Generalized Hyperalgesia in Children and Adults Diagnosed With Hypermobility Syndrome and Ehlers‐Danlos Syndrome Hypermobility Type: A Discriminative Analysis

Scheper, Mark; Pacey, Verity; Rombaut, Lies; Adams, Roger; Tofts, Louise; Nicholson, Leslie L.; Engelbert, Raoul

doi:10.1002/acr.22998

Cited by 52 publications

(47 citation statements)

References 49 publications

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“…It has recently been postulated that chronic widespread pain in JHS/EDS‐HT is due to the persistent nociceptive input as a result of joint abnormalities that triggers central sensitization in the dorsal horn neurons . Moreover, such central sensitization may also be the cause of generalized hyperalgesia, which has been demonstrated in both children and adults with JHS/EDS‐HT . It should be noted however that other factors than musculoskeletal pain may contribute to chronic pain in patients with JHS/EDS‐HT.…”

Section: Resultsmentioning

confidence: 99%

Gastrointestinal disorders in joint hypermobility syndrome/Ehlers‐Danlos syndrome hypermobility type: A review for the gastroenterologist

Beckers

Keszthelyi

Fikree

et al. 2017

Neurogastroenterology Motil

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The aim of this review was to examine the nature of GI symptoms and their underlying pathophysiology in JHS/EDS-HT. In addition, we consider the clinical implications of the diagnosis and treatment of JHS/EDS-HT for practicing clinicians in gastroenterology. Observations summarized in this review may furthermore represent the first step toward the identification of a new pathophysiological basis for a substantial subgroup of patients with functional GI disorders.

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Section: Resultsmentioning

confidence: 99%

Gastrointestinal disorders in joint hypermobility syndrome/Ehlers‐Danlos syndrome hypermobility type: A review for the gastroenterologist

Beckers

Keszthelyi

Fikree

et al. 2017

Neurogastroenterology Motil

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“…Osteoarthritis, secondary to joint instability, is also a likely factor. Central sensitization, generalized hyperalgesia, chronic regional pain syndrome, and similar systemic or regional pathogenic mechanisms may contribute in later stages [Castori et al, ; Rombaut et al, ; Scheper et al, , ; Di Stefano et al, ]. See also “Pain Management in the Ehlers–Danlos Syndromes” by Chopra et al, this issue.…”

Section: Clinical Descriptionmentioning

confidence: 99%

Hypermobile Ehlers–Danlos syndrome (a.k.a. Ehlers–Danlos syndrome Type III and Ehlers–Danlos syndrome hypermobility type): Clinical description and natural history

Tinkle

Castori²,

Berglund

et al. 2017

American J of Med Genetics Pt C

372

348

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The hypermobile type of Ehlers-Danlos syndrome (hEDS) is likely the most common hereditary disorder of connective tissue. It has been described largely in those with musculoskeletal complaints including joint hypermobility, joint subluxations/dislocations, as well as skin and soft tissue manifestations. Many patients report activity-related pain and some go on to have daily pain. Two undifferentiated syndromes have been used to describe these manifestations-joint hypermobility syndrome and hEDS. Both are clinical diagnoses in the absence of other causation. Current medical literature further complicates differentiation and describes multiple associated symptoms and disorders. The current EDS nosology combines these two entities into the hypermobile type of EDS. Herein, we review and summarize the literature as a better clinical description of this type of connective tissue disorder.

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“…Parent and child self‐reported pain intensity is highly correlated [Pacey et al, ], although parents have been shown to underestimate their child's perception of pain [Kemp et al, ]. Recently it was shown that children and adults diagnosed with JHS/hEDS are not only characterized by GJH, and chronic pain, but also by the presence of generalized hyperalgesia (GHA) [Scheper et al, ]. The presence of GHA may indicate involvement of the central nervous system in the development of chronic pain and may not only provide insights as to the phenotype of GJH related disorders, but also indicates diagnostic qualities that may be useful in clinical practice [Scheper et al, ].…”

Section: Jhs/heds In Childhoodmentioning

confidence: 99%

The evidence‐based rationale for physical therapy treatment of children, adolescents, and adults diagnosed with joint hypermobility syndrome/hypermobile Ehlers Danlos syndrome

Engelbert

Juul-Kristensen²,

Pacey

et al. 2017

American J of Med Genetics Pt C

134

114

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New insights into the phenotype of Joint Hypermobility Syndrome (JHS) and Ehlers‐Danlos Syndrome‐hypermobile type (hEDS) have raised many issues in relation to classification, diagnosis, assessment, and treatment. Within the multidisciplinary team, physical therapy plays a central role in management of individuals with hypermobility related disorders. However, many physical therapists are not familiar with the diagnostic criteria, prevalence, common clinical presentation, and management. This guideline aims to provide practitioners with the state of the art regarding the assessment and management of children, adolescents, and adults with JHS/hEDS. Due to the complexity of the symptoms in the profile of JHS/hEDS, the International Classification of Functioning, Disability and Health (ICF) is adopted as a central framework whereby the umbrella term of disability is used to encompass functions, activities and participation, as well as environmental and personal factors. The current evidence‐based literature regarding the management of JHS/hEDS is limited in size and quality and there is insufficient research exploring the clinical outcomes of a number of interventions. Multicenter randomized controlled trials are warranted to assess the clinical and cost‐effectiveness of interventions for children and adults. Until further multicenter trials are conducted, clinical decision‐making should be based on theoretical and the current limited research evidence. For all individuals diagnosed with JHS/hEDS, international consensus and combined efforts to identify risk profiles would create a better understanding of the pathological mechanisms and the potential for optimizing health care for affected individuals. © 2017 Wiley Periodicals, Inc.

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Generalized Hyperalgesia in Children and Adults Diagnosed With Hypermobility Syndrome and Ehlers‐Danlos Syndrome Hypermobility Type: A Discriminative Analysis

Abstract: Children and adults with HMS/EDS-HT are characterized by hypermobility, chronic pain, and generalized hyperalgesia. The presence of generalized hyperalgesia may indicate involvement of the central nervous system in the development of chronic pain.

Cited by 52 publications

References 49 publications

Gastrointestinal disorders in joint hypermobility syndrome/Ehlers‐Danlos syndrome hypermobility type: A review for the gastroenterologist

Gastrointestinal disorders in joint hypermobility syndrome/Ehlers‐Danlos syndrome hypermobility type: A review for the gastroenterologist

Hypermobile Ehlers–Danlos syndrome (a.k.a. Ehlers–Danlos syndrome Type III and Ehlers–Danlos syndrome hypermobility type): Clinical description and natural history

The evidence‐based rationale for physical therapy treatment of children, adolescents, and adults diagnosed with joint hypermobility syndrome/hypermobile Ehlers Danlos syndrome

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