Complex regional pain syndromes—reflex sympathetic dystrophy and causalgia

Reflex sympathetic dystrophy (RSD) was the term applied to a variety of unrelated disorders having strikingly similar clinical features.The problem with the term RSD is that not all cases meet the classical case scenario.The umbrella term Complex Regional Pain Syndromes (CRPS) now includes causalgia and RSD and excludes sympathetically mediated pain, neuropathic pain, inflammatory pain, and phantom pain. Complex Regional Pain Syndromes includes the features of inflammation, autonomic, cutaneous, motor and dystrophic changes which distinguish this from other forms of neuropathic pain. Because the pathophysiology of CRPS is predominantly a hyperactivity of the regional sympathetic nervous system, pain management in such patients should focus on interrupting the activity of the sympathetic nervous system.The interruption can be produced by different modalities classified as pharmacologic, nerve blocks, sympathectomy, physical therapy and psychological therapeutic measures. Physical therapy to regain function is an important endeavor for CRPS patients. In spite of acute and vigorous therapeutic modalities practiced on these patients, early and multidisciplinary treatment holds the best promise.     

Complex regional pain syndromes

Complex regional pain syndromes (CRPS) (formerly reflex sympathetic dystrophy and causalgia) are neuropathic pain conditions that are initiated by an extremity trauma or peripheral nerve lesion. Clinical definition and scientific understanding of CRPS are still evolving; however, both the clinical picture and therapeutic options are significantly influenced by a dysfunction of the sympathetic nervous system. Recent investigations suggest functional central abnormalities and a peripheral inflammatory component in the pathophysiology of CRPS. Interdisciplinary treatment includes physical, pharmacologic, and invasive interventional therapy, as well as stimulation techniques.     

Pathologic alterations of cutaneous innervation and vasculature in affected limbs from patients with complex regional pain syndrome

Complex regional pain syndromes (CRPS, type I and type II) are devastating conditions that can occur following soft tissue (CRPS type I) or nerve (CRPS type II) injury. CRPS type I, also known as reflex sympathetic dystrophy, presents in patients lacking a well-defined nerve lesion, and has been questioned as to whether or not it is a true neuropathic condition with an organic basis. As described here, glabrous and hairy skin samples from the amputated upper and lower extremity from two CRPS type I diagnosed patients were processed for double-label immunofluorescence using a battery of antibodies directed against neural-related proteins and mediators of nociceptive sensory function. In CRPS affected skin, several neuropathologic alterations were detected, including: (1) the presence of numerous abnormal thin caliber NF-positive/MBP-negative axons innervating hair follicles; (2) a decrease in epidermal, sweat gland, and vascular innervation; (3) a loss of CGRP expression on remaining innervation to vasculature and sweat glands; (4) an inappropriate expression of NPY on innervation to superficial arterioles and sweat glands; and (5) a loss of vascular endothelial integrity and extraordinary vascular hypertrophy. The results are evidence of widespread cutaneous neuropathologic changes. Importantly, in these CRPS type I patients, the myriad of clinical symptoms observed had detectable neuropathologic correlates.     

Complex regional pain syndromes in children and adolescents: regional and systemic signs and symptoms and hemodynamic response to tilt table testing

OBJECTIVE: Complex regional pain syndromes (CRPS) involve neuropathic limb pain and localized circulatory abnormalities. The authors hypothesized that (1) pediatric CRPS patients exhibit systemic autonomic symptoms and orthostatic and/or cardiac sympatho-vagal dysregulation and (2) their orthostatic regulation differs from healthy controls and pediatric patients with postural orthostatic tachycardia syndrome (POTS). METHODS: CRPS children and adolescents (n=20) underwent a 6-week trial of physical therapy and cognitive-behavioral treatment. Measures included pain and function scores, regional and systemic autonomic symptom profiles, heart rate and blood pressure with tilt, heart rate variability indices, and baroreflex gain. Systemic autonomic symptoms were recorded in 55 healthy pediatric controls. Tilt responses in CRPS patients were compared with those of 21 POTS patients and 39 healthy controls. RESULTS: CRPS patients' regional autonomic symptoms, pain, and limb function improved over 6 weeks (P<0.01). At baseline CRPS patients reported more systemic autonomic symptoms than controls (P<0.05). Tilt table test showed orthostatic stability, but the mean heart rate increase with tilt was greater in CRPS patients than controls (P<0.001). POTS patients showed significant increases with tilt in mean heart rate and diastolic and systolic blood pressures compared with controls (P<0.001). There were significant increases in the mean systolic and diastolic blood pressures in POTS compared with CRPS patients but no difference in the mean heart rate between groups. DISCUSSION: CRPS patients reported multiple regional and systemic autonomic symptoms that improved during the study course, and they experienced minimal and transient tilt table-induced hemodynamic changes compared with POTS patients but relatively similar to controls.     

