Benign Joint Hypermobility Syndrome: What Women Need to Know - Part 1

What is Benign Hypermobility Syndrome (BHS)?

Well, I’m here to tell you that there’s a whole lot more to what the name suggests. You may be thinking that having flexible and hypermobile joints is a key factor in reducing the chance for injury, but what if you express other symptoms or signs that warrant a considerable amount of attention? For those women who have consulted with me, you might recall the examination history to be quite broad, in the sense that it really didn’t have much to do directly with your reccurent neck or knee symptoms.
Hypermobility refers to an increased range of motion in one or more joints. Benign joint hypermobility syndrome (BJHS) is a disorder associated with musculoskeletal symptoms affecting hypermobile individuals without any systemic rheumatic disease. BJHS results from an abnormality of the structure of type-1 collagen, which is the most abundant collagen in the human body. Type-1 collagen is mostly found in tendons, ligaments, joint capsules, skin, and bone. The altered structure of collagen in BJHS results in less stiffness, more flexibility, and hypermobility.

Joint hypermobility is more common in females compared to males, with symptomatic disease affecting 5 % of the female population and 0.6 % of the male population.

You may have found that you are double jointed or at one stage during pre-adolescent years, able to amuse your friends with tangling your body into certain positions or even excel at such a sport like gymnastics or ballet. BHS can either be hereditary or congenital. According to the revised Brighton diagnostic criteria (below) for BHS, diagnosis is made in the presence of 2 major criteria or one major and two minor criteria. This means you don’t need to have super flexible joints to have BHS! In fact, in the past 6 weeks, two women presented in the clinic with general joint achiness in the neck, shoulder, wrists and knees along with short-sightedness. One of these women also presented with a recurring history of right shoulder dislocation, whilst the other presented with a very tall stature and large arm span width.
So why is BHS underdiagnosed most of the time? The problem is that these patients did not present classically with severely defective connective tissue disorders. However, it takes a careful and thorough history such that we provide to detect the not so clinically obvious.

So what signs and symptoms do patients with severely defective collagen disorders present with?

  1. Ocular (eye) disorders
  2. Varicosities
  3. Excessive bruising
  4. Tall slim stature
  5. Multiple dislocations and subluxations
  6. Cardiac valve defects
  7. Excessive nose bleeds and prolonged bleeding times.
  8. Foot deformities
  9. Abnormally hyper-elastic and stretchable skin
  10. Multiple pelvic organ prolapses and hernias

So what’s wrong with having inherently super flexible joints?

Pain may be the end result of a decrease in the sense of joint position that causes joints to become more susceptible to injury. Patients with BHS have impaired ability to localize where their joints are in the absence of vision. This is defined as proprioception. Abnormal stress and strain occurs to the ligaments and soft tissue as a result. Osteoarthritis may occur in the long term due to excessive movement

You can easily perform the Beighton 9-point scoring system yourself:

Beighton 9-point Scoring System

Manoeuvre

Right Left
Ability to passively dorsiflex the 5th metacarpophalangeal joint to 90° 1 point 1 point
Ability to appose the thumb to the volar aspect of the ipsilateral forearm 1 point 1 point
Ability to hyperextend the elbow joint to beyond 10° 1 point 1 point
Ability to hyperextend the knee joint to beyond 10° 1 point 1 point
Ability to place the hands flat on the floor by bending forward with knees fully extended 1 point 1 point

Total

If you scored 4/9 or greater and experience general muscle and joint pain in 4 or more joints, then categorically you have BHS. Alternatively, if you fit only one of these major criteria with 2 additional minor criteria, then you also via definition have BHS. However, the minor criteria must be assessed and examined in my clinic as it’s hard to perform by yourself.

Beighton criteria-diagnostic criteria for hypermobility syndrome
Major Criteria
1. A Beighton Score of 4/9 or greater.
2. Arthralgia for longer than 3 months in four or more joints.
Minor Criteria
1. A Beighton Score of 1, 2 or 3/9 (0, 1, 2 or 3 if aged 50+).
2. Arthralgia (for 3 months or longer) in one to three joints or back pain (for 3 months or longer).Spondylosis, spondylolysis or spondylolisthesis.
3. Dislocation/subluxation in more than one joint or in one joint on more than one occasion.
4. Three or more soft tissue lesions (e.g. epicondylitis, tenosynovitis and bursitis.
5. Marfanoid habitus (tall, slim, span/height ratio>1.03; upper/lower segment ratio<0.89 and arachnodactyly (positive Steinberg/wrist signs).
6. Abnomal skin striae, hyperextensibility, thin skin or abnormal scarring.
7. Ocular signs, drooping eyelids or myopia or antimongoloid slant.
8. Varicose veins or hernia or uterine/rectal prolapse.

Joint hypermobility syndrome patients also tend to have decreased muscle mass and muscle strength. This may be partly due to increased laxity of tendons, which are unable to transmit power produced by muscles. Generalized muscle and joint hypersensitivity is a common complaint and may serve as a compensatory mechanism to prevent joint instability and altered joint position sense. Furthermore, Knee joints were the most painful joints for the BJHS subjects, followed by the wrists and lower back when compared to the lower back, knees and neck in that order in the normal group. Knee extensor muscle strength was significantly lower in the patient group compared with the controls.

Whilst patients have enjoyed the many positive effects of manipulation to the neck with regard to pain outcomes and functioning, symptom recurrence is likely. There is nothing more frustrating than making repeated visits to a physio or chiro despite enjoying short term positive changes without being given the insight as to what is underlying the symptoms. If you know any women that have recurrent neck, back or joint pain accompanied by increased joint laxity, feel free to share this blog with them.

Please stay tuned for Part 2 of this blog to find out how BHS is involved with other systems of the body.

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