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Hypermobility (EDS III) Syndrome INFO Page

There is much controversy over Hypermobility Syndrome (HMS) especially since there is no standardized test for it's diagnosis or treatment. There is also some question as to whether HMS and EDS III are one and the same; most authorities consider them to be and after much research, so do I. One such authority that believes they are the same is The Ehlers-Danlos National Foundation who on their web site lists Hypermobility as formerly being called EDS type III.  HMS affects the collagenous tissues of the body which include bone, cartilage, tendons, ligaments, and skin.

The following information seen in green has been copied directly from the The Elhers-Danlos Nation Foundation site, though parts not pertaining to EDS III hypermobility have been omitted.

Types of Ehlers-Danlos Syndrome
Ehlers-Danlos syndrome (EDS) is a heterogeneous group of heritable connective tissue disorders characterized by articular (joint) hypermobility, skin extensibility and tissue fragility. Individuals with EDS have a defect in their connective tissue. It is this tissue that provides support to many body parts such as the skin, muscles, ligaments and organs. The fragile skin and unstable joints found in EDS are due to faulty collagen. Collagen is a protein that acts like glue in the body adding strength and elasticity to connective tissue.

There are six major types of EDS. The different types of EDS are classified according to the signs and symptoms that are manifested. Each type of EDS is a distinct disorder that "runs true" in a family. This means that an individual with Vascular Type EDS will not have a child with Classical Type EDS.

Hypermobility (Formerly EDS Type III)
Joint hypermobility is the dominant clinical manifestation. Generalized joint hypermobility that affects large (elbows, knees) and small (fingers and toes) joints is evident in the Hypermobility Type. Recurring joint subluxations and dislocations are common occurrences. Certain joints, such as the shoulder, patella(knee cap), and temporomandibular(jaw) joint dislocate frequently. The skin involvement (hyperextensibility and/or smooth velvety skin) as well as bruising tendencies in the Hypermobility Type are present but variable in severity.

Chronic joint and limb pain is a common complaint amongst individuals with the Hypermobility Type. Skeletal X-rays are normal. Musculoskeletal pain is early onset, chronic and may be debilitating. The anatomical distribution is wide and tender points can sometimes be elicited.

To date, no distinctive biochemical collagen finding has been identified by researchers. The Hypermobility Type of EDS is inherited in an autosomal dominant manner.

The following information in red is from the Site called Health A to Z you can link there to read the rest of the information.

Definition

The Ehlers-Danlos syndromes (EDS) refer to a group of inherited disorders that affect collagen structure and function. Genetic abnormalities in the manufacturing of collagen within the body affect connective tissues, causing them to be abnormally weak.

Description

Collagen is a strong, fibrous protein that lends strength and elasticity to connective tissues such as the skin, tendons, organ walls, cartilage, and blood vessels. Each of these connective tissues requires collagen tailored to meet its specific purposes. The many roles of collagen are reflected in the number of genes dedicated to its production. There are at least 28 genes in humans that encode at least 19 different types of collagen. Mutations in these genes can affect basic construction as well as the fine-tuned processing of the collagen.

EDS was originally described by Dr. Van Meekeren in 1682. Dr. Ehlers and Dr. Danlos further characterized the disease in 1901 and 1908, respectively. Today, according to the Ehlers-Danlos National Foundation, one in 5,000 to one in 10,000 people are affected by some form of EDS.

EDS is a group of genetic disorders that usually affects the skin, ligaments, joints, and blood vessels. Classification of EDS types was revised in 1997. The new classification involves categorizing the different forms of EDS into six major sub-types, including classical, hypermobility, vascular, kyphoscoliosis, arthrochalasia, and dermatosparaxis, and a collection of rare or poorly defined varieties. This new classification is simpler and based more on descriptions of the actual symptoms.

 

There are many people in society that are hypermobile, meaning that they have one or more joints that can move beyond the normal range considering their age, gender and ethnicity (populations of Asian descent are most mobile while those of European descent are the least) , but they are healthy and have little or no problems arising from their increased mobility. Some of these people are not born hypermobile but acquire it through training, like many dancers for example. HMS, however, is a pain syndrome that affects the hypermobile and you do not have to be hypermobile in all joints to be a sufferer. Some rheumatologists see HMS more as a physiological problem than a pathological one. Meaning that they think that the hypermobility seen in HMS patients is nothing more than a variation in joint motion in the uppermost distribution of a given population. They acknowledge that exercise could cause pain in these individuals but that this is a minor problem unlikely to impair their quality of life. Therefore making HMS a condition not to be taken seriously. BUT there are other rheumatologists that view HMS as a pathological entity and consider it to be a part of a group called "Heritable Connective Tissue Disorders" or HDCT's. This view is based on the evidence of skin involvement seen in HMS patients such as increased skin stretchiness, thin crinkly scars and stretch marks (often in places where no significant weight gain has occurred to warrant them, most often on thighs and lower back)

