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Vascular abnormalities in reflex sympathetic dystrophy (CRPS I): mechanisms and diagnostic value.

Complex regional pain syndrome type I (CRPS I, formerly known as reflex sympathetic dystrophy) is a painful neuropathic disorder that develops after trauma affecting the limbs without overt nerve injury. Clinical features are spontaneous pain, hyperalgesia, impairment of motor function, swelling, changes in sweating, and vascular abnormalities. In this study, the pathophysiological mechanisms of vascular abnormalities were investigated. Furthermore, the incidence, sensitivity and specificity of side differences in skin temperature were defined in order to distinguish patients with definite CRPS I from patients with extremity pain of other origin. In 25 CRPS I patients and two control groups (20 healthy subjects and 15 patients with other types of extremity pain), cutaneous sympathetic vasoconstrictor activity was altered tonically by the use of controlled thermoregulation. Whole-body temperature changes were induced with a thermal suit in which cold or hot water circulated. The vascular reflex response (skin blood flow, laser Doppler flowmetry, skin temperature, infrared thermometry) was analysed to quantify sympathetic outflow. Measurements were performed during a complete thermoregulatory cycle, i.e. during the entire spectrum of sympathetic vasoconstrictor activity from high (whole-body cooling) to low sympathetic activity (whole-body warming). Venous noradrenalin levels were determined bilaterally in five CRPS patients. (i) Three distinct vascular regulation patterns were identified related to the duration of the disorder. In the "warm" (acute) type of regulation, the affected limb was warmer and perfusion values were higher than in the contralateral limb during the entire spectrum of sympathetic activity. In the "intermediate" type of regulation the limb was either warmer or colder. In the "cold" (chronic) type of regulation, skin temperature and perfusion values were lower on the affected side during the entire spectrum of sympathetic vasoconstrictor activity. (ii) Noradrenalin levels were lower on the affected side, even in chronic patients with considerable cutaneous vasoconstriction. (iii) Temperature and blood flow differences between the two sides were dynamic and most prominent at a high to medium level of vasoconstrictor activity. (iv) In both control groups, there were only minor side differences in flow and temperature. In conclusion, it is suggested that, in CRPS I, unilateral inhibition of sympathetic vasoconstrictor neurones leads to a warmer affected limb in the acute stage. Secondary changes in neurovascular transmission may lead to vasoconstriction and cold skin in chronic CRPS I, whereas sympathetic activity is still depressed. Vascular abnormalities are dynamic. The maximal skin temperature difference that occurs during the thermoregulatory cycle distinguishes CRPS I from other extremity pain syndromes with high sensitivity and specificity.

Adult↗

Reflex sympathetic dystrophy in the hands: clinical and scintigraphic criteria.

In an attempt to establish specific scintigraphic criteria for the reflex sympathetic dystrophy syndrome (RSD) as defined by a group of specialized hand surgeons, 145 consecutive patients, 23 of whom had clinical RSD, underwent three phase radionuclide bone scanning (TPBS). Specific patterns for positive radionuclide angiogram, blood pool, and delayed images were established. The delayed images were sensitive (96%), specific (97%), and had a valuable negative predictive value (99%). We concluded that TPBS could provide an objective marker for RSD, and it could also be used to exclude RSD in patients who had less specific signs and symptoms.

Bone and Bones↗

Munchausen's syndrome simulating reflex sympathetic dystrophy.

A 15 year old girl who had pain, oedema of her left hand, and fever of four months' duration is described. Marked demineralisation of her hand was shown by radiography, and increased articular uptake by technetium-99m bone scan. All these changes were indistinguishable from reflex sympathetic dystrophy. After two admissions to hospital and multiple explorations we discovered that she had induced her symptoms herself and a diagnosis of Munchausen's syndrome was made. As far as we know this presentation has not been previously reported and might help to explain the physiopathology of some signs of reflex sympathetic dystrophy.

Adolescent↗

Current management of reflex sympathetic dystrophy syndrome (complex regional pain syndrome type I).

