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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.

It remains a clinical challenge identifying when joint hypermobility (JH) is responsible for pain. Previous nomenclature utilized terms such as (benign) joint hypermobility syndrome (JHS) but this was updated in 2017 as advances in genetics provide a basis for nearly all variants of Ehlers-Danlos syndrome (EDS) with the exception of hypermobile EDS (hEDS). New terminology describes hypermobility spectrum disorders (HSDs) as the updated term for JHS. Diagnosis of a subtype of HSDs should be considered in patients who have JH coupled with the presence of secondary musculo-skeletal manifestations (trauma, chronic pain, disturbed proprioception, and other manifestations) and at the exclusion of hEDS. Extra-articular manifestations are common. Treatment relies on management strategies for other chronic pain syndromes with a multidisciplinary approach likely optimal. Lifestyle modifications focus on weight loss and exercise. Physical therapy helps strengthen periarticular muscles, improving mobility. Pharmacologic therapies focus on judicious use of non-steroidal anti-inflammatory drugs and acetaminophen. Serotonin and norepinephrine reuptake inhibitor may help widespread pain. Avoidance of opioids remains prudent. The purpose of this review is to provide clinicians the rationale for the update in nomenclature, understand the musculoskeletal and extra-articular manifestations of the subtypes of HSDs, considerations when making the diagnosis, and treatment.

Background: The purpose of this study was to determine the prevalence of hypermobility in randomly selected healthy children, without previous trauma or disease process affecting the joints and whether other demographic variables (age, sex, BMI) had an impact on Beighton scores and range of motion (RoM) in children between 6 and 10 years of age. Results: 286 children were included; 27.3% of them had a Beighton score ≥7/9 and 72% would be classified hypermobile if we had used a Beighton cut-off score ≥4/9. Prevalence declined with increasing age. Girls were more often hypermobile (34%) than boys (20%) and this was mainly caused by increased RoM in the knees. Positive scores of finger items of the Beighton were more common than on the other items, leading to a high prevalence of peripheral hypermobility. Localized hypermobility was only found in the fifth MCP joint. A total of 15% of the children with normal mobility reached 20 excess degrees RoM of the left and right fifth MCP. Pain was present in 12 of the 239 children but was not linked to the level of mobility. Conclusion: Hypermobility is the rule in this pain-free population of children with GJH.

Background Joint Hypermobility Syndrome (JHS) is a Heritable Disorder of Connective tissue characterised by joint laxity and chronic widespread arthralgia. People with JHS exhibit a range of other symptoms including balance problems. To explore balance further, the objective of this study is to compare responses to forward perturbations between three groups; people who are hypermobile with (JHS) and without symptoms and people with normal flexibility. Methods Twenty-one participants with JHS, 23 participants with Generalised Joint Hypermobility (GJH) and 22 participants who have normal flexibility (NF) stood on a platform that performed 6 sequential, sudden forward perturbations (the platform moved to the anterior to the participant). Electromyographic outcomes (EMG) and kinematics for the lower limbs were recorded using a Vicon motion capture system. Within and between group comparisons were made using Kruskal Wallis tests. Results There were no significant differences between groups in muscle onset latency. At the 1st perturbation the group with JHS had significantly longer time-to-peak amplitude than the NF group in tibialis anterior, vastus medialis, rectus femoris, vastus lateralis, and than the GJH group in the gluteus medius. The JHS group showed significantly higher cumulative joint angle (CA) than the NF group in the hip and knee at the 1st and 2nd and 6th perturbation, and in the ankle at the 2nd perturbation. Participants with JHS had significantly higher CA than the GJH group at the in the hip and knee in the 1st and 2nd perturbation. There were no significant differences in TTR. Conclusions The JHS group were able to normalise the timing of their muscular response in relation to control groups. They were less able to normalise joint CA, which may be indicative of impaired balance control and strength, resulting in reduced stability.