It is often unrecognized and often difficult to diagnose resulting in the sufferer often being labeled as "neurotic" or a "hypochondriac". HMS/EDS III goes unrecognized because suffers often have a wide variety of seemingly unrelated symptoms. Also they often cannot connect the pain with a specific event or action, and if they can the pain seems disproportionately high when compared to the event and the currently mobility of the area. Often HMS/EDS III sufferers feel pain and stiffness but retain enough mobility in the joint to be seen as normal. However, for the individual the movement may be greatly restricted in comparison with their normal hypermobile range. HMS sufferers do not tolerate repetitive or excessive activity well and tend to suffer symptoms after the activity rather than during it (24-48 hrs later). This makes it hard to connect the pain to the cause. Especially since the excessive activity could have been an everyday activity done a little longer than normal or a little more frequently. The symptoms are often similar to those seen is soft tissue strain but there is often no accompanying signs of inflammation. HMS patients also note that they cannot maintain a sustained posture such as sitting or standing for prolonged periods. They are often considered fidgety people who feel like they are constantly trying to get comfortable.

Since HMS affects collagen tissues there can be associated problems in other systems of the body. Patients report that they bruise more easily and often have no memory of how or when they could have done it. There is reports of higher incidences of varicose veins in HMS and the suggestion that HMS can also have laxity involving blood vessels. There is also evidence to suggest a higher incidence of uterine prolapse and abdominal hernias. Getting back to joints, dislocations and subluxations are more common in those with HMS. Spontaneous relocation often occurs (specially the first time). Dislocations and subluxations thereafter often occur with no trauma and some people can sublux and relocate joints at will with little pain or discomfort (most commonly the shoulder or patella=knee cap ).

There are other physiological and psychological abnormalities that have come to light in connection with HMS. Impaired joint proprioceptive acuity (the proprioceptive system communicates with the brain so that even if your eyes are closed, you can tell which direction your arms are pointed, whether you are upside-down or just feel the presence of your own body. HMS sufferer often have an impaired ability to accurately know where their limbs etc are in space without looking at them. This makes it more easy to over extend joints and not know it). People with HMS also experience a lack of efficacy of local anesthetics either given topically or by injection. This is often problematic and distressing when having dental surgery, during childbirth (using epidural) or in minor surgery. Pain enhancement is another common problem leading to the development of a full chronic pain syndrome, resulting in acute and chronic pain being the dominant factor of every day life. There is also a significant association between HMS and anxiety  states with panic attacks and phobic states. Panic disorder, agoraphobia and simple phobias are four(4) times more common among HMS patients. Recently Autonomic dysfunction was added to the list since HMS sufferer can complain of palpitations, light-headedness, dizziness or fainting, which are similar symptoms to those of patients with autonomic nervous system dysfunction.

Diagnosis of HMS often made by ruling out other rheumatological disorders, taking family history and rating hypermobility using one of the scales such as the beighton score or the more comprehensive Contompasis semi-qualitative scoring system. The most fruitful areas of the body to find hypermobility are the following

*hands with fingers outstretched

* wrist flexion and extension

* lateral rotation of the shoulder (90 degrees or more)

* cervical spine = neck (rotation to right or left to 90 deg or more or lateral flexion to left or right greater than 60 deg)

*temporomandibular joint = jaw joint (ability to insert four finger vertically stacked into mouth)

*Dorsal spine ( ability to rotate passively to 90deg)

*hip rotation (medial + lateral rotation = 150 deg)

*ankle (flexion + extension = 90 deg or more)

*Passive hyperextension of the MTP of big toe joint to 90 deg or more

(passive means that another person moves or you move it without help or resistance from the muscles of the joint in question)

For the most up to date compiled information on HMS get the following book

"Hypermobility Syndrome: Recognition and Management for Physiotherapists"

by Rosemary Keer and Rodney Grahame (almost all of the information and research findings found here have been compiled by them)

As far as I can tell it is one of the few books out their dedicated to this topic

I have it and it has been a great information source and explanation source.

Also for updates and information on current research see Hypermobility Association  and The Ehlers-Danlos National Foundation web sites

 

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