Although no major advances have occurred in the curative treatment of reflex sympathetic dystrophy syndrome (RSDS), new pathogenic insights may soon lead to innovative approaches, which may also prove effective in alleviating some forms of neuropathic pain. Preventing nerve compression and ischemia-reperfusion injury constitute valuable measures for preventing RSDS. Vitamin C administration can also prevent RSDS, together with clonidine in high-risk patients. Short-term glucocorticoid therapy has been found effective in preventing RSDS after stroke but has not been evaluated in other situations. Beneficial effects of bisphosphonates have been documented in several placebo-controlled trials. Placebo-controlled trials of ketamine and spinal cord stimulation are in order to confirm or refute the promising results obtained in open-label studies. Mirror visual feedback was introduced recently for the rehabilitation of patients with RSDS but needs to be evaluated in randomized controlled trials.

Analgesics↗

Dual energy X-ray absorptiometry in patients with lower limb reflex sympathetic dystrophy syndrome.

OBJECTIVE: To assess the difference in bone mineral density (BMD) between pathologic and healthy legs in reflex sympathetic dystrophy syndrome (RSDS) using a whole body BMD with dual energy X-ray absorptiometry (DEXA). METHODS: Cross sectional evaluation of BMD in 55 patients with RSDS compared BMD of the affected and the healthy limb with 121 controls. Followup was performed on 21 patients treated with intravenous pamidronate. RESULTS: In the cross sectional study, BMD was reduced in the dystrophic affected extremities and this reduction correlated with the duration of evolution of the disease. In the longitudinal study, BMD remained stable in patients treated with pamidronate. CONCLUSION: The cross sectional study confirms that DEXA is not a diagnostic tool. The longitudinal study confirms DEXA is accurate, nontraumatic, rapid, and safe for longterm quantitative assessment of unilateral bone loss caused by lower limb RSDS.

Absorptiometry, Photon↗

Reflex sympathetic dystrophy revisited: MR imaging findings before and after infusion of contrast material.

PURPOSE: To determine the appearance of reflex sympathetic dystrophy (RSD) at magnetic resonance (MR) imaging. MATERIALS AND METHODS: Fifty-one patients with suspected RSD were prospectively evaluated at MR imaging with T1- and T2-weighted sequences and T1-weighted sequences with fat suppression before and after the intravenous administration of contrast material. RESULTS: RSD was confirmed in 45 patients. In 35 patients with stage 1 RSD, skin thickening (31 patients), tissue enhancement with contrast material (31 patients), and soft-tissue edema (six patients) were demonstrated. In five patients with stage 2 RSD, skin thickening (two patients), skin thinning (two patients), and infrequent contrast material enhancement (one patient) were demonstrated. There was no edema in this group of patients. In five patients with stage 3 RSD, inconsistent skin changes were also demonstrated; however, muscle atrophy (four patients) was demonstrated in this stage only. CONCLUSION: MR imaging was beneficial in the demonstration of soft-tissue abnormalities in patients with RSD. MR imaging may also help stage RSD, particularly stages 1 and 3.

Adult↗

Abnormal thermoregulatory responses in patients with reflex sympathetic dystrophy syndrome.

OBJECTIVE: To look for asymptomatic thermoregulatory abnormalities in the noninvolved limbs in patients with well established reflex sympathetic dystrophy syndrome (RSDS). The study was prompted by the observation of development of severe upper limb Raynaud's phenomenon in a young woman with a history of RSDS affecting both lower limbs. METHODS: Eleven patients and 11 age and sex matched control subjects underwent a standard thermal stress test: cold water immersion of a hand unaffected by RSDS in water at 15 degrees C for one min. All controls had had injuries uncomplicated by RSDS. RESULTS: Lag time (the lag phase between the end of the cold challenge and the onset of rewarming) was significantly increased (p < 0.01) in the patient group (median 5.67 min, range 0.50 to > 15) compared to the control group (median 0.50 min, range 0.50 to 12.92). The median maximum temperature recovery was lower in the patient group (81.6%, range 0 to 170.4) than in the control group (119.3%, range 49.1 to 153.1). CONCLUSION: Our findings support an association between RSDS and a generalized abnormal response to cold challenge. Further studies are required to examine the temporal relationship underlying this. Early screening for thermoregulatory dysfunction of patients after fractures might allow identification of patients at risk of developing RSDS.

