Joint hypermobility: bibliography

Latest publications -  updated June 2021

Cattalini, M., Khubchandani, R., & Cimaz, R. (2015). When flexibility is not necessarily a virtue: a review of hypermobility syndromes and chronic or recurrent musculoskeletal pain in children. Pediatric rheumatology online journal13(1), 40. https://doi.org/10.1186/s12969-015-0039-3

de Vries, J., Verbunt, J., Stubbe, J., Visser, B., Ramaekers, S., Calders, P., & Engelbert, R. (2021). Generalized Joint Hypermobility and Anxiety in Adolescents and Young Adults, the Impact on Physical and Psychosocial Functioning. Healthcare (Basel, Switzerland)9(5), 525. https://doi.org/10.3390/healthcare9050525

Sobhani-Eraghi, A., Motalebi, M., Sarreshtehdari, S., Molazem-Sanandaji, B., & Hasanlu, Z. (2020). Prevalence of joint hypermobility in children and adolescents: A systematic review and meta-analysis. Journal of research in medical sciences : the official journal of Isfahan University of Medical Sciences25, 104. https://doi.org/10.4103/jrms.JRMS_983_19

Malek, S., Reinhold, E. J., & Pearce, G. S. (2021). The Beighton Score as a measure of generalised joint hypermobilityRheumatology international, 10.1007/s00296-021-04832-4. Full text  https://link.springer.com/article/10.1007/s00296-021-04832-4

The Beighton Score (BS) is a set of manoeuvres in a nine-point scoring system, used as the standard method of assessment for Generalised Joint Hypermobility (GJH). It was originally developed as an epidemiological tool used in screening large populations for GJH, but later adopted as a clinical tool for diagnostic purposes. Its ability to truly reflect GJH remains controversial, as joints within the scoring system are predominantly of the upper limb and disregard many of the major joints, preventing a direct identification of GJH. Furthermore, a consistent finding in the literature whereby the BS failed to identify hypermobility in joints outside the scoring system suggests its use as an indirect indicator of GJH is also not viable. As such, the collective findings of this review demonstrate a need for a change in clinical thinking. The BS should not be used as the principle tool to differentiate between localised and generalised hypermobility, nor used alone to exclude the presence of GJH. Greater emphasis should be placed on a clinician’s judgement to identify or exclude GJH, according to its full definition.

Nikolajsen, H., Juul-Kristensen, B., Hendriksen, P. F., & Jensen, B. R. (2021). No difference in knee muscle activation and kinematics during treadmill walking between adolescent girls with and without asymptomatic Generalised Joint Hypermobility. BMC musculoskeletal disorders22(1), 170. https://doi.org/10.1186/s12891-021-04018-w

Conclusion: Muscle activity during gait was not different between participants with GJH and non-GJH participants. However, participants with GJH displayed minor but statistically significant increased knee flexion during gait

Jensen, B. R., Sandfeld, J., Melcher, P. S., Johansen, K. L., Hendriksen, P., & Juul-Kristensen, B. (2016). Alterations in neuromuscular function in girls with generalized joint hypermobility. BMC musculoskeletal disorders, 17(1), 410. https://doi.org/10.1186/s12891-016-1267-5

Girls with GJH and non-GJH (NGJH) performed isometric knee flexions (90°,110°,130°), and extensions (90°) at 20 % Maximum Voluntary Contraction, and explosive isometric knee flexions while sitting. EMG was recorded from knee flexor and extensor muscles.

Results: Early rate of torque development was 53 % faster for GJH. Reduced hamstring muscle activation in girls with GJH was found while knee extensor and calf muscle activation did not differ between groups. Flexion-extension and medial-lateral co-activation ratio during flexions were higher for girls with GJH than NGJH girls.

Conclusions: Girls with GJH had higher capacity to rapidly generate force than NGJH girls which may reflect motor adaptation to compensate for hypermobility. Higher medial muscle activation indicated higher levels of medial knee joint compression in girls with GJH. Increased flexion-extension co-activation ratios in GJH were explained by decreased agonist drive to the hamstrings

Steinberg N, Tenenbaum S, Zeev A, Pantanowitz M, Waddington G, Dar G, Siev-Ner I. Generalized joint hypermobility, scoliosis, patellofemoral pain, and physical abilities in young dancers. BMC Musculoskelet Disord. 2021 Feb 9;22(1):161. 

A high prevalence of young girls participating in dance classes had GJH, as the increased joint flexibility probably provides them with some esthetic advantages. The high prevalence of scoliosis found in these young dancers might be attributed to their relatively low body mass, their delayed maturation, and the selection process of dancers. Dancers with both GJH and scoliosis had decreased muscle strength, reduced postural balance, reduced proprioception, with higher risk of PFP. The main clinical implications are the need to reduce the risk of PFP among dancers by developing appropriate strength and stabilizing exercises combined with proprioceptive and postural balance training, to improve the correct alignment of the hyperextended and hypermobile joints, and to improve their supporting muscle strength.

