What Is Hypermobility/Hypermobility Syndrome?:

What Is Hypermobility/Hypermobility Syndrome?: en.wikipedia.org/wiki/Hypermobility

Hypermobility (also called double-jointedness, hypermobility syndrome or hyperlaxity) describes joints that stretch farther than is normal.

For example, some hypermobile people can bend their thumbs backwards to their wrists, or bend their knee joints backwards.

It can affect a single joint or multiple joints throughout the body.

Hypermobility Syndrome: en.wikipedia.org/wiki/Hypermobility#Hypermobility_Syndrome

Hypermobility syndrome (known by a variety of other names, including Benign Joint Hypermobility Syndrome) is generally considered to comprise hypermobility together with other symptoms, such as myalgia and arthralgia, and extra-articular features such as skin hyperextensibility and varicose veins.

It affects more females than males.

The current diagnostic criteria for hypermobility syndrome are the Brighton criteria, which incorporates the Beighton score[1].

Hypermobility syndrome is considered by many doctors expert in hypermobility (e.g. Professor Rodney Grahame) to be equivalent to the Hypermobile Type of Ehlers-Danlos Syndrome.

People with hypermobility syndrome may develop other conditions caused by their lax connective tissues.

These conditions include:

*Gastroesophageal Reflux Disease (GERD)

*Irritable Bowel Syndrome (IBS)

*Varicose Veins

*Flat feet, pronated feet, plantar fasciitis or sesamoiditis and unsupportive shoes

*Idiopathic scoliosis

*Joint instability causing frequent sprains, tendinitis, or bursitis when doing activities that would not affect the normal individual.

*Early-onset osteoarthritis

*There is evidence linking hypermobility syndrome to anxiety and depression. [2][3]

*Subluxations or dislocations, especially in the shoulder.

*Knee pain

*Back pain, prolapsed discs or spondylolisthesis

*Hernias

*Bruising easily

*Worsening of symptoms in cold weather

*Joints that make clicking noises

*Headaches

*Susceptibility to whiplash

*Temperomandibular Joint Syndrome also known as TMJ

*Increased nerve compression disorders (i.e. carpal tunnel syndrome)

*Mitral valve prolapse

*Uterine prolapse

*Flu [Formal Larinal Usekretinals]

What Is Hypermobility/Hypermobility Syndrome?: www.hypermobility.org/whatishms.php

Connective tissue proteins such as collagen give the body its intrinsic toughness.

When they are differently formed, the results are mainly felt in the \"moving parts\" - the joints, muscles, tendons, ligaments - which are laxer and more fragile than is the case for most people.

The result is joint laxity with hypermobility and with it comes vulnerability to the effects of injury.

The Hypermobility Syndrome is said to exist when symptoms are produced, a state of affairs that may affect only a minority of hypermobile people.

It is probably more correct to refer to Hypermobility Syndromes (in the plural) as a family of related genetically-based conditions which differ not only in the particular protein affected, but also in the degree of difference of formation.

Thus at one end of the spectrum are the diseases with the potentially serious complications such as Marfan Syndrome or Ehlers-Danlos Syndrome Vascular Type (formally EDS IV).

At the other end are what is now called on good evidence Benign Joint Hypermobility Syndrome (BJHS) and Ehlers-Danlos Hypermobile Type (formerly EDS III), which may be one and the same.

These may cause troublesome and persistent problems, but do not affect the vital organs and thus do not pose a serious threat to life.

Although there is still much to learn, understanding of these conditions is advancing and the knowledge gained can help people deal with the various aspects.

What Is Hypermobility/Hypermobility Syndrome?: www.bbc.co.uk/dna/h2g2/A20701496

A surprising number of people have joint hypermobility and suffer no ill effects. This is probably why not many people know, nor have heard of, the syndrome (members of the medical profession included).

Hypermobility is caused when the muscles, tendons and ligaments around the joints are far more supple than is usual for the majority of people.

You may have been able to move your joints into odd positions as a child and thought that you were 'double-jointed'.