Complex regional pain syndrome and chiropractic

OBJECTIVE: Complex regional pain syndromes (CRPS) represent curious and difficult syndromes for both patient and clinician. CRPS presents as a triad of signs and symptoms, usually after a seemingly trivial injury to a peripheral joint or appendage. The clinical triad includes severe pain, vasomotor changes in and around the affected area, and trophic changes in the affected limb. Many of the acute symptoms are similar to those seen after many acute injuries, which makes an early diagnosis often times difficult. Current treatment protocols revolve around aggressive physical therapy plus pharmacologic interventions aimed at limiting sympathetic nervous system activity. OBJECTIVE: To review the literature on CRPS regarding symptoms, diagnosis, treatment, and causal mechanisms and to discuss alternative treatment approaches and the possible role of chiropractic care in patient rehabilitation. Data Sources: Texts, review articles, and randomized clinical trials investigating treatments, causes, and epidemiology. CONCLUSIONS: Recent research calls into question the predominant theories that view excessive sympathetic nervous system activity as the cause of CRPS. No evidence of an increase in sympathetic nervous system activity has been found, and new theories suggest that an increase in the sensitivity of neurotransmitter receptors may be the cause of CRPS. Alternatively, other research has suggested that a local inflammatory process may in fact cause CRPS. Although no research has been completed examining the role of chiropractic care in the treatment of CRPS, there is reason to believe that spinal manipulation may be beneficial to patients with CRPS.     

Complex regional pain syndrome in adults: concise guidance

Complex regional pain syndrome (CRPS) is a debilitating, painful condition in a limb associated with sensory, motor, autonomic, skin and bone abnormalities. Pain is typically the leading symptom, but is often associated with limb dysfunction and psychological distress. Prompt diagnosis and early treatment is required to avoid secondary physical problems related to disuse of the affected limb and the psychological consequences of living with undiagnosed chronic pain. UK guidelines have recently been developed for diagnosis and management in the context of primary and secondary care. The purpose of this concise guideline is to draw attention to these guidelines. Information in this article has been extracted from the main document and adapted to inform the management of CRPS as it presents to physicians in the course of their daily practice.     

Complex regional pain syndrome of the knee: early and beneficial action of diphosphonates on pain and function]

We report the case of a 24-years-old man with parcellar Complex Regional Pain Syndrome I (CRPS I) of the patella, responsible for major functional limitation. The diagnosis was based on physical exam and X-ray, in the absence of other articular or peri-articular diseases. The patient received two pamidronate perfusions over a week, with a spectacular decrease of pain, which allowed him to follow the rehabilitation program in good conditions. He was therefore able to go back to work. The place of this treatment in CRPS I is discussed.     

Complex regional pain syndrome in children and adolescents

Complex regional pain syndrome (CRPS) is a disorder characterised by pain, sensory and motor disturbances and represents a significant medical entity. This report discusses two cases of CRPS in children and adolescents, highlighting several critical issues for clinicians in the diagnosis and management of CRPS in these populations. Early diagnosis, referral and appropriate intervention are essential in decreasing pain, suffering and resorting function for children and adolescents with CRPS.     

