HYPERMOBILITY Jean Oliver, MCSP, SRP Chartered Physiotherapist Achilles Physiotherapy Clinic, Cambridge October 2005 No 7

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1 HYPERMOBILITY Jean Oliver, MCSP, SRP Chartered Physiotherapist Achilles Physiotherapy Clinic, Cambridge October 2005 No 7 Introduction Hypermobility (HM) excessive mobility can occur in a single joint, or it can be more widespread throughout the body. Usually HM joints cause no problems and are even an asset in sports such as gymnastics and artistic professions such as ballet and music. However, in a significant percentage of people HM joints do cause symptoms ranging from minor sprains to more persistent arthralgia and chronic pain to such an extent that their quality of life may be jeopardised. Since there are often no demonstrable signs HM is underdiagnosed as a cause of symptomatology. 1 In the main, HM is genetically determined, those affected having loose connective tissue in particular, ligaments and articular capsules. This connective tissue is fragile and is more easily injured, 2 taking longer to heal than the norm. There is now evidence that proprioception in some HM joints is also impaired. 3 Women are affected more than men. HM patients fall into two groups: 1. Overuse/malalignment These patients have one or several HM joints that suffer the consequences of overuse or malalignment caused by e.g. leg-length inequality, asymmetrical hip rotation or flat feet. HM patients experience the ill-effects of malalignment far earlier than people without HM. 2. Hypermobility syndrome (HMS) This syndrome was defined by Kirk et al 4 as generalised joint laxity with associated musculoskeletal complaints in the absence of any systemic disease. Arthralgia is a common feature of this syndrome. The suffering of some of these patients is incalculable and their quality of life may be jeopardised by the syndrome. Diagnosing hypermobility Questions to ask The diagnosis is often arrived at by the patient s affirmative answers to the following questions regarding their childhood and teenage years. 5 Were you supple in your teens? Did you have any knee problems in your teens? Were you ever able to get your hands flat on the floor when your knees were straight? Did you go over on your ankles a lot? Did you have growing pains in your legs? Did your joints click a lot? Did you fidget a lot as a child? Did you bump into things/fall over a lot as a child? Symptoms Pain and stiffness related to soft tissue damage and proprioceptive impairment/lack of warning signs. The patient is often alarmed at the rate at which pain in one joint appears to spread to other areas of the body, e.g. the patient may sprain an ankle and shortly afterwards his/her knee, hip and back feel the strain as his/her body attempts to compensate for the original injury. Statements such as I feel as if I m falling to bits and I feel as if I can t hold up my head express the patient s anxiety but often sound bizarre. Arthralgia, the severity of which is often influenced by the phases of the menstrual cycle and by changes in the weather. 6 Signs Testing for HM should be a routine part of the examination of patients with musculoskeletal problems. Range of movement The patient may exhibit what appears to be a normal range of movement, e.g. touching the toes. However, this may be very limited for an HM

2 patient, who previously may have been able to get the palms of his/her hands on the floor. The patient will nonetheless complain of feeling stiff. Many scales have been devised to screen for the hypermobility syndrome (see Further reading ), the Beighton score, which is a 9-point scale, being the most well known. Although this score includes the spinal hyperflexion and thumb abduction with wrist hyperflexion tests two high indicators for HM many HM patients would in fact score 0 in this test because too few joints are included. This is why the BSR Special Interest Group on Hypermobility proposed the use of the Brighton score to help in diagnosis (Table 1). Importantly this scoring system reminds us that organs may be involved. Joint effusions sometimes, but not always, in the acute stage. Easy bruising Striae atrophicae TABLE Brighton revised diagnostic criteria for benign joint hypermobility syndrome. (Reprinted from Grahame R et al, J Rheumatol 2000;27(7):1777-9, with permission.) Major criteria 1. A Beighton score of 4/9 or greater (either currently or historically) 2. Arthralgia for longer than 3 months in 4 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 1 3 joints, or back pain ( 3 months), spondylosis, spondylolysis/spondylolisthesis 