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Multiple reflex sympathetic dystrophy. Which patients are at risk for developing a recurrence of reflex sympathetic dystrophy in the same or another limb.

Many aspects of bilateral presentation or recurrence of reflex sympathetic dystrophy (RSD) are unknown. For this reason 1183 consecutive patients with RSD were analyzed. In 10 patients RSD started in symmetrical limbs. In 34 patients RSD recurred in the same limb after a period of no or few complaints and in 76 patients RSD recurred in one or more limbs other than the first limb. Compared to 1065 patients with RSD without these features, these patients were younger (P < 0.01) and RSD started more frequently with a cold skin temperature (P = 0.02). Patients did not differ in gender or primary localization of RSD. Involvement of a second limb concerned in 47% the symmetrical limb. Recurrences were in 53% of spontaneous origin and often characterized by few signs and symptoms. The incidence of a recurrence was 1.8% per patient per year. No measures are known to prevent recurrence. Reflex sympathetic dystrophy may recur in the same or in another limb, although only in a minority of patients. Recurrences occur especially in younger patients and in the symmetrical limb. Diagnosis of a recurrence is difficult, for often the recurrence is spontaneous and presents with few signs and symptoms.

Adolescent↗

Is fibromyalgia a generalized reflex sympathetic dystrophy?

Fibromyalgia and reflex sympathetic dystrophy share defining characteristics, namely chronic pain and allodynia, as well as other important clinical features such as onset after trauma, female predominance, paresthesias, vasomotor instability, response to sympathetic blockade and anxiety/depression. Recent research using heart rate variability analysis demonstrated that patients with fibromyalgia have changes consistent with relentless circadian sympathetic hyperactivity. I propose that fibromyalgia is a sympathetically maintained pain syndrome in which ongoing sympathetic hyperactivity sensitises the primary nociceptors and induces widespread pain and allodynia.

Chronic Disease↗

Ketorolac in reflex sympathetic dystrophy.

Treatment of reflex sympathetic dystrophy (RSD) is primarily surgical. Typically, regional sympathetic blockade or sympathectomy is employed. Alternatives to neurosurgery, such as pharmacotherapy, include systemic corticosteroids and antidepressants. However, side effects and nonresponders to these approaches are not uncommon. Recently, ketorolac was demonstrated to relieve pain in RSD patients when administered by intravenous regional block (IVRB), yet this method has obvious limitations in the outpatient setting. The following case demonstrates marked improvement in treatment-resistant RSD pain with intramuscular ketorolac.

Adult↗

Norepinephrine in reflex sympathetic dystrophy: an hypothesis.

Reflex sympathetic dystrophy (RSD) usually occurs in an individual who has been experiencing significant personal stress, a state associated with increased discharge of norepinephrine (NE) from perivascular postganglionic sympathetic neurons. RSD is often precipitated by this sequence: traumatic arterial spasm, regional ischemia, neurogenic inflammation, and ischemic/edematous damage to membranes of preterminal perivascular nociceptive neurons. In the natural repair of these membranes, it is suggested that adrenoceptors appear and are ordinarily transitory; but in RSD, they are retained by the increased adjacent NE. This process delays further healing, produces pain, and releases inflammatory substances, resulting in interacting pathophysiologic vicious cycles.

Humans↗

Reflex sympathetic dystrophy in childhood.

Reflex sympathetic dystrophy is characterized by constant burning pain and hyperesthesia in an extremity. Lower extremities are usually affected. Pain is accompanied by swelling, sweating, vasomotor instability and sometimes trophic changes. There may be a history of minor injury or not. Muscle spasms, myoclonus or focal dystonia may occur. Diffuse pain, loss of function and autonomic dysfunction are three main criteria suggested for diagnosis. Symptoms can last a few days to as long as a year. In this report we present a girl with multiple limb involvement of stage I RSD. The sympathetic skin responses were tested during a remission period. She had milder attacks with a recurrence rate of 4 per year in the following three years from onset.

