View clinical trials related to Ehlers-Danlos Syndrome.
Filter by:The management of patients with Ehlers-Danlos syndrome (EDS) is still underdeveloped in healthcare institutions in France. Although multidisciplinary management through exercise rehabilitation has demonstrated its benefits in many chronic pathologies, it has not been evaluated for EDS. As a result, to date there is no evidence of its effectiveness in patients with EDS. The objective of this study is therefore to objectively evaluate the effectiveness of such a treatment on the different dimensions of these patients' health.
Cranio-cervical instability (CCI) has been well identified in diseases regarding connective tissue, such as Ehlers-Danlos Syndrome/Hipermobility Type (EDS-HT). These patients frequently suffer from severe widespread pain with very difficult management and control. Chronic neuroinflamation, opioid-induced hyperalgesia, and central sensitization phenomena may explain this complex painful condition. A retrospective, observational, consecutive case series study is designed to determine if opioid-free anesthetic management shows a reduction in postoperative pain and opioid rescues needs in comparison with opioid-based anesthesia management for patients with EDS-HT undergoing crano-cervical fixation.
In the absence of a medical consensus, the current management of Ehlers-Danlos Syndrome hypermobility type (hEDS) remains very speculative That's why investigators want in this study to demonstrate the beneficial contribution of an innovative therapeutic strategy combining reprogramming and substitution somatosensory. This therapy is based primarily on the somesthetic substitution provided by compression garments(VC). Indeed, the VC port associated with stimulation of the somesthetic system via a specific program of physiotherapy (e.e. centered on body awareness through movement) could potentiate the effectiveness of care, and therefore enhance its long-term beneficial effect. The patient could thus regain his mobility, his functional independence, and thus considerably increase its quality of life. Thereafter, the practice of an adapted physical activity (APA), by the sensory stimulation induced and its playful nature, will allow patients to preserve and sustain the benefits of taking load previously carried out. Validation of this therapeutic approach would offer an effective solution may subsequently be proposed to the entire medical profession as reference support in the treatment of the hEDS. This study plans to include, over a period of two years, 40 patients with hypermobile Ehlers-Danlos syndrome and 40 healthy volunteers controls. Patients will be followed for a period of one year divided into 3 periods of 4 months, between which they will be evaluated in order to quantify the impact of the intervention performed at each stage of the protocol. Each patient will benefit successively: from a classic care, over a period of 2 to 4 months, then will be treated with compressive garments or with low compressive garment during the next 4 months in association with proprioceptive physical therapy. Finally, the 4 last months, patient will participate in an APA with both dance sessions strengthening the body diagram and stretching sessions. The aim of this work is therefore to evaluate the effect of a treatment specifically oriented towards somatosensory remediation.
Studies have shown the existence of a proprioceptive deficit in patients with Ehlers Danlos Syndrome (EDS) (genetic pathology of connective tissue with hypermobility, multifactor joint instability). A study dating from 2010 showed a qualitative improvement in disability when wearing compression garments (CG) in this pathology, particularly in the fields of proprioception and balance. The purpose of this study is to quantify the effect of CG on standing static balance in patients with EDS.
People with POTS, autoimmune autonomic neuropathy (AAN), pure autonomic failure (PAF), SFN and Ehlers Danlos Syndrome (EDS) do not only suffer from orthostatic symptoms such as dizziness, headache, neck pain, blurred vision or (pre-) syncope. They also experience deficits in attention and concentration (more precisely deficits in selective perspective, operating speed, executive functions and memory performance) mainly in upright position. Only few studies concerning cognitive impairment in autonomic neuropathies, their frequency, aetiology and therapy exist. Many patients concerned, especially with POTS, report attention deficits and "brain fog" with problems in their everyday life and work, predominantly in upright posture. Specific symptomatic or medical therapies do not exist. Medical treatment with Modafinil is discussed and part of a current study at Vanderbilt Autonomic Dysfunction Centre (1-5). The investigators want to investigate if problems of concentration, attention and/or cognitive dysfunction exist in people with POTS, AAN, SFN and EDS compared to healthy controls (HC). Thus the investigators use detailed clinical, autonomic and neuropsychological tests in different body positions (lying, sitting and standing) as also acute therapy (leg crossing).
The purpose of this study is to evaluate cognition in patients with postural tachycardia syndrome (POTS) while lying down and standing and to assess the prevalence of hypermobile Ehlers-Danlos Syndrome in POTS.
EDS (Ehlers-Danlos syndromes) form a heterogeneous group of hereditary connective tissue pathologies, which present a characteristic triad: cutaneous hyperelasticity, articular hyperlaxity and connective tissue fragility. The innumerable sprains and dislocations of all the joints require recurrent immobilizations and are responsible for a musculoskeletal handicap, chronic pains and a great fatigability, resounding on the quality of life of the patient. The use of custom-made compression garments seems to provide relief from pain, especially nociceptive pain and in stabilizing joints by proprioceptive effect. The SEDCARE study is an observational monocentric, non-comparative study. 76 patients will be followed in this study for 2 years during which they will wear compression custom-made garments (Cerecare®).The main objective of this study is to demonstrate the effectiveness of pressure garments Cerecare in the EDS, especially in terms of joint pain.
Sudoscan™ (Impeto Medical, Paris France) uses electrochemical skin conductance as a novel noninvasive method to detect sudomotor dysfunction. Several small studies have recently shown that Sudoscan use in the assessment of small fiber polyneuropathy (in diabetes mellitus) can be performed non-invasively, quickly and effectively. The investigators aim to study the use of Sudoscan in rare disease condition associated with small fiber polyneuropathy.
The Ehlers-Danlos Syndrome (SED) comprises a group of clinically and genetically heterogeneous, inherited connective tissue diseases. The hypermobility type is the most frequent. It is characterized by a generalized joint hypermobility and a hyperextension skin. Chronic pain syndrome is often present. Its psychological impact can be significant (anxiety, depression, impact on the quality of life of the patients). The disease also requires changes and a permanent adaptability (coping). It can lead to feelings of isolation and misunderstanding. The French association of the Ehlers-Danlos Syndromes and the rehabilitation center of the 'Croix-Rouge Française des Massues' propose a patient education program for the patients with a hypermobility type SED (the PrEduSED program). This education program is open to patients and their caregivers located in France. The research hypothesis is that the PrEduSED program improves their coping and reduces patient anxiety at 6 months (these are the two clinical criteria on which the PrEduSED could have the most impact, given the results of the first sessions).
Ehlers-Danlos Syndrome (EDS) is a clinically and genetically heterogeneous group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. EDS features such as genetically related cartilage defects, craniofacial abnormalities and increased pharyngeal collapsibility have been proposed to cause obstructive sleep apnoea (OSA). There is evidence from studies based on questionnaires that EDS patients might be more frequently affected by OSA and sleep disturbances than the general population. However, the actual prevalence of OSA in children and adolescents with EDS is unknown. The primary objective of this study is to assess the prevalence of OSA in children and adolescents with EDS (25) compared to a matched control group (25). The secondary objective of this pioneer study is to assess the quality of life in children and adolescents in EDS in comparison to healthy children and adolescents.