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hypermobility syndrome

Definition of

Also known as familial hypermobility syndrome, familiar articular hypermobility syndrome, hypermobility syndrome, hypermobile joint syndrome or benign hypermobile joint syndrome, this is a non-uniform group of congenital disorders in the area of connective tissue that manifest a general hypermobility in the joints, often accompanied by muscular complaints. The diagnosis requires the exclusion of all diseases relevant to the differential diagnosis, such as RA, Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, fibromyalgia and growth-related pain. Kirk described this disorder in otherwise completely healthy people in 1967, before that there were only isolated case descriptions. Hypermobility of a joint is described as active if it can cause the abnormal joint position itself, otherwise, if the abnormal position can only be achieved with the use of external force, it is referred to as passive hypermobility. The diagnosis can only be made after puberty, for example, because age-related hypermobility is inherent in children. The incidence in Europe is 3%, W:M 3-5:1. The Beighton Score is generally used for classification, which awards one point for each of the following:

1. The ability to place the palms of the hands on the floor with the knees extended
2. any elbow joint that can be hyperextended by at least 10
3. any thumb that can touch the forearm
4. each little finger that can be hyperextended by at least 90° in the base joint
5. any knee joint that can be hyperextended by at least 10

The score classifies with the sum formed in

1. 0-2: not hypermobile
2. 3-4: moderately hypermobile
3. from 5: generalized hypermobility

This at least covers the first criterion, which is already present in people who are well trained in flexibility for purely muscular reasons and without any special features of a joint. Generalized hypermobility is not pathogenic per se, but clearly predisposes to painful synovial, capsular, periarticular enthesiopathic (painfulness of a tendinous bone attachment) and muscular reactions as a result of functional and morphological overuse due to instability, dislocations and traumatic vulnerability. The score obtained does not allow any conclusions to be drawn about the symptoms or prognosis for development, nor vice versa. If the Beighton score is embedded in the Beighton criteria, these achieve a sensitivity and specificity of 93% each: Main criteria:

1. Beighton score of at least 4 current or earlier
2. Arthralgia in at least 4 joints for at least 3 months

Secondary criteria:

1. Beighton from 1-3, from 50 years of age also 0
2. Arthralgia for at least 3 months in 1-3 joints, back pain, spondylolisthesis or spondylarthrosis
3. Dislocations or subluxations in more than one joint or several times in one joint on different occasions
4. „Soft tissue rheumatism“ such as tennis elbow or golfer’s elbow, tendovaginitis or bursitis at more than 3 sites
5. Marfanoid habitus, i.e. tall, slender, ratio of arm span to body height of at least 1.03, ratio of legs to torso of at least 0.89, spider fingers
6. Abnormal skin with stretch marks, thin, peelable skin or cigarette paper scars
7. Eye abnormalities such as drooping eyelids, antimongoloid eye axis
8. Varicose veins, hernias, uterine or rectal prolapse

The diagnosis of hypermobility syndrome is made in the presence of

1. two main criteria
2. 1 main criterion and 2 secondary criteria
3. 4 Secondary criteria
4. 2 secondary criteria and positive family history in the first degree

Hypermobility syndrome is still little known among doctors as a connective tissue disorder, which is why it is often not correctly diagnosed and treated.

ICD M35.7

Cause

1. congenital

Symptoms

1. Usually completely asymptomatic, but various excessive movements caused by abnormal connective tissue structures are noticeable

Complications

1. Difficulties learning to walk
2. Normal life expectancy except for cases of vascular involvement
3. Reduced quality of life due to pain and functional limitations
4. Strokes due to changes in blood vessels
5. Pain due to sprains and dislocations even without trauma or external impact, even in the absence of radiology findings
6. Prescribed pain therapy is preferable to self-medication with NSAIDs due to the risk of long-term side effects
7. Nerve compression due to tense muscles or spondylolisthesis, triggering even without trauma

Therapy

1. No known causal therapy
2. Treatment of orthopaedic problems, preferably conservative
3. Physiotherapy
4. Posture training. Avoid overstretching. Be careful with weight training, NO contact sports!
5. Orthosis if necessary
6. Behavioral training
7. Avoidance of terminal joint positions
8. Be careful with stretching exercises!
9. Tai chi, yoga, strengthening movement therapies without the risk of dislocationand end-degree movements

NHK

1. Tai Chi
2. Strengthening movement therapies without the risk of dislocationand final movements