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An 11-year-old girl with reflex sympathetic dystrophy successfully treated by thoracoscopic sympathectomy.

We report on an 11-year-old girl with reflex sympathetic dystrophy (RSD) complaining of severe pain in her right upper extremity. Oral administration of narcotics or non-steroid anti-inflammatory drugs gave no relief in pain. Thoracoscopic electrocauterization of the thoracic sympathetic ganglion at the level of T3 was performed 3 months after the start of symptoms, and brought complete resolution of pain.

Arm↗

Spontaneous reflex sympathetic dystrophy (Sudeck's atrophy) syndrome associated with idiopathic thrombocytopenia.

A 37-year-old woman developed reflex sympathetic dystrophy (RSD) (Sudeck's atrophy) syndrome in the right foot. Simultaneously, she had idiopathic thrombocytopenia. There was no history of injury or bone fracture before onset of RSD. Spontaneous RSD associated with idiopathic thrombocytopenia has rarely been described. A possible relationship between the 2 conditions is discussed.

Adult↗

Prolonged stellate block in treatment of reflex sympathetic dystrophy.

Prolonged continuous stellate ganglion blockade has been found useful in treatment of post-traumatic reflex sympathetic dystrophy under the following circumstances: 1) When conservative treatment with outpatient physical therapy, tranquilizers and mild analgesics has failed. 2) When conservative therapy plus intermittent stellate blocks (q.l to 4 weeks) has failed. A three-year average follow-up of a group of 26 unselected patients has shown 25% relapse rate and 75% marked to complete improvement.

Autonomic Nerve Block↗

Reflex sympathetic dystrophy syndrome and osteogenesis imperfecta. A report and review of the literature.

A case of multifocal reflex sympathetic dystrophy (RSDS) in a patient with the tarda form of osteogenesis imperfecta is described, followed by a review of the literature. Microfractures of the trabecular bone in these patients are proposed to be the initial stimulus in the pathogenesis of RSDS by a mechanism similar to the one involved in the pathogenesis of RSDS complicating other diseases associated with bone fragility.

Adult↗

Reflex sympathetic dystrophy syndrome due to arteriovenous fistula.

A patient with end-stage renal disease presented with reflex sympathetic dystrophy syndrome (RSDS) on her left hand 1 month after arteriovenous fistula (AVF) surgery. Magnetic resonance angiography revealed steal syndrome at the AVF level. Bone scintigraphy revealed early-stage RSDS. We considered that arterial insufficiency because of steal phenomenon following AVF surgery and underlying occlusive arterial disease triggered RSDS development.

Arteriovenous Fistula↗

A 2 1/2-year-old girl with reflex sympathetic dystrophy syndrome (CRPS type I): case report.

An unusual case of a 2 1/2-year-old girl with reflex sympathetic dystrophy (RSD) of the left arm is described. She is the youngest RSD case ever presented in the literature. Upper extremity involvement is also rare in childhood RSD. She had both physical and psychological trauma in an earthquake preceding the disease. The association of RSD with a psychological disorder is stressed and awareness of the condition to the general paediatrician is recommended for early diagnosis and successful treatment.

Administration, Intranasal↗

Reflex sympathetic dystrophy: treatment with long-acting intramuscular corticosteroids.

In an 8-year period, 69 patients with reflex sympathetic dystrophy, who were followed at least 1 year, were treated with active exercises and injections of long-acting methylprednisolone. Of these, 22 patients did not respond to this treatment and 47 patients obtained significant improvement. All 47 were relieved of night and rest pain. Proximal interphalangeal motion improved from an average of 39 degrees to an average of 75 degrees. Grip strength was an average of 7 lb. before treatment and 29 lb. after treatment.

Anti-Inflammatory Agents↗

Profile of Caucasian women with possible genetic predisposition to reflex sympathetic dystrophy: a pilot study.

