Since the early musings in the mid-1800s of Claude Bernard and his French neurological colleagues on the association of pain with the sympathetic nervous system, complex regional pain syndrome (CRPS) has both fascinated and perplexed practitioners. Some of the clearest and most interesting descriptions of ‘causalgia’ come from the American Civil War by one of Bernard’s students, Silas Weir-Mitchell. The low-velocity, high-mass missiles used in this confrontation (the ‘Minnie ball’) seemed to be particularly effective in inducing neuropathic pain associated with intense autonomic dysregulation. Weir-Mitchell’s depictions are clear and elegant, and as good as any clinical description that can be found in this century. 64 Many great minds have struggled with the pathophysiology of what came to be called ‘reflex sympathetic dystrophy’ in the later part of the 1900s and what has, since the Orlando consensus-based workshop of 1999, come to be called complex regional pain syndrome (CRPS) (Table 1). 37 63 85 From Leriche 46 and his vicious circles we have progressed through Livingston 47 and Sunderland 88 with the turbulence theory, and finally to the solid physiological information generated by the various animal models of causalgia, especially the chronic constriction injury model of Bennett and Xie. 5 Recently, the effort to understand the syndrome has turned towards consensus symposia. The first of these concerned taxonomy, as above. 37 85 A second Dahlem-type conference was conducted in regard to the guidelines for therapy, 84 and recently the International Association for the Study of Pain (IASP) sponsored a symposium in Cardiff, Wales in 2000 to discuss issues of pathophysiology and to amend the diagnostic considerations. 83 The epidemiology of the syndrome is very unclear. Although the syndrome has traditionally been considered rare, its ‘discovery’ by personal injury lawyers in the United States has caused a radical increase in the reporting of the syndrome (at least in the USA). The current diagnostic criteria, as set forth by the Committee of Classification of Chronic Pain of the IASP, have contributed to the liberalization of the diagnosis (Table 1). 63 This effort was extremely important in providing standardized diagnostic criteria, and caused a vast improvement in clinical communication and research homogeneity. It provided the hope that results could be generalized across studies, and in fact widespread use of these standardized criteria has helped all these things considerably. These criteria, while being very sensitive, greatly lack specificity. 92 1 31 The intent of the Orlando conference in 1994 was that these criteria should evolve on the basis of experience and empirical testing, and that they should be subject to systematic validation research over time. 37 62 85 This has been accomplished to some extent, and through a process of internal and external validation the opportunity to improve the specificity of the bedside diagnostic criteria is available. 92 1 31 Although the original IASP criteria required only subjective and potentially only historical signs and symptoms, the suggestions for improving these criteria are that some objectification and observed evidence be included. It is recommended that the diagnostic criteria be modified to include at least one symptom in each of the four diagnostic categories derived by factor analysis: sensory

Complex Regional Pain Syndrome

Objective This is the fourth edition of diagnostic and treatment guidelines for complex regional pain syndrome (CRPS; aka reflex sympathetic dystrophy).

Methods Expert practitioners in each discipline traditionally utilized in the treatment of CRPS systematically reviewed the available and relevant literature; due to the paucity of levels 1 and 2 studies, less rigorous, preliminary research reports were included. The literature review was supplemented with knowledge gained from extensive empirical clinical experience, particularly in areas where high-quality evidence to guide therapy is lacking.

Results The research quality, clinical relevance, and “state of the art” of diagnostic criteria or treatment modalities are discussed, sometimes in considerable detail with an eye to the expert practitioner in each therapeutic area. Levels of evidence are mentioned when available, so that the practitioner can better assess and analyze the modality under discussion, and if desired, to personally consider the citations. Tables provide details on characteristics of studies in different subject domains described in the literature.

Conclusions In the humanitarian spirit of making the most of all current thinking in the area, balanced by a careful case-by-case analysis of the risk/cost vs benefit analysis, the authors offer these “practical” guidelines.

/pdf/complex-regional-pain-syndrome-practical-diagnostic-and-1e38f98gld.pdf

Complex regional pain syndrome: practical diagnostic and treatment guidelines, 4th edition.

Neuropathic pain, caused by various central and peripheral nerve disorders, is especially problematic because of its severity, chronicity and resistance to simple analgesics. The condition affects 2%-3% of the population, is costly to the health care system and is personally devastating to the people who experience it. The diagnosis of neuropathic pain is based primarily on history (e.g., underlying disorder and distinct pain qualities) and the findings on physical examination (e.g., pattern of sensory disturbance); however, several tests may sometimes be helpful. Important pathophysiologic mechanisms include sodium-and calcium-channel upregulation, spinal hyperexcitability, descending facilitation and aberrant sympathetic-somatic nervous system interactions. Treatments are generally palliative and include conservative nonpharmacologic therapies, drugs and more invasive interventions (e.g., spinal cord stimulation). Individualizing treatment requires consideration of the functional impact of the neuropathic pain (e.g., depression, disability) as well as ongoing evaluation, patient education, reassurance and specialty referral. We propose a primary care algorithm for treatments with the most favourable risk-benefit profile, including topical lidocaine, gabapentin, pregabalin, tricyclic antidepressants, mixed serotonin-norepinephrine reuptake inhibitors, tramadol and opioids. The field of neuropathic pain research and treatment is in the early stages of development, with many unmet goals. In coming years, several advances are expected in the basic and clinical sciences of neuropathic pain, which will provide new and improved therapies for patients who continue to experience this disabling condition.

