TITLE: Chronic pain in hypermobility syndrome and Ehlers–Danlos syndrome (hypermobility type): it is a challenge
PUBLICATION: Dove Press, Journal of Pain Research
DATE: August 2015
AUTHORS : University of Applied Sciences in Amsterdam, Mark C Scheper, Janneke E de Vries, Jeanine Verbunt, Raoul HH Engelbert
Beighton score: considered the gold standard and the most used in classifying GJH from infancy to old age. It consists of five clinical maneuvers performed bilaterally, in which a positive score in ≥4 joints indicates the presence of GJH. The higher the score, the higher the laxity.
- One point is given if palms touch the floor in a standing forward bend with legs straight.
- One point for each elbow that bends backwards
- One point for each knee that bends backwards
- One point for each thumb that touches the forearm when bent backwards
- One point for each little finger that bends backwards beyond 90 degrees.
Brighton criteria: a set of clinical criteria and morphological features used for diagnosing Hypermobility Syndrome (HMS) that includes a Beighton score of 4 or higher and joint pain in 4 or more joints for more than 3 months. It can also include abnormalities in the skin, marfanoid habitus and other signs of tissue laxity.
Central Sensitization (CS): condition of the nervous system that is associated with the development and maintenance of chronic pain. When CS, the nervous system goes through a process called “wind-up” and gets regulated in a persistent state of high reactivity.
Copers: are those that have GJH but don’t have chronic musculoskeletal complaints lasting longer than 3 months. GJH is very common in athletes, dancers, martial artists, and gymnasts. They are at more risk for musculoskeletal pain but are high functioning in performance and motor control. Poor proprioception may be present as well as other symptoms of GJH.
Ehlers-Danlos syndrome: a collection of heritable connective tissue disorders thought to alter the biology of collagen in the body (the most abundant protein), which can lead to multi-systemic symptoms, such as: hypermobile joints and soft, stretchy skin, easy bruising, easy wounding, poor wound healing and/or atrophic scarring.
Ehlers–Danlos syndrome hypermobility type (EDS-HT): within the Ehlers–Danlos spectrum, a similar subcategory of patients having similar clinical features as HMS but lacking a specific genetic profile.
Generalized Joint Hypermobility (GJH): common with patients who have chronic pain. It’s a prevalent clinical characteristic that is present in 2%–57% (that’s a huge range) of the healthy population and is dependent on age, sex, and ethnicity. Since 1967 it’s been documented as being associated with chronic pain. Because clinicians are not as aware of GJH-related chronic pain, patients tend to search for years before being diagnosed. It’s common in Marfan syndrome, osteogenesis imperfecta and Ehlers-Danlos syndrome.
Hyperalgesia: increased sensitivity to pain.
Hypermobility Syndrome (HMS): When generalized joint hypermobility is accompanied by pain in 4 or more joints for more than 3 months.
Marfan Syndrome: a genetic disorder that affects the body’s connective tissue. Marfan syndrome is caused by a defect (or mutation) in the gene that tells the body how to make fibrillin-1. This mutation results in an increase in a protein called transforming growth factor beta.
Osteogenesis Imperfecta (OI): a genetic bone disorder characterized by fragile bones that break easily. It is also known as “brittle bone disease.”