Complex regional pain syndrome: A review of evidence-supported treatment options

Complex regional pain syndrome consists of pain and other symptoms that are unexpectedly severe or protracted after an injury. In type II complex regional pain syndrome, major nerve injury, often with motor involvement, is the cause; in complex regional pain syndrome I, the culprit is a more occult lesion, often a lesser injury that predominantly affects unmyelinated axons. In florid form, disturbances of vasoregulation (eg, edema) and abnormalities of other innervated tissues (skin, muscle, bone) can appear. Because of these various symptoms and the difficulty in identifying causative lesions, complex regional pain syndrome is difficult to treat or cure. Complex regional pain syndrome has not been systematically investigated; there are few controlled treatment trials for established complex regional pain syndrome. This article reviews the existing studies (even if preliminary) to direct clinicians toward the best options. Treatments for other neuropathic pain syndromes that may be efficacious for complex regional pain syndrome also are discussed. Some common treatments (eg, local anesthetic blockade of sympathetic ganglia) are not supported by the aggregate of published studies and should be used less frequently. Other treatments with encouraging published results (eg, neural stimulators) are not used often enough. We hope to encourage clinicians to rely more on evidence-supported treatments for complex regional pain syndrome.     

Complex regional pain syndrome—2: physical therapy management

Part I of this article reviewed the history, etiology and underlying mechanisms of CRPS I and II. The current article reviews the available research of physical therapy treatment interventions for patients with CRPS. As outlined in Part 1 of this article, there continues to be much uncertainty about the underlying mechanisms of CRPS. It remains challenging to develop evidence-based guidelines for physical therapy or for any other discipline. There is a paucity of prospective randomized clinical trials. The majority of published reports are case reports or consensus-based. Although the article is written primarily from a physical therapy perspective, the clinical guidelines are also of interest to other health care providers. Given the complexity and scope of CRPS, an interdisciplinary management approach is recommended.     

Spatial and temporal summation of sensory and affective dimensions of deep somatic pain

There is considerable evidence in support of differential information processing of the sensory-discriminative and motivational-affective meanings of pain. The purpose of this work was to examine whether temporal (acute, tonic, persistent) and spatial (local, regional, widespread) aspects of deep somatic pain influence the sensory and affective dimensions of pain. Acute pain consisted of a short bout of pain, lasting about 100 s. Tonic pain was the experience of experimentally maintained pain for 18 min. Both acute and tonic pain were induced by infusion of an algesic or control substance into muscle with the subject blinded with respect to the type of infusion and randomization of the application sequence. Comparing the response of experimental subjects to a group of matched cases with persistent masticatory myalgia alone or in combination with widespread musculoskeletal pain, we examine whether the experimental state is different from the matched clinical condition, and whether there is a difference between the condition being restricted to the face or not. The McGill pain questionnaire was used to assess the sensory and affective correlates of pain. The normalized sensory score for acute/unilateral face pain was different from that established for tonic/unilateral face pain (P = 0.055, borderline s.), and so was the normalized affective score (P = 0.009, s.). When comparing tonic/unilateral versus tonic/bilateral face pain, the affective scores increased with increased pain involvement (P = 0.009, s.) while the sensory sores were unaffected by the additional pain induced in the contralateral masseter muscle (P = 0.357, n.s). Notably, sensory and affective scores for tonic/bilateral and persistent/bilateral face pain were not statistically different (sensory: P = 0.169, n.s.; affective: P = 0.643, n.s). On the other hand, when contrasting persistent/bilateral face pain with persistent/ widespread musculoskeletal pain, both scores were significantly different (sensory: P < 0.001, s.; affective: P = 0.041, s.). Time in and spread of pain influenced the perceptual correlates of pain to a significant degree. The major increase in the sensory dimension occurred from 'no pain' to 'acute pain'. Affective scores showed the most significant increases from acute to tonic pain, particularly with greater spatial involvement. The significant increases in sensory scores observed when contrasting persistent facial pain alone and in combination with widespread musculoskeletal pain was attributed to the broader body experience. Because the perceptual correlates of tonic and matched persistent (chronic) pain states were similar, we concluded that it does not require months for the development of the sensory and affective meaning of persistent pain as assumed.     