/pdf/neuropathic-pain-a-practical-guide-for-the-clinician-2h41r8sccj.pdf

Neuropathic pain: a practical guide for the clinician

Treatment of complex regional pain syndrome type I (CRPS-I) is subject to discussion. The purpose of this study was to develop multidisciplinary guidelines for treatment of CRPS-I. A multidisciplinary task force graded literature evaluating treatment effects for CRPS-I according to their strength of evidence, published between 1980 to June 2005. Treatment recommendations based on the literature findings were formulated and formally approved by all Dutch professional associations involved in CRPS-I treatment. For pain treatment, the WHO analgesic ladder is advised with the exception of strong opioids. For neuropathic pain, anticonvulsants and tricyclic antidepressants may be considered. For inflammatory symptoms, free-radical scavengers (dimethylsulphoxide or acetylcysteine) are advised. To promote peripheral blood flow, vasodilatory medication may be considered. Percutaneous sympathetic blockades may be used to increase blood flow in case vasodilatory medication has insufficient effect. To decrease functional limitations, standardised physiotherapy and occupational therapy are advised. To prevent the occurrence of CRPS-I after wrist fractures, vitamin C is recommended. Adequate perioperative analgesia, limitation of operating time, limited use of tourniquet, and use of regional anaesthetic techniques are recommended for secondary prevention of CRPS-I. Based on the literature identified and the extent of evidence found for therapeutic interventions for CRPS-I, we conclude that further research is needed into each of the therapeutic modalities discussed in the guidelines.

http://rsds.org/wp-content/uploads/2015/02/Perez_Zollinger_BMCNeurol_2010.pdf

Evidence based guidelines for complex regional pain syndrome type 1

Complex regional pain syndrome is a chronic pain condition characterized by autonomic and inflammatory features. It occurs acutely in about 7% of patients who have limb fractures, limb surgery, or other injuries. Many cases resolve within the first year, with a smaller subset progressing to the chronic form. This transition is often paralleled by a change from “warm complex regional pain syndrome,” with inflammatory characteristics dominant, to “cold complex regional pain syndrome” in which autonomic features dominate. Multiple peripheral and central mechanisms seem to be involved, the relative contributions of which may differ between individuals and over time. Possible contributors include peripheral and central sensitization, autonomic changes and sympatho-afferent coupling, inflammatory and immune alterations, brain changes, and genetic and psychological factors. The syndrome is diagnosed purely on the basis of clinical signs and symptoms. Effective management of the chronic form of the syndrome is often challenging. Few high quality randomized controlled trials are available to support the efficacy of the most commonly used interventions. Reviews of available randomized trials suggest that physical and occupational therapy (including graded motor imagery and mirror therapy), bisphosphonates, calcitonin, subanesthetic intravenous ketamine, free radical scavengers, oral corticosteroids, and spinal cord stimulation may be effective treatments. Multidisciplinary clinical care, which centers around functionally focused therapies is recommended. Other interventions are used to facilitate engagement in functional therapies and to improve quality of life.

https://rsds.org/wp-content/uploads/2014/12/CRPS-bruehl.pdf

Complex Regional Pain Syndrome.

:Objective:To report the initial and long-term outcome after an intensive exercise therapy program for childhood complex regional pain syndrome, type I (CRPS).Design:Prospective follow-up.Setting:A children's hospital.Subjects:We followed 103 children (87 girls; mean age = 13.0 years) with C

Short- and Long-term Outcomes of Children with Complex Regional Pain Syndrome Type I Treated with Exercise Therapy

OBJECTIVE: The presence of pain over an enthesis defines enthesitis and is a major (or sole) clinical feature of childhood spondyloarthropathies. However, the presence or degree of tenderness of the entheses in healthy children is unknown. We studied the prevalence of enthesalgia and pain thresholds over entheses in healthy children and whether these sites are different from those in patients diagnosed with seronegative enthesopathic arthropathy (SEA syndrome). METHODS: We examined 234 schoolchildren aged 8 to 16 years for enthesalgia; those reporting tenderness were compared to randomly selected patients with SEA syndrome previously diagnosed in a tertiary outpatient clinic. RESULTS: Enthesalgia in at least one site was found in 68 children (29%). Schoolchildren had fewer sites than patients (mean 1.2 +/- 2.8 sites vs 8.1 +/- 4.5 sites; p

https://www.jrheum.org/content/jrheum/30/6/1335.full.pdf

Enthesalgia in childhood: site-specific tenderness in healthy subjects and in patients with seronegative enthesopathic arthropathy.

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Short- and Long-term Outcomes of Children with Complex Regional Pain Syndrome Type I Treated with Exercise Therapy

Enthesalgia in childhood: site-specific tenderness in healthy subjects and in patients with seronegative enthesopathic arthropathy.