When excessive mobility becomes a problem

The 2017 international classification replaces previous terms for symptomatic joint hypermobility. Ehlers-Danlos syndrome (EDS) and hypermobility spectrum disorder (HSD) are clinical diagnoses. The primary therapeutic goals are to alleviate the symptoms, prevent joint injuries and provide the best possible education for those affected.

Hypermobility is the umbrella term for a greater range of motion in one or more joints. Around 10-20% of the general population exhibit generalized or local hypermobility, reported Dr. Gere Luder, physiotherapist in Bern [1]. Hypermobility with functional limitations and pain is much rarer, with a prevalence of 0.1% [2]. The new international classification criteria published in 2017 replace previous terms for symptomatic joint hypermobility and introduce the term hypermobility spectrum disorder ( HSD) for patients who do not meet the diagnostic criteria for Ehlers-Danlos syndrome (EDS) [3,4].

Hypermobile EDS is the most common – assessment of findings using a checklist

This international classification describes thirteen different subtypes of EDS, with hypermobile EDS (hEDS) accounting for around 80-90% of all cases and is therefore by far the most common (Overview 1) [3,4]. Further efforts to advance research into EDS and HDS and the clinical management of the diseases are summarized under the slogan “The Road to 2026”. In particular, the aim is to shorten the diagnosis latency and improve treatment options [5]. Genetic testing can be performed to differentiate between specific subtypes of EDS, although there are no genetic tests for the most common subtype, hEDS [6,7]. This subtype is characterized by joint hypermobility, skin findings and joint pain or recurrent dislocations and is clinically diagnosed on the basis of specific criteria [3]. A summary of the criteria according to the 2017 international classification is as follows (all three must be met for a confirmed hEDS) [1,3]:

Generalized joint hypermobility: Nowadays, age-dependent limits are common for the Breighton score; for 20-50-year-olds, the critical limit is ≥5 (Table 1)
Systemic criteria: Two or more features from three groups of systemic manifestations (skin, connective tissue), family history and/or musculoskeletal complications
Exclusion of other diagnoses: absence of unusual skin vulnerability; exclusion of other connective tissue diseases and/or alternative diagnoses/EDS subtypes.

A checklist for a structured assessment of these three criteria is available at the following web link: www.ehlers-danlos-initiative.de/images/mwb/pdf/hEDS_Diagnose_Checkliste_v2.pdf

What are the possible consequences of hypermobility?

Hypermobile people often exhibit reduced muscle strength and altered kinematics when walking. “Injuries are a big issue,” says Dr. Luder [1]. The risk of cruciate ligament ruptures is several times higher. Studies have shown that hypermobility is associated with impaired proprioception. In the long term, there is an increased risk of osteoarthritis, particularly in the knee and back. Hypermobile people are more likely to develop chronic pain and fibromyalgia than healthy people. The latter is about four times as common with hypermobility. Those affected often avoid activities and exercise, which can lead to deconditioning. Psychological side effects have also been described, with hypermobile people suffering more frequently from anxiety disorders and depression, for example. A specific questionnaire developed for people with hypermobility is the Bristol Impact on Hypermobility [8]. It is a valid and reliable tool for recording pain, activities and limitations in a structured way, and a German translation is planned, the speaker reported [1].

How useful is strength training?

There are several reasons why people with hypermobility can benefit from strength training, explained Dr. Luder [1]. Strengthening exercises can improve proprioception and stability, among other things. Strength training also makes sense in terms of motor control, body awareness and self-efficacy. Experience and studies show that strength training is feasible and safe for women with joint hypermobility. The risk of pain and injury does not appear to be increased. Whether strength and muscle mass can be increased by 12 weeks of strength training cannot be clearly answered on the basis of the available studies. Although a randomized study in women with joint hypermobility found a higher percentage increase in the muscle cross-sectional area of the thigh (^mm2) in the intervention group compared to the control group (+2.4% vs. +0.6%), this was not one of the target parameters targeted in this study [1,9]. No significant effects were achieved with regard to a measurable increase in strength or outcome parameters such as stair climbing. As possible explanations for this, the speaker mentioned too low a training intensity with too little increase in resistance [1]. This may be due to a lack of motivation and fear of pain. More guidance is probably needed to adapt the training program to individual requirements and needs. In a case series published in 2022 by Henriksen et al. a 12-week strength training program was able to reduce pain, increase strength and improve quality of life in 16 participants (women with generalized hypermobility) [10]. In this study, 5 exercises were carried out twice a week, with close supervision.

In general, it is important to supplement strength training with functional exercises and proprioceptive training (balance or jumping exercises). End positions of joints should be avoided as far as possible, which also applies to people with hypermobility in everyday life, says Dr. Luder [1].

Literature:

“Physiotherapy for hypermobility: When mobility becomes a problem”, Dr. Gere Luder, Symposium for Musculoskeletal Medicine, 11.04.2024.
Mariacher-Gehler S: Hyperlaxity syndrome, https://rheuma-schweiz.ch/krankheitsbilder/fachbereiche/weichteilrheumatismus/hyperlaxitaetssyndrom-2021,(last accessed, 12.06.2024).
Malfait F, et al: The 2017 International Classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet 2017; 175(1): 8-26.
Ehlers-Danlos organization: clinical picture, https://ehlers-danlos-organisation.de/index.php/erkrankung/krankheitsbild,(last accessed 12.06.2024).
Ehlers-Danlos Organization: The road to 2026, www.ehlers-danlos.com/road-to-2026/de,(last accessed 12.06.2024).
Miklovic T, Sieg VC: Ehlers-Danlos syndrome. 2023 May 29. in: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. PMID: 31747221.
Mayo Clinic: Ehlers-Danlos Syndrome, www.mayoclinic.org/diseases-conditions/ehlers-danlos-syndrome/diagnosis-treatment/drc-20362149,(last accessed 12.06.2024).
Palmer S, et al: Test-retest reliability and smallest detectable change of the Bristol Impact of Hypermobility (BIoH) questionnaire. Musculoskelet Sci Pract 2017; 32: 64-66.
Luder G, et al: Effect of resistance training on muscle properties and function in women with generalized joint hypermobility: a single-blind pragmatic randomized controlled trial. BMC Sports Sci Med Rehabil 2021; 13(1): 10.
Henriksen P, et al: Supervised, Heavy Resistance Training Is Tolerated and Potentially Beneficial in Women with Knee Pain and Knee Joint Hypermobility: A Case Series. Transl Sports Med 2022 Dec 30; 2022: 8367134.
Malek S, Reinhold EJ, Pearce GS: The Beighton Score as a measure of generalized joint hypermobility. Rheumatol Int 2021; 41(10): 1707-1716.