Elsevier

Research in Developmental Disabilities

Volume 33, Issue 6, November–December 2012, Pages 1914-1918
Research in Developmental Disabilities

Relationship between fatigue and gait abnormality in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility type

https://doi.org/10.1016/j.ridd.2012.06.018Get rights and content

Abstract

Ehlers-Danlos syndrome (EDS) is a clinically and genetically heterogeneous group of inherited connective tissue disorders characterised by joint hypermobility, skin hyperextensibility and tissue fragility. It has recently been shown that muscle weakness occurs frequently in EDS, and that fatigue is a common and clinically important symptom. The aim of this study was to investigate the relationship between fatigue severity and the gait pattern using 3D Gait Analysis (GA).

Eleven individuals with Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility type (JHS/EDS-HT) were investigated using muscle strength measured with standardised questionnaire measuring fatigue (Fatigue Severity Scale, FSS) and quantitative 3D GA. Our data showed that FSS value well correlated with the peak of vertical component of ground reaction force (r = −0.66, p < 0.05). The negative correlation gives evidence that the higher the fatigue is the more reduced force is during gait. Our results showed that the ground reaction force has been applied as a functional evaluation score for detecting pathology in gait of JHS/EDS-HT participants and the found correlation between vertical force and fatigue demonstrated that muscle fatigue may be associated with a loss of proprioceptive acuity in lower limb muscles.

Highlights

In patients with Ehlers-Danlos syndrome fatigue is an important symptom. ► Fatigue Severity Scale value correlated with peak of vertical component of force. ► The ground reaction force during gait may be applied as a functional evaluation score.

Introduction

Joint Hypermobility Syndrome (JHS) is a probably common and largely unrecognised heritable connective tissue disorder mainly characterised by joint hypermobility (JHM), joint instability and chronic pain (Voermans et al., 2010). Recently, the JHS has been considered the same condition, with extreme clinical variability, with the Ehlers-Danlos Syndrome Hypermobility type (EDS-HT) (Tinkle et al., 2009). Actually the JHS/EDS-HT is recognised as a severely and disabling condition with chronic pain and fatigue as major determinants for such severe deterioration of quality of life (Castori et al., 2010, Voermans et al., 2010).

Fatigue has been described as an associated feature with a positive and direct relationship with muscle weakness; in particular fatigue has a high prevalence among EDS individuals with a most often severely fatigue among the hypermobility type (Castori et al., 2011, Voermans and Knoop, 2011, Voermans et al., 2010, Voermans et al., 2011). Fatigue is considered the reduced capacity to sustain force or power output (physiological) reduced capacity to perform multiple tasks over time (psychological) and simply a subjective experiences of feeling exhausted, tired, weak or having lack of energy (Wessely, 1995). The perception of fatigue is subjective and no exact definition exists because of overlap between the lay notion of tiredness and the clinically relevant symptom of fatigue (Chaudhuri & Behan, 2004). Understanding if the main component in perception of fatigue is central (subjective sense of fatigue) of peripheral (objective reduction in motor power in muscle fatigability) may help physician in the EDS-HT/JHS management.

Muscle fatigue in fact adversely affects movement coordination and reaction times (Lin et al., 2009), which are considered important components of gait control. The literature provides some evidence of fatigue-related changes of the biomechanical characteristics of gait even if previous studies were limited in numbers and used widely different protocols to induce fatigue and dependent variables. Strikingly the most commonly studied variables, gait speed, step or stride length and stride time appeared not affected in a majority of studies (Granacher et al., 2010, Helbostad et al., 2007, Kavanagh et al., 2006, Olson, 2010, Parijat and Lockhart, 2008, Yoshino et al., 2004). In addition, these studies were conducted on healthy subjects, younger and/older but not in pathological individuals. Fatigue has a positive and direct relationship with muscle weakness and from literature we know that ankle muscle weakness has been suggested as a possible cause of reduced ankle power output in late stance in children with Cerebral Palsy (Gage, 2004) and in elderly gait (Kerrigan et al., 1998, Winter et al., 1990).

From these considerations and from the clinical need to better analyse the element in JHS/EDS-HT individuals, the aim of this work was to investigate the possible relation between the intensity of fatigue and main gait pattern assessed with quantitative Gait Analysis (GA) in EDS-HT/JHS individuals. Although in fact, the poor postural control and the abnormal gait pattern of these participants have been demonstrated in literature (Cimolin et al., 2011, Galli et al., 2011a, Galli et al., 2011b, Rigoldi et al., 2012), their relationship with fatigue is still lacking.

Section snippets

Participant selection

We studied 11 participants (10 female, 1 male; mean age ± standard deviation: 43.08 ± 6.78 years), all directly evaluated in a multidisciplinary outpatient clinic dedicated to hypermobility. Subjects were selected on the basis of a clinically confirmed diagnosis of EDS-HT/JHS; diagnosis was established on both the Villefranche and Brighton criteria (Beighton et al., 1998, Grahame et al., 2000) and participants were considered affected if meeting at least one of the two sets of diagnostic criteria.

Results

FSS mean score in our population shows a statistically significant difference if compared to those of normal healthy adults (6.2 ± 0.9 versus 2.3 ± 0.7; p < 0.05).

The research of correlations between the FSS scores and GA measures revealed that no significant correlation were found with Ankle Power parameters (A1, A2 and MPO indices) from a statistical point of view (r = 0.06 in FSS and A1; r = −0.19 in A2 and FFS; r = −0.20 in MPO and FSS; p > 0.05). On the contrary, the maximum of ground reaction force

Discussion

In the present study we investigated the possible correlation between gait pattern and intensity of fatigue in EDS-HT/JHS individuals. Fatigue has been recognised as a serious symptom of many chronical illnesses that can significantly impair a person's functioning and negatively impact their quality of life (Chaudhuri & Behan, 2004). Focusing on EDS-HT/JHS individuals, fatigue is considered one of the major determinants for such severe deterioration of quality of life in this individuals (

Acknowledgments

The authors would like to acknowledge Dr. Nunzio Tenore for his valuable contribution in data collection.

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      Some possible contributors to fatigue-related disability have been investigated in EDS and include sleep disturbances, concentration problems, social functioning, self-efficacy concerning fatigue, and pain severity [45]. A few experimental studies demonstrate that fatigue associates with muscle weakness [49,50], worsens with exercise [51] and affects gait pattern [52]. Recently, dysautonomia has been recognized as one of the most relevant pathogenic factors influencing fatigue onset and evolution.

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