OBJECTIVES: To test possible human lymphocyte antigen (HLA) associations in subjects with reflex sympathetic dystrophy (RSD), and to determine correlation of HLA associations to treatment outcomes. DESIGN: Identification of class I (HLA-A, B, C) and class II (HLA-DR and DQ) (MHC) antigens by well-defined reagents in patients with RSD. SETTING AND PATIENTS: The HLA analysis was performed on 15 Caucasian women attending a university pain clinic and diagnosed with RSD on the basis of strict inclusion and exclusion criteria. OUTCOME MEASURES: Resistance to treatment was defined on the basis of lack of response to conservative management, failure to experience long-term symptom relief after sympathetic blocks, recurrence of pain after sympathectomy, need for palliative treatment, and degree of residual disability at the end of all treatments. RESULTS: A twofold increase of A3, B7, and DR2(15) MHC antigens was observed in the study population compared to control frequencies. Eighty (five of six) of DR2(15)-positive patients proved to be resistant to treatment. CONCLUSIONS: The results of this pilot study are the first to suggest a possible genetic diathesis in RSD patients with poor treatment outcome. If this finding can be confirmed in larger studies, strictly defined RSD could constitute the third neuroimmune disorder (besides multiple sclerosis and narcolepsy) associated with DR2(15). Gene(s) conferring susceptibility to RSD may be present within or near the MHC region of the short arm of chromosome 6. Due to the small size of our study group it is imperative that larger studies be done in RSD patients employing strict diagnostic criteria to confirm or refute our original observations.

Adolescent↗

Ureaplasma urealyticum as a possible cause of reflex sympathetic dystrophy syndrome.

We describe the cases of two patients with clinical and radiological findings of the reflex sympathetic dystrophy syndrome (RSDS) in whom the history of a previous genito-urinary inflammation and high levels of ESR lead us to suspect a hidden reactive arthritis. However, instrumental examinations showed a characteristic picture of RSDS without evident signs of arthritis. In both patients we decided a treatment with quinolones because of detection of an ureaplasma urealyticum genito-urinary infection. This brought to complete remission of the joint symptoms in a few days. Our findings suggest that ureaplasma urealyticum can cause and sustain a RSDS picture, maybe with a reactive arthritis-like mechanism, and that an antibiogram-driven antimicrobial treatment can be rapidly effective against this disorder.

Adult↗

Reflex sympathetic dystrophy in a teenaged girl.

A patient had pain, a cold feeling, and absent pulses in the lower part of the right leg. Minimal change was present on Doppler artery examination. She was diagnosed as having reflex sympathetic dystrophy and was treated with nifedipine (Adalat, Procardia). Symptoms gradually diminished, and after three months the results of physical examination were normal, although she still had an occasional cool feeling in the right foot. Reflex sympathetic dystrophy seems to be a common condition, but it is not widely understood and is probably underdiagnosed. It may be seen by primary care physicians as well as by specialists. Treatment is fairly simple. When pain with vasospasm is the presenting manifestation, use of a calcium channel blocker or prazosin (Minipress) in addition to physical therapy should be considered.

Adolescent↗

Acceptance of the different denominations for reflex sympathetic dystrophy.

OBJECTIVE: To elucidate the real impact in the medical literature of the different denominations for reflex sympathetic dystrophy (RSD). METHODS: A search was performed through the Medline database (WinSPIRS, SilverPlatter International, NS), from 1995 to 1999, including the following descriptors: RSD, complex regional pain syndrome (CRPS), CRPS type I, algodystrophy, Sudeck, shoulder-hand syndrome, transient osteoporosis, causalgia, and CRPS type II. RESULTS: The descriptor RSD was detected in 576 references, algodystrophy in 54, transient osteoporosis in 42, CRPS type I in 24, Sudeck in 16, and shoulder-hand syndrome in 11. One hundred records were obtained for the descriptor causalgia and five for CRPS type II. The descriptor RSD was detected in the title of 262 references, algodystrophy in 29, transient osteoporosis in 29, CRPS type I in 15, Sudeck in 3, shoulder-hand syndrome in 5, causalgia in 17, and CRPS type II in 3 references. CONCLUSIONS: The new CRPS terminology has not effectively replaced the old one. RSD and causalgia are the most used denominations.

Diffusion of Innovation↗

Three-phase bone scanning in reflex sympathetic dystrophy of the hand.

