Are You Hyperflexible? The Neurology of Hypermobility

Hypermobility Syndromes and Hypermobility Spectrum Disorder

What are Hypermobility Syndromes:  Benign Hypermobility Syndrome affects perhaps 5% of the population, and is diagnosed when joint hypermobility is present a simple joint flexibility score called the Beighton Score, is equal to or greater than 5.  (see below). In order for the joints to be overly “stretchy,” the ligaments and muscle tendons which stabilize those joints must also be “stretchy.”

However, when multiple other systems are involved along with joint hypermobility, like those that affect blood pressure regulation, the gastrointestinal system, reproductive system, the joints of the body, and blood vessels, then Ehlers-Danlos Syndrome, Hypermobile Type (previously thought to be a purely genetic disorder) may be present.  But often this distinction is not clear-cut.

The diagnostic criteria separating simple Joint Hypermobility from the more serious disorders are still murky, so an all-encompassing diagnosis, Hypermobility Spectrum Disorder, has recently been suggested. (link to American Journal of Genetics, March), since it is recognized that there is no genetic testing specific to Ehlers-Danlos syndrome.

It is not clear whether dietary exposure to agents can worsen the disorder and affect the immune system, whether some sort of toxin exposure, or specific dietary deficiencies, or even manipulation of the bacteria that live in our gut, can affect how Hypermobility Spectrum Disorders play out in any one particular patient.

What is becoming clear, though, is that it is important to recognize the role of joint hypermobility and its other associated symptoms in the role of headache, neck and back and other joint pain, fatigue and sleep problems, dizziness, cardiac rhythm problems, blood vessel abnormalities including cavernous hemangioma, aneurysm, stroke, aortic root dilation and arterial dissection, kidney disease, reproductive problems, and sweating and Raynaud’s syndrome.

Joint hypermobility can truly be a gift, because people with flexible joints bend instead of break.  Sometimes they can be thrown through the windshield of a car in a head-on collision accident and walk away from the scene, when anyone with normal joint flexibility would be dead or paralyzed.  They are the stars of the Yoga class, not realizing that they are over-stretching their already flexible joints.  They can be gifted athletes in gymnastics, cheerleading, dance, and team sports, because they can do things that people with average flexibility cannot do.

On the other hand, patients with neck pain, back pain, joint pain, nerve pain, and headache can be over-stretched by aggressive chiropractic or physical therapy maneuvers, and nerves and already somewhat fragile arteries can be damaged by aggressive maneuvers, and symptoms made worse.

This same excessive connective tissue flexibility that is present in the joints can be associated with many other symptoms, which must be diagnosed and treated individually. Again, no single genetic change has been associated with this increased connective tissue flexibility, though Dr. Rowe is working with a genetics company to provide as complete a diagnostic gene set as possible for these patients.

When the complex of clinical symptoms warrant the diagnosis of Ehlers-Danlos Syndrome, Hypermobile Type (Type III), then abnormalities of the arteries supplying blood to the brain and other parts of the body can be present.  Since it’s important to know about these abnormalities, MRI and MRA scans of the brain and other parts of the body are frequently done.  This is especially true in patients with headache, neck pain and back pain, and pinched nerves in the neck, back, arms, and legs.

Symptoms Associated with Hypermobility Syndromes:

