Interdisciplinary pain rehabilitation for patients with Ehlers-Danlos syndrome and hypermobility spectrum disorders.

OBJECTIVE: Chronic pain is a common manifestation of Ehlers-Danlos syndrome and hypermobility spectrum disorders; thus it is often suggested that patients undergo generic interdisciplinary pain rehabilitation, despite there being little evidence to support this decision. The aim of this study is to examine the effectiveness of standard rehabilitation programmes for chronic pain on patients with Ehlers-Danlos syndrome and hypermobility spectrum disorders, compared with patients with other chronic pain disorders. SUBJECTS: Data, collected between 2008 and 2016, were extracted from a Swedish national registry. The patient data comprised of 406 cases with Ehlers-Danlos syndrome or hypermobility spectrum disorders, 784 cases with a whiplash-related diagnosis, 3713 cases with diagnoses relating to spinal pain, and 2880 cases of fibromyalgia. METHODS: The differences between groups on key outcome measures from pre- to 1-year follow-up after interdisciplinary pain rehabilitation were analysed using linear mixed effects models. Sensitivity analysis in the form of pattern-mixture modelling was conducted to discern the impact of missing data. RESULTS: No significant differences were found in improvements from pre- to 1-year follow-up for patients with Ehlers-Danlos syndrome or hypermobility spectrum disorder compared with other diagnostic groups regarding measures of health-related quality of life, mental health, or fatigue. At follow-up, differences in pain interference (d = -0.34 (95% confidence interval [95% CI] -0.5 to -0.18)), average pain (d = 0.22 (95% CI 0.11-0.62)) and physical functioning (d = 2.19 (95% CI 1.61-2.77)) were detected for the group with spinal-related diagnoses in relation to those with EDS/HSD, largely due to pre-treatment group differences. Sensitivity analysis found little evidence for missing data influencing the results. CONCLUSION: This study suggests that patients with Ehlers-Danlos syndrome/hypermobility spectrum disorders may benefit from inclusion in an interdisciplinary pain rehabilitation programme.

Multiple Sustainable Benefits of a Rehabilitation Program in Therapeutic Management of Hypermobile Ehlers-Danlos Syndrome: A Prospective and Controlled Study at Short- and Medium-Term.

OBJECTIVE: To evaluate the effects of a 9-week rehabilitation program (RP) for patients with hypermobile Ehlers-Danlos syndrome (hEDS) in the short- and medium-term. DESIGN: Nonrandomized controlled trial with 6 months follow-up. SETTING: Outpatient rehabilitation program. PARTICIPANTS: A referred sample of 36 hEDS patients were assessed for eligibility (N=36), 25 were included, 22 completed the RP and 19 completed the follow-up. INTERVENTIONS: A 9 -week control period without intervention followed by a 9-week RP. MAIN OUTCOME MEASURE: Functional exercise capacity was used as a primary outcome measure. Balance, kinesiophobia, fatigue, pain, quality of life, anxiety, depression, and hyperventilation were measured as secondary outcomes. RESULTS: No significant change was observed during the 9-week control period before the RP. There was a significant improvement immediately after the RP for the functional exercise capacity, balance with eyes closed, fatigue, and quality of life (P<.05). Even more improvements were found 6 weeks after the end of the RP, and there was still an improvement after 6 months in functional exercise capacity, kinesiophobia, depression, hyperventilation, and some components of the quality of life. CONCLUSION: This study supports the effectiveness of an RP as a useful management tool for hEDS patients.

Pain Management through Neurocognitive Therapeutic Exercises in Hypermobile Ehlers-Danlos Syndrome Patients with Chronic Low Back Pain.