Three-phase bone scanning was performed in 181 patients suffering from reflex sympathetic dystrophy (RSD) of the hand. Four quantitative parameters were defined as follows: (a) hemovelocity and (b) blood pool (determined from the Fourier processing of angiographic data); (c) early (3-5 min) and (d) delayed (2-3 hr) bone fixation. Three significant stages of RSD were demonstrated scintigraphically. Stage I (0-20 wk from onset) demonstrated increases in velocity, blood pool, and early and delayed fixations. At stage II (20-60 wk) blood velocity and blood pool were normalized, but early and delayed hyperfixation persisted. During stage III (60-100 wk) blood velocity and blood pool were reduced on the affected hand, and early and delayed fixations were normalized. Such abnormality of decreased hemodynamic parameters may become associated with bone hypofixation in stage III. Early treatment of RSD (as compared with delayed treatment) has been demonstrated to induce normalization of hemovelocity (p less than 0.05), blood pool (p less than 0.02), and joint stiffness (p less than 0.001) without any change in the bone fixation; therefore, three-phase bone scanning may provide useful information regarding the pathophysiologic and clinical evolution of RSD.

Bone and Bones↗

Sensory testing of pathophysiological mechanisms of pain in patients with reflex sympathetic dystrophy.

The incidence of 3 sensory abnormalities was studied among 17 patients with a diagnosis of reflex sympathetic dystrophy (RSD) and 14 patients with persistent limb pain following trauma; the extent to which the 3 abnormalities were associated with each other and with the intensity of spontaneous clinical pain were also studied. These abnormalities included (1) heat-induced hyperalgesia (54.8% of 31 patients tested); (2) low-threshold A beta-mediated (45.2%) or high-threshold (54.8%) mechanical allodynia; and (3) slow temporal summation of mechanical allodynia (10 of 29 patients tested). These 3 sensory abnormalities occurred to widely varying extents and were not reliably associated with each other. As hypothesized, patients with temporal summation had significantly more intense spontaneous pain than those who did not demonstrate this sensory characteristic. In contrast, the presence or absence of thermal hyperalgesia and type of allodynia did not appear to influence the intensity of spontaneous pain. These results indicate that variable types of primary afferents (i.e., A beta versus A delta, C) and/or varying extents of abnormal spatial summation mechanisms trigger pain responses among RSD patients and that at least one of these, slow temporal summation, is likely to contribute to the intensity of a patient's ongoing pain.

Adult↗

Interobserver reproducibility in the interpretation of bone scans from patients suspected of having reflex sympathetic dystrophy.

OBJECTIVE: This work was undertaken to identify scintigraphic patterns obtained in patients suspected of having reflex sympathetic dystrophy (RSD), now often referred to as complex regional pain syndrome, whose interpretations could be difficult. METHODS: Ten patients had bone scans because of clinical suspicion of RSD in the lower legs. They were selected retrospectively to have a wide sample of scintigraphic patterns. The radionuclide images and a multiple-choice questionnaire were presented as a PowerPoint file that was sent electronically on the Internet to 54 Belgian nuclear medicine physicians. They had to determine whether the images were in favor of the diagnosis of RSD. RESULTS: Twenty-eight answers (52%) were received. There was near-complete interobserver agreement for perfectly normal scans, for scans showing diffuse uptake with enhancement of periarticular activity, and for scans showing only focal hyperactivity at the site of previous trauma. Results were more discordant when the hyperactivity was mild and when there was a diffuse hypoactivity, with or without focal hyperactivity. CONCLUSION: This study shows that using very simple methodology, it is possible to identify some scintigraphic patterns in which there is disagreement among observers and whose interpretations vary. As the results are returned to the participants, they can compare their own interpretations with those of their peers. This aspect could be useful in continuing education in medical imaging.

Adult↗

Intravenous guanethidine in patients with reflex sympathetic dystrophy.

BACKGROUND: Intravenous regional guanethidine Bier block (IVRGBB) has been used predominantly in Europe for treating reflex sympathetic dystrophy (RSD). Our experience in the United States, where its use has been limited, is reported. METHODS: Fifty-five patients received IVRGBB for RSD. Upper extremities received 20 mg (10 mg/ml) of guanethidine in 30-50 ml of 0.5% lidocaine; lower extremities received 40 mg in 40-75 ml of lidocaine (volume adjusted for size, weight, or prior adverse effect). Pain severity (mild, moderate, severe, excruciating) was obtained pretreatment. Pain severity