3. Dislocation/subluxation in more than one joint, or in one joint on more than one occasion 4. Soft tissue rheumatism 3 lesions (e.g. epicondylitis, tenosynovitis, bursitis) 5. Marfanoid habitus: tall, slim, span:height ratio >1.03, upper:lower segment ratio <0.89, arachnodactyly (+ Steinberg/wrist signs) 6. Abnormal skin: striae, hyperextensibility, thin skin, papyraceous scarring 7. Eye signs: drooping eyelids or myopia or antimongoloid slant 8. Varicose veins or hernia or uterine/rectal prolapse Benign joint hypermobility syndrome (BJHS) is diagnosed in the presence of 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 the presence of Marfan s or Ehlers Danlos syndrome (EDS) [other than the EDS Hypermobility type (formerly EDS III) as defined by the Ghent 1996 and the Villefranche 1998 criteria, respectively]. Criteria Major 1 and Minor 1 are mutually exclusive, as are Major 2 and Minor 2. Varicose veins Hernias Risk factors Unaccustomed physical exercise A change of occupation/studying, e.g. to an office job involving a lot of sitting, or pre-exam time Premenstrual Pregnancy (effect of the hormone relaxin) Sudden gain in weight Repetitive movements Malalignment Cold weather (usually) Unsupportive sandals or flat deck shoes Sleeping prone (neck) Hypermobility in children and adolescents Symptoms usually start during the adolescent growth spurt in the knees or spine but they can occur much earlier. At first they come on after activity sport, or walking or after a protracted period of sitting. HM children may be thought to be lazy, or be trying to avoid going to a gym lesson. They often fidget and sit in distorted postures to try to gain stability. Problems with writing are common; when children move on from using thicker pencils they experience difficulties in gripping a thinner pen. Some children have associated dyspraxia. Hypermobility in adults HM in men is less easily recognised because their increased muscle bulk reduces joint range of movement. The conventional explanation for the observed clinical differences between men and women is the effect of the female hormones on the collagen (and muscles). HM people working with computers or performing repetitive movements are at a higher risk from work-related upper limb syndrome (WRULS) formerly known as repetitive strain injury (RSI) in their hands, wrists and forearms, which can strike suddenly and without any warning signs. Although range of movement decreases with age, some elderly HM people still maintain impressive flexibility, that is unless pathology has stiffened their joints. Differential diagnosis Rheumatoid arthritis Juvenile idiopathic arthritis 7,8 Pathology Although not unique to them, the following pathology occurs more frequently in HM patients: 2

3 FEET : excessive calcaneal eversion (Figure 1.1) and flat feet Ankle sprains Hallux abductovalgus or osteoarthritis (OA) of the 1st metatarsophalangeal joint Heel pain: plantar fasciitis 1. Excessive calcaneal eversion KNEES : hyperextension (Figure 1.2) Medial knee pain Anterior pain (chondromalacia patellae) Anterior cruciate ligament ruptures Joint effusions HIPS : anteversion (Figure 1.3) ( squinting patellae when standing) Clicking hip Trochanteric bursitis Associated with low back/pelvic pain and inability to stand for long periods LOW BACK : increased lumbar lordosis/sway back in teens, able to get hands flat in forward flexion (Figure 1.4) and/or do the crab (hyperextension) (Figure 1.5) Lower two lumbar levels show disc narrowing and facet joint arthrosis Pars defects/spondylolisthesis 10 Spinal stenosis PELVIS Sacroiliac joint instability or hypomobility, especially during pregnancy Symphysis pubis diastasis Weakness of pelvic floor muscles/uterine prolapse 2. Hyperextension of the knees 3. Anteversion of the hip 4. Spinal hyperflexion THORACIC SPINE : stiffness/scoliosis The thoracic spine is often the first area to stiffen up NECK : forward head posture Acute wryneck episodes Susceptible to whiplash injuries Headaches, often associated with tight ligamentum flavum on neck flexion Low cervical disc with nerve root entrapment 5. Spinal hyperextension TEMPEROMANDIBULAR JOINT Clicking and subluxation/dislocation HANDS : excessive thumb abduction and wrist flexion (Figure 1.6) OA of the metacarpophalangeal joint of the thumb Repetitive strain 6. Thumb abduction with wrist hyperflexion ELBOWS : hyperextension Tennis elbow (lateral epicondylitis) Golfer s elbow (medial epicondylitis) SHOULDERS: hyperextension (Figure 1.7), excessive external rotation Rotator cuff tendinitis Subluxation/dislocation of glenohumeral joint Crepitus on scapulae movement 7. Shoulder hyperextension FIGURE 1. Hypermobile joints 9 frequently associated with pathology. 3