Adolescent↗

Intravenous pamidronate for treatment of reflex sympathetic dystrophy during breast feeding.

A 39-year-old woman presented in the first month of pregnancy with reflex sympathetic dystrophy involving both lower legs. Symptoms became so severe that she could not walk unassisted, and the pain worsened after delivery. Radiographs showed patchy reduction in apparent density in the tarsal bones and around the ankles and knees. Uptake was increased in these areas on technetium methylene diphosphonate bone scan. Bone density (dual-energy X-ray absorptiometry) was reduced in the spine, hip, and radius. Biochemical tests were normal except for an increase in urinary excretion of the N-telopeptide cross-linking region of type I collagen (NTx). Because the patient wanted to continue breast-feeding, intravenous pamidronate was administered at monthly intervals. Breast milk was collected for 48 h after the infusion. The pain began to decrease soon after drug administration was initiated, and it was virtually gone by 6 months. NTx excretion fell by 78% and bone density increased by as much as 18.9% over the 6-month treatment interval. The baby was healthy and grew normally. Milk expressed after the first treatment was assayed for pamidronate content by high-performance liquid chromatography with fluorescence detection. None was detected (limit of quantitation, 0.4 micromol/liter). This case shows that pamidronate may be considered for treatment of lactating women.

Absorptiometry, Photon↗

The effect of gabapentin in earlier stage of reflex sympathetic dystrophy.

The objective of this paper is to investigate the effect of gabapentin in the earlier stage of reflex sympathetic dystrophy syndrome (RSD). Twenty-two patients diagnosed with RSD were enrolled. Initial gabapentin dosage was 600 mg/day. This dosage is increased gradually until a satisfactory pain level was reached. After this level, this dosage was maintained throughout the study. An exercise program was also applied to the patients. Provoked and static pain scores of the patients were obtained initially, at 3-day intervals for maintenance dosage determining, and at 6 weeks after the discharge. Functional improvement parameters were volumetric measurement; dynamometric measurement and third finger pulp-distal palmar crease distance measurement for hands; and metric circumferential measurement and range of motion for elbow, knee, and foot initially, at baseline, on the tenth day, upon discharge, and 6 weeks after the discharge. The mean maintenance dose of gabapentin was 1,145.46+/-377.6 mg/day (range, 900-1,800 mg/day). Improvements in spontaneous and provoked pain intensities were statistically significant. No statistically significant difference was obtained in functional improvement parameters. Dizziness in three patients, headache in two patients, and mild burning feeling in the tongue in one patient were the reported side effects. These symptoms resolved spontaneously in few days. Gabapentin cannot be recommended as the drug of choice, but it may be considered as one of the therapeutic alternatives in the management of pain due to RSD. We suggest that it is effective only for the pain and not for other symptoms of RSD. Serious side effects that will cause the patient to stop using the drug are rare.

Adult↗

Cost-effectiveness analysis of adjuvant physical or occupational therapy for patients with reflex sympathetic dystrophy.

OBJECTIVE: To study from a societal viewpoint the cost-effectiveness of adjuvant treatment for patients with reflex sympathetic dystrophy (RSD) of one upper extremity. DESIGN: A two-center randomized clinical trial comparing pairwise physical therapy (PT), occupational therapy (OT), and control treatment (CT). PATIENTS: One hundred thirty-five patients with RSD for less than 1 year participated. INTERVENTIONS: PT and OT were given according to protocols. For CT, services by social workers were offered. MAIN OUTCOME MEASURES: The Impairment-level Sum Score (ISS), the modified Greentest, and the Sickness Impact Profile (SIP) were used to determine effectiveness. Real medical costs, nonmedical costs, and productivity costs were distinguished and incremental cost-effectiveness ratios were calculated. Sensitivity analyses were performed on cost estimates. RESULTS: The ISS, but not the Greentest and SIP, showed a significant difference between PT versus OT and CT. The mean adjuvant treatment costs were significantly higher for PT (Netherlands Guilders [NLG] 1,726) and OT (NLG 2,089) compared with CT (NLG 903). The mean total medical costs were not significantly different for the groups (PT, NLG 8,692; OT, NLG 13,023; and CT, NLG 7,888) (intention-to-treat analysis). The sensitivity analyses showed a moderate influence of the cost estimates. CONCLUSIONS: PT results in clinically relevant improvement in RSD. Costs associated with adjuvant treatment are moderate compared to other medical costs. The incremental cost-effectiveness ratios of PT versus OT and CT were moderate or even dominant, thus PT was both more effective and less costly than its comparators.

