Chronic widespread pain is highly present in patients with the Ehlers-Danlos syndrome hypermobility type (EDS-HT), but up to now, evidence for generalized hyperalgesia is lacking. The aim of this study is to investigate whether pressure pain thresholds (PPTs) at both symptomatic and asymptomatic body areas differ in EDS-HT patients compared to healthy subjects. Twenty-three women with EDS-HT and 23 gender- and age-matched healthy controls participated. All subjects marked on Margolis Pain Diagram where they felt pain lasting longer than 24 h in the past 4 weeks. Then, they completed several questionnaires assessing pain cognitions, fatigue, disability, and general health status, in order to take the possible influence of these factors on PPTs into account. Patients also completed a form concerning the type of pain they experienced. Thereupon, a blinded researcher assessed PPTs at 14 body locations on the trunk and extremities. PPTs were compared for the two complete groups. In addition, PPTs of patients and controls who did not report pain in a respective zone were compared. PPTs of the patients were significantly lower compared to those of the control group, also when pain-free samples per zone were compared. The mean (SD) PPT was 2.9 (1.62) kg/cm(2) in the EDS-HT patients and 5.2 (1.88) kg/cm(2) in the controls (P < 0.001). No confounding factors responsible for the observed differences could be revealed. In half of the patient group, a predominantly neuropathic pain component was likely present. This study provides evidence for the existence of hyperalgesia even in asymptomatic areas (generalized secondary hyperalgesia). The generalized hyperalgesia may represent the involvement of a sensitized central nervous system, which inquires an adapted pain management for this patient group.
DOCUMENT
INTRODUCTION: Lowered pressure pain thresholds have been demonstrated in adults with Ehlers-Danlos Hypermobility type(EDS-HT), however it remains unclear if these findings are also present in children. Therefore, the objectives of the study were to: (1)determine if generalized hyperalgesia is present in Hypermobility syndrome (HMS)/EDS-HT children, (2)explore potential differences in pressure pain thresholds between HMS/EDS-HT children and adults, and (3)determine the discriminative value of generalized hyperalgesia.METHODS: Patients classified in one of three groups: HMS/EDS-HT, hypermobile (Beighton score ≥4/9) and healthy controls. Descriptive data of age, gender, body mass index, Beighton score, skin laxity and medication usage were collected. Generalized hyperalgesia was quantified by the averaged pressure pain thresholds collected from 12 locations. The following confounders were collected: pain locations/intensity, fatigue, psychological distress. Comparisons between HMS/EDS-HT children and normative values, between children and adults with HMS/EDS-HT, corrected confounders, were analysed with MANCOVA. The discriminative value of generalized hyperalgesia employed in order to differentiate between HMS/EDS-HT, hypermobile and controls was quantified with logistic regression.RESULTS: Significantly lower pressure pain thresholds were found in children with HMS/EDS-HT compared to normative values (range: -22.0% to -59.0%, p=<.05). When applying a threshold of 30.8 N/cm(2) for males and 29.0 N/cm(2) for females, the presence of generalized hyperalgesia discriminated between individuals with HMS/EDS-HT, hypermobile and healthy controls (odds ratio=6.0).CONCLUSION: Children and adults with HMS/EDS-HT are characterized by hypermobility, chronic pain, as well as generalized hyperalgesia. The presence of generalized hyperalgesia may indicate involvement of the central nervous system in the development of chronic pain. This article is protected by copyright. All rights reserved.
DOCUMENT
ObjectiveMajor issues in children with Marfan syndrome (MFS) are pain, fatigue and joint hypermobility which might influence functional ability. We recently studied these aspects in children with Ehlers-Danlos syndrome hypermobility type (EDS-HT) and hypermobility spectrum disorders (HSD). What can be learned from this research regarding diagnostics and treatment for children with MFS and children with EDS other than EDS-HT? Methods We followed 101 children and adolescents with EDS and HSD for three years and studied the presence of pain, fatigue and functional ability as well as the presence of multi-systemic complaints (eg. dysautonomia). In 255 adolescents and adults with EDS and HSD, we studied the presence of pain, fatigue and functional ability. In 19 children and their parents with Marfan syndrome, we performed qualitative research regarding pain, fatigue and functional ability.ResultsChildren with a high incidence of multi-systemic complaints, and significant pain and fatigue at baseline were most likely to have a deteriorating trajectory of functional impairment. Besides joint hypermobility and chronic pain, generalized hyperalgesia was also found, possibly indicating central nervous system involvement.We stated that in children and adolescents with EDS-HT and HSD, identifying risk profiles are important to clarify the pathological mechanisms involved, and to develop interdisciplinary treatment strategies. Parents perceive a large impact of MFS on daily life of children, parents and family. Adolescents with MFS perceived problems in keeping up with peers in school, work, sports, leisure and friendships/relationships and perceived to be and feel different from peers due to appearance and disabilities. Conclusion Based on lessons learned in EDS-HT and HSD, we designed a longitudinal follow-up study in 150 children and adolescents with MFS and EDS in the Netherlands and Belgium. Disease characteristics, functional ability, exercise capacity, symptoms of pain and fatigue and psycho-social wellbeing will be studied. We will identify risk profiles for functional decline and develop therapeutic rehabilitation strategies. The first results of qualitative research in MFS adolescents warrants awareness and individual tailored physical, psychosocial, educational and environmental support programs to improve physical functioning and empowerment of teenagers with MFS. Longitudinal studies have been started.
DOCUMENT