Adolescent↗

Complex regional pain syndromes: including "reflex sympathetic dystrophy" and "causalgia".

"Reflex sympathetic dystrophy" and "causalgia" are now classified by the International Association for the Study of Pain as Complex Regional Pain Syndromes I and II. Sympathetically maintained pain is a frequent but variable component of these syndromes, as the sympathetic and somatosensory pathways are no longer functionally distinct. Pain is the cardinal feature of CRPS, but the constellation of symptoms and signs may also include sensory changes, autonomic dysfunction, trophic changes, motor impairment and psychological changes. Diagnosis is based on the clinical picture, with additional information regarding the presence of sympathetically maintained pain or autonomic dysfunction being provided by carefully performed and interpreted supplemental tests. Clinical experience supports early intervention with sympatholytic procedures (pharmacological or nerve block techniques), but further scientific data is required to confirm the appropriate timing and relative efficacy of different procedures. Patients with recurrent or refractory symptoms are best managed in a multi-disciplinary pain clinic as more invasive and intensive treatment will be required to minimize ongoing pain and disability.

Causalgia↗

Reflex sympathetic dystrophy and electroacupuncture.

Reflex sympathetic dystrophy (RSD) is a painful and progressively debilitating condition. Untreated, it leads to changes in the patient's lifestyle that are consistent with chronic pain syndrome. This general overview of contributing factors, signs and symptoms, diagnosis, and treatment of RSD presents three consecutive cases for which electroacupuncture (EA) proved to be an effective treatment for RSD pain. Possible underlying neuroanatomical mechanisms of RSD are addressed and the scientific basis for EA is considered briefly.

Adult↗

Reflex sympathetic dystrophy: diagnostic controversies.

Reflex sympathetic dystrophy, (RSD) is a complex physiologic response of the body to an external stimulus resulting in sympathetically mediated, usually nonanatomic pain, which is out of proportion to the inciting event or expected healing response. This complex entity has been the focus of much investigation, leading however to somewhat confusing and conflicting results and theories about the etiology and pathophysiology. There is even significant conflict about what characteristics define the clinical entity called RSD, and if these characteristics vary with the specific site of involvement. We have examined the current literature regarding these fundamental conflicts, and in addition we have evaluated the current controversies surrounding the role of Three Phase Radionuclide Bone Imaging (TPBI) for diagnosis, prognosis, and patient management. These controversies include the role of scintigraphy, the various criteria for scintigraphic diagnosis, and the reported variations in sensitivity and specificity of TPBI in RSD. We have examined several factors that may have affected these results, and potentially underestimated the value of scintigraphy in the diagnosis of RSD. In addition to the heterogeneous patient populations used to establish the diagnosis by different subspecialty physicians, these factors include duration of patient's symptoms, age of the patient population evaluated, location of the disease, and the varying scintigraphic scan interpretation criteria used.

Arm↗

Reflex sympathetic dystrophy in children.

Reflex sympathetic dystrophy (RSD) is an uncommonly reported entity in children. This paper reports five cases of RSD in children and summarizes 80 cases of pediatric RSD reported in the literature. The diagnosis is based on the clinical findings of pain, dysesthesia, and autonomic instability. Tache cérébrale, not previously described in the diagnosis of RSD, is a helpful sign of vasomotor dysfunction. RSD in childhood frequently affects the lower extremities, in contrast to the adult localization around the shoulders and hands. Noninvasive, nonpharmacologic management is generally successful. A simple outpatient program of massage and mobilization was beneficial in four of the five patients treated in this study.

Adolescent↗

[Reflex sympathetic dystrophy in children].

