Abstract

It has been suggested that people with Ehlers–Danlos syndrome (EDS), or other similar connective tissue disorders, may have proprioceptive impairments, the reason for which is still unknown. We recently found that EDS patients were less precise than healthy controls when estimating their felt hand’s position relative to visible peripheral reference locations, and that this deficit was positively correlated with the severity of joint hypermobility. We further explore proprioceptive abilities in EDS by having patients localize their non-dominant left hand at a greater number of workspace locations than in our previous study. Additionally, we explore the relationship between chronic pain and proprioceptive sensitivity. We found that, although patients were just as accurate as controls, they were not as precise. Patients showed twice as much scatter than controls at all locations, but the degree of scatter did not positively correlate with chronic pain scores. This further supports the idea that a proprioceptive impairment pertaining to precision is present in EDS, but may not relate to the magnitude of chronic pain.

Highlights

  • Ehlers–Danlos syndrome (EDS) is a group of genetic connective tissue disorders that can afflict up to 2% of individuals (Castori 2012)

  • Given that EDS patients, who exhibit joint hypermobility, seem to have some proprioceptive impairments that vary across the workspace (Clayton et al 2013), our goal was to further examine proprioceptive sensitivity in EDS across a greater number of workspace locations, and to see how they differ compared to healthy controls

  • Since many EDS patients suffer from chronic pain (Sacheti et al 1997; Voermans et al 2010), and proprioceptive deficits have been observed in other chronic pain populations (Gill and Callaghan 1998; Knox et al 2006; Lewis et al 2010; Tsay et al 2015), we considered the possibility that pain might be influencing our results, as well

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Summary

Introduction

Ehlers–Danlos syndrome (EDS) is a group of genetic connective tissue disorders that can afflict up to 2% of individuals (Castori 2012). Using a threshold-detection paradigm in which participants indicated when they could feel movement in the knee joint and were asked to report the direction of the Clayton et al SpringerPlus (2015) 4:323 movement, the study revealed that hypermobile subjects showed significantly higher threshold detection levels at knee flexion angles of 5° and 30° in comparison to age and gender-matched control subjects. These findings are similar to those reported by Sahin et al (2008) who found that HMS patients had significantly higher absolute angular errors than age and gender-matched control subjects during a knee joint matching task. They found that EDS patients could detect tactile stimuli just as well as controls (using vibratory perception threshold at these same joints), suggesting that cutaneous receptors in the skin may not be contributing to the observed proprioceptive deficit in EDS; it could be that tendon and joint receptors are the most likely contributors to the proprioceptive impairment found in EDS (Hall et al 1995; Sahin et al 2008; Rombaut et al 2010a)

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