Are You Hyperflexible? The Neurology of Hypermobility

Hypermobility Syndromes and Hypermobility Spectrum Disorder

Benign Hypermobility Syndrome affects perhaps 5% of the population, and is diagnosed when joint hypermobility is present on a simple joint flexibility score called the Beighton Score.  When this score is equal to or greater than 5, with a maximum possible being 9, then the connective tissues of the body, including the ligaments that help the muscles and their tendons stabilize the joints and spine, are overly “stretchy,” and a person is referred to as being “Hypermobile.”

Hypermobility can be associated with other systems of the body, however, and when these other systems are involved, like those that affect blood pressure regulation, the gastrointestinal system, reproductive system, the joints of the body, blood vessels, and the immune system, then Ehlers-Danlos Syndrome (EDS) may be present.  Many of the thirteen recognized types of this disorder have genetic mutations that can be tested for.  Far and away, though, the most common type, or the Hypermobile Type, has no definite genetic correlation known at this time.  and so can not be specifically identified with genetic testing.

So the specific diagnostic criteria separating Benign Joint Hypermobility from the more serious disorders are still murky.  Since many patients have complex symptoms involving the gut, sleep, immune system, headache and other pain, blood pressure and other autonomic (automatic) nervous system functions, but do not have a specific gene abnormality that can be identified yet, Hypermobility Spectrum  Disorder is an all-encompassing name that can be applied to to these Complex Neurology patients.   (link to American Journal of Genetics EDS classification)

Patients with Hypermobility Spectrum Disorder, and those with one of the genetically identifiable types of EDS oftentimes have disorders of the immune system, and can benefit from dietary treatment.  Whether the Autoimmune Protocol Diet only affect the immune system, or whether it avoids some sort of  toxin exposure or specific dietary deficiencies, or even manipulation of the bacteria that live in our gut, or substances that “leak” through the gut, is not at all clear at this time.  However, what is clear is that the AIP diet can improve the quality of life for these complex patients.   

What is becoming increasingly clear, though, is that these patients with EDS and other complex Neurology issues and hypermobility DO NOT DO WELL WITH STANDARD BACK SURGERY, and surgery should be entertained only as a last resort to help them with their symptoms.  Also, these patients seem to be sensitive to all sorts of surgically implanted materials in their body.

So it is important to recognize the presence of joint hypermobility and its other associated connective tissue and immune 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, so that surgical overtreatment can be avoided.

Joint hypermobility can truly be a gift, because people with flexible joints bend instead of break.  This can be “a feature and not a bug,” so to speak, because sometimes joint hypermobility allows for better athletic performance in certain sports (everything from ballet and gymnastics to volleyball).  These folks may be 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.

But it is important to recognize when coaching these gifted athletes, that irreversible damage to joints and spine can occur when those joints are repeatedly overstretched, and that sometimes the ultimate price for a gifted performance now will not be paid until years later.  So the muscles themselves must be trained to protect the joints from overstretch injury.

Simlarly, 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  somewhat fragile arteries can be damaged by aggressive maneuvers.  This results in a worsening of the symptoms.  Thus, one size cannot fit all in physical therapy and other interventions for these patients!.

And likewise, the same excessive connective tissue flexibility that is present in the joints can be associated with many other symptoms.  This association must be recognized, and patients treated as the unique individuals they are.

When the complex of clinical symptoms warrant the diagnosis of Ehlers-Danlos Syndrome, Hypermobile Type (hEDS in the newer nomenclature, Type III in the older nomenclature), then abnormalities of the arteries supplying blood to the brain and other parts of the body can be present.  Genetic testing can reveal the vascular variant of EDS.  However, hEDS can be associated with blood vessel abnormalities, such as fibromuscular hyperplasia, which can be associated with vascular symptoms.  MRI and MRA scans of the brain and other parts of the body are frequently done in these patients, depending on their major symptoms.  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:

1. Headache.  Over the years, we at the RNI made tThe association between headache and hypermobility syndromes because many of our patients presented to our Headache Center with Intractable Headache.  They often had been treated with multiple medication regimens with little relief.  We found that many such patients of these patients had excessive neck motion during the day, disordered breathing with neck hyperextension for airway clearance during the night.  We found that if those two problems are diagnosed and treated appropriately, headaches markedly improved.  (Link to AHS abstract and JON paper in preparation.)  We found that sometimes small hemorrhages and silent stroke can occur from blood vessel abnormalities, and that these could be associated with headache.
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.  (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  therapy in 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 must 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.  Now that the Covid-19 pandemic is coming under better control in some parts of the world, research into the molecular basis of the hypermobility syndromes, and the aberrant connections between the cytoskeleton and extracellular elements in these disorders, may be able to be continued.

Vernon Rowe, M.D.

You can click to see the patient Handouts:  Hypermobility Handout 09252017  and the Autoimmune Diet Protocol

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)