BACKGROUND: The hypermobile type of Ehlers-Danlos syndrome (hEDS) is likely the most common hereditary disorder of connective tissue mainly characterized by joint hypermobility. Patients with hEDS suffer joint pain, in particular low back pain, commonly resistant to drug therapy. The aim of this research was to evaluate a neurocognitive rehabilitation approach based not only on the motion and function recovery but also on the pain management. METHODS: In this nonrandomized clinical trial, eighteen hEDS patients (4 males and 14 females) with mean age 21 years (range 13-55) were recruited and evaluated before and after three months of rehabilitation treatment. RESULTS: The outcome scores showed significant statistical results after treatment in reducing pain symptoms (numerical rating scale, P = 0.003; McGill (total score), P = 0.03), fatigue (fatigue severity scale, P = 0.03), fear of movement (Tampa scale, P = 0.003), and pain-associated disability (Oswestry disability index, P = 0.03). CONCLUSION: The clinical results observed in our study seem to confirm the role of a specific neurocognitive rehabilitation program in the chronic pain management in the Ehlers-Danlos syndrome; the rehabilitation treatment should be tailored on patient problems and focused not only in the recovery of movement but also on pain perception.

Practical management strategies for benign hypermobility syndromes.

PURPOSE OF REVIEW: Patients with symptomatic hypermobility syndrome such as hypermobile Ehlers-Danlos syndromes (hEDS) and hypermobility spectrum disorders (HSD) commonly present to rheumatologists with joint pain and functional disability. Providers often have difficulty with diagnosis due to a lack of knowledge on the range of associated manifestations and the available therapeutic modalities. This review will discuss recent updates on diagnostic measures and treatment options for rheumatologists to help patients navigate hEDS/HSD. RECENT FINDINGS: This article describes newer diagnostic measures and assessment of hEDS/HSD manifestations. Evidence supporting physical therapy and occupational therapy is provided, as well as recent updates on assistive devices, compressive garments, orthoses, and surgical interventions. Given patient heterogeneity specific guidance about the amount and type of therapies required to produce a beneficial effect is lacking. Treatment should be individualized, and many of the studies focus on regional joint complaints rather than a whole-body approach. SUMMARY: Physical therapy and occupational therapy remain the cornerstone of treatment.

Immediate and 6-week after effects of a rehabilitation program for Ehlers-Danlos syndrome hypermobile type patients: A retrospective study.

Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders with an impaired quality of life in association with fatigue, pain, and kinesiophobia. A retrospective evaluation of the effects of an outpatient rehabilitation program (RP) was performed in Ehlers-Danlos syndrome hypermobile type (hEDS) patients. The 6-minute walk test (6MWT) was used to evaluate functional capacity. Kinesiophobia, fatigue, pain, and quality of life were self-evaluated at the start, at the end, and 6 weeks after the end of the RP. The retrospective analysis of patients' records showed significant improvement for the walked distance during the 6MWT (491.8 ± 72.5 vs. 439.4 ± 100.9 m) maintained at 6-week follow-up (p = .001), significant improvement for kinesiophobia (p = .033) and the impact of fatigue on activity (p = .01), and significant increase for quality of life with in particular improvements of vitality (p = .001). This retrospective study showed encouraging results of a RP for hEDS patients on functional capacity and quality of life, and prospective studies with long-term follow-up are needed to confirm them.

Custom-Made Foot Orthoses Reduce Pain and Fatigue in Patients with Ehlers-Danlos Syndrome. A Pilot Study.

BACKGROUND: Pain and fatigue are major clinical manifestations in patients with Ehlers-Danlos Syndrome (EDS). The aim of this study is to measure change of the effects of custom-made foot orthotics on some manifestations related to EDS, such as foot pain, foot functionality, fatigue, and quality of life. METHODS: Thirty-six patients with EDS wore foot orthoses for three months. Foot pain, foot-related disability, foot functionality, fatigue, and quality of life were measured using the 11-point Numeric Rating Scale, the Manchester Foot Pain and Disability Index, the Foot Function Index, the Fatigue Severity Score, and the 12-Item Short Form Health Survey questionnaires, respectively, at the beginning and after 3 months. RESULTS: Participants demonstrated significantly improved foot pain (p = 0.002), disability related to foot pain (p < 0.001), foot functionality (p = 0.001), fatigue (p < 0.007), and mental health-related quality of life (p = 0.016). The physical health-related quality of life did not show significant changes. CONCLUSIONS: The use of custom-made foot orthoses help in the management of the symptoms by participants. This study could contribute to the foot specialists being considered as an additional member in multidisciplinary teams that are trying to develop an approach for patients with EDS.

The effect of five isometric exercises on glenohumeral translations in healthy subjects and patients with the hypermobility type of the ehlers-danlos syndrome (heds) or hypermobility spectrum disorder (hsd) with multidirectional shoulder instability: an observational study.

