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Sympathetic skin response in patients with reflex sympathetic dystrophy.

The sympathetic skin response (SSR) was recorded in four patients diagnosed with reflex sympathetic dystrophy (RSD) in one upper limb using Kozin's clinical criteria in conjunction with a three-phase bone scan. All patients had sustained cerebral vascular accidents and were classified as Stage I RSD. The SSR was recorded in both hands after each of ten contralateral median nerve surface stimulations and in both feet after each of ten contralateral peroneal nerve surface stimulations. Amplitude, onset latency, and number of phases were recorded for all responses in each limb. Mean amplitude, onset latency, and the number of phases of the five largest potentials were then determined. In all patients, there was a statistically significant difference in the amplitude and latency of the SSR in the involved limb compared with the uninvolved limb; mean amplitude of the involved limb was greater than the mean amplitude of the uninvolved limb (P < 0.001), and latency to onset of the SSR in the involved limb was shorter than that of the uninvolved limb (P < 0.001). There was no statistically significant difference in mean amplitude and latency between the involved side and uninvolved side responses as measured at the feet.

Cerebrovascular Disorders↗

Traumatic neuralgias: complex regional pain syndromes (reflex sympathetic dystrophy and causalgia): clinical characteristics, pathophysiological mechanisms and therapy.

Complex regional pain syndromes (CPRS) may develop as a disproportionate consequence of a trauma affecting the limbs without (CRPS I, reflex sympathetic dystrophy) or with (CRPS II, causalgia) obvious nerve lesions. The clinical picture of CRPS consists of asymmetrical distal extremity pain, swelling, and autonomic (sympathetic) and motor symptoms. Changes in the peripheral and central somatosensory, autonomic and motor processing, and a pathologic interaction of sympathetic and afferent systems are discussed as underlying pathophysiologic mechanisms. Therapeutic strategies include pharmacologic pain relief, sympatholytic interventions, and rehabilitation.

Analgesics↗

Quantitative bone scintigraphy in reflex sympathetic dystrophy.

We have investigated the appearances of the technetium-99m labelled methylene diphosphonate bone scan in 29 scans performed in 16 patients with post-fracture reflex sympathetic dystrophy (RSD) of the hand. There was a close correlation between increased uptake in the metacarpophalangeal joints and the metacarpal bones, suggesting that the increased uptake in RSD is not confined to the periarticular areas as has been previously reported but occurs throughout the affected region. In seven cases examined 3 months after fracture, RSD was associated with a significant increase in uptake at all sites in the hand on the delayed scan which was not seen in matched post-fracture controls, confirming that increased uptake on delayed bone scintography is a sensitive test for the presence of RSD even in the presence of a fracture of the wrist. The early increased uptake gradually returned to normal. Local tenderness assessed by dolorimetry was correlated with increased uptake, suggesting that the bone scan is not only useful as a diagnostic tool but also provides a quantitative indication of the severity of the condition which may be useful in planning and assessing treatments.

Bone and Bones↗

Impact of three-phase bone scintigraphy on the diagnosis and treatment of complex regional pain syndrome type I or reflex sympathetic dystrophy.

OBJECTIVE: To determine the impact of three-phase bone scintigraphy (TPBS) on the diagnosis and management of complex regional pain syndrome type I (CRPSI) or reflex sympathetic dystrophy (RSD). SUBJECTS AND METHODS: Twenty consecutive patients with a recent clinical evidence of CRPSI were referred for TPBS as part of their routine management plan. All patients underwent neurological examinations with special attention to the evaluation of clinical features of vasomotor, sudomotor, motor and sensory dysfunction. Patients were followed prospectively. When both the clinical and TPBS results supported the diagnosis of CRPSI, patients were started on treatment. RESULTS: Of the 20 patients, TPBS supported the diagnosis of RSD in 9 who were treated with steroids and physiotherapy. Complete follow-up was available for 7 of them and all had a satisfactory response to treatment. For the remaining 11 patients RSD was diagnosed clinically but not confirmed by TPBS. On follow-up there was no evidence that TPBS failed to identify RSD in these 11 patients. CONCLUSION: The results indicate that TPBS confirmed the clinical diagnosis of RSD, and, more importantly, had a significant impact on its management.

Adult↗

Migraine headache following stellate ganglion block for reflex sympathetic dystrophy.