Reflex sympathetic dystrophy (RSD) in children is a clinical syndrome characterized by pain in the extremities associated with hyperesthesia and vasomotor changes. The symptoms frequently result in serious impairment in function of the involved extremity. Treatment should be initiated as soon as possible and include intensive physical therapy; analgesics and transcutaneous nerve stimulation can be added as needed. Compared with the adult, childhood RSD is of unknown etiology and has a better prognosis. RSD has attracted little attention in clinical pediatric practice and in pediatric textbooks and the literature, so the diagnosis may be missed. We report 2 girls with RSD, aged 12 and 15 years, respectively, who were successfully treated with conservative measures; both recovered, with no sequelae.

Adolescent↗

Axillary brachial plexus blockade for the reflex sympathetic dystrophy syndrome.

The reflex sympathetic dystrophy syndrome (RSD) is a neurogenic pain syndrome that is characterized by pain, vasomotor and dystrohic changes and often motor impairments. Although the exact pathogenesis of RSD is unknown, for long the sympathetic nervous system was thought to play a dominant role and pharmacological and surgical sympathectomies have been a mainstay in treatment procedures. However, there is growing evidence of a pivotal role of C- and A delta-fibres in the aetiology of RSD. These fibres subserve a dual sensory-effector function. Besides the initiation of afferent impulses, they release neuropeptide mediators that cause a peripheral neurogenic inflammatory reaction and central neuroplastic reactions. Brachial plexus blockade (BPB) with local anaesthetic drugs interferes with the conduction of action potentials along both sympathetic efferents and the somatosensory C- and A delta-afferents and therefore seems a potential treatment modality in RSD. The aim of this study was to draw attention on this regional anaesthetic technique that is not commonly used in RSD. In this study six patients with severe RSD of an upper extremity in varying stages were treated with BPB in the multidisciplinary setting of an out-patient rehabilitation clinic with a follow-up of 12 to 21 months. The study was not placebo controlled. Three patients responded well. In these cases the treatment interval varied from 3 to 6 months, one case had RSD stage 1 and the two others stage 2. Three patients showed poor response. In one of these patients the initial effect was good but due to an infection at the insertion site of the catheter BPB had to be discontinued. The other two poor-responders had treatment intervals of 7 and 25 months and both had stage 3 RSD. We conclude that there is theoretical and clinical support to further evaluate the effect of BPB as a treatment modality in the early stages of RSD.

Adult↗

Technetium-99m sestamibi limb scintigraphy in post-traumatic reflex sympathetic dystrophy: preliminary results.

Reflex sympathetic dystrophy (RSD) has widely variable clinical manifestations. Its pathogenesis remains partially unexplained. RSD is commonly divided into three stages; these stages are not always clearly separable, but staging remains important for correct treatment. Since the disease involves soft tissue alterations as well as bone changes, we decided to investigate whether technetium-99m sestamibi limb imaging can be used to evaluate the soft tissue appearance. Fifteen patients (seven females and eight males; age range 12-68 years) with clinically significant post-fracture RSD were evaluated with both three-phase bone scan (TPBS) and 99mTc-sestamibi limb scintigraphy. Although, in general, patients with similar duration of disease, clinical stage and TPBS activity tended to have similar patterns of sestamibi uptake, discordant uptake patterns were observed in some patients with clinical stage 1. Thus, of 12 patients with stage I disease, eight had increased 99mTc-sestamibi activity in the distal part of the affected limb, while three had normal activity and one had decreased activity. All three patients with stage II disease showed normal 99mTc-sestamibi uptake. Although most of the patients with increased 99mTc-sestamibi uptake had increased activity on all three phases of the bone scan, there were discordant results between the scan patterns in other patients. On the basis of these findings, we suggest that 99mTc-sestamibi imaging may contribute to the differentiation between clinical stages and may permit evaluation of the disease course and selection of appropriate therapy. 99mTc-sestamibi imaging is not, however, a primary diagnostic procedure for RSD.

Adolescent↗

Psychological dysfunction in patients with reflex sympathetic dystrophy.