OBJECTIVES: To examine whether patients, diagnosed with the hypermobility type of the Ehlers-Danlos syndrome (hEDS) or Hypermobility Spectrum Disorder (HSD), with multidirectional shoulder instability (MDI) have increased humeral head translations compared to healthy controls and to describe the direction of the humeral translations during five exercises. DESIGN: Observational study. SETTING: Ghent University Hospital. PARTICIPANTS: Twenty-seven female patients (aged mean (SD) 35 (13) years) with hEDS/HSD and MDI and 20 female healthy controls (aged 34 (11) years) participated in this study. INTERVENTIONS: The acromiohumeral (AHD) and humeralglenoid distance (HGD) were measured using ultrasound during five isometric exercises: shoulder external rotation, shoulder extension, shoulder flexion, elbow extension and holding a 2kg dumbbell. MAIN OUTCOME MEASURES: Ultrasound measures of the AHD and HGD. RESULTS: During isometric shoulder extension, elbow extension and dumbbell loading, patients had a significantly larger change in AHD compared to controls. In patients, the AHD was significantly smaller during isometric shoulder flexion, extension and elbow extension compared to the AHD measured in rest. By contrast, the AHD was significantly larger during isometric external rotation and dumbbell loading compared to the AHD measured in rest. Regarding the HGD, no significant differences between patients and controls were observed. However, significantly smaller HGD values were found in patients during isometric shoulder flexion compared to the HGD in rest. CONCLUSIONS: Isometric external rotation and holding a 2kg dumbbell caused an inferior translation in patients with hEDS/HSD and MDI, whereas isometric shoulder flexion and shoulder/elbow extension respectively led to an anterior-superior and superior translation.

Postural analysis in a pediatric cohort of patients with Ehlers-Danlos Syndrome: a pilot study.

BACKGROUND: The Ehlers-Danlos Syndrome (EDS) is a rare disorder affecting the connective tissue. EDS patients may suffer of proprioception and balance impairment but all the studies dealing with such symptoms have been addressed to adult subjects. The Study of such impairment in younger patients may lead to a better awareness of own motor abilities and to a focused rehabilitative intervention. Therefore, our work aims to assess the occurrence of these alterations in a pediatric cohort of EDS patients. METHODS: The Research was designed as a cross-sectional study with a matching control group and performed on a pediatric cohort of 12 subjects with Ehlers-Danlos Syndrome (Classic and Hypermobility type) and on 12 healthy controls, during a follow-up visit at the Department of Pediatrics and at the Rehabilitation Unit of the Foundation IRCCS Policlinico San Matteo, in Pavia from April 2015 to October 2015. A square forceplatform was used to obtain the CoP (center of pressure) displacement during quiet standing during an open and a closed eyes trials. The comparisons between EDS and control group were performed using a t-test for independent data. P<0.05 was considered statistically significant. All tests were two-sided. RESULTS: All the postural parameters considered raised at closed eyes, no significant modifications without vision were found only for the standard deviation along the antero-posterior (AP) axis for the two groups. Both at open eyes and at closed eyes, Patients with EDS showed the postural parameters significantly greater than controls (P≤0.05) and this observation was most notably for the Sway. CONCLUSIONS: According to our results, a planned monitoring of age-related changes in postural parameters of patients with EDS could be really interesting to provide a perspective of the development of postural control in these patients. In fact, considering our results, it could be interesting to apply rehabilitative strategies to enhance motor coordination and postural reflexes so improving their postural control as soon as possible. Further studies about the postural control in EDS children and adolescents are required to confirm our results.

Exercise beliefs and behaviours of individuals with Joint Hypermobility syndrome/Ehlers-Danlos syndrome - hypermobility type.

