Joint Hypermobility Syndrome (JHS) Part 1 – what are the problems?

I see many people who are hypermobile, in other words, are able to move certain joints through a greater range. But what is normal? With so many being able to do this including some of the best known athletes, what is the problem? Diagnosis Usually when I see that the person in front of me is able to bend forward and touch the floor without bending their knees, extend their elbows beyond straight as do the knees, bend their 5th fingers backwards more than 90 degrees and that they can touch their thumb to the forearm, you would be right in thinking that they are hypermobile. This is the Beighton classification and in most cases that I see, the patient demonstrates some of these but not necessarily all. The diagnosis of Joint Hypermobility Syndrome comes when there is greater movement at these and other joints (e.g. thumb, foor arches) but associated with other findings such as joint pain and skin that is more elastic. Below are the diagnostic criteria: REVISED DIAGNOSTIC CRITERIA FOR THE BENIGN JOINT HYPERMOBILITY SYNDROME (BJHS) Major Criteria

  • A Beighton score of 4/9 or greater (either currently or historically)
  • Arthralgia for longer than 3 months in 4 or more joints

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, spondylolysis/spondylolisthesis.
  • Dislocation/subluxation in more than one joint, or in one joint on more than one occasion.
  • Soft tissue rheumatism. > 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis).
  • Marfanoid habitus (tall, slim, span/height ratio >1.03, upper: lower segment ratio less than 0.89, arachnodactyly [positive Steinberg/wrist signs].
  • Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring.
  • Eye signs: drooping eyelids or myopia or antimongoloid slant.
  • Varicose veins or hernia or uterine/rectal prolapse.

The BJHS is diagnosed in the presence two major criteria, or one major and two minor criteria, or four minor criteria. Two minor criteria will suffice where there is an unequivocally affected first-degree relative. BJHS is excluded by presence of Marfan or Ehlers-Danlos syndromes (other than the EDS Hypermobility type (formerly EDS III) as defined by the Ghent 1996 (8) and the Villefranche 1998 (9) criteria respectively). Criteria Major 1 and Minor 1 are mutually exclusive as are Major 2 and Minor 2.

Prof. Rodney Grahame – UCL

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Observations

Spatial awareness

When I chat to a patient with BJHS I usually discover that they have poor spatial awareness, highlighted by regular mini-incidents such as walking into doorways and catching the edge of tables. On testing indeed I often find that proprioception (knowing where your joints are in space), 2-point discrimination and left/right recognition are all poor. These clinical tests all highlight an issue with the planning and execution of movement.

Pain

A few years ago a study was performed that looked at the effects of creating incongruence between the actual movement of an arm and what it appeared to be doing in normal subjects. They did this by setting up a system of mirrors so that the individual observed a different movement compared to the real movement. As a result of the incongruence the subjects experienced both pain and sensory disturbances due to conflict occurring in the brain thereby creating a model implicating the cortex. In relation to JHS, if the joint position sense and processing of sensory information form the body does not match that expected by the brain, the same conflict can arise and potentially explain a component of the pain. Additionally, it is considered that there maybe stresses and strains that create sensitivity as a result of taking joints through greater ranges but without the necessary control or awareness of the movement as it feels normal. When I am talking to a patient about their pain, I am seeking to unravel the underlying mechanisms. Many people who I see have had pain for some time, often for months to years. In these cases, central sensitisation can be a feature. Central sensitisation is described by Clifford Woolf as ‘an increase in the excitability of neurons within the central nervous system, so that normal inputs begin to produce abnormal responses’ – read the full article here. The central nervous system (brain and spinal cord) have the ability to amplify the danger signals (increased sense of pain) and modify normal signals (normal stimuli such as light touch now hurt). There is good research to suggest that central sensitisation underpins many functional pain syndromes such as fibromyalgia, migraine, chronic back pain, pelvic pain, dysmenorrhoea, IBS and other persisting pains. Those who present with pain as the primary complaint may begin by describing one area of the body but soon begin to unravel a range of aches and pains at different joints. The examination reveals varying degrees of mobility and the complete picture emerges.

Control of movement

The concept of ‘body ownership’ is both fascinating and relevant. How we feel and sense our body and consequently know where it is in relation to our environment is important for everyday movement and activity. The brain constructs the feeling of our body by integrating information that comes from the tissues with that which is already known. This is a feedback-feedforward system that is affected when the information is not as accurate as it may otherwise be. Without a good ‘view’ on the body, the ability to control movement is impaired.

Between our ears in the cortex of the brain sits a map of our body. This is very well defined and maintained by the regular flow of information from the body. In cases where we move differently or have pain we know that the map is less well defined. This is known as ‘smudging’. Patients will tell me that their sense of a particular body part is altered, e.g. missing, poorly defined, difficulty imaging moving the joint. Clearly this ties in with spatial awareness.

Flat feet and gait

Often there is noticeable flat feet where the arches are low or non-existent. Many people have a variation and this is not necessarily a problem in itself. However, when this is combined with a movement compensation or there are issues with walking and running, then flat feet will be addressed.

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These are some of the common problems that I see in relation to hypermobility. In the next blog I will discuss what we can do to tackle the issues.

If you have any questions or would like to book an appointment, please call us on 07518 445493. Our clinics for hypermobility are in London: Harley Street, Chelsea, Temple; and in New Malden – click here for details

 

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