Hypermobility Spectrum Disorder

Procedure

Ask five yes/no questions: 1. Can you now (or could you ever) place your hands flat on the floor without bending your knees? 2. Can you now (or could you ever) bend your thumb to touch your forearm? 3. As a child, did you amuse your friends by contorting your body into strange shapes, or could you do the splits? 4. As a child or teenager, did your shoulder or kneecap dislocate on more than one occasion? 5. Do you consider yourself double-jointed?

Positive Finding

Yes to two or more questions.

Sensitivity / Specificity

Reference: Glans M, Humble MB, Elwin M, Bejerot S. Self-rated joint hypermobility: the five-part questionnaire evaluated in a Swedish non-clinical adult population. BMC Musculoskelet Disord. 2020;21(1):174. Schlager A, Ahlqvist K, Pingel R, Nilsson-Wikmar L, Olsson CB, Kristiansson P. Validity of the self-reported five-part questionnaire as an assessment of generalized joint hypermobility in early pregnancy. BMC Musculoskelet Disord. 2020;21(1):514.

Interpretation

A quick self-report screen for generalised joint hypermobility, including hypermobility the patient had in the past. A positive result should be followed by a Beighton examination. From two studies comparing the questionnaire with a hands-on Beighton examination: 284 adults from the general population (Glans 2020; 23 with hypermobility) and 301 women in early pregnancy (Schlager 2020; 44 with hypermobility). Two or more "yes" answers counted as positive. A negative result makes generalised hypermobility unlikely; a positive result needs a Beighton examination. The original 2003 study reported higher figures, but it compared clinic patients with healthy volunteers, which makes a test look better than it is.

Procedure

Score 1 point for each side: little finger bends back beyond 90° at the knuckle; thumb can be pulled down to touch the forearm; elbow straightens more than 10° past neutral; knee straightens more than 10° past neutral. Score 1 point for placing the palms flat on the floor with the knees straight. Maximum 9.

Positive Finding

A score at or above the age-based cut-off in the 2017 criteria.

Interpretation

Identifies generalised joint hypermobility. HSD is diagnosed when symptomatic hypermobility (pain, instability, fatigue) is present but the criteria for hypermobile EDS are not met. A score below the cut-off does not exclude hypermobility confined to a few joints (localised or peripheral HSD), and older people lose range over time, so ask about past flexibility too. The Beighton score is itself the standard used to define generalised joint hypermobility in the 2017 international criteria (Malfait et al.), so it has no separate accuracy figure. Adults up to 50 usually need 5 or more of 9; from 50, 4 or more. Generalised hypermobility on its own is common and is not a diagnosis.

Procedure

Older criteria for benign joint hypermobility syndrome, combining the Beighton score, joint pain, dislocations, soft tissue problems, skin features and marfanoid body shape.

Positive Finding

Meeting the Brighton combination of major and minor criteria.

Reference: Grahame et al., 2000, Rheumatology; Castori et al., 2017, American Journal of Medical Genetics

Interpretation

Replaced in 2017 by the international criteria for hypermobile EDS and hypermobility spectrum disorders. Useful only for reading older research.

Procedure

Ask the patient to rate pain from 0 to 10 and shade where they feel it on a body chart. Record how long it has lasted and what makes it worse or better.

Positive Finding

Not a test with a positive result: it records pain intensity and spread.

Reference: Castori et al., 2017, American Journal of Medical Genetics; See current literature for pain mapping protocols

Interpretation

Tracks pain over time and shows whether it is localised or widespread. Widespread long-standing pain is common in HSD and may involve a sensitised nervous system, which shapes treatment (education, graded activity) rather than diagnosis.

Procedure

Ask about joints that dislocate, partly dislocate or give way, and how often. At symptomatic joints, use the relevant instability tests (for example shoulder apprehension and relocation, patellar apprehension).

Positive Finding

Recurrent instability episodes, or a positive instability test that reproduces the patient's symptoms.

Reference: Castori et al., 2017, American Journal of Medical Genetics; See current literature for joint-specific instability tests

Interpretation

Supports symptomatic hypermobility. Rehabilitation focuses on strength, control and pacing rather than end-range stretching.

Procedure

Gently lift the skin on the inner forearm and watch how far it stretches and springs back. Look for stretch marks without weight change, easy bruising, wide scars and marfanoid features (tall, long limbs and fingers, chest wall shape).

Positive Finding

Soft or mildly stretchy skin, unexplained stretch marks, or wide scars.

Reference: Castori et al., 2017, American Journal of Medical Genetics; Malfait et al., 2017, Genetics in Medicine

Interpretation

Mild skin features fit hypermobile EDS or HSD. Very stretchy, fragile skin suggests classical EDS; marfanoid features suggest Marfan syndrome, which needs heart and eye checks. Thin, see-through skin with visible veins, very easy bruising, or a family history of sudden death or artery or bowel rupture suggests vascular EDS: avoid high-velocity thrust techniques and refer for medical and genetic assessment.