PURPOSE: To explore exercise beliefs and behaviours of individuals with Joint Hypermobility syndrome/Ehlers-Danlos syndrome - hypermobility type and to explore patient experiences of physiotherapy. METHODS: A cross sectional questionnaire survey design was used to collect quantitative and qualitative data from adult members of the Hypermobility Syndromes Association and Ehlers-Danlos Syndrome Support UK. Descriptive and inferential statistics were used to analyse the data. Qualitative data was analysed thematically. RESULTS: 946 questionnaires were returned and analysed. Participants who received exercise advice from a physiotherapist were 1.75 more likely to report high volumes of weekly exercise (odds ratio [OR] = 1.75, 95% confidence interval [CI] = 1.30-2.36, p < 0.001) than those with no advice. Participants who believed that exercise is important for long-term management were 2.76 times more likely to report a high volume of weekly exercise compared to the participants who did not hold this belief (OR = 2.76, 95% CI = 1.38-5.50, p = 0.004). Three themes emerged regarding experience of physiotherapy; physiotherapist as a partner, communication - knowledge, experience and safety. CONCLUSION: Pain, fatigue and fear are common barriers to exercise. Advice from a physiotherapist and beliefs about the benefits of exercise influenced the reported exercise behaviours of individuals with Ehlers-Danlos syndrome - hypermobility type in this survey. Implications for rehabilitation Exercise is a cornerstone of treatment for Ehlers-Danlos syndrome/Ehlers-Danlos syndrome - hypermobility type. Pain, fatigue and fear of injury are frequently reported barriers to exercise. Advice from physiotherapists may significantly influence exercise behaviour. Physiotherapists with condition specific knowledge and good verbal and non-verbal communication facilitate a positive therapeutic experience.

Physical and mechanical therapies for lower limb symptoms in children with Hypermobility Spectrum Disorder and Hypermobile Ehlers-Danlos Syndrome: a systematic review.

BACKGROUND: Hypermobility Spectrum Disorder and Hypermobile Ehlers Danlos Syndrome are two common heritable genetic disorders of connective tissue. Both conditions are characterised by excessive joint range of motion and the presence of musculoskeletal symptoms, and are associated with joint instability, motion incoordination, decreased joint position sense, and musculoskeletal pain. Hypermobility Spectrum Disorder is the new classification for what was previously known as Joint Hypermobility Syndrome. This systematic review evaluates the evidence for physical and mechanical treatments for lower limb problems in children with Hypermobility Spectrum Disorder and Hypermobile Ehlers Danlos Syndrome. METHODS: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, PUBMED and CINAHL were searched to October 2017 for randomised controlled trials (RCT) and quasi-RCTs evaluating physical and mechanical interventions for lower limb problems in children with hypermobility. Two authors independently screened studies for eligibility for inclusion and three review authors independently assessed risk of bias of included studies. One author extracted and analysed statistical data, which were checked by a second author. RESULTS: Two RCTs including a total of 86 participants were eligible for inclusion. Trials evaluated differences between generalised versus targeted physiotherapy programs and between performing knee extension exercises to the neutral versus hypermobile range. There was no clear benefit of any of the physical therapies evaluated. CONCLUSION: There is very limited evidence to guide the use of physical and mechanical therapies for lower limb problems in children with Hypermobility Spectrum Disorder and Hypermobile Ehlers Danlos Syndrome. Mechanical therapies have not been evaluated in RCTs and results of the two RCTs of physical therapies do not definitively guide physical therapy prescriptions. Current studies are limited by small sample sizes and high attrition rates. No physical therapy has been compared to a sham intervention no intervention or no intervention, so overall effectiveness is unknown.

A multimodal physical therapy approach utilizing the Maitland concept in the management of a patient with cervical and lumbar radiculitis and Ehlers-Danlos syndrome-hypermobility type: A case report.

The purpose of this case report is to present a multimodal approach for patient management using the Maitland concept framework for cervical and lumbar radiculitis with an underlying diagnosis of Ehlers-Danlos Syndrome-Hypermobility Type (EDS-HT). This case presents care guided by evidence, patient values, and rationale for the selected course of physical therapy treatment provided by therapist experience. A 35-year-old female with a 2-year history of worsening lumbar and cervical pain was referred to physical therapy to address these musculoskeletal issues concurrent with diagnostic testing for EDS. A multimodal approach including manual therapy, therapeutic exercise, postural and body mechanics education, and a home exercise program was used. The patient specific functional scale (PSFS) was used to gauge patient's perceived improvements which were demonstrated by increased scores at reevaluation and at discharge. Following the Maitland concept framework, the physical therapist was able to make sound clinical decisions by tracking the logical flow of constant patient assessment. A 10-month course of treatment designed to maximize recovery of function was successful with a chronic history of pain and the EDS-HT diagnosis. The role of education and empowering the patient is shown to be of utmost importance. Optimizing therapeutic outcomes long-term for this patient population requires maintaining a home exercise program, adaptation and modifications of work and lifestyle activities.