ObjectivesTo describe the epidemiology of diagnosed hypermobility spectrum disorder (HSD) and Ehlers-Danlos syndromes (EDS) using linked electronic medical records. To examine whether these conditions remain rare and primarily affect the musculoskeletal system.DesignNationwide linked electronic cohort and nested case–control study.SettingRoutinely collected data from primary care and hospital admissions in Wales, UK.ParticipantsPeople within the primary care or hospital data systems with a coded diagnosis of EDS or joint hypermobility syndrome (JHS) between 1 July 1990 and 30 June 2017.Main outcome measuresCombined prevalence of JHS and EDS in Wales. Additional diagnosis and prescription data in those diagnosed with EDS or JHS compared with matched controls.ResultsWe found 6021 individuals (men: 30%, women: 70%) with a diagnostic code of either EDS or JHS. This gives a diagnosed point prevalence of 194.2 per 100 000 in 2016/2017 or roughly 10 cases in a practice of 5000 patients. There was a pronounced gender difference of 8.5 years (95% CI: 7.70 to 9.22) in the mean age at diagnosis. EDS or JHS was not only associated with high odds for other musculoskeletal diagnoses and drug prescriptions but also with significantly higher odds of a diagnosis in other disease categories (eg, mental health, nervous and digestive systems) and higher odds of a prescription in most disease categories (eg, gastrointestinal and cardiovascular drugs) within the 12 months before and after the first recorded diagnosis.ConclusionsEDS and JHS (since March 2017 classified as EDS or HSD) have historically been considered rare diseases only affecting the musculoskeletal system and soft tissues. These data demonstrate that both these assertions should be reconsidered.

Purpose This study aimed to determine the relationship between kinaesthesia, motor performance, fitness, and joint mobility in children. Methods A descriptive cross-sectional study was conducted involving children from two primary schools in the South-Eastern part of Nigeria. The Beighton criteria were used to measure joint mobility. Motor performance, fitness, and kinaesthesia were measured in all the children. Spearman’s rank correlation was used to evaluate the relationship between the outcomes. Results A total of 91 children (51.6% girls) participated in the study. The mean age of the children was 8.20 ± 1.98 years. Using a Beighton score of ≥ 6, Generalized Joint Hypermobility (GJH) was identified in a total of 35 (38.46%) children and was more prevalent in females (60.0%). Joint mobility had significant correlations with most fitness and motor performance items, but not kinaesthesia. Agility & power, and motor performance seem to be reduced if mobility is larger. Kinaesthesia was correlated with most fitness and motor performance items, indicating that better fitness and better motor performance cooccur with better kinaesthesia or vice versa. Conclusion Joint mobility may have a significant influence on fitness and motor performance in children. Hence, it may be useful for future studies to investigate how fitness and motor performance modulate the onset and progression of musculoskeletal symptoms in GJH.

To examine the perspective of individuals with Hypermobile Ehlers–Danlos Syndrome (hEDS) and Generalized Hypermobility Spectrum Disorder (G-HSD), needed to help identify priorities and improve the lives of people living with these conditions. We conducted an online survey between November 2021 and January 2023. Participants were recruited through the Ehlers-Danlos Society’s Research Surveys website. We obtained 483 responses and 396 were analyzed. 80% of the survey’s respondents were individuals with hEDS, 90% were females, 30% were between 21 and 30, and 76% lived in North America, with 85% of the participants living in North America indicated being White or European American. Participants reported exercising from none to less than three times per week, with no physical therapy treatment. Ninety-eight percent of participants reported pain mainly at the neck (76%), lower (76%) and upper back (66%), knees (64%), shoulders (60%), and hips (60%). Approximately 80% of participants reported fatigue, joint hypermobility, joint instability, interference with daily activities, gastrointestinal problems, orthostatic hypotension, muscle weakness, and emotional distress. About 60% of respondents indicated walking issues, balance issues, and reduced joint proprioception. Nearly 40% of participants reported pelvic floor dysfunction and cardiovascular problems. Participants with hEDS and G-HSD reported pain on an average (SD) of 6.4 (± 1.3) and 5.9 (± 1.5) days in a typical week, respectively. People with hEDS and G-HSD desperately need more effective treatment options, a better diagnostic process, and education among health care providers.