4 Psychological aspects A higher incidence of panic attacks and anxiety states has been found in people with HM. 11 Many HM patients are thought, erroneously, to be neurotic or at best to be exaggerating their symptoms. Investigations X-rays and magnetic resonance imaging (MRI) scans are usually negative in the acute stage, but may show premature OA in patients in early middle age. Treatment Reassurance is extremely important and the patient will be relieved that a diagnosis has at last been made to explain his/her symptoms and that the condition is not lifethreatening. From the outset, the importance of self-management should be stressed, but the patient (and sometimes his/her family) will need guidance and some initial monitoring. Periodic treatment for acute episodes may also be necessary. An increasing number of physiotherapists are gaining expertise in treating hypermobility, but it is easy to exacerbate the patient s symptoms if the therapist is not familiar with HM. Rest In the short term for acute episodes of soft tissue lesions. Alignment check with podiatrist or physiotherapist Correction with a heel raise and orthotics if necessary. Physiotherapy To teach self-management and back care. Exercise forms the basis of treatment, in particular reeducating the deep stabilising muscles of the affected joints and pelvic floor muscles. Some patients may be able to progress to joining a Pilates exercise class, while others fare better with swimming/hydrotherapy or gym ball exercises. Aerobics are often contraindicated. Pacing of activities is essential because HM patients often do not get warning signs of overuse and suffer a delayed reaction to overactivity. Stretching after warm-up, gentle stretching of the superficial muscles to the end of the patient s hypermobile range is pain-relieving. Overstretching is to be avoided. Hatha (gentle) yoga helps patients with minor problems. Stretching before going to bed will help relieve morning stiffness when getting dressed. Balance training, including t ai chi. Passive mobilisation for symptomatic relief, especially to areas that are difficult for the patient to reach, such as the thoracic spine. Repeated manipulation is harmful and creates dependency on the therapist. Ergonomic advice, especially apropos working with computers. The Alexander Technique Posture correction through sensory feedback. The technique suits the more reflective type of patient and is best taught when the patient is pain-free. Medication The usefulness of drugs in controlling pain in HM patients is limited. Analgesics Paracetamol with or without codeine are the first choices. NSAIDs/COX-2s should be reserved for situations where inflammation is suspected, especially in view of the recent European Medicines Agency (EMEA) 12 review of COX-2s and ongoing NSAIDs review. Opioid matrix patches are an alternative to simple or compound analgesics. Local steroid injections These may be useful, especially for trochanteric pain in patients with hypermobile hips. Inject knees as little as possible to decrease the risk of dependency and theoretical risks of weakening collagen, but removing large effusions is usually worthwhile. Pain management and self-management Most primary care organisations employ or will soon appoint pain management nurses. HM patients may well benefit from a consultation. Support group The Hypermobility Syndrome Association can be recommended for the more severely affected patients. ( Prognosis The earlier the diagnosis of HM is made, the more effective are treatment outcomes. Many symptoms from HM joints (especially if there is associated malalignment) can be successfully managed if the patient complies with weight reduction, alignment correction, exercise, and life-style modifications. However, after injury the rate of healing is slower than average; this is particularly noticeable after road traffic accidents and WRULS. It is likely that HM predisposes to premature osteoarthrosis. A minority of patients will have severe arthrosis in many joints. Further reading Keer R, Grahame R (ed). Hypermobility syndrome: recognition and management for physiotherapists. Edinburgh/ London/New York: Butterworth Heinemann; Bird HA. Heritable collagen disorders. Reports on the Rheumatic Diseases (Series 5), Topical Reviews 5. Arthritis Research Campaign; 2005 Feb. Bird HA. Joint hypermobility in children. Rheumatology 2005;44(6): Joint hypermobility. Information Booklet (for patients). Arthritis Research Campaign;