Features that exacerbate fatigue severity in joint hypermobility syndrome/Ehlers-Danlos syndrome - hypermobility type.

AIM: To assess the prevalence, severity and impact of fatigue on individuals with joint hypermobility syndrome (JHS)/Ehlers-Danlos syndrome - hypermobility type (EDS-HT) and establish potential determinants of fatigue severity in this population. METHODS: Questionnaires on symptoms and signs related to fatigue, quality of life, mental health, physical activity participation and sleep quality were completed by people with JHS/EDS-HT recruited through two social media sites. Multiple regression analysis was performed to identify predictors of fatigue in this population. RESULTS: Significant fatigue was reported by 79.5% of the 117 participants. Multiple regression analysis identified five predictors of fatigue severity, four being potentially modifiable, accounting for 52.3% of the variance in reported fatigue scores. Predictors of fatigue severity were: the self-perceived extent of joint hypermobility, orthostatic dizziness related to heat and exercise, levels of participation in personal relationships and community, current levels of physical activity and dissatisfaction with the diagnostic process and management options provided for their condition. CONCLUSION: Fatigue is a significant symptom associated with JHS/EDS-HT. Assessment of individuals with this condition should include measures of fatigue severity to enable targeted management of potentially modifiable factors associated with fatigue severity. Implications for rehabilitation Fatigue is a significant symptom reported by individuals affected by joint hypermobility syndrome/Ehlers-Danlos syndrome - hypermobility type. Potentially modifiable features that contribute to fatigue severity in this population have been identified. Targeted management of these features may decrease the severity and impact of fatigue in joint hypermobility syndrome/Ehlers-Danlos syndrome - hypermobility type.

The evidence-based rationale for physical therapy treatment of children, adolescents, and adults diagnosed with joint hypermobility syndrome/hypermobile Ehlers Danlos syndrome.

New insights into the phenotype of Joint Hypermobility Syndrome (JHS) and Ehlers-Danlos Syndrome-hypermobile type (hEDS) have raised many issues in relation to classification, diagnosis, assessment, and treatment. Within the multidisciplinary team, physical therapy plays a central role in management of individuals with hypermobility related disorders. However, many physical therapists are not familiar with the diagnostic criteria, prevalence, common clinical presentation, and management. This guideline aims to provide practitioners with the state of the art regarding the assessment and management of children, adolescents, and adults with JHS/hEDS. Due to the complexity of the symptoms in the profile of JHS/hEDS, the International Classification of Functioning, Disability and Health (ICF) is adopted as a central framework whereby the umbrella term of disability is used to encompass functions, activities and participation, as well as environmental and personal factors. The current evidence-based literature regarding the management of JHS/hEDS is limited in size and quality and there is insufficient research exploring the clinical outcomes of a number of interventions. Multicenter randomized controlled trials are warranted to assess the clinical and cost-effectiveness of interventions for children and adults. Until further multicenter trials are conducted, clinical decision-making should be based on theoretical and the current limited research evidence. For all individuals diagnosed with JHS/hEDS, international consensus and combined efforts to identify risk profiles would create a better understanding of the pathological mechanisms and the potential for optimizing health care for affected individuals. © 2017 Wiley Periodicals, Inc.

The association between muscle strength and activity limitations in patients with the hypermobility type of Ehlers-Danlos syndrome: the impact of proprioception.

