BACKGROUND: Generalized joint hypermobility as a characteristic feature of Ehlers-Danlos syndromes (EDS) is among the factors contributing to temporomandibular disorders (TMD).
OBJECTIVE: To evaluate the prevalence of TMD symptoms and their risk factors among women born in Sweden or Finland who were 27- to 78-year-olds with diagnosed hypermobile EDS (hEDS).
METHODS: A cohort of women with confirmed hEDS (n = 185) was constructed from the members of the National EDS Associations in both countries. Based on questionnaire data, frequency of independent variables in terms of socio-demographic, general health and oral health-related factors, comorbid symptoms and psychological distress for self-reported TMD symptoms as the dependent variables, were calculated first. Prevalence ratios (PR) and their 95% confidence interval (95% CI) were estimated for the association between independent and dependent variables.
RESULTS: Nearly all participants reported TMD symptoms (98%) with TMD pain (95%), TMJ clicking (90%) and jaw fatigue (80%) as the most common symptoms and TMJ crepitation (63%) and luxation (44%) as the least common symptoms. Risk factors for TMD among 27- to 50-year-olds participants were Finland as a country of birth, living alone and self-reported worst pain in the body (not the joints). The respective risk factors among the 51- to 78-year-olds were Finland as a country of birth, family history of EDS, tinnitus and regularly taking contraceptives.
CONCLUSIONS: Among adult women with confirmed hEDS, socio-demographic and health-related factors and comorbid symptoms were significantly associated with TMD but with differences regarding age group. Therefore, management of TMD requires a multidisciplinary approach among the affected.

BACKGROUND: The Ehlers-Danlos Syndromes (EDS) are a group of connective tissue disorders that are hereditary in nature and characterized by joint hypermobility and tissue fragility. The complex nature of this unique patient population requires multidisciplinary care, but appropriate centers for such care do not exist in large portions of the country. Need for more integrated services has been identified in Chicagoland, or Chicago and its suburbs. In order to explore and begin to address barriers to seeking appropriate care facing EDS patients in this region, we developed an online survey which we circulated through EDS social media groups for Chicagoland patients.
RESULTS: Three hundred and nine unique respondents participated. We found that there exists a strong medical need for and interest in the development of a center in the region, and participants reported that, if made available to them, they would make extensive and regular use of such a facility.
CONCLUSIONS: We conclude that the establishment of a collaborative medical center specializing in the diagnosis and treatment of EDS, Hypermobility Spectrum Disorder, and related disorders in the Chicagoland area would greatly benefit patients by providing comprehensive care, alleviate the burden on overworked healthcare providers, and contribute to the sustainability of medical facilities.

Joint hypermobility is a spectrum of symptoms associated with connective tissue disorders. The main feature is the increased range of joint mobility. Hypermobility is rarely recognised in clinical practice. The diagnosis is based on the evaluation of diagnostic tests, mainly the Beighton score. The divergence of research methods means that patients do not receive a proper diagnosis and treatment.
The study used the Beighton score and the Sachse scale. Both tests are \"all-or-none-tests\". Non-parametric correlations were used to assess the concordance effect. To this end, two methods were adopted, i.e., the Spearman Rank Correlation and Kendall tau Rank Correlation. The values of correlation coefficients were calculated, respectively, rho and Kendall tau. The study involved 30 women working as fitness instructors.
Consent results of hypermobility assessment for both methods were obtained in 3 cases, while the discrepancy in the hypermobility statement concerns measurements made in 10 participants. This cursory assessment already indicates a significant differentiation of results obtained for both methods.
To the best of our knowledge, there are not many studies comparing different HSD diagnostic methods. The Beighton score is the most commonly used, but the selection of only 5 joints for the examination does not show the systemic nature of hypermobility. A reliable methodology should be based not only on goniometric measurements of selected joints.
The expanded correlation analysis of Beighton and Sachse hypermobility tests indicates their poor compliance. Therefore, there is a need to standardise hypermobility spectrum disorder diagnostics, which may affect the objectification and credibility of these diagnostics.

Children with hypermobility spectrum disorder/hypermobile Ehlers-Danlos syndrome (HSD/hEDS) have a high prevalence of chronic pain, which may influence gait dynamics. However, little is known about pain outcomes and their association with gait spatiotemporal parameters in children with HSD/hEDS.
Does pain correlate with gait spatiotemporal parameters in children with HSD/hEDS?
Eighteen children with HSD/hEDS and eighteen typically developing (TD) children participated in the study. The current level of pain (0-10 on the numeric rating scale), modified Brief Pain Inventory, and Pain Catastrophizing Scale-Child version were implemented to assess pain in children with HSD/hEDS. All children completed a gait analysis at a self-selected speed. Mean and variability (measured using the coefficient of variation) of gait spatiotemporal parameters were analyzed. Gait parameters included stride length, stride time, gait speed, percent stance time, and step width. A Mann-Whitney U-test was used to compare the gait parameters between children with HSD/hEDS and TD children. Spearman correlations were used to examine the relationships between pain and gait spatiotemporal parameters in children with HSD/hEDS.
Children with HSD/hEDS had a longer percent stance time compared to TD children (p = 0.03). Lower pain interference in relationships with other people was significantly associated with faster gait speeds (ρ = -0.55, p = 0.03). Children with HSD/hEDS also had greater pain interference during mobility (ρ = 0.5, p = 0.05) and going to school (ρ = 0.65, p = 0.01), which were significantly correlated with greater stride length variability. Greater pain interference during enjoyment of life was significantly associated with greater percent stance time variability (ρ = 0.5, p = 0.05). Greater pain catastrophizing was correlated with decreased step width variability in children with HSD/hEDS (ρ = -0.49, p = 0.05).
Pain interference and catastrophe were significantly associated with gait spatiotemporal variability. Our findings suggest that assessing pain-associated gait alterations may help understand the clinical features and gait kinematics of children with HSD/hEDS.

