Tag Archives: POTS

Autonomic Neuropathy

Autonomic Neuropathy

Autonomic neuropathies are a collection of syndromes and diseases affecting the autonomic neurons, either parasympathetic or sympathetic, or both. Autonomic neuropathies can be hereditary or acquired in nature. Most often, they occur in conjunction with a somatic neuropathy, but they can also occur in isolation.

The autonomic nervous system modulates numerous body functions; therefore, autonomic dysfunction may manifest with numerous clinical phenotypes and various laboratory and neurophysiologic abnormalities. Although a patient may present with symptoms related to a single portion of the autonomic system, the physician must be vigilant for other affected parts of the autonomic system.In some forms, the degree and type of autonomic system involvement varies extensively.

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POTS: Association with EDS and Orthopaedic Considerations.

Postural Orthostatic Tachycardia Syndrome (POTS): Association with Ehlers-Danlos Syndrome and Orthopaedic Considerations [Clin Orthop Relat Res. 2014] – PubMed – NCBI.

BACKGROUND

Postural orthostatic tachycardia syndrome (POTS) is the most common of several types of dysautonomia, characterized by dysfunction of the autonomic nervous system manifesting with symptoms of orthostatic intolerance with or without associated orthostatic hypotension and excessive autonomic excitation.

Given the numerous presenting musculoskeletal symptoms of POTS and its known associations with other clinical entities like Ehlers-Danlos syndrome, POTS constitutes an unusual treatment challenge of which the orthopaedic surgeon and other related healthcare providers should be aware.

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EDS and Postural Tachycardia Syndrome

Ehlers-Danlos Syndrome and Postural Tachycardia Syndrome: A Relationship Study

OBJECTIVE: This study examines a possible relationship between Ehlers-Danlos Syndrome (EDS) and Postural Tachycardia Syndrome (POTS)

BACKGROUND: POTS is a common form of orthostatic intolerance with an unknown incidence across various ages. EDS is a heterogeneous group of inherited abnormalities of connective tissue characterized by skin hyper-extensibility, joint hypermobility, and connective tissue fragility. The mechanistic relationship between EDS and POTS has been suggested without definitive results.

CONCLUSIONS: The prevalence of EDS is significantly higher in patients with EDS than that of the general population and in autonomic patients without POTS. We suspect an additional underlying mechanism of POTS caused by the change in connective tissue from EDS exists

Autonomic symptom burden in EDS and FMS

Autonomic symptom burden in the hypermobility type of Ehlers–Danlos syndrome:  A comparative study with two other EDS types, fibromyalgia, and healthy controls – Seminars in Arthritis and Rheumatism

Aim:  This study provides insight into the profile and importance of autonomic symptoms in the hypermobility type (HT) of Ehlers–Danlos syndrome (EDS). The impact of these symptoms is put into perspective by comparing with fibromyalgia (FM) and two other EDS types.

Results:  The total autonomic symptom burden was higher in EDS-HT (57.9 ± 21.57) than in controls (11.3 ± 19.22), cEDS (32.3 ± 19.47), and vEDS (29.1 ± 19.18), but comparable to FM (53.8 ± 19.85). Especially orthostatic and gastrointestinal complaints were prevalent. The importance of autonomic symptoms in EDS-HT was emphasized by the correlation with lowered QOL (r = −0.402), fatigue (r = 0.304), and pain severity (r = 0.370). Although affective distress and decreased physical activity are often suggested as possible causes for dysautonomia, the ASP did not correlate with the HADS and Baecke score. By contrast, the correlation of the GHQ (r = 0.298) and PDQ (r = 0.413) with the ASP supports the hypothesis that joint hypermobility and neuropathy may play a role in the development of autonomic symptoms.

Conclusion: Autonomic symptoms, especially orthostatic and gastrointestinal complaints, are frequent extraarticular manifestations of EDS-HT and contribute to the disease burden.

Sympathetic nervous system dysfunction in FMS and CFS

Sympathetic nervous system dysfunction in fibromyalgia, chronic fatigue syndrome, irritable bowel syndrome, and interstitial cystitis: a review of case-control studies – PubMed

Fibromyalgia often coexists and overlaps with other syndromes such as chronic fatigue, irritable bowel syndrome, and interstitial cystitis. Chronic stress has been implicated in the pathogenesis of these illnesses. The sympathetic nervous system is a key element of the stress response system. Sympathetic dysfunction has been reported in these syndromes, raising the possibility that such dysautonomia could be their common clustering underlying pathogenesis.

