Abstract
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 rehabilitationReducing 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.
Highlights
Patients diagnosed with Ehlers–Danlos syndrome, are characterized by an altered structural integrity of connective tissue [1,2] resulting in frailty [3] and multi-systemic manifestations like orthostatic intolerance,[4] hyper-elastic skin,[5] organ dysfunction [6] and joint instability.[7]
Clinical diagnosis of Ehlers–Danlos Syndrome Hypermobility Type (EDS-HT) is based on the Villefranche criteria, a validated set of clinical features, that are specific to EDS-HT, in which the presence of Generalized Joint Hypermobility (GJH), hyper-elastic skin, pain, form the mainstay of diagnostic criteria.[8,9,10]
Muscle strength was found to be associated with activity limitations in EDS-HT patients
Summary
Patients diagnosed with Ehlers–Danlos syndrome, are characterized by an altered structural integrity of connective tissue [1,2] resulting in frailty [3] and multi-systemic manifestations like orthostatic intolerance,[4] hyper-elastic skin,[5] organ dysfunction [6] and joint instability.[7] The phenotype of EDS-HT is heterogeneous, in which the severity of complaints varies from mild to severe.[5,8] Despite of this, a specific clinical pattern is present on which the diagnosis is established.[8] Clinical diagnosis of EDS-HT is based on the Villefranche criteria, a validated set of clinical features, that are specific to EDS-HT, in which the presence of Generalized Joint Hypermobility (GJH), hyper-elastic skin, pain, form the mainstay of diagnostic criteria.[8,9,10]. Pain and fatigue,[10,11] combined with multi-systemic dysfunction, may cause severe limitations in daily activities. The underlying mechanisms of the musculoskeletal complaints and functional decline remain unknown.[15]
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