When the Hypermobility Syndrome was first put on the medical map
in 1967, it was defined as the presence of musculoskeletal symptoms
(predominantly pain) occurring in otherwise healthy individuals. Thirty years
down the line we now think that there are probably two types of
hypermobility.
The first is a milder type occurring in people whose
joints are just like everyone else's but which have the capacity to move more
than most people's joints. The other, a more marked form, has features that
suggest that it may be part of an inherited connective tissue disorder similar
to the hypermobile form of the Ehlers-Danlos Syndrome, formerly called EDS III.
It probably is EDS. At the present time we simply do not know for certain
whether or not HMS is merely a less severe type of EDS III. Pain can occur in
other forms.
It is often accompanied by an intense sense of exhaustion.
the severity of the pain we feel is greatly influenced by our state of mind.
If we are upset or agitated it tends to increase. If we are content, relaxed
or just happy it tends to diminish. The HMS/EDS people are often in the former
category, and for good reason! Lack of understanding of the condition is
widespread, and this, coupled with failure to receive adequate treatment for
relief of symptoms, leads to frustration, resentment, anger (and lots more
emotions which I could list but readers know them all only too well!) and,
ultimately depression. These emotional influences can amplify pain, but they do
not cause it.(Prof R Grahame CBE, MD, FRCP, FACP. University College Hospital,
LONDON)
http://www.ehlersdanlos.ca/hyperpain.htm
Joint
hypermobility results from genetic variations in connective tissue matrix
proteins resulting in stretchier tissues. For many it is an asset that confers
greater facility for physical prowess. Others, less fortunate, fall prey to the
associated effects of tissue fragility. The most frequently encountered
constellation of traumatic and overuse injuries is termed the (benign joint)
hypermobility syndrome (BJHS). This condition, poorly understood, frequently
overlooked, misdiagnosed and inappropriately treated, is the cause of much
needless suffering and anguish. Accumulating evidence suggests that JHS
represents a forme fruste of an heritable disorder of connective alongside
Marfan syndrome, Ehlers-Danlos syndrome and osteogenesis imperfecta, with which
it shares many overlapping features, but from which it can be phenotypically
distinguished on the basis of clinical features and prognosis. The responsible
gene defects have yet to be elucidated.(Grahame R. "Hypermobility--not a circus
act" Int J Clin Pract. 2000
Jun;54(5):314-5.
This article seeks to
draw readers' attention to the importance of the heritable disorders of
connective tissue in clinical practice. It describes the principal features of
the Marfan and Ehlers-Danlos syndromes, and osteogenesis imperfecta, their
clinical and prognostic similarities and differences, and their distinguishing
features. Recently revised international classifications drawing on advances in
molecular genetics are described in detail. Wherever possible, patients'
symptoms are explained on the basis of the altered biomechanics of genetically
aberrant connective tissue matrix proteins. Finally, the chapter draws attention
to the often unrecognized burden of chronic pain borne by patients with these
conditions, a feature of which many rheumatologists seem unaware, and sets out a
rational and holistic approach to treatment and management that is based on the
best currently available evidence. (Grahame R.Heritable disorders of connective
tissue. Baillieres Best Pract Res Clin Rheumatol. 2000 Jun;14(2):345-61).
