Hypermobility Spectrum disorder Flashcards
What is hypermobility?
- Pain syndrome in people with joints that mvoe beyond normal limits
- Often referred to as being double jointed
- No precise definition as joint mobility is graduated
- Can affect any joints and any number
- Due to laxity of ligaments, capsules and tendons
Where does pain arise from in hypermobility?
Microtrauma
Testing for hypermobility - genetic?
No genetic test
Who gets hypermobility?
10% population
Only few are symptomatic
Familial
More common women and asian population
When does hypermobility present/occur?
Presents in childhood or young adulthood
What can be a feature of hypermobility/complications?
Recurrent subluxations or disclocations
Main symptom of hypermoblity
- Pain around joints
- Worse after activity
- Pain may generalise though
- Fatigue
Other features of hypermobility
- Soft tissue rheumatism - epicondylitis (eg tennis elbow, tendon inflamed)
- Abnormal skin - papyraceous scars, hyperextensible, thin, striae
- Marfanoid habitus
- Arachnodactyly
- Drooping eyelids or myopia
- Hernias and uterine/rectal prolapses
What is Marfanoid habitus?
Features suggesting Marfans eg:
* Tall stature
* Long limbs
* Pectus excavatum or pectus carinatum (sternum sunken and protruding)
* High arched palate
* Scoliosis
Treatment for hypermobility - aims
- Improve pain
- Reduce disability
Non-drug therapy for hypermobility
- Main treatment
- Strengthening exercises to prevent joint subluxation
- Work on posture and balance
- Splinting or surgical interventions may be needed
- Goal setting
- Specialist pain management
Pharmacological therapy
No good evidence on which
Paracetamol should be main treatment
Scoring system for hypermobility
Beighton score has these domains, score 1 for left and one for right, total / 9 :
* Can put hands flat on floor with knees straight
* Bend elbows backwards (hand on wall)
* Bend knee backwards? (lift foot)
* Bend thumb back onto front of forearm?
* Bend little finger up at 90 degrees to the back of your hand
Heritable connective tissue disorders
- Ehlers Danlos
- Marfan syndrome
- Hypermobility spectrum disorder
How to distinguish between the connective tissue disorders
Genetic testing - lots of phenotypic overlap