PURPOSE: The patients diagnosed with Ehlers-Danlos Syndrome Hypermobility Type (EDS-HT) are characterized by pain, proprioceptive inacuity, muscle weakness, potentially leading to activity limitations. In EDS-HT, a direct relationship between muscle strength, proprioception and activity limitations has never been studied. The objective of the study was to establish the association between muscle strength and activity limitations and the impact of proprioception on this association in EDS-HT patients. METHODS: Twenty-four EDS-HT patients were compared with 24 controls. Activity limitations were quantified by Health Assessment Questionnaire (HAQ), Six-Minute Walk test (6MWT) and 30-s chair-rise test (30CRT). Muscle strength was quantified by handheld dynamometry. Proprioception was quantified by movement detection paradigm. In analyses, the association between muscle strength and activity limitations was controlled for proprioception and confounders. RESULTS: Muscle strength was associated with 30CRT (r = 0.67, p = <0.001), 6MWT (r = 0.58, p = <0.001) and HAQ (r = 0.63, p= <0.001). Proprioception was associated with 30CRT (r = 0.55, p < 0.001), 6MWT (r = 0.40, p = <0.05) and HAQ (r = 0.46, p < 0.05). Muscle strength was found to be associated with activity limitations, however, proprioceptive inacuity confounded this association. CONCLUSIONS: Muscle strength is associated with activity limitations in EDS-HT patients. Joint proprioception is of influence on this association and should be considered in the development of new treatment strategies for patients with EDS-HT. Implications for rehabilitation Reducing activity limitations by enhancing muscle strength is frequently applied in the treatment of EDS-HT patients. Although evidence regarding treatment efficacy is scarce, the current paper confirms the rationality that muscle strength is an important factor in the occurrence of activity limitations in EDS-HT patients. Although muscle strength is the most dominant factor that is associated with activity limitations, this association is confounded by proprioception. In contrast to common belief proprioception was not directly associated with activity limitations but confounded this association. Controlling muscle strength on the bases of proprioceptive input may be more important for reducing activity limitations than just enhancing sheer muscle strength.

Quality of life, unmet needs, and iatrogenic injuries in rehabilitation of patients with Ehlers-Danlos Syndrome hypermobility type/Joint Hypermobility Syndrome.

Ehlers-Danlos Syndrome, hypermobility type (EDS-HT) and the joint hypermobility syndrome (JHS) are connective tissue disorders that form an overlapping clinical syndrome and are associated with frequent medical visits and substantial morbidity. EDS-HT/JHS-associated pain correlates with poor quality of life. While physical therapy is the recommended treatment for EDS-HT/JHS, little is known about therapy-related patient experiences and iatrogenic injuries. We studied 38 adult EDS-HT/JHS patients, eliciting health-related quality of life (HRQoL) from 28 patients through the RAND SF-36 questionnaire. We also explored physical therapy experiences through focus groups with 13 patients. Our patients displayed poor HRQoL, with 71% reporting worse health over the past year. SF-36 scores were significantly lower than the scores of the average American population (P < 0.001 for 8 of 10 categories assessed), but were comparable to EDS-HT/JHS populations in Belgium, the Netherlands, Sweden, and Italy. Focus groups identified factors associated with: negative past physical therapy experiences, iatrogenic joint injuries, positive treatment experiences, and unmet rehabilitation needs. This group of EDS-HT/JHS patients has significant decrements in HRQoL and many unmet treatment needs, as well as a risk for iatrogenic injuries. We identify several approaches to help meet patients' needs and improve joint rehabilitation in patients with EDS-HT/JHS. © 2016 Wiley Periodicals, Inc.

Disability in Adolescents and Adults Diagnosed With Hypermobility-Related Disorders: A Meta-Analysis.

OBJECTIVE: To (1) establish the association of the most common reported symptoms on disability; and (2) study the effectiveness of treatment on disability in patients with Ehlers-Danlos syndrome-hypermobility type (EDS-HT)/hypermobility syndrome (HMS). DATA SOURCES: An electronic search (Medical Subject Headings and free-text terms) was conducted in bibliographic databases CENTRAL/MEDLINE. STUDY SELECTION: Comparative, cross-sectional, longitudinal cohort studies and (randomized) controlled trials including patients with HMS/EDS-HT aged ≥17 years were considered for inclusion. A class of symptoms was included when 5 publications were available. In regards to treatment (physical, cognitive interventions), only (randomized) controlled trials were considered. Surgical and medicinal interventions were excluded. DATA EXTRACTION: Bias was assessed according to the methodological scoring tools of the Cochrane collaboration. Z-score transformations were applied to classify the extent of disability in comparison with healthy controls and to ensure comparability between studies. DATA SYNTHESIS: Initially, the electronic search yielded 714 publications, and 21 articles remained for analysis after selection. The following symptoms were included for meta-analysis: pain (n=12), fatigue (n=6), and psychological distress (n=7). Pain (r=.64, P=.021), fatigue (r=.91, P=.011), and psychological distress (r=.86, P=.018) had a significant impact on disability. Regarding treatment, a significant pain reduction was achieved by a variety of physical and cognitive approaches. Treatment effectiveness on disability was not established. CONCLUSIONS: Disability can affect patients with HMS/EDS-HT significantly and is highly correlated with both physical and psychological factors. Although evidence is available that physical and psychological treatment modalities can induce significant pain reduction, the evidence regarding disability reduction is lacking.