Patients with reflex sympathetic dystrophy (RSD) often present with pain and disability that cannot be explained on the basis of objective physical findings. This has led some to speculate that RSD may be caused or mediated by non-organic factors. Unfortunately, there have been few studies using standardized measures of mood and illness behavior that have compared patients with RSD to patients with other chronic pain disorders. The goal of the present study, therefore, was to compare the pattern of psychological dysfunction in patients with RSD to the pattern of dysfunction in patients with chronic back pain and local neuropathic pain. Patients with back pain resemble those with RSD in that both may report symptoms that cannot be reconciled with objective physical findings. Patients with local neuropathy, by contrast, report pain that is both circumscribed and consistent with a known organic cause. The records of 253 patients attending a tertiary pain service were retrospectively reviewed and three distinct (non-overlapping) diagnostic groups were formed: 25 were assigned to the RSD group; 44 to the back pain group; and 21 to the local neuropathy group. Using a set of stringent criteria to diagnose RSD and an analysis of covariance to control for differences in symptom duration and age, the present study found no evidence to suggest that patients with RSD were psychologically unique. Instead, RSD patients were remarkably similar to those with local neuropathy in terms of their symptom reporting, illness behavior, and psychological distress. The only exception was that RSD patients had more disability days during the preceding 6 months than those with local neuropathy (P < 0.05). The back pain group, on the other hand, presented with more diffuse pain complaints (P < 0.05) and had a greater number of non-specific medical symptoms (P < 0.05) compared to either the RSD or local neuropathy group. In contrast to previous research using less stringent diagnostic criteria, there was no evidence of higher pain scores or lower levels of psychological distress among patients with RSD. In addition, a validated survey of childhood trauma found that sexual abuse, physical abuse, emotional abuse, and cumulative trauma were evenly distributed among all three diagnostic groups. The burden of proof would appear to be upon those who advocate the non-organic hypothesis to provide credible evidence of psychological involvement in the etiology of RSD.

Adaptation, Psychological↗

Vertebral crush fracture syndrome and reflex sympathetic dystrophy.

The association of reflex sympathetic dystrophy in one or more extremities with vertebral crush fracture syndrome is reported in six cases. In two of them the reflex sympathetic dystrophy preceded the vertebral crush fractures. The 99mTc-methylene diphosphonate scintigraphy results of the skeletons of 42 consecutive patients suffering from vertebral collapse and of 30 matched controls without osteoporosis have been evaluated by three independent observers for abnormal uptakes in the extremities. An abnormal radioactive bone tracer uptake in the extremities has been observed in 15 osteoporotics (36%) and 8 controls (29%). In most cases, osteoporotics and controls, the abnormal uptake was of the zonal type in a localized area, probably reflecting bone remodeling due to localized degenerative changes. An abnormal uptake of the segmental type involving multiple joints and even multiple extremities, as seen in reflex sympathetic dystrophy, was observed in 7 patients of the osteoporotic group (17%) and in none of the control group (P less than 0.05). The patients with an abnormal segmental uptake were younger and had a significantly higher mean 24 h calcium:creatinine ratio as well as a higher urinary hydroxyproline excretion. These observations and results suggest that in some cases of idiopathic osteoporosis there might be a relation between reflex sympathetic dystrophy and vertebral crush fracture syndrome, the vertebral crush fracture syndrome being the axial type of reflex sympathetic dystrophy.

Adult↗

Sudomotor function in sympathetic reflex dystrophy.

