Hypermobility – Does it Cause Joint Symptoms?
Such scoring systems imply that a high score is required before symptoms arising from joints can safely be attributed to hyperlaxity. This misses the crucial point that even a single joint that, for whatever reason, is particularly hyperlax, may be specifically symptomatic and also fails to take on board the increasing clinical clues that accumulate when careful examination of other organ systems is conducted in subjects with hyperlax joints. To take account of this, the Brighton scoring criteria were developed and validated.6
These are listed in Table
2. By way of screening, a five-point questionnaire has been developed by Grahame and can be used quickly and routinely in clinics (shown in Table 3).7
Subjects responding in the affirmative to two or more of the five questions should be considered for more detailed screening.
Two other nosological points have recently been discussed. When joints that are hyperlax become symptomatic – particularly when supporting extra-articular clues – the term ‘joint hypermobility syndrome’ is preferred to ‘joint hypermobility’. There has also been controversy on whether benign joint hypermobility syndrome (BJHS) overlaps with the mildest hypermobile variant (type III) of EDS. The root of this confusion is that rheumatologists prefer the term ‘joint hypermobility’ and clinical geneticists classify according to the many variants of EDS. A survey of British rheumatologists showed that opinion was divided.8
Although a sizeable minority consider that the
two conditions can be separated on careful clinical examination (especially in terms of the skin), political expediency determines that facilities for subjects may improve if separation is not attempted, not least because management of the two conditions is similar.9
Causes of Hyperlaxity at Individual Joints Essentially any separation follows careful clinical examination, often inadequately taught in the rheumatology curriculum. In general, joint hyperlaxity consequent upon collagen structure is likely to be widespread throughout most joints in the body; the degree of laxity displayed at each of these joints is approximately the same. Hormonal effects on symptoms provide a further clue to this aetiology, as does the presence of clinical features in other organs that can be attributed to widespread laxity in collagen. In the vascular system these may include hypotension and Raynaud’s syndrome, at the urethral sphincter some stress incontinence and easy bruising in the capillaries.
Joint hyperlaxity as a consequence of unusual bony/cartilaginous shape of the articulating surfaces, probably a mild variant of the acetabular dysplastic group of syndromes, tends to be severe at a small number of joints and absent at others, sometimes the majority. Ball and socket joints or saddle joints seem to be rather susceptible to this variant of hyperlaxity, which is much less likely to be hormonally influenced, which is not associated with features of lax collagen elsewhere and may well be accompanied by a familial orthopaedic history. These are probably the two commonest causes of hyperlaxity and both may coexist in the same individual. A careful family history often suggests each parent has contributed a separate type.10
Hyperlaxity, as an acquired type, may also arise from regular stretching or training. Studies on the tensile properties of collagen have been carried out on tendons in the tails of rats to show that a degree of extension can be achieved and maintained by stretching. The extent to which neuromuscular control may then contribute remains somewhat controversial but may sometimes mimic laxity that results in inherited factors alone. One clue here is the dependence upon ‘warm-up’ for the laxity to be achieved.11
EUROPEAN MUSCULOSKELETAL REVIEW
Table 1: The Nine-point Beighton Scoring System for Joint Hypermobility Scale
R
The ability to 1. Passively dorsiflex the fifth metacarpophalangeal joint to ≥90º
2. Oppose the thumb to the volar aspect of the ipsilateral forearm 3. Hyperextend the elbow to ≥10º 4. Hyperextend the knee to ≥10º
5. Place hands flat on the floor without bending the knees
Maximum total 1
1 1 1
1 9
One point may be gained for each side for manoeuvres 1–4 so that the hypermobility score will have a maximum of 9 points if all are positive.
Figure 1: Manoeuvres used in the Beighton Scoring System
L 1
1 1 1
Table 2: 1998 Brighton Revised Diagnostic Criteria for Benign Joint Hypermobility Syndrome
Major Criteria 1. A Beighton score of 4/9 or greater (either currently or historically) 2. Arthralgia for longer than three 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 (> 3 months) in one to three joints or back pain (> 3 months) 3. Dislocation or subluxation in more than one joint, or in one joint on more than one spondylosis, spondylolysis or spondylolisthesis occasion
4. Soft tissue rheumatism – more than three lesions (e.g. epicondylitis, tenosynovitis, bursitis)
5. Marfanoid habitus: tall, slim, span/height ratio >1.03, upper/lower segment ratio <0.89, arachnodactyly (positive Steinberg or wrist signs)
6. Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring 7. Eye signs: drooping eyelids, myopia or antimongoloid slant 8. Varicose veins, hernia or uterine or rectal prolapse
Benign joint hypermobility syndrome (BJHS) is diagnosed in the presence of two major criteria, 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 the presence of Marfan’s or Ehlers–Danlos syndrome (EDS) other than EDS hypermobility type (formerly EDS III) as defined by the Ghent 199623
and the Villefranche 199822 criteria respectively. Criteria major 1 and minor 1 are mutually exclusive as are major 2 and minor 2.
A further factor is the increasing realisation that proprioception may be an influence, either as a primary or secondary event.12
In addition,
impairment and abnormality in the reflex arc serving the hyperlax joint has also been established13
and recent work from Ghent, Belgium, has
further defined proprioceptive abnormalities, suggesting that the abnormality in proprioception arises from deficits in joint and muscle
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