Hypermobility can affect one or all of your joints.

Mild hypermobility in the most part will probably not affect your everyday life, but in severe cases it can cause a lot of joint pain and may even cause you to dislocate a joint, and not only your shoulder.

Hypermobility has also been connected to Fibromyalgia, osteogenesis imperfecta, Marfan Syndrome and Ehlers-Danlos Syndrome.

In fact some doctors think that Hypermobility Syndrome may be a mild form of Ehlers-Danlos Syndrome.

What Is Hypermobility/Hypermobility Syndrome?: www.cafamily.org.uk/Direct/h56.html

condition in which the protein, collagen, which makes up the supporting tissues and gives the body its intrinsic toughness, is more flexible than usual.

A person's joints are lax because they have inherited looser and more brittle connective tissue, particularly their ligaments, tendons, joints and muscles.

This makes the joints more mobile, sometimes unstable, thus more prone to injury.

Some children may have more severe problems that may lead to subluxations (excessive movement of the joint) or dislocations.

The degree of difference and hence tendency towards painful symptoms, varies from individual to individual, even in the same family.

HMS is a composite term bringing together all the various difficulties that may arise in people with hypermobile or lax joints.

It is probably more correct to refer to the Hypermobility syndromes (in the plural) as a family of genetically based conditions which differ not only in the particular protein affected, but also in the degree of difference of formation.

At one end of the spectrum are the diseases with potentially serious complications such as Marfan syndrome or Ehlers-Danlos syndrome Vascular Type (formally EDS IV) see entries.

At the other end, are what is now called, on good evidence, the Benign Joint Hypermobility syndrome (BJHS) or Ehlers-Danlos Hypermobile Type (formerly EDS III).

These may cause troublesome and persistent problems but do not affect the vital organs and thus do not pose a serious threat to life.

Because joints do not look arthritic, the problem is often overlooked by health professionals.

Although some people with BJHS have little or no trouble, in others, pain can be a recurring or, in some, even a constant, problem.

This renders them prone to the effects of injury and over-use resulting in acute (short-term) pain on prolonged and unaccustomed exercise.

More chronic day-in day-out pain is also seen and may require a variety of measures, which can vary from one person to another in order to control it.

About seven to ten per cent of the population of school age children has been found to have loose joints and occasional pain in the joints and muscles, especially after exercise or at night.

Most children with HMS complain of joint pains in the evening or sometime after exercise.

These mostly occur in the knees, ankles or non specifically in the legs.

Sometimes the joints may appear to be swollen.

Swelling should be treated as with any injury: RICE (rest, ice, compression, and elevation).

Young children generally do not like ice, but it can be used with older children.

If the joint pain persists, a doctor should be consulted.

Children with HMS often wake up in the night complaining of pain in the legs.

For the majority, symptoms will improve as they grow older as their supporting muscles and ligaments get tighter and this is why this used to be called 'Growing Pains' which we now feel does not exist.

Others may need to have physiotherapy, occupational therapy, podiatry and pain management through psychology, to help strengthen specific muscles that stabilise joints and cope with their pain.

It is important that children continue to exercise and do sport to build up their strength and muscles.

What Is Hypermobility/Hypermobility Syndrome?: www.benefitsnow.co.uk/handbook/hypermobility.asp

The hypermobility syndrome (HMS) is one of a group of inherited diseases which affect the connective tissues of the body.

It is a multi-system disorder which may result in a wide variety of clinical features and disabilities.

Fibrous proteins (collagens, elastins, fibrillins) give the body its strength. A defect in genetic information which determines the biochemical structure and strength of those proteins may cause structural weakness in muscle, tendon, ligament cartilage, bone, the blood vessels, eyes and skin.

The clinical effects depend on the function of the particular tissue affected.

Joints may become lax, unstable and hypermobile with increased tendency to dislocation and vulnerability to the effects of injury.

Bones may become osteoporotic, predisposing to fractures.