Complex regional pain syndrome. Sympathetic reflex dystrophy and causalgia

Complex regional pain syndromes (CRPS) occur as the inadequate response to painful trauma in a distal extremity. With CRPS I (sympathetic reflex dystrophy), no lesion of the nerve is present. Aside from sensory disturbances, burning deep spontaneous pain and mechanical allodynia are characteristic. Disturbances in the skin blood circulation,sweating,edema,and trophic disturbances of the skin, joints, and bones are typical. Reduction in muscle strength, tremor, and late dystonic changes comprise the motor disturbances. All symptoms are distributed in the distal extremity and not limited to the region of the peripheral nerves. Complex regional pain syndrome II (causalgia),develops following a partial peripheral nerve lesion. The distally generalized symptoms are identical. Successful therapy depends on an early start of interdisciplinary treatment. In addition to the pain therapy,physiotherapy plays a decisive role in rehabilitation. During the acute phase, freedom from pain at rest and retrogression of the edema must be achieved. With slight spontaneous pain, a conservative therapeutic method may be applied (analgesics, rest, raised position). In case of insufficient improvement and in difficult cases, the effect of intervention (sympathetic blockade) should be tested and possibly a blockade series performed. After reduced spontaneous pain,physiotherapy should be increased stepwise.     

Cutaneous sensory abnormalities in children and adolescents with complex regional pain syndromes

Complex regional pain syndromes (CRPS) have been recognized with increasing frequency in children. These disorders appear to differ markedly from those observed in adults. The International Association for the Study of Pain diagnostic criteria for CRPS were developed based on adult studies; these criteria have not been validated for children. We performed standardized neurological examination and quantitative sensory testing (QST) in a group of pediatric patients to characterize features of sensory dysfunction. Forty-two patients, with unilateral lower extremity CRPS of a mean duration of the pain and symptoms of 12.6 months, who met IASP adult-based criteria for CRPS underwent standardized neurological examination and QST. QST parameters were compared to values previously derived from age- and sex-matched pediatric healthy controls. In most respects, QST parameters did not differ significantly between patients and the normal reference values except for cold and heat pain detection thresholds. Allodynia to cold and/or heat (P<0.001) occurred in 21 patients. Cold allodynia was the most common QST abnormality in our patients. Twenty-six patients showed a combination of mechanical dynamic and static allodynia and allodynia to punctate temporal summation. There was a significant correlation between mechanical dynamic allodynia and allodynia to punctate temporal summation (P<0.001). As with adult CRPS, the thermal and mechanical sensory abnormalities appear in different combinations in different patients with similar clinical presentations. In a majority of patients, the pathogenesis of pain is seemingly of central origin.     

Complex regional pain syndrome type I of the knee: A systematic literature review

In our Center for Pain Medicine, a group of patients reported to have symptoms possibly attributable to complex regional pain syndrome (CRPS) of only the knee(s). Therefore, this study aimed to investigate whether the literature reports on patients with CRPS type I in the knee(s) alone and, if so, to summarize the reported diagnostics, aetiology and treatment strategies of CRPS of the knee(s). Medline, Embase, Cochrane Library, PubMed and Web of Science were searched for articles focusing on a painful disorder of the knee, most likely CRPS type I. Screening on title and abstract was followed by full‐text reading and searching of reference lists to determine the final set of relevant articles. Of the 513 articles identified, 31 met the inclusion criteria. These articles reported on a total of 368 patients diagnosed with CRPS of the knee(s) based on the diagnostic criteria used at the time of publication. Knee surgery, especially arthroscopic surgery, was the most common inciting event in developing CRPS of the knee(s). Various treatment strategies were applied with variable outcomes. In conclusion, the scientific literature does report cases of CRPS type I of only the knee(s). This applies when using the diagnostic criteria prevailing at the time of publication and, obviously for a smaller number of cases, also when using the current Budapest criteria set. Arthroscopic knee surgery is described multiple times as the inciting event. We recommend to include CRPS of the knee in future research on the aetiological mechanisms of and optimal treatment for CRPS.     

Complex regional pain syndrome--type I: research relevance, practice realities.

Because complex regional pain syndrome--type I usually occurs following minor trauma, clients will most likely be encountered in community settings. Nurses will come into contact with them as family members, friends, neighbors, and coworkers. It is important that nurses become knowledgeable about this syndrome to assist in early recognition, diagnosis, and treatment. The latest research findings have relevance for current practice, but many questions remain unanswered.