Hypermobility Syndrome (Joint Hypermobility Syndrome)

Hypermobility, HSD and hEDS

"Joint hypermobility syndrome" and "benign joint hypermobility syndrome" are older names now largely replaced by HSD and hEDS. Management is very similar.

The Beighton score

One point for each:

1. bending the little finger back beyond 90 degrees (each hand: 2 points)
2. bringing the thumb to touch the forearm (each side: 2 points)
3. elbow bending backward more than 10 degrees (each side: 2 points)
4. knee bending backward more than 10 degrees (each side: 2 points)
5. placing palms flat on the floor with straight knees (1 point)

A score of 6 or more in children, 5 or more in adults up to 50, and 4 or more over 50 suggests generalized hypermobility. Flexibility decreases with age, so a history of being "double-jointed" also counts.

Symptoms

• joint pain, often in knees, hips, shoulders, wrists and fingers, worse after activity
• frequent sprains, partial dislocations (subluxations) and dislocations
• soft tissue problems: tendinitis, bursitis
• back and neck pain
• clumsiness and poor sense of joint position
• fatigue
• dizziness and fast heart rate on standing (POTS)
• digestive problems: reflux, constipation, bloating
• bladder problems and pelvic floor weakness
• anxiety, which is more common in people with hypermobility
• soft, slightly stretchy skin and easy bruising

Causes

Joint flexibility depends on the collagen in ligaments, the shape of joint surfaces, and muscle tone. Hypermobility often runs in families. It is more common in women, children, and people of Asian and African ancestry. hEDS is thought to be genetic, but no gene has yet been identified. Other inherited conditions with hypermobility include Marfan syndrome and other EDS types, which need different monitoring.

Diagnosis

There is no blood test for HSD or hEDS. Diagnosis is clinical:

• Beighton score and history of hypermobility
• assessment of skin, scars and body proportions
• review of symptoms and family history
• 2017 international criteria to decide between HSD and hEDS
• genetic testing when a rarer EDS type, Marfan syndrome or another condition is suspected
• echocardiogram if Marfan syndrome or valve problems are possible

Other causes of joint pain, such as inflammatory arthritis, should be ruled out.

Treatment

Exercise and physical therapy

The core of treatment. A physical therapist with experience in hypermobility can design a program that:

• builds muscle strength around joints, especially core, hips and shoulders
• trains proprioception and balance
• starts gently and progresses slowly to avoid flare-ups
• includes low-impact activities such as swimming, cycling and Pilates

Stretching is generally less helpful; the focus is on stability.

Pain and fatigue management

• pacing activities and planning rest
• heat, gentle movement and simple pain relief (acetaminophen (paracetamol) or ibuprofen, following the label dose)
• occupational therapy for joint protection and aids
• treating sleep problems, anxiety and depression
• pain management programs for chronic pain

Other symptoms

• POTS: fluids, salt (if advised), compression garments, and sometimes medicines
• gut symptoms: dietary changes and treatment of reflux or constipation
• pelvic floor physical therapy

Surgery to tighten joints has mixed results and is reserved for specific problems.

Outlook

Many hypermobile people have no problems or only mild symptoms. For those with HSD or hEDS, symptoms can vary over time. A well-structured exercise program and good self-management lead to improvement for most people.

Frequently asked questions

What is hypermobility syndrome?

It is a condition in which joints move further than normal and this causes symptoms such as pain, frequent sprains or dislocations, and fatigue. Doctors now usually call it hypermobility spectrum disorder or, if strict criteria are met, hypermobile Ehlers-Danlos syndrome.

What is the Beighton score?

The Beighton score is a 9-point test of joint flexibility, checking the little fingers, thumbs, elbows, knees and whether you can place your palms flat on the floor. A score of 5 or more in adults suggests generalized hypermobility.

Is being double-jointed a problem?

Usually not. Many flexible people never have symptoms and may even benefit in sports or dance. It becomes a problem only when it causes pain, repeated injuries or other symptoms, which is when it is called a hypermobility disorder.

What is the difference between hypermobility syndrome and Ehlers-Danlos syndrome?

Hypermobility spectrum disorder covers symptomatic hypermobility that does not meet criteria for hypermobile Ehlers-Danlos syndrome. hEDS has stricter criteria, including skin and family features. Treatment is very similar for both.

What is the best treatment for hypermobility?

Strengthening exercises guided by a physical therapist are the most effective treatment. They stabilize joints, reduce pain and lower injury risk. Pacing, pain management, and treatment of related issues such as POTS also help.