The body-shape may take on characteristic body proportions (called \"Marfanoid\") with long slender limbs, twisting of the spine and chest deformity. Skin shows increased stretchiness and the blood vessels (vasculature) may also be affected in certain of the diseases associated with HMS, such as the Ehlers-Danlos syndrome, with involvement of the heart and major vessels.

Eye involvement may occur as dislocation of the lens in the Marfan syndrome where the lens ligament is lax and unable to hold the lens in a stable manner.

Approximately 10% of the adult population is hypermobile.

The prevalence varies among different ethnic groups and is greater in women.

The majority of affected people have no significant disability.

People in some occupations or pursuits find it an asset, for example ballet dancers, gymnasts, yoga, violin players, flautists, snooker players.

However, any hypermobile joint is vulnerable.

The hypermobile back if used excessively, may be subject to prolapsed discs, stress fractures, spinal narrowing and other mechanical problems.

People with severe forms of hypermobility syndromes (particularly those with greater degrees of tissue laxity and fragility) may lead a restricted life because their tissues are so fragile.

Symptoms tend to be similar in hypermobility syndromes irrespective of the cause.

Due to the weaknesses in muscle, ligaments, tendons and cartilage, etc, there may be joint pain, dislocations of joints, and fractures.

In any hypermobile joint, \"over-use\" injury can cause pain and loss of function. The same is true of the back.

Stress fractures of bone are not uncommon.

Joint and/or muscle pain may be a prominent symptom. Hypermobility may be a serious potential source of problems in children. Many will develop osteoarthritis in time.

With age, joint hypermobility declines, but other complications resulting from HMS may arise, such as secondary osteoarthritis; osteoporosis with resultant fractures; and loss of balance particularly in the older person, which may result in falls, especially if there is also impaired vision.

What Is Hypermobility/Hypermobility Syndrome?: www.arc.org.uk/arthinfo/patpubs/6019/6019.asp

Introduction: www.arc.org.uk/arthinfo/patpubs/6019/6019.asp

If you have joint hypermobility, this booklet will help you, your family and friends.

It explains what joint hypermobility is, what causes it, the usual symptoms, and what can be done to treat it.

It also explains what you can do to help yourself – such as avoiding certain sports which will make your symptoms worse.

Joint hypermobility is not a type of arthritis (it just means that you can move some or all your body joints in a way that most people cannot) and it only affects a small number of people.

It can be very mild with few symptoms and not need treatment, or it can be more severe in which case the joints may be easily dislocated.

It can also help some people, for example dancers and musicians, who need flexibility in their joints in order to perform.

Very rarely, joint hypermobility is part of a more serious inherited illness. We discuss this briefly at the end of this booklet.

The 'Useful addresses' section includes details of organizations which can provide further information on these rarer conditions, as well as details of how to contact the Arthritis Research Campaign (arc).

What is joint hypermobility?: www.arc.org.uk/arthinfo/patpubs/6019/6019.asp

If you have joint hypermobility, some or all of your joints will have an unusually large range of movement.

You may have known that your joints were very 'supple' even from an early age.

You may have been 'double-jointed', or able to twist your limbs into unusual positions.

Athletes sometimes train to achieve what they call 'flexibility'. Some doctors call it 'joint hyperlaxity'.

Women are often more supple than men of the same age, and certain racial groups are more supple than others.

In general, we become less supple as we get older.

But there are always exceptions – the music hall contortionist, Norwood the Amazing Flexible Fellow, enjoyed a long career as a circus performer and could twist his body into amazing shapes until he was aged 80.

The Beighton Score - The New Diagnostic Criteria for HMS @ tinyurl.com/6u7ke3

The Brighton Score - The New Diagnostic Criteria for HMS

An important landmark was passed in July 2000 with the publication in the Journal of Rheumatology (2000; 27: 1777-1779) of the Brighton Diagnostic criteria for the Benign Joint Hypermobility Syndrome (BJHS).
WHY IS IT IMPORTANT TO HAVE SUCH A SET OF CRITERIA FOR THE BJHS?