  1. Headache.  Most patients come to our Neurology Institute because of Intractable Headache.  They often have been treated with multiple medication regimens with little relief.  We recently have found that most of these patients have excessive neck motion and sleep problems, and if those two problems are diagnosed and treated, their headaches markedly improve.  (Link to AHS abstract and JON paper in preparation.)  Sometimes small hemorrhages and silent stroke can occur from blood vessel abnormalities, and these can produce headaches.
  2. Autonomic, or “automatic” nervous system symptoms. These include blood pressure regulation problems (POTS, or Postural Orthostatic Tachycardia Syndrome), which can lead to passing out and dizziness.  Sweating problems (hyperhidrosis).  Hands and feet that turn red or purple in the shower, and white in the cold (Raynaud’s Syndrome,) and indicate problems with the regulation of blood flow to the small vessels in the hands and feet.  Wound healing can be a problem, as well as sometimes stretch marks appearing for no reason.
  3. Gastrointestinal problems, with delayed stomach emptying or gastroparesis, treated paradoxically with small amounts of acid in vinegar in these patients, instead of the anti-acid therapy usually used in other patients. Irritable Bowel Syndrome (IBS) with alternating diarrhea and constipation.  These GI symptoms may be a part of the dysautonomia present in these patients.
  4. Mast Cell Activation Disorder. Skin that turns red easily with mechanical stimulation (dermographia) because of sensitive histamine-containing mast cells in the skin, that release histamine with all sorts of stimulation (food dyes, non-steroidal anti-inflammatory agents, or NSAIDS like aspirin, ibuprofen, and naproxen sodium—common joint pain and headache over-the-counter pain relievers.)
  5. Auto-immune disorders, like Hashimoto’s thyroiditis which causes hypothyroidism, and can be associated with many symptoms. Other auto-antibodies can also be present (ANA, Sjogren’s, and others.)
  6. Endometriosis and Polycystic Ovarian Syndrome—Very painful periods because of endometrial rests in the pelvic region, and irregular periods with ovarian cysts.
  7. Poor wound healing, and stretch marks in the absence of pregnancy or previous obesity.
  8. Joint pain and early onset osteoarthritis because of excessive joint stretching.
  9. Mononeuropathy (carpal tunnel syndrome and ulnar neuropathy at the elbow) caused by stretching of nerves that cross joints being over-stretched, with subsequent injury to that nerve. This can result in pain, numbness, and weakness in the skin and muscles supplied by the injured nerve.
  10. Sleep Disordered Breathing—with fatigue, restless and non-restorative sleep, and excessive daytime sleepiness present in teens and young adults, even without being overweight. (link to RNI AASM abstracts and Journal of Clinical Sleep Medicine Submission)

This is only a partial list of symptoms and signs that patients with joint hypermobility can have.  The connection between these symptoms and hypermobility is rarely appreciated by health professionals.  In fact, Dr. Rowe of the RNI recently described their association in papers to be presented at the American Academy of Sleep Medicine and American Headache Society meetings in Boston in June, 2017.  (Diagnosis and Treatment of Sleep Disorders in Patients with Hypermobility, and the Role of Hypermobility in Headache Disorders.)

The recognition of the syndrome of Hypermobility, Sleep Disorder, and Dysautonomia, can have profound implications for the therapy of patients with this syndrome, depending on the individual patient and the specific symptoms that disrupt their quality of life the most:

  1. Unnecessary surgery of many types can be avoided.
  2. Harmful stretching of joints and nerves can be avoided, in favor of joint stabilization.
  3. The real causes of symptoms such as neck pain, back pain, fatigue, and headache can be diagnosed and treated.
  4. Foods provocative of autoimmune disorders, should be avoided. The Autoimmune Protocol Diet or Paleo-type diet may help, especially with GI Symptoms.
  5. Hypothyroid states can be treated, and Hashimoto’s Thyroiditis checked for.
  6. Sleep problems can be diagnosed and treated, and the real cause of fatigue and non-restorative sleep can be identified and treated.
  7. Joint Supplements and others may help, though that topic needs to be studied further.
  8. Blood pressure regulation can be improved, and epilepsy and seizures ruled out.
  9. Other disorders like Multiple Sclerosis can be ruled out, or appropriately treated if present.
  10. Unsuspected causes of stroke in young people can be identified, and secondary stroke prevention therapy initiated.
  11. The true causes of headache syndromes can be identified.

As with all the rest of medicine, diagnosis is critical to decide on appropriate treatment of any disorder.  And further research into the cause of worsening symptoms, as discussed above, must be done.  But recognition of the syndrome as an association of particular symptoms and signs, is a start.  

Vernon Rowe, M.D.

The Beighton Score

How much flexibility makes you “hyperflexible”? You can test yourself by answering a few questions to determine your Beighton score. The Beighton score is used to measure a person’s degree of hypermobility.

One point is assigned for the ability to accomplish each of the following
movements:

  1. Bending your small finger back further than 90 degrees (1 point each side)
  2. Bending your elbow beyond a straight line (1 point each side)
  3. Bending your knee beyond a straight line (1 point each side)
  4. Putting your palms flat on the floor without bending your knees
  5. Bending your thumb back to touch the front of your forearm (1 point each side)

Do you think you have hypermobility syndrome? Contact us to find out how we can help.

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