The alteration of extracranial blood flow in conjunction with clinical signs of autonomic nervous system dysfunction have led to various explanations concerning the pathophysiology of migraine headache. Reflex sympathetic dystrophy, a painful disorder of the sympathetic nervous system, can be treated by blocking the sympathetic nerves located in the stellate ganglion, resulting in vasodilation, ptosis, miosis, and anhydrosis. In theory, these changes could trigger a migraine headache attack secondary to autonomic dysfunction reflecting an imbalance between sympathetic and parasympathetic nervous systems. This may be especially true in a patient with a previous history of meningitis that may have resulted in a disorder of cerebrovascular regulation. We report a 56-year-old man with no previous history of migraine who developed migraine with aura after a stellate ganglion block. These episodic headaches occurred with decreasing frequency and severity for over 6 months, with eventual complete resolution. This interesting phenomenon has not been reported in the English literature and may help to better understand the pathophysiology of migraine.

Humans↗

Pattern recognition in five-phase bone scintigraphy: diagnostic patterns of reflex sympathetic dystrophy in adults.

The objective of this study was to assess qualitative and quantitative patterns of tracer accumulation to increase the diagnostic utility of bone scintigraphy in reflex sympathetic dystrophy (RSD). Of 120 patients with high clinical suspicion for RSD, 96 were confirmed as having RSD during follow-up, while the remaining 24 were used as controls. Clinical parameters were measured and correlated to five activity ratios (0-30 s, 0.5-5 min, 5-15 min, 3 h, 24 h) and five scintigraphic signs. Monitoring three dynamic phases revealed different tracer kinetics of potential diagnostic utility; however, the 24-h bone phase offered no additional diagnostic contribution and can be omitted. Quantification provided objective parameters for the duration of symptoms, pain and impairment of movement but not for surface temperature differences, swelling and impairment of physical force. It is of limited use for diagnosis except for the exclusion of disease. Discriminant analysis revealed the combination of three signs (diffuse uptake in carpus/tarsus+diffuse uptake in all small joints+increased activity ratio in the late blood pool phase) to be the pattern with the highest diagnostic accuracy independent of localisation, sex, age and precipitating factors. It is concluded that the scintigraphic confirmation of RSD is based on lateralisation in the late blood pool phase and the described pattern in the early bone phase.

Adult↗

Severe complications of reflex sympathetic dystrophy: infection, ulcers, chronic edema, dystonia, and myoclonus.

OBJECTIVE: To determine the prevalence, type of complication, predisposing factors, and treatment for severe complications in a population of reflex sympathetic dystrophy (RSD) patients. DESIGN: Retrospective analysis of the data from RSD patients collected over a 12-year period, to investigate the involvement of predisposing factors in an RSD population without severe complications compared with an RSD population with severe complications. SETTING: Outpatient clinic of a department of surgery of a university hospital. PATIENTS: A total of 1,006 patients with the diagnosis of RSD established according to prospectively defined criteria. MAIN OUTCOME MEASURES: The signs and symptoms of every RSD patient who visited the department were prospectively documented in the medical history; these data were retrospectively analyzed with special regard to RSD with severe complications-infection, ulcers, chronic edema, dystonia, and/or myoclonus-for prevalence, type of complication, and treatment. RESULTS: Seventy-four RSD patients who were mostly young and female developed severe complications. More than one complication occurred in 91% of the affected extremities. Severe complications developed more frequently in the lower extremity (65%). In patients in whom the acute RSD started with a decreased skin temperature of the affected extremity, severe complications developed significantly more often than in acute RSD patients with a warm skin temperature of the extremity from the onset of the disease (p < .001). CONCLUSIONS: It is important to recognize "cold" RSD immediately at the onset of the disease because this group of RSD patients has a higher risk of developing a severe complication, mostly followed by a severe disability that is resistant to therapy.

Adolescent↗

The reflex sympathetic dystrophy syndrome (RSDS). III. Scintigraphic studies, further evidence for the therapeutic efficacy of systemic corticosteroids, and proposed diagnostic criteria.

