BackgroundIn adolescents with non-pathological and pathological joint hypermobility, gait deviations have been associated with pain and fatigue. It remains unclear what distinguishes the non-pathological form of joint hypermobility (JH) from pathological forms (i.e. hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorders (HSD). Our objective was to identify discriminative clinical characteristics and biomechanical gait features between adolescents with hEDS/HSD, JH, and healthy controls (HC).MethodsThirty-two adolescents were classified into three subgroups (hEDS/HSD=12, JH=5, HC=15). Clinical characteristics (e.g. pain intensity and surface, fatigue, functional disability) were inventoried.The gait pattern was assessed using a three-dimensional, eight-camera VICON MX1.3 motion capture system, operating at a sample rate of 100 Hz (VICON, Oxford, UK). Spatiotemporal parameters, joint angles (sagittal plane), joint work, joint impulse, ground reaction force and gait variability expressed as percentage using Principal Component Analysis (PCA) were assessed and analysed using multivariate analysis. Multivariate analysis data is expressed in mean differences(MD), standard error(SE) and P-values.ResultsThe hEDS/HSD-group had significantly higher fatigue score (+51.5 points, p = <0.001) and functional disability (+1.6, p < .001) than the HC-group. Pain intensity was significantly higher in the hEDS/HSD-group than the other subgroups (JH; +37 mm p = .004, HC; +38 mm, p = .001). The hEDS/HSD-group showed significantly more gait variability (JH; +7.2(2.0)% p = .003, HC; + 7.8(1.4)%, p = <0.001) and lower joint work (JH; −0.07(0.03)J/kg, p = .007, HC; − 0.06(0.03)J/kg, p = .013) than the other subgroups. The JH-group showed significantly increased ankle dorsiflexion during terminal stance (+5.0(1.5)degree, p = .001) compared to hEDS/HSD-group and knee flexion during loading response compared to HC-group (+5.7(1.8) degree, p = .011).SignificanceA distinctive difference in gait pattern between adolescents with non-pathological and pathological joint hypermobility is found in gait variability, rather than in the biomechanical features of gait. This suggests that a specific gait variability metric is more appropriate than biomechanical individual joint patterns for assessing gait in adolescents with hEDS/HSD.
BACKGROUND: Generalized Joint Hypermobility (GJH) has been found to be associated with musculoskeletal complaints and disability. For others GJH is seen as a prerequisite in order to excel in certain sports like dance. However, it remains unclear what the role is of GJH in human performance. Therefore, the purpose of the study was to establish the association between GJH and functional status and to explore the contribution of physical fitness and musculoskeletal complaints to this association.METHODS: A total of 72 female participants (mean age (SD; range): 19.6 (2.2; 17-24)) were recruited among students from the Amsterdam School of Health Professions (ASHP) (n = 36) and the Amsterdam School of Arts (ASA), Academy for dance and theater (n = 36) in Amsterdam, The Netherlands. From each participant the following data was collected: Functional status performance (self-reported Physical activity level) and capacity (walking distance and jumping capacity: side hop (SH) and square hop (SQH)), presence of GJH (Beighton score ≥4), muscle strength, musculoskeletal complaints (pain and fatigue) and demographic characteristics (age and BMI).RESULTS: GJH was negatively associated with all capacity measures of functional status. Subjects with GJH had a reduced walking distance (B(SE):-75.5(10.5), p = <.0001) and jumping capacity (SH: B(SE):-10.10(5.0), p = .048, and SQH: B(SE):-11.2(5.1), p = .024) in comparison to subjects without GJH, when controlling for confounding: age, BMI and musculoskeletal complaints. In participants with GJH, functional status was not associated with performance measures.CONCLUSION: GJH was independently associated with lower walking and jumping capacity, potentially due to the compromised structural integrity of connective tissue. However, pain, fatigue and muscle strength were also important contributors to functional status.
Generalized joint hypermobility (GJH) is highly prevalent among patients diagnosed with chronic pain. When GJH is accompanied by pain in ≥4 joints over a period ≥3 months in the absence of other conditions that cause chronic pain, the hypermobility syndrome (HMS) may be diagnosed. In addition, GJH is also a clinical sign that is frequently present in hereditary diseases of the connective tissue, such as the Marfan syndrome, osteogenesis imperfecta, and the Ehlers-Danlos syndrome. However, within the Ehlers-Danlos spectrum, a similar subcategory of patients having similar clinical features as HMS but lacking a specific genetic profile was identified: Ehlers-Danlos syndrome hypermobility type (EDS-HT). Researchers and clinicians have struggled for decades with the highly diverse clinical presentation within the HMS and EDS-HT phenotypes (Challenge 1) and the lack of understanding of the pathological mechanisms that underlie the development of pain and its persistence (Challenge 2). In addition, within the HMS/EDS-HT phenotype, there is a high prevalence of psychosocial factors, which again presents a difficult issue that needs to be addressed (Challenge 3). Despite recent scientific advances, many obstacles for clinical care and research still remain. To gain further insight into the phenotype of HMS/EDS-HT and its mechanisms, clearer descriptions of these populations should be made available. Future research and clinical care should revise and create consensus on the diagnostic criteria for HMS/EDS-HT (Solution 1), account for clinical heterogeneity by the classification of subtypes within the HMS/EDS-HT spectrum (Solution 2), and create a clinical core set (Solution 3).