Sudomotor functions were studied in 27 patients suffering from reflex sympathetic dystrophy (RSD) according to the criteria established by Bonica (18 women, 9 men; mean age 50 +/- 12.3 years; median duration of disease 8 weeks, range 2-468 weeks). To measure local sweating rates, two small chambers (5 cm2) were affixed to corresponding areas of hairy skin on the affected and unaffected limbs. Dry nitrogen gas was passed through the chambers (270 ml/min) and evaporation was recorded at both devices with hygrometers. Thermoregulatory sweating (TST) was induced by raising body temperature (intake of 0.5 1 hot tea and infra-red irradiation). Local sweating was also induced through an axon reflex (QSART) by transcutaneous iontophoretic application of carbachol (5 min, 1 mA). In addition, skin temperature was measured on the affected and unaffected side by infra-red thermography. Mean skin temperature was significantly higher on the affected side (P < 0.003). In spite of the temperature differences, there was no difference in basal sweating on the affected and unaffected side. However, both methods of sudomotor stimulation lead to significantly greater sweating responses on the affected compared to the unaffected side (TST: P < 0.05, QSART: P < 0.004). Latency to onset of sweating was significantly shorter on the affected side under both test conditions (P < 0.04 and P < 0.003, respectively). Sweat responses were not correlated to absolute skin temperature but were probably related to the increased blood flow on the affected side. Our findings imply a differential disturbance of vasomotor and sudomotor mechanisms in affected skin. Whereas vasoconstrictor activity is apparently lowered, sudomotor output is either unaltered or may even be enhanced.

Axons↗

[Acupuncture and reflex sympathetic dystrophy].

The term "reflex sympathetic dystrophy" (RSD) is used for various syndromes, e.g. posttraumatic edema, shoulder-hand syndrome, algodystrophy and causalgia. The clinical symptoms of RSD are characterized by a triad of autonomic, sensory and motor disturbances, which usually develop in the distal region of an affected extremity. The main symptoms are swelling, a side difference in skin temperature (autonomic symptoms), reduced active movements and muscular strength (motor symptoms) and spontaneous, deep, diffuse pain with an orthostatic component (sensory system). As soon as possible the treatment of RSD should include sympatholytic strategies and obligatory physical therapy. Acupuncture has also been reported to reduce sympathetic activity. The analgesic effect of acupuncture is well known, and therefore from an at least theoretical point of view, it should make sense to use acupuncture in the treatment of RSD. To date, however, no prospective, randomized, controlled, clinical long-term studies have been done. One short-term study showed promising results, but did not reach statistical significance. In that study acupuncture treatment seemed to alleviate the major symptoms of RSD. Since acupuncture rarely has side effects, its role as an additional option in the treatment of RSD should be further investigated.

English Abstract↗

[Distal edema and hyperhidrosis of the arm. Symptoms of reflex sympathetic dystrophy (Sudeck's disease)].

Reflex sympathetic dystrophy is characterized clinically by the triad of autonomic sympathetic dysfunction, and motor and sensory disturbances of the affected extremity. Typical symptoms are distal generalized edema with cyanotic skin, pathologic function of eccrine sweat glands and diffuse dull pain. If reflex sympathetic dystrophy is not recognized an irreversible stage may be reached, with atrophic pale, cool, and anhidrotic skin, contractures and diffuse osteoporosis. The syndrome can be idiopathic but can also be precipitated by a variety of factors, including banal trauma, bone fracture, and traumatic nerve lesions. Pathophysiologically, a functional disturbance of sympathetic nerve fibres may result in a vicious circle of blood flow dysfunction, excitation of afferent nociceptors and maintenance of sympathetic dysfunction at the level of the spinal or central nervous system. In the patient presented in this paper, sympathetic dysregulation of reflex sympathetic dystrophy was cured by means of blockades of the stellate ganglion.

Adult↗

[Sympathetic reflex dystrophy in circumscribed stenosis of the abdominal aorta. Case report and discussion of pathophysiologic principles].

Reflex sympathetic dystrophy (RSD) is a pain syndrome characterized by somatosensory and motor disturbances, as well as by autonomic and trophic changes. The term is used in a descriptive sense and does not imply specific mechanisms of pathogenesis. We report on a patient who fulfilled the clinical criteria of RSD and who also displayed increasing impairment of peripheral blood supply. Angiography revealed a circumscribed stenosis of the abdominal aorta adjacent to the bifurcation. Disturbances in peripheral circulation as a potential cause of RSD are discussed.

Aorta, Abdominal↗