5 Scoring systems for hypermobility: Beighton score: Beighton P, Solomon L, Soskolne CL. Articular mobility in an African population. Ann Rheum Dis 1973; 32(5): Brighton criteria: Grahame R, Bird HA, Child A, Dolan AL, Edwards-Fowler A, Ferrell W et al. The revised (Brighton 1998) criteria for the diagnosis of benign joint hypermobility syndrome (BJHS). J Rheumatol 2000;27(7): References 1. Beighton P, Grahame R, Bird H. Hypermobility of joints. 3rd edn. London: Springer Verlag; Grahame R, Jenkins JM. Joint hypermobility asset or liability? A study of joint mobility in ballet dancers. Ann Rheum Dis 1972; 31(2): Hall MG, Ferrell WR, Sturrock RD, Hamblen DL, Baxendale RH. The effect of the hypermobility syndrome on knee joint proprioception. Br J Rheumatol 1995;34(2): Kirk JA, Ansell BM, Bywaters EG. The hypermobility syndrome: musculoskeletal complaints associated with generalized joint hypermobility. Ann Rheum Dis 1967;26(5): Oliver J. Hypermobility: recognition and management. In Touch (The Journal of the Organisation of Chartered Physiotherapists in Private Practice) 2000;94(2): Grahame R. Hypermobility syndrome. Reports on the Rheumatic Diseases (Series 2), Topical Reviews 25. Arthritis & Rheumatism Council; 1993 Sept. 7. Bird HA, Wright V. Joint hypermobility mimicking pauciarticular juvenile polyarthritis. Br Med J 1978;2(6134): Bird HA. Joint hypermobility in children. Rheumatology 2005;44(6): Oliver J. Back in line. Oxford: Butterworth Heinemann; Morgan AW, Gibbon W, Bird HA. A controlled study of spinal laxity in subjects with joint hyperlaxity and Ehlers Danlos syndrome. Br J Rheumatol 1997;36 Suppl 1:59(S136). 11. Bulbena A, Duro JC, Porta M et al. Anxiety disorders in the joint hypermobility syndrome. Psychiatry Res 1993;46(1): European Medicines Agency (EMEA) website: COMMENT Howard Bird Professor of Pharmacological Rheumatology University of Leeds This article provides comprehensive practical advice from the physiotherapy perspective. Intriguingly, consideration is given to some joints (e.g. the ankle) that are indeed important, though which have lapsed from conventional scoring systems. It should be remembered that adjacent hypermobile joints often complement each other. The illustration denotes spinal hyperflexion and spinal hyperextension, i.e. reflecting laxity of the hip almost as much as the spine. It should also be recalled that extreme hyperlaxity localised to a single joint, perhaps as a result of bony abnormality or neuropathy, may give just as much trouble as a more generalised syndrome. The physician is often more aware than the physiotherapist of the implications of collagen-linked laxity at organs other than the joints. These patients may have cardiac murmur, severe Raynaud s phenomenon and asthma in addition to features mentioned in the article. It is for this reason that the Brighton scoring system has recently been proposed, which although it incorporates the Beighton system also allows in patients on the basis of their organ involvement or extreme localised hyperlaxity at a small number of sites. Although the spectrum of benign familial hypermobility syndrome undoubtedly accounts for the majority of patients described in this issue, the physician may also encounter one of a very small number of patients in whom the hypermobility is a feature of a more serious inherited abnormality of connective tissue such as Ehlers Danlos syndrome, Marfan s syndrome or osteogenesis imperfecta. These appear to have their various additional problems, specific for each condition, which require specialist advice. Extreme examples can be associated with maternal and foetal mortality in pregnancy or at childbirth and then from vascular accident at a relatively young age. Patients also ask about analgesics to control pain. The self-management activity described should always be tried before resorting to drugs but a large proportion of these patients will still need background analgesics to control their symptoms and allow them a reasonable quality of life. NSAIDs are rationally restricted to shortterm use to quieten episodes of inflammation that sometimes follow traumatic dislocation. I would be less happy with the regular use of local steroid injections since this has the side-effect of reducing collagen content in the joint capsule and may therefore aggravate the hypermobility. The article makes the important point that repetitive movement can aggravate hypermobility. I see many overuse syndromes in my hypermobility clinic, presumably because extra muscular work is needed to stabilise the hypermobile joint before it can be used. 5

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