Sixty-four patients were evaluated prospectively for a reflex sympathetic dystrophy syndrome (RSDS), using quantitative clinical measurements, high-resolution roentgenography and scintigraphy. Five separate groups were identified by their clinical features, allowing us to distinguish patients with definite or incomplete forms of the RSDS as well as 16 patients with other disorders. Scintigraphy was found to be a useful diagnostic study that may also provide a method of predicting therapeutic response. Systemic corticosteroid therapy proved to be a highly effective mode of treatment for up to 90 percent of the patients with the RSDS.

Diagnosis, Differential↗

Reflex sympathetic dystrophy and repetitive strain injury: temperature and microcirculatory changes following mild cold stress.

Temperature and blood flow studies were performed in the upper limbs of six patients with reflex sympathetic dystrophy (RSD), nine patients with repetitive strain injury (RSI) and 12 control subjects using thermography, laser Doppler flowmetry, infrared photoplethysmography and venous occlusion strain gauge plethysmography. The contralateral responses of the symptomatic and asymptomatic limbs were examined after being subjected, separately, to mild cold stress (20 degrees C for 1 min). Altered thermoregulation and haemodynamics were evident in RSD. Though the pattern of response to contralateral cold challenge is similar to normal in RSI, vasodilatation and reduced vasomotion appears to be characteristic in this condition. Such changes may assist in distinguishing between RSD and RSI from other causes of chronic upper limb pain.

Adult↗

Causalgia and reflex sympathetic dystrophy: does the sympathetic nervous system contribute to the generation of pain?

The striking response of causalgia and reflex sympathetic dystrophy (RSD) to sympatholytic procedures together with signs of autonomic nervous system abnormalities suggest that the sympathetic efferent system can generate or enhance pain (sympathetically maintained pain, SMP). This concept is supported by human and animal experiments indicating that sympathetic activity and catecholamines can activate primary afferent nociceptors. Some clinical evidence, however, calls the SMP concept into question and alternative explanations have been advanced. In this review, we describe the clinical features of causalgia and RSD and the evidence for sympatholytic efficacy. The major barrier to proving the SMP concept is that all available sympatholytic procedures are problematic. We conclude that, although the weight of current evidence supports the SMP concept and its relevance to causalgia and RSD, it remains unproven by scientific criteria. More careful adherence to diagnostic criteria and well-controlled trials of sympatholysis are needed to finally settle the issue.

Animals↗

Reflex sympathetic dystrophy: misdiagnosis in patients with dysfunctional postures of the upper extremity.

The purpose of this case-control study was to assess the frequency of the inappropriate diagnosis of reflex sympathetic dystrophy (RSD) in patients who presented with dysfunctional postures of the upper extremity (n = 43). This group of patients with a dysfunctional posture was compared with a randomly selected control group of patients who presented with pain but no dysfunctional posture (n = 88). The patients underwent radiographic evaluation after review of previous medical records and history and physical examination. Patients with dysfunctional postures had a significantly higher frequency (63%) of a previous inappropriate diagnosis of RSD compared with the control group (6%). None of the patients in either group had objective findings consistent with a diagnosis of RSD. Patients presenting with dysfunctional postures of the upper extremity may be misdiagnosed as having RSD and rarely meet the criteria for this diagnosis.

Adolescent↗

Quantitative three-phase bone scintigraphy in the evaluation of intravenous regional blockade treatment in patients with stage-I reflex sympathetic dystrophy of upper extremity.

OBJECTIVE: To investigate the role of quantitative three phase bone scintigraphy (QTPBS) in the evaluation of efficacy of intravenous regional blockade treatment in patients having reflex sympathetic dystrophy (RSD) of the upper extremity. MATERIAL AND METHODS: Twenty-six patients with stage-I RSD were focused on in this study. Patients were treated with physical therapy and intravenous (I.V.) regional blockade therapy consisting of dexamethasone and lidocaine. All patients were clinically evaluated before and 1 month after the completion of the therapy protocol. QTPBS was applied to patients before therapy and 1 month after the therapy. As a control group, 11 healthy subjects also underwent QTPBS. Perfusion, hyperemic and fixation indices were calculated from three-phase bone scintigraphy. RESULTS: All patients showed statistically significant clinical improvement after the therapy (p < 0.01). Pre-treatment, perfusion (1.67 +/- 0.63), hyperemic (1.44 +/- 0.48) and fixation (1.69 +/- 0.48) indices of patients were higher than those of healthy subjects (PI: 0.95 +/- 0.05, HI: 0.94 +/- 0.06, FI: 1.01 +/- 0.2) (p < 0.01) and all indices significantly decreased after the treatment (PI: 1.33 +/- 0.46, HI: 1.18 +/- 0.23, FI: 1.42 +/- 0.26) (p < 0.01). CONCLUSION: I.V. regional blockade therapy combined with corticosteroids is a simple, safe and effective method for the treatment of patients with stage-I RSD in the upper extremity. QTPBS is a valuable and objective method to evaluate the response to therapy and may be useful for staging of patients and predicting the response to therapy.