Background Previous research has demonstrated impaired proprioception and poorer responses to tactile deep pressure, visual-tactile integration, and vestibular stimuli in individuals with generalized hypermobility, potentially leading to sensory processing issues. Therefore, we aimed to explore the influence of hypermobility on somatognosia and stereognosia. Methods Forty-six participants were assessed using the Beighton score and categorized into three groups: non-hypermobile ( n  = 20), symptomatic hypermobile ( n  = 13), and asymptomatic hypermobile ( n  = 13). Somatognosia was evaluated using the shoulder width test in the vertical plane and pelvic width test in the vertical and horizontal planes. Stereognosia was assessed with Petrie’s test. Spearman’s rank correlation coefficient was examined the relationship between the Beighton score and measures of somatognosia and stereognosia. An unpaired t-test was used to compare variables between hypermobile (both symptomatic and asymptomatic) and non-hypermobile individuals, while a one-way ANOVA was used to compare data between the three groups. Results No significant relationship was observed between Beighton scores and measures of somatognosia and stereognosia. The t-test revealed no statistically significant differences between hypermobile and non-hypermobile groups in the shoulder width, two pelvic widths, and Petrie’s tests (all p  ≥ 0.105). Similarly, one-way ANOVA showed no statistically significant differences between the three groups across these tests (all p  ≥ 0.177). Conclusions The results indicate that somatognosia and stereognosia are not significantly related to the Beighton score and do not significantly differ between the groups studied. These sensory processing functions are unlikely to contribute to the common complaints reported by hypermobile individuals. Clinical trial number Not applicable.

Purpose To identify the nature and extent of the evidence on psychological interventions among individuals with Ehlers-Danlos Syndrome (EDS) and Hypermobility Spectrum Disorder (HSD). Materials and methods Eligible studies reported on psychological interventions for individuals of all ages with EDS and/or HSD. All studies published in English were included, with no restrictions to publication year or status. MEDLINE, CINAHL, EMBASE, and PsycINFO were searched. Two reviewers independently screened studies and abstracted data. Results This scoping review included 10 studies reporting on EDS, HSD, or both. Only cohort studies and case studies were identified. Four studies investigated Cognitive Behavioural Therapy (CBT), one investigated Dialectical Behavioural Therapy (DBT), two investigated psychoeducation, two investigated Intensive Interdisciplinary Pain Treatment (IIPT), and one investigated Acceptance Commitment Therapy (ACT). Interventions targeted pain management, self-destructive behaviours, and related psychological issues (e.g., depression/anxiety). Sample sizes were small (n < 50) for most studies and interventions were generally poorly described. Conclusions There is a critical need for high-quality research surrounding psychological interventions for individuals with EDS/HSD. Psychological interventions for these individuals are understudied and existing studies lack validity. Researchers should investigate psychological interventions for individuals with all types of EDS/HSD with high-quality studies to validate findings from the existing studies.

Ehlers–Danlos syndromes (EDSs) constitute a heterogeneous group of connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. Asymptomatic EDSs, joint hypermobility without associated syndromes, EDSs, and hypermobility spectrum disorders are the commonest phenotypes associated with joint hypermobility. Joint hypermobility syndrome (JHS) is a connective tissue disorder characterized by extreme flexibility of the joints, along with pain and other symptoms. JHS can be a sign of a more serious underlying genetic condition, such as EDS, which affects the cartilage, bone, fat, and blood. The exact cause of JHS could be related to genetic changes in the proteins that add flexibility and strength to the joints, ligaments, and tendons, such as collagen. Membrane proteins are a class of proteins embedded in the cell membrane and play a crucial role in cell signaling, transport, and adhesion. Dysregulated membrane proteins have been implicated in a variety of diseases, including cancer, cardiovascular disease, and neurological disorders; recent studies have suggested that membrane proteins may also play a role in the pathogenesis of JHS. This article presents an exploration of the causative factors contributing to musculoskeletal pain in individuals with hypermobility, based on research findings. It aims to provide an understanding of JHS and its association with membrane proteins, addressing the clinical manifestations, pathogenesis, diagnosis, and management of JHS.