Contents
Definition
Hypermobility is excessively mobile joints and back without another explanatory disease, i.e. absence of the syndromes Marfan's-, Ehlers-Danlos, Loeys-Dietz- og Osteogenesis imperfecta. The hypermobility syndrome is also characterized by Pain in joints og muscles. The condition is often genetic and not one autoimmune systemic connective tissue disease.
Occurrence
Common occurrence. Most women. One study showed a degree of hypermobility in as many as 17,6-21,2% in European populations using the Beighton score (see below) (reference: Mulvey MR, 2013).
Symptoms
Over-movable, unstable joints that easily come "out of joint" (luxates): "Stepping over" with ankle, kneecap out of joint, shoulder out of joint, jaw out of joint, whip lash (neck pain after injury). Someone with hypermobility reports Pain in joints og muscles or backache, but repeated episodes of "out of joint" dispose too soon Osteoarthrtitis which can be painful.
Beighton's hypermobility score
To determine hypermobility is often used Beighton hypermobility score (reference: B Juul-Kristensen, 2007). 4 / 9 points indicate generalized hypermobility:
- Little finger can be stretched (extension) to more than 90 degrees (2 points if present in both arms)
- Opposes thumbs against the forearm (2 points if both sides)
- Elbow more than 10 degrees overextension (2 points if both)
- Knee more than 10 degrees overextension (2 points if both)
- Palms against the floor (standing with straight knees) 1 points
The The hypermobility syndrome in addition to hypermobility it should be symptoms such as pain in joints or muscles or sensible skin.
There are several diagnostic criteria for hypermobility syndrome. A set of current criteria here (Reference: Graham R, 2000)
Diagnostic criteria for hypermobility syndrome (Brighton 1998) for EDS (Grahame R, Bird HA, Child A 2000)
| Major criteria | Minor criteria |
| Beighton score ≥4/9 | Beighton scores 1–3/9 |
| Joint pain >3 months in >4 joints | Joint pain in 1–3 joints |
| Medical history of "out of joint" / dislocations | |
| >3 soft tissue injuries | |
| Marfanoid Habitus | |
| Skin stretch marks () striae, stretchable, thin skin, abnormal scars | |
| Eye symptoms: drooping eyelids, myopia, anti-mongoloid droop. Varicose veins, pelvic prolapse or hernia in the abdominal area (abdominal hernias) |
Causes of hypermobility
Usually there are no signs of disease (idiopathic), but is a normal variant which is then often seen when other close relatives have the same. Listed below are rare associated conditions:
- Hereditary (genetic) connective tissue diseases (Marfan's syndrome, Ehlers-Danlos syndrome, Osteogenesis imperfecta)
- Williams syndrome is caused by a gene defect on chromosome 7 and is characterized by short stature, mild to moderate mental retardation.
- Stickler syndrome: Hereditary arthro-ophthalmopathy: Stickler 1965. Early onset of osteoarthritis, myopia with later, retinal detachment, skeletal changes in the form of irregular epiphyses and enlarged metaphyses, possibly deafness. Hypermobility, scoliosis, micrognathia. Genetic test (reference: Klingenberg C, 2001; Boothe M, 2020).
- Chromosome disorders (downs Syndrome, Killian-Tescler-Nicola syndrome. Palister mosaic syndrome: due to tetrosomy of chromosome 12, localized alopecia, pigment changes, mental retardation, broad hands and feet with short fingers and toes, congenital hip joint dislocation.
- Other genetic causes
- Myotonica congenita
- Metabolic conditions (homocysteineuria, hyperlysinemia)
Treatment
Interdisciplinary treatment with physical treatment, initial instruction by a physiotherapist may be applicable (reference: Simmonds JV, Keer RJ Man Ther. 2007). It is important to maintain physical strength and form to reduce the risk of injury. Regular exercise, possibly under the guidance of a physiotherapist. Avoid maximum range of motion and overstretching. Adapted to everyday life and work that does not overload joints, back and muscles. No effective drug treatment, diet/dietary supplement or gene therapy is known.
Literature
- Tofts LJ, 2009 (differential diagnosis in children)
- Scheper MC, 2014 (Prognosis / Complications)
- Grans Reef Compendium