Adult↗

Reflex sympathetic dystrophy of the head: case report and discussion of diagnostic criteria.

The case of a patient who was involved in a car crash is presented. On the right side her wrist and zygomatic arch were fractured. At both localizations she developed a painful syndrome. The diagnosis of reflex sympathetic dystrophy of her arm was made within 3 weeks after the injury and therapy instituted. The pain in her face and skull were accompanied by vasomotor changes which increased after exercise and by hypesthesia, hyperpathia, paresis, and hyperhidrosis. Disorders of the cranial nerves were excluded by physical examination and electromyography and structural disorders by CT scanning. The diagnosis reflex sympathetic dystrophy of the head was made one year after the injury. The problems in diagnosis are discussed.

Accidents, Traffic↗

Reflex sympathetic dystrophy of the upper extremity. Clinical features and response to multimodal management.

The weight of available evidence suggests that reflex sympathetic dystrophy (RSD) is a complex clinical entity that (1) occurs predominantly in young adult women; (2) has five clinical types but presents most frequently as minor traumatic dystrophy; (3) has primary signs and symptoms (e.g., pain, edema, stiffness, and discoloration) that are expressed highly in each clinical type, whereas secondary signs and symptoms are variable; (4) responds well to treatment, regardless of its clinical type; and (5) is managed best when treatment is started early. It can be concluded that RSD is a multifaceted disease that responds well when managed with a multimodal treatment program aimed at the various interacting components of the disorder. The recognition and documentation of the variation of the clinical features of RSD may allow for its earlier diagnosis and treatment and thus significantly improve the chances for a successful outcome.

Analysis of Variance↗

Experimental pressure pain in patients with complex regional pain syndrome, Type I (reflex sympathetic dystrophy).

Research in animals shows that the levels of neuropathic pain expression is genetically associated with a characteristic response profile to sensory stimuli. The aim of the present investigation was to examine if pressure algometry can identify a specific pain sensitivity profile in patients with complex regional pain syndrome, Type I (reflex sympathetic dystrophy), and to distinguish complex regional pain syndromes from other chronic pain dysfunction syndromes. Pressure pain threshold and pain tolerance measured at the sternum in 17 patients with complex regional pain syndrome, Type I (reflex sympathetic dystrophy), were compared with values obtained in 13 patients suffering from other chronic pain dysfunction syndromes and in a control group of 24 pain-free volunteers. The pressure algometer consisted of a force displacement transducer with a 0.25 cm2 tip connected to a recorder. The rate of force application was 1 kg/0.25 cm2/s. The difference between threshold and tolerance was defined as the pain sensitivity range. Young patients with complex regional pain syndrome (<40 yr) demonstrated a significantly higher mean pain sensitivity range compared with young subjects who had chronic pain or who were pain-free. Mean threshold and tolerance values were significantly lower in patients with complex regional pain syndrome (2.7+/-1.0 kg (mean +/- standard deviation) and 5.4+/-2.0 kg, respectively) and in patients suffering from other chronic pain syndromes (2.6+/-1.1 and 4.6+/-1.7 kg) than in healthy subjects (5.4+/-2.3 and 8.4+/-2.6 kg). Women in the chronic pain group exhibited a significantly lower pressure pain threshold than all other subgroups. Regardless of group, women exhibited lower pressure pain tolerance than men. In conclusion, the study contained herein shows a specific pain sensitivity profile to experimental stimuli behavior in young patients with complex regional pain syndrome expressed by a large pressure pain sensitivity range, at a location away from the painful area. However, one single pressure pain measurement over the sternum is insufficient for differentiation of patients with complex regional pain syndrome from those with chronic pain because of intersubject variation.

Adult↗