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

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 ¼ 0.67, p ¼ <0.001), 6MWT (r ¼ 0.58, p ¼ <0.001) and HAQ (r ¼ 0.63, p¼ <0.001). Proprioception was associated with 30CRT (r ¼ 0.55, p < 0.001), 6MWT (r ¼ 0.40, p ¼ <0.05) and HAQ (r ¼ 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. ä 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. ARTICLE HISTORY

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

“…The 6MWT distance was likely enhanced by an improvement of the aerobic pathway. This improvement of physical functional capacity can also be explained by the content of the program in which muscle strengthening and proprioception are important elements and have been suggested to be related to activity limitations (Scheper et al, 2017). We found a significant mean improvement for the 6MWT distance higher than the MCID.…”

“…All assessments were conducted on the baseline (t0), at the end of the program (t9), and 6 weeks after the end (t15). These assessments have been already used in hEDS in the literature (e.g., Scheper et al (2017);Celletti et al (2013)) and all the questionnaires have been validated in French.…”

Ehlers–Danlos Syndrome—Hypermobility Type: A Much Neglected Multisystemic Disorder

“…Impaired proprioceptive acuity is thought to influence muscle strength. Therefore, improving muscle strength on the basis of proprioceptive impairment may be more important for reducing activity limitations than just improving muscle strength 80. Chiari 1 malformation type 1 was found in 4.7% of EDS-HT patients19 and may be associated with cranio-cervical instability and/or the tethered cord syndrome.…”

Symptoms in Daily Life and Activity Level of Women with and without Hypermobility

“…Also, some evidence was found that muscle strength and functional performance of the lower extremity is reduced in women with hypermobile Ehlers-Danlos syndrome [15]. The same group found in a previous study that patients with hypermobile Ehlers-Danlos syndrome demonstrated a significant impairment in knee joint reposition sense and a lower level of sports and physical activity than controls [16]. A systematic review and metaanalysis showed that lower limb proprioception is reduced in people with GJH [17].…”

“…The second study was done with adults suffering from Ehlers-Danlos syndrome type III (hypermobility type). In this study the hypermobile patients showed a lower activity level in sports than controls [16]. Finally Schmidt et al [23] investigated how people with JHS make decisions about physical activity.…”