“Joint hypermobility syndrome, alternatively termed Ehlers-Danlos syndrome hypermobility type (JHS/EDS-HT), is likely the most common, though the least recognized heritable connective tissue disorder.” ~ Joint hypermobility syndrome (a.k.a. Ehlers-Danlos Syndrome, Hypermobility Type): an updated critique. Castori M
Frequently hypermobility syndrome is not recognized or is considered unimportant unless a family member has a brain or aortic aneurysm due to congenital collagen weakness. I see quite a few novice runners in the training team who have problems caused by hypermobility – more than would be expected in the general population. It makes sense since I am looking at a subgroup that has problems.
There is a broad spectrum of hypermobility syndromes ranging from a little more than normal flexibility to named genetic syndromes that weaken multiple systems like Marfan Syndrome and Ehlers-Danlos Syndrome.
The term, “hypermobility syndrome” is old and has been replaced in much of the literature but is still in common use. The terminology is currently too nonspecific, lumping together some high risk systemic collagen problems along with more common situations that require only recognition of lower stress tolerance.
People with hypermobility syndrome often seek our help for multiple problems. Some examples are:
- Joint instability causing frequent sprains, muscle strains and overstress, tendinitis and bursitis.
- Increased injury by impacts in contact sports or accidents.
- Fibromyalgia Syndrome (FMS).
- Joint pain including wrists, shoulders, hips, knees, feet (bunions, pronation)
- Early-onset degenerative joint disease (DJD) (Osteoarthritis)
- Early, wide spread spinal disc degeneration
- Temporomandibular Joint Syndrome (TMJ syndrome)
- Carpal tunnel syndrome
The more severe conditions warrant monitoring the patient for vascular problems. They also require chiropractors and PTs to be more selective and gentle with manipulation / mobilization, stretching and exercises. It generally takes more effort to develop an effective treatment program and more work and time on the patient’s part to manage the situation.
Your brain and spinal cord know where all your parts are and how they are moving by way of sensory input. Much of this is from stretch sensation which is diminished in very flexible people. If your central nervous system does not know your joint and body position instantly and there is a delay due to increased stretching, it can move farther before reacting. This increases damage during impact and also during walking and running. It forces your muscles to tighten on both sides of a joint during motion to stiffen it and provide information. This creates tight sore muscles and tendinitis or bursitis. These muscles and tendons as well as ligaments and joint capsules can overstretch as well, which promotes further displacement especially in the ankle and foot.
Diagnosing HMS
I test single leg standing on almost all of my patients. Their ability to balance with minimal ankle wobbling is important. When I test patients with eyes closed and, in the extreme, with their head tilted back, they are prevented from relying on vision or inner ear for balance. They are then forced to rely on information from their foot and the rest of their body to determine where they are and maintain balance. Eyes closed is a good reflection of how stable you are with the lights off or when texting while standing or walking.
I look at their posture for knees that go backward (Genu recurvatum) and elbows that hyper extend or thumbs that can easily reach their wrist. Excessive ankle pronation with arch flattening and bunions is an indication of overstretch and excessive shearing motion at the big toe. These are indications that their collagen is not supporting their structure well.
Female hormones (just before their period) and to a much greater extent, during later pregnancy, greatly increase mobility. Pregnant women have run Marathons but the question as to whether it is good for them or the baby remains controversial. Children are often more mobile. There also seems to be a difference between ethnicities around the world. This needs to be taken into account when determining whether the patient is normal or hypermobile and what activities are appropriate.
Since July 2000, hypermobility has frequently been diagnosed using the Beighton criteria. The Beighton criteria incorporate the Beighton score along with other symptoms.
The Beighton score: Add 1 point for each of the following:
- Placing flat hands on the floor with straight legs
- Left knee bending backward (passive knee hyperextension over 10°)
- Right knee bending backward (passive knee hyperextension over 10°)
- Left elbow bending backward (passive hyperextension of the elbows over 10°)
- Right elbow bending backward (passive hyperextension of the elbows over 10°)
- Left thumb touching the forearm (passive flexion of the thumbs to the flexor surface of the forearms)
- Right thumb touching the forearm (passive flexion of the thumbs to the flexor surface of the forearms)
- Left little finger bending backward past 90 degrees (Passive dorsiflexion of the fifth finger of the hands over 90)
- Right little finger bending backward past 90 degrees (Passive dorsiflexion of the fifth finger of the hands over 90)
HMS is diagnosed in the presence of two major criteria, one major and two minor criteria, or four minor criteria. The Beighton criteria have not been very well supported in the literature in that the minor criteria do not add much to the correlations.
Major criteria:
- A Beighton score of 4/9 or more (either current or historic)
- Arthralgia in four or more joints for more than three months.
- Criteria major 1 and minor 1 are mutually exclusive as are major 2 and minor 2.
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, spondylolisthesis/lysis.
- Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.