Adult↗

Reflex sympathetic dystrophy of the left hand and motor impairments of the unaffected right hand: impaired central motor processing?

OBJECTIVE: To test whether central motor processing can be impaired in chronic reflex sympathetic dystrophy (RSD). DESIGN: Experimental 2-group analysis. SETTING: Tertiary care center in the Netherlands. PARTICIPANTS: Five patients with stage 3 RSD of the left forearm, free of symptoms and complaints in the right forearm; and 10 healthy control subjects. INTERVENTION: On a digitizer, RSD patients and controls had to draw 3 sequences of graphemes of different complexity with their (unaffected) dominant right hand. The drawing tracks were segmented in time periods between points of velocity minima of the pen tip. MAIN OUTCOME MEASURES: Mean velocity, coefficients of variation of both length and movement time per segment, and mean intersegmental pausing time were calculated for each sequence. RESULTS: A repeated-measures analysis of variance by using the multivariate method yielded a 35% lower mean velocity (F(1,13) = 5.83, P =.031), a 110% larger segment length variability (F(1,13) = 9.72, P =.008) and a 60% larger variability of movement time per segment (F(1,13) = 5.78, P =.032) in RSD patients. No group difference was found for intersegmental pausing time or any interaction effect with the type of task. CONCLUSION: Patients with chronic RSD have a normal ability to preprogram sequential movements of their unaffected hand; but with impaired temporospatial coding and movement execution. We concluded that cortical mechanisms may be involved in motor impairments in patients with chronic RSD.

Adult↗

Münchausen's syndrome simulating reflex sympathetic dystrophy.

Rheumatologic presentations of factitious illness such as Münchausen's syndrome are rare. We describe a case of alternate edema of the hands in a 25-year-old man, diagnosed as reflex sympathetic dystrophy. This patient eventually presented all clinical characteristics of Münchausen's syndrome.

Adult↗

Treatment of severe, recalcitrant reflex sympathetic dystrophy: assessment of efficacy and safety of the second generation bisphosphonate pamidronate.

The objective of the study was to assess the efficacy and the safety of pamidronate (APD) in recalcitrant reflex sympathetic dystrophy (RSD). Ten women and 13 men with a mean (+/-standard deviation, SD) age of 44 +/- 11 years were included. The involved sites were: the ankle (n = 10), the foot (n = 7), the hand (n = 3), the hip (n = 2), the knee (n = 2) and the shoulder (n = 1). Some patients had more than one site involved. Mean (+/-SD) duration of the disease was 15 +/- 13 months. RSD was in pseudo-inflammatory phase in 16 patients and in ischaemic phase in 7 patients. RSD was post-traumatic in 17 cases; 11 patients have been previously treated unsuccessfully by sympathetic blockades. APD was administered intravenously (perfusion) to a dose of 1 mg/kg/day during 3, 2 or one day. Fourteen patients received APD during 3 consecutive days whereas 7 patients have been treated during 2 consecutive days and 2 patients only during 1 day mainly due to adverse events. Efficacy was assessed by a decrease of pain = visual analogic scale (VAS, 0-100 mm) and verbal scale (PVS, range 0-3). Moreover, the patient and the observer have estimated the efficacy of the treatment on a verbal scale (EVS, range 0-3). Measurements of these parameters were performed immediately before the treatment and 7, 30, 60 and 90 days later. The maximum duration after treatment was 9 months. A significant decrease of VAS and PVS were observed between D0 and D30 (p = 0.0002 and p = 0.0002 respectively), D0 and D60 (p = 0.0004, p = 0.0004 respectively), and D0 and D90 (p = 0.00003, p = 0.0001 respectively). A significant increase of EVS was only observed between D0 and D90 (p = 0.03). Adverse events were noted in 14 patients: transient fever (n = 6), venous inflammation (n = 2), transient symptomless hypocalcaemia (n = 3), nausea (n = 1), lymphopenia (n = 1), transient hypertension (n = 1). These results suggest an efficacy of APD in recalcitrant RSD. Double-blind placebo controlled studies are required to back up these preliminary results.

