hyper mobility spectrum disorders - not on exam Flashcards
what is hyper mobility and what needs to be taken into consideration?
-joints moving beyond the normal limits taking into consideration the age, gender and ethnic background of the person
what are examples of specific disorders related to hyper mobility spectrum disorders?
-ehlers danlos syndrome
-marfans
-osteogenesis imperfecta
what are the symptoms of ehlers danlos syndrome EDS?
-joint hypermobility
-stretchy skin
-fragile skin that breaks or bruises easily
what is the name of the most common type of ehlers danlos syndrome?
hypermobile EDS
other than hyper mobile EDS, what are other examples of EDS?
-classical skin
-vascular
-hyphoscoliotic
what is vascular EDS?
a more severe type of EDS
-affects the walls of major blood vessels & organs
what is EDS characterised by?
a defect in collagen production which affects the bodies connective tissue eg skin, joints and blood vessels
what is the cause of EDS?
its usually inherited
describe marfans
a disorder that involves a mutation in a gene that affects the protein fibrillin which is essential for connective tissue integrity
what are examples of symptoms associated with marfans?
-tall, slender, square shoulders
-aortic enlargement
-eye problems (increased risk of cataracts)
-crowded teeth
-arachnodactily (long fingers) and flat feet
-lumbar hyper lordosis
describe osteogenesis imperfecta
-also called ‘brittle bone disease’
-genetic disorder that affects collagen production which leads to fragile bone
-there is an increased fracture rusk due to osteoporosis
-there are different types - type I to IV
what are musculoskeletal features of HSDs and HEDs?
-chronic/widespread pain due to mechanical overload
-recurrent STIS
-joint instability
-dislocations
-crepitus
-reduced proprioceptive sense
-hyperalgesia
-risk of developing early onset arthritis in shoulders and knees esp
other than MSK, what other features can be seen in HSD or HEDs?
-GI eg reflux, abdominal pain etc
-incontinence
-skin problems eg skin fragility and bruising
-dysautonomia - eg reduced BP, bradycardia, fatigue etc
-headaches
-internal organ problems
what are examples of questions to ask patients regarding joint stability?
-can you now or could you ever place your hands flat on the floor w/o bending knees?
-as a child did you amuse your friends by controlling your body into strange shapes or could you do the splits?
-do you consider yourself double jointed?
-as a child or teen, did your kneecap or shoulder dislocate on more than 1 occasion
what are examples of other important subjective features in HSD or HEDs?
-fatigue levels
-PMH eg skin, GI , eyes etc
-FH of hypermobilioty disorders
-other - important to screen biopsychosocial factors eg anxiety, depression, social engagement etc
what is the beighton scoring system?
a screening tool for hyper mobility (not recommended to use in isolation)
-if greater than 6 for pre-pubertal children and adolescents etc
what is important to assess in a physical exam for HSD or HEDs?
-affected joint flexibility / instability
-muscle tone and control
-strength and endurance
-CVS ditness
-proprioception
-other - skin, scars stretchy
-posture - curves of spine
who might these patients be referred onto?
-cardiology
-rheumatology
-pain services
-gastroenterology
what is the research behind strengthening exercise for HSD or HEDs?
-strengthening exercise is beneficial & helps with pain.
- proprioception can be improved with exercise, but exact type and parameters are unknown
are HSDs inflammatory conditions?
no
so inflammatory markers eg CRP or ESR in the blood shouldn’t be raised
what does physio Rx involve for HSDs?
-advice regarding rest, pacing activities etc
-tape and splinting
-care should be taken with manual therapy etc
what kind of therapeutic exercise is good for HSD>
-closed kinetic chain esp LL
-co-ordination