- Soft tissue rheumtism. > 3 lesions (eg. 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.
Check back tomorrow for part two: Managing HMS
Image courtesy of Ambro at FreeDigitalPhotos.net
Frequently hypermobility syndrome is not recognized or is considered unimportant unless a family member has a brain or aortic aneurysm due to congenital collagen weakness. I see quite a few novice runners in the training team who have problems caused by hypermobility – more than would be expected in the general population. It makes sense since I am looking at a subgroup that has problems.
There is a broad spectrum of hypermobility syndromes ranging from a little more than normal flexibility to named genetic syndromes that weaken multiple systems like Marfan Syndrome and Ehlers-Danlos Syndrome.
The term, “hypermobility syndrome” is old and has been replaced in much of the literature but is still in common use. The terminology is currently too nonspecific, lumping together some high risk systemic collagen problems along with more common situations that require only recognition of lower stress tolerance.
People with hypermobility syndrome often seek our help for multiple problems. Some examples are:
- Joint instability causing frequent sprains, muscle strains and overstress, tendinitis and bursitis.
- Increased injury by impacts in contact sports or accidents.
- Fibromyalgia Syndrome (FMS).
- Joint pain including wrists, shoulders, hips, knees, feet (bunions, pronation)
- Early-onset degenerative joint disease (DJD) (Osteoarthritis)
- Early, wide spread spinal disc degeneration
- Temporomandibular Joint Syndrome (TMJ syndrome)
- Carpal tunnel syndrome
The more severe conditions warrant monitoring the patient for vascular problems. They also require chiropractors and PTs to be more selective and gentle with manipulation / mobilization, stretching and exercises. It generally takes more effort to develop an effective treatment program and more work and time on the patient’s part to manage the situation.
Your brain and spinal cord know where all your parts are and how they are moving by way of sensory input. Much of this is from stretch sensation which is diminished in very flexible people. If your central nervous system does not know your joint and body position instantly and there is a delay due to increased stretching, it can move farther before reacting. This increases damage during impact and also during walking and running. It forces your muscles to tighten on both sides of a joint during motion to stiffen it and provide information. This creates tight sore muscles and tendinitis or bursitis. These muscles and tendons as well as ligaments and joint capsules can overstretch as well, which promotes further displacement especially in the ankle and foot.
Diagnosing HMS
I test single leg standing on almost all of my patients. Their ability to balance with minimal ankle wobbling is important. When I test patients with eyes closed and, in the extreme, with their head tilted back, they are prevented from relying on vision or inner ear for balance. They are then forced to rely on information from their foot and the rest of their body to determine where they are and maintain balance. Eyes closed is a good reflection of how stable you are with the lights off or when texting while standing or walking.
I look at their posture for knees that go backward (Genu recurvatum) and elbows that hyper extend or thumbs that can easily reach their wrist. Excessive ankle pronation with arch flattening and bunions is an indication of overstretch and excessive shearing motion at the big toe. These are indications that their collagen is not supporting their structure well.
Female hormones (just before their period) and to a much greater extent, during later pregnancy, greatly increase mobility. Pregnant women have run Marathons but the question as to whether it is good for them or the baby remains controversial. Children are often more mobile. There also seems to be a difference between ethnicities around the world. This needs to be taken into account when determining whether the patient is normal or hypermobile and what activities are appropriate.
Since July 2000, hypermobility has frequently been diagnosed using the Beighton criteria. The Beighton criteria incorporate the Beighton score along with other symptoms.
The Beighton score: Add 1 point for each of the following:
- Placing flat hands on the floor with straight legs
- Left knee bending backward (passive knee hyperextension over 10°)
- Right knee bending backward (passive knee hyperextension over 10°)
- Left elbow bending backward (passive hyperextension of the elbows over 10°)
- Right elbow bending backward (passive hyperextension of the elbows over 10°)
- Left thumb touching the forearm (passive flexion of the thumbs to the flexor surface of the forearms)
- Right thumb touching the forearm (passive flexion of the thumbs to the flexor surface of the forearms)
- Left little finger bending backward past 90 degrees (Passive dorsiflexion of the fifth finger of the hands over 90)
- Right little finger bending backward past 90 degrees (Passive dorsiflexion of the fifth finger of the hands over 90)
HMS is diagnosed in the presence of two major criteria, one major and two minor criteria, or four minor criteria. The Beighton criteria have not been very well supported in the literature in that the minor criteria do not add much to the correlations.
Major criteria:
- A Beighton score of 4/9 or more (either current or historic)
- Arthralgia in four or more joints for more than three months.
- Criteria major 1 and minor 1 are mutually exclusive as are major 2 and minor 2.
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, spondylolisthesis/lysis.
- Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.
- Soft tissue rheumtism. > 3 lesions (eg. 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.
Check back tomorrow for part two: Managing HMS
Image courtesy of Ambro at FreeDigitalPhotos.net