Adult↗

Diagnostic approach to reflex sympathetic dystrophy after fracture: radiography or bone scintigraphy?

The aim of this paper was to compare the value of bone scintigraphy and radiography in the early diagnosis of post-fracture reflex sympathetic dystrophy (RSD). Thirty-seven adult patients with post-fracture RSD (28 in the first and nine in the second clinical stage of RSD), as well as seven patients with fracture but without RSD (control group), were investigated by radiography and bone scintigraphy. All of them were immobilized (duration of immobilization: 4-22 weeks). In 21 persons three phase bone scintigraphy was performed. The best distinction between the control group and the RSD patients was achieved by delayed bone scintigrams. The sensitivity (97%), positive predictive value (97%) and accuracy (95%) of delayed bone scintigraphy were very high compared to the values for radiography, which were 73%, 90% and 70% respectively. Bone scintigraphy also displayed higher specificity (86%) and negative predictive value (86%) than radiography (57% and 29% respectively). In the first clinical stage the difference between the accuracy of bone scintigraphy (97%) and radiography (63%) was greater than for the whole group. In the second stage of RSD the accuracy of bone scintigraphy (86%) and radiography (81%) was similar. Three-phase bone scintigraphy is not necessary for the diagnosis of post-fracture RSD: it is sufficient to perform delayed bone scintigraphy. It is concluded that bone scintigraphy is to be preferred to radiography for the early diagnosis of post-fracture RSD in the first clinical stage. In the second stage the diagnostic capabilities of bone scintigraphy and radiography are more comparable.

Adult↗

Surgical sympathectomy for reflex sympathetic dystrophy syndromes.

PURPOSE: The purpose of this study was the assessment of the efficacy of thoracoscopic cervicodorsal and open lumbar sympathectomy for the reduction of pain severity and disability associated with reflex sympathetic dystrophy (RSD). METHODS: From 1992 to 2000, 73 patients with RSD underwent 46 video-assisted thoracoscopic (first to fourth thoracic ganglion) or 37 surgical lumbar (first to fourth lumbar ganglion) sympathetic chain resections. The patients were referred from multidisciplinary pain clinics with documented sympathetically maintained pain syndrome on the basis of reproducible more than 50% reduction in pain severity score (0, no pain; 10, most severe pain imaginable) for more than 2 days after sympathetic block therapy. The mean duration of the RSD symptoms before sympathectomy was 26 plus minus 14 months (range, 6 to 100 months). Postoperative pain severity score, limb disability, and overall patient satisfaction were assessed by an independent third-party observer at a mean follow-up period of 30 months. RESULTS: No operative mortality or serious morbidity (Horner's syndrome, bleeding that needed transfusion, wound infection) occurred. Transient (<3-month) postprocedural sympathalgia developed in one third of the patients for cervicodorsal sympathectomy and 20% of the patients for lumbar sympathectomy and was treated effectively with trigger point/proximal ganglion block therapy or transcutaneous electrical nerve stimulation. At 3 months after sympathectomy, 10% of the patients had conditions that were judged treatment failures with no reduction in pain severity or limb disability. The remaining patients testified to more than 50% pain reduction, with pain severity scores decreasing from a mean of 8.7 before surgery to 3.4 after sympathectomy. At 1 year, one quarter of the patients had continued significant pain relief (pain severity score, <3) and an additional 50% of the patients indicated continued but reduced pain severity and an increase in daily/work activities. Overall, patient satisfaction (willingness to have procedure again, benefit from sympathectomy) was 77% and was not significantly influenced by patient age, RSD duration/stage, or extremity involvement (lumbar, 84%; cervicodorsal, 72%). CONCLUSION: Patients with RSD with a confirmed sympathetically maintained pain syndrome can realize long-term benefit from surgical sympathectomy. Procedural efficacy was similar for both upper limb and lower limb RSD syndromes, although the level of pain reduction did deteriorate with time. After sympathectomy, the patients with RSD had a low incidence rate (7%) of "new" complex regional pain or disabling compensatory sweating syndromes.

Adolescent↗