Hitherto, there has been no consensus view on how the BJHS should be defined. Without generally agreed criteria for the diagnosis, it is very much a hit and miss affair and doctors confronted with an affected patient may come to very different conclusions regarding whether BJHS is present. We know that that happens quite a lot. To take one example, doctors differ considerably in the number of hypermobile joints they require to be present before they accept the diagnosis on hypermobility syndrome. In a recent survey out of 319 consultant rheumatologists 185 required a Beighton score of 5 while 92 required 3, 3 required 1, while 35 preferred not commit themselves.
WHY ARE THE BRIGHTON CRITERIA REPLACING THE WELL TRIED BEIGHTON SCORE?

The answer is that they are not. The Beighton scoring system has been used for over 30 years and have withstood the test of time. But as Professor Beighton has pointed out they were never designed for assisting in diagnosis in the clinical situation. They were in fact developed for epidemiological studies and for this they were invaluable.

They are not adequate for diagnosis for two reasons. Firstly, they only cover a sample of joints in the body, and reliance on them may lead to hypermobility being missed in other joints: those not covered in the Beighton 9-point score. Secondly, as we always knew, hypermobility syndrome means hypermobility + symptoms, and we now know there other features in BJHS (changes in skin – increase skin stretchiness, thin scar formation, marfanoid body shape) so that a measurement of joint hypermobility alone is no longer enough. The new criteria include symptoms and these other features. The Beighton score remains an integral component. It is not being abandoned!
HOW RELIABLE ARE THE NEW BRIGHTON CRITERIA?

The new criteria have been validated in adults but not yet in children below the age of 16 years. The process of validation requires the criteria to be “tested” among a group of BJHS patients and also among a group of volunteers who have not got the syndrome. For criteria to declared valid they have to show that they are capable of picking up the condition in the majority who have it (sensitivity), but also of ruling out the condition in the majority of people who do not (specificity). In the case of the Brighton criteria the sensitivity and specificity were both very high (93% each).
HOW WILL THEY HELP?

Doctors and research workers can use the criteria in their work. If a person fulfils the criteria, then, for all intents and purposes they may be confidently considered to have the BJHS. We therefore now have a reliable benchmark that we did not have before. It is anticipated that they will be widely used in future research. Indeed, since their publication they have already been incorporated into research protocols. For example, they will constitute an important linchpin in the gene-mapping project reported elsewhere in the website.
WHAT DO THE BRIGHTON CRITERIA CONSIST OF?

The actual criteria have been reproduced (as published) below.
REVISED DIAGNOSTIC CRITERIA FOR THE BENIGN JOINT HYPERMOBILITY SYNDROME (BJHS)

Major Criteria

* A Beighton score of 4/9 or greater (either currently or historically)
* Arthralgia for longer than 3 months in 4 or more joints

Minor Criteria

* 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, arachnodactily [positive Steinberg/wrist signs].
* Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring.
* Eye signs: drooping eyelids or myopia or antimongoloid slant.
* Varicose veins or hernia or uterine/rectal prolapse.

The BJHS is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative.

BJHS is excluded by presence of Marfan or Ehlers-Danlos syndromes (other than the EDS Hypermobility type (formerly EDS III) as defined by the Ghent 1996 ( and the Villefranche 1998 (9) criteria respectively). Criteria Major 1 and Minor 1 are mutually exclusive as are Major 2 and Minor 2.

Professor Rodney Grahame

UCL Hospitals, LONDON

The Beighton Score @ tinyurl.com/3k97ak

Beighton Score

The Beighton modification of the Carter & Wilkinson scoring system has been used for many years as an indicator of widesparead hypermobility.

A high Beighton score by itself does not mean that an individual has HMS.

It simply means that the individual has widespread hypermobility.

Diagnosis of Hypermobility Syndrome or HMS should be made using the Brighton Criteria.

The Beighton score is calculated as follows @ tinyurl.com/3k97ak