypermobility causes physical trauma (in the form of joint dislocations, joint subluxations, joint instability, sprains, etc.). These conditions often, in turn, cause physical and/or emotional trauma and are possible triggers for conditions such as fibromyalgia.[2]

During pregnancy, the body releases certain hormones that alter ligament physiology easing the stretching needed to accommodate fetal growth as well as the birthing process. For women with hypermobility conditions pregnancy-related pelvic girdle, pain during pregnancy can be debilitating due to these two converging factors. Pain often inhibits such women from standing up or walking during pregnancy. The pregnant patient may be forced to use a bedpan and/or a wheelchair during pregnancy (possibly associated with permanent disabilities). The pregnant woman with hypermobile joints will often be in significant pain as muscles and joints adapt to the pregnancy.

Symptoms of hypermobility include a dull but intense pain around the knee and ankle joints and the soles of the feet. The pain and discomfort affecting these body parts can be alleviated by using custom orthoses.

Anxiety and joint hypermobility[edit]

A 1998 study linked panic disorders and joint hypermobility. The prevalence of joint hypermobility syndrome among patients with a panic disorder was 67.7% compared to the control psychiatric group (10.1%). Women and younger subjects were found to be over 20 times more likely to have hypermobile joints than their counterparts in the control group. The study also found a higher prevalence for mitral valve prolapse (8%). Depression and anxiety were other correlated symptoms.[3]

A 2003 study found that 78% of people with hypermobility also had orthostatic intolerance, which can lead to chronically high adrenalin and chronic anxiety.[4]

Hypermobility syndrome[edit]

Hypermobile metacarpo-phalangeal joints
Hyperextension thumb


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]

Current thinking suggests four causative factors:

Hypermobility can also be caused by connective tissue disorders, such as Ehlers-Danlos Syndrome (EDS) and Marfan syndrome. Joint hypermobility is a common symptom for both. EDS has numerous sub-types; most include hypermobility in some degree. When hypermobility is the main symptom, then EDS/hypermobility type is likely. People with EDS-HT suffer frequent joint dislocations and subluxations (partial/incomplete dislocations), with or without trauma, sometimes spontaneously. Commonly, hypermobility is dismissed by medical professionals as nonsignificant.[7]

Symptoms[edit]

People with hypermobility syndrome may develop other conditions caused by their unstable joints. These conditions include:

Diagnosis[edit]

Joint hypermobility shares symptoms with other conditions such as Marfan syndrome, Ehlers-Danlos Syndrome, and osteogenesis imperfecta. Experts in connective tissue disorders formally agreed that severe forms of Hypermobility Syndrome and mild forms of Ehlers-Danlos Syndrome Hypermobility Type are the same disorder.[9]

Generalized hypermobility is a common feature in all these hereditary connective tissue disorders and many features overlap, but often features are present that enable differentiating these disorders.[10] The inheritance pattern of Ehlers-Danlos syndrome varies by type. The arthrochalasia, classic, hypermobility and vascular forms usually have an autosomal dominant pattern of inheritance. Autosomal dominant inheritance occurs when one copy of a gene in each cell is sufficient to cause a disorder. In some cases, an affected person inherits the mutation from one affected parent. Other cases result from new (sporadic) gene mutations. Such cases can occur in people with no history of the disorder in their family.

The dermatosparaxis and kyphoscoliosis types of EDS 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, both 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.

Brighton criteria[edit]

As of July 2000, hypermobility was diagnosed using the Brighton criteria.[11] The Brighton 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:

Major criteria[edit]
Minor criteria[edit]

Beighton score[edit]

The Beighton score is an edited version of the Carter/Wilkinson scoring system which was used for many years as an indicator of widespread hyper-mobility. 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:

Treatments[edit]

Physical therapy[edit]

It is important that hypermobile individuals remain fit - even more so than the average individual - to prevent recurrent injuries. Regular exercise and physical therapy or hydrotherapy can reduce symptoms 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 as they are 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. In many cases alternating the two (hot and cold) helps relieve the pain.

Medication[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 sufferers may benefit from medications such as steroid injections or gabapentin, a drug originally used for treating epilepsy. 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 muscle spasms around loose joints.[citation needed]

Lifestyle modification[edit]

For some hypermobiles, lifestyle changes decrease symptom severity. In general activity that increases pain is to be avoided. For example:

Other treatments[edit]

References[edit]

  1. ^ a b c d Simpson, MR (September 2006). "Benign joint hypermobility syndrome: evaluation, diagnosis, and management.". The Journal of the American Osteopathic Association 106 (9): 531–536. PMID 17079522. 
  2. ^ "Fibromyalgia: Possible Causes and Risk Factors". Webmd.com. 2008-05-21. Retrieved 2014-03-06. 
  3. ^ 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. 
  4. ^ Gazit Y, Nahir AM, Grahame R, Jacob G. (2003). "Dysautonomia in the joint hypermobility syndrome". American Journal of Medicine 115: 33–40. doi:10.1016/s0002-9343(03)00235-3. PMID 12867232. 
  5. ^ 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." 
  6. ^ "Joint hypermobility". Arthritis Research UK. 
  7. ^ Levy, Howard (2004). “The Ehlers Danlos Syndrome, Hypermobility Type.” University of Washington: NIH. Retrieved from
  8. ^ "1.00 Musculoskeletal System-Adult". Ssa.gov. 2013-05-31. Retrieved 2014-03-06. 
  9. ^ "The lack of clinical distinction between the hypermobility type of Ehlers–Danlos syndrome and the joint hypermobility syndrome (a.k.a. hypermobility syndrome)". 
  10. ^ 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. 
  11. ^ Grahame R. The revised (Brighton 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]