This review demonstrates that sympathetic nervous system predominance is common in fibromyalgia, chronic fatigue syndrome, irritable bowel syndrome, and interstitial cystitis. This concordance raises the possibility that sympathetic dysfunction could be their common underlying pathogenesis that brings on overlapping clinical features. The recognition of sympathetic predominance in these 4 syndromes may have potential clinical implications. It may be worth exploring the use of nonpharmacological measures as well as drug therapies aimed to regain autonomic balance.

Dysautonomia linked to Ehlers-Danlos syndrome

Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers-Danlos syndrome – PubMed – NCBI

OBJECTIVES: Many non-musculoskeletal complaints in EDS-HT may be related to dysautonomia. This study therefore aims to investigate whether dysautonomia is present and to explore the underlying mechanisms.

RESULTS: The EDS-HT group showed autonomic deregulation with increased sympathetic activity at rest and reduced sympathetic reactivity to stimuli.

Orthostatic intolerance was significantly more prevalent in EDS-HT than controls

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Joint hypermobility syndrome – easily missed

Joint hypermobility syndrome | BMJ

Joint hypermobility syndrome (JHS), previously known as benign joint hypermobility syndrome (BJHS), is a heritable disorder of connective tissue that comprises symptomatic hypermobility predisposing to arthralgia, soft tissue injury, and joint instability.1

It is indistinguishable from the hypermobility type of Ehlers-Danlos syndrome.2 Complications may include autonomic dysfunction, proprioceptive impairment, premature osteoarthritis, intestinal dysmotility, and laxity in other tissues causing hernias or uterine or rectal prolapse. Symptoms are often minimal or mild, but 168 out of 700 patients with joint hypermobility syndrome (24%) attending the UCH Hypermobility Clinic already had an established chronic pain syndrome at the time of their first outpatient attendance. These patients were experiencing serious pain, disability, and impairment of the quality of life, some patients becoming chairbound or even bedbound.3

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EDS III: unifying concept for various functional somatic syndromes

Ehlers–Danlos syndrome hypermobility type: a possible unifying concept for various functional somatic syndromes – Springer

This excellent article  explains all the possible syndromes that could be caused by or related to Hypermobility, like Fibromyalgia, POTS, and Chronic Fatigue.

It is only available in PDF format, so you can click the pages below to view them as images and then enlarge them to be able to read them.

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Joint hypermobility syndrome: easily missed?

Joint hypermobility syndrome: easily missed? | BMJ

Joint hypermobility syndrome (JHS), previously known as benign joint hypermobility syndrome (BJHS), is a heritable disorder of connective tissue that comprises symptomatic hypermobility predisposing to arthralgia, soft tissue injury, and joint instability.1 It is indistinguishable from the hypermobility type of Ehlers-Danlos syndrome.2

Complications may include autonomic dysfunction, proprioceptive impairment, premature osteoarthritis, intestinal dysmotility, and laxity in other tissues causing hernias or uterine or rectal prolapse. Symptoms are often minimal or mild, but 168 out of 700 patients with joint hypermobility syndrome (24%) attending the UCH Hypermobility Clinic already had an established chronic pain syndrome at the time of their first outpatient attendance. These patients were experiencing serious pain, disability, and impairment of the quality of life, some patients becoming chairbound or even bedbound.3

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Causes of Amplified Musculoskeletal Pain Syndrome

Causes of Amplified Musculoskeletal Pain Syndrome | The Children’s Hospital of Philadelphia

This is post expands upon a previous post: Have Pain? Are You Crazy?.  Dr. Sherry has decided that CRPS is actually a mental disorder which he calls amplified musculoskeletal pain syndrome (AMPS).  He advocates against treating the pain:

There are three major causes for the abnormal reflex in children with amplified musculoskeletal pain syndrome (AMPS): injury, illness and psychological stress.

Types of injuries that lead to AMPS Injury is the cause of AMPS in approximately 10 to 20 percent of children.

llness is an infrequent cause of amplified musculoskeletal pain syndrome. It is most commonly seen in conjunction with four inflammatory illnesses of the musculoskeletal system: arthritis, tendonitis, myositis or enthesitis. Other illnesses may be the initial cause of pain; most are infections such as mononucleosis, influenza or gastroenteritis. Constipation can also lead to amplified abdominal pain.

In at least 80 percent of children with amplified musculoskeletal pain syndrome, psychological factors seem to play a role

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