Kindgren, E., Quiñones Perez, A., & Knez, R. (2021). Prevalence of ADHD and Autism Spectrum Disorder in Children with Hypermobility Spectrum Disorders or Hypermobile Ehlers-Danlos Syndrome: A Retrospective Study. Neuropsychiatric disease and treatment, 17, 379–388. https://doi.org/10.2147/NDT.S290494

All EDS cases could be classified as hEDS. Of the entire study cohort, 16% had a verified ADHD diagnosis and a further 7% were undergoing ADHD diagnostic investigation. Significantly more children with hEDS had ADHD compared to children with HSD (p=0.02). In the age group 15-16 years, 35% of those with hEDS had ADHD and, among those aged 17-18 years, ADHD was present in 46%. Children with coexisting ADHD showed a significantly higher proportion of associated symptoms such as fatigue, sleep-problems, and urinary tract problems. ASD had been verified in 6% of the children. Of those with ASD, 92% had sleep problems.

Conclusion: This study shows a strong association between HSD or hEDS and ADHD or ASD. Therefore, children with HSD or hEDS may need to be routinely screened for neuropsychiatric symptoms.

Bibliography

De Wandele, I., Calders, P., Peersman, W., Rimbaut, S., De Backer, T., Malfait, F., … Rombaut, L. (2014). Autonomic symptom burden in the hypermobility type of Ehlers-Danlos syndrome: a comparative study with two other EDS types, fibromyalgia, and healthy controls. Seminars in Arthritis and Rheumatism, 44(3), 353–61.

Murray, K. J. (2006). Hypermobility disorders in children and adolescents. Best Practice & Research. Clinical Rheumatology, 20(2), 329–51.

Pacey, V., Adams, R. D., Tofts, L., Munns, C. F., & Nicholson, L. L. (2014). Proprioceptive acuity into knee hypermobile range in children with joint hypermobility syndrome. Pediatric Rheumatology Online Journal, 12, 40. doi:10.1186/1546-0096-12-40

Pacey, V., Adams, R. D., Tofts, L., Munns, C. F., & Nicholson, L. L. (2015). Joint hypermobility syndrome subclassification in paediatrics: a factor analytic approach. Archives of Disease in Childhood, 100(1), 8–13. doi:10.1136/archdischild-2013-305304

Pacey, V., Tofts, L., Wesley, A., Collins, F., & Singh-Grewal, D. (2014). Joint hypermobility syndrome: A review for clinicians. Journal of Paediatrics and Child Health, 1–8. doi:10.1111/jpc.12731

Plackett, T. P., Kwon, E., Gagliano, R. a, & Oh, R. C. (2014). Ehlers-danlos syndrome-hypermobility type and hemorrhoids. Case Reports in Surgery, 2014, 

Remvig L, Jensen DV, Ward RC. Epidemiology of general joint hypermobility and basis for the proposed criteria for benign joint hypermobility syndrome: review of the literature. J Rheumatol.2007;34:804–809.

Rombaut, L., Scheper, M., De Wandele, I., De Vries, J., Meeus, M., Malfait, F., … Calders, P. (2014). Chronic pain in patients with the hypermobility type of Ehlers-Danlos syndrome: evidence for generalized hyperalgesia. Clinical Rheumatology. doi:10.1007/s10067-014-2499-0

Scheper, M. C., Engelbert, R. H., Rameckers, E. A., Verbunt, J., Remvig, L., & Juul-Kristensen, B. (2013). Children with generalised joint hypermobility and musculoskeletal complaints: state of the art on diagnostics, clinical characteristics, and treatment. BioMed research international2013, 121054. https://doi.org/10.1155/2013/121054

Shirley, E. D., Demaio, M., & Bodurtha, J. (2012). Ehlers-danlos syndrome in orthopaedics: etiology, diagnosis, and treatment implications. Sports Health, 4(5), 394–403. doi:10.1177/1941738112452385

Smits-Engelsman, B., Klerks, M., & Kirby, A. (2011). Beighton score: a valid measure for generalized hypermobility in children. The Journal of Pediatrics, 158(1), 119–23, 123.e1–4. doi:10.1016/j.jpeds.2010.07.021

Tofts, L. J., Elliott, E. J., Munns, C., Pacey, V., & Sillence, D. O. (2009). The differential diagnosis of children with joint hypermobility: a review of the literature. Pediatric Rheumatology Online Journal7, 1. doi:10.1186/1546-0096-7-1  Link to article 

Voermans, N. C., Knoop, H., van de Kamp, N., Hamel, B. C., Bleijenberg, G., & van Engelen, B. G. (2010). Fatigue is a frequent and clinically relevant problem in Ehlers-Danlos Syndrome. Seminars in Arthritis and Rheumatism, 40(3), 267–74. doi:10.1016/j.semarthrit.2009.08.003