UNASSIGNED: Hypermobility spectrum disorders (HSD) and Ehlers-Danlos syndromes (EDS) are multisystem conditions marked by dysfunctional connective tissue. This feasibility study evaluated a 9-week integrative medicine program in this population.
UNASSIGNED: Using a single-arm study design, adults with HSD or EDS were given recommendations for an anti-inflammatory Mediterranean diet and self-management with additional behavioral and psychosocial support. Preliminary data on feasibility based on recruitment and retention, adherence to the diet, mobile app tracking, changes to perceived well-being via health outcomes, and satisfaction with care were obtained.
UNASSIGNED: Thirteen participants were enrolled within a 4-month timeframe. Eight participants completed the study. Three participants met dietary tracking requirement in at least 4 of 8 intervention weeks and met the macronutrient requirements in at least half of the weeks tracked. No decreases in VAS pain scores after 5 and 9 weeks were noted; however, 62.5% (n = 5) of participants had decreased pain at 9 weeks, compared to baseline. There were significant improvements (p<.05) in six of twelve measurements of satisfaction with care at the end of the intervention.
UNASSIGNED: This study provides a foundation for future research on patient experience and introduces a novel treatment paradigm focused on nutrition and self-management.
Trial Registration: National Institutes of Health clinicaltrials.gov; identifier: NCT04734041.
UNASSIGNED: Lifestyle and dietary interventions are relatively safe and well tolerated in the hypermobility spectrum disorder (HSD) and Ehlers-Danlos disorder (EDS) population.Participants in our 9-week integrative medicine program actively engaged in self-management of their condition and showed promising adherence to dietary and tracking requirements.Effective treatment of the intricacies and dynamics of these highly variable and clinically heterogeneous disorders may require a network of healthcare providers, integrative healthcare, as well as behavioral and psychosocial support.Dietary tracking through mobile apps might help promote self-efficacy and adherence to dietary changes.Symptom tracking might be an effective way for patients to track changes to their health and could provide valuable information for health professionals engaged in managing the disorders.

This study aimed to determine the relationship between kinaesthesia, motor performance, fitness, and joint mobility in children.
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.
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.
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.

UNASSIGNED: Hypermobile Ehlers-Danlos Syndrome (hEDS) and Hypermobility Spectrum Disorders (HSD) are understudied conditions characterized by hallmark hypermobility and chronic pain. Disease manifestations lead to significant disability. Understanding predictors of disability, over and above the univariate construct of pain severity, is necessary to tailor treatment. Thus, the current study examined the impact of the Fear-Avoidance Model [FAM] on disability in hEDS/HSD. Fear of falling was included as a novel fear-avoidance factor impacting disability.
UNASSIGNED: A total of 168 individuals with hEDS/HSD answered a cross-sectional online survey regarding FAM constructs, fear of falling, disability, and clinical-demographic factors. A hierarchical regression analysis was used to assess whether FAM constructs and fear of falling significantly predicted disability, over and above pain severity and age.
UNASSIGNED: Pain catastrophizing, anxiety, and fear of falling contributed significant unique predictive relations, above age and average pain severity. Pain severity and fear of falling were the strongest unique predictors of disability.
UNASSIGNED: This is the first study to assess the relations among FAM constructs, pain severity, and disability in hEDS/HSD, and introduces fear of falling as a novel fear-avoidance factor specific to this population. Future research should apply these findings towards individualized interventions to improve disability in hEDS/HSD.
Disability is significant in hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders, and may be predicted by psychosocial factors of anxiety, pain catastrophizing, and fear of falling.Our findings suggest that improving disability in hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders involves the consideration of physical factors, such as pain severity, along with psychosocial factors, the latter of which are amenable to change through individualized intervention plans.Fear of falling is a novel fear-avoidance construct that should be considered in future research and clinical settings to increase functionality in this frequently falling population.

To identify the nature and extent of the evidence on psychological interventions among individuals with Ehlers-Danlos Syndrome (EDS) and Hypermobility Spectrum Disorder (HSD).
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.
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.
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.

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: 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.
CONCLUSIONS: Hypermobility is the rule in this pain-free population of children with GJH.