The effectiveness of therapeutic exercise for joint hypermobility syndrome: a systematic review.

BACKGROUND: Joint hypermobility syndrome (JHS) is a heritable connective tissue disorder characterised by excessive range of movement at multiple joints accompanied by pain. Exercise is the mainstay of management yet its effectiveness is unclear. OBJECTIVES: To establish the effectiveness of therapeutic exercise for JHS. DESIGN: Systematic literature review. DATA SOURCES: A search of nine online databases, supplemented by a hand search and snowballing. STUDY ELIGIBILITY CRITERIA (PARTICIPANTS AND INTERVENTIONS): People diagnosed with JHS (rather than asymptomatic generalised joint laxity); therapeutic exercise (of any type) used as an intervention; primary data reported; English language; published research. STUDY APPRAISAL AND SYNTHESIS METHODS: Methodological quality was appraised by each reviewer using Critical Appraisal Skills Programme checklists. Articles were then discussed collectively and disagreements resolved through debate. RESULTS: 2001 titles were identified. Four articles met the inclusion criteria, comprising one controlled trial, one comparative trial and two cohort studies. All studies found clinical improvements over time. However there was no convincing evidence that exercise was better than control or that joint-specific and generalised exercise differed in effectiveness. LIMITATIONS: The studies used heterogeneous outcome measures, preventing pooling of results. Only one study was a true controlled trial which failed to report between-group statistical analyses post-treatment. CONCLUSIONS AND IMPLICATIONS OF KEY FINDINGS: There is some evidence that people with JHS improve with exercise but there is no convincing evidence for specific types of exercise or that exercise is better than control. Further high quality research is required to establish the effectiveness of exercise for JHS.

The multifaceted and complex hypermobility syndrome (a.k.a. Ehlers-Danlos Syndrome Hypermobility Type): evaluation and management through a rehabilitative approach.

Joint hypermobility syndrome (JHS) is a hereditary disorder of connective tissue recently considered the one and the same as the Ehlers-Danlos Syndrome Hypermobility Type (EDS-HT). The JHS/EDS-HT is mainly characterized by joint hypermobility, chronic pain and a variable skin involvement. Clinical manifestations expressed by patients are multiple and varied. The rehabilitative approach may play a fundamental role in the understanding and management of symptoms and clinical manifestation. Aim of this study is to make a literature revision of all the aspects of this not so rare disease.

Measuring regularity of human postural sway using approximate entropy and sample entropy in patients with Ehlers-Danlos syndrome hypermobility type.

Ligament laxity in Ehlers-Danlos syndrome hypermobility type (EDS-HT) patients can influence the intrinsic information about posture and movement and can have a negative effect on the appropriateness of postural reactions. Several measures have been proposed in literature to describe the planar migration of CoP over the base of support, and the most used in clinical field are the CoP excursions in antero-posterior and medio-lateral direction. In recent years a growing number of studies have been designed to explore the complexity of the COP trajectories during quiet standing. We assessed 13 adults with EDS-HT (EDSG) and 20 healthy adults (CG) during static posture, evaluating the CoP using time and frequency domain analysis and entropy analysis (SampEn and ApEn parameters). Higher values of CoP displacements in medio-lateral and anterior-posterior directions for EDSG than CG were found; no differences were observed in CoP frequency. The entropy analysis showed lower value for EDSG than CG, pointing out the needing of EDSG to concentrate more attention on postural control, loosing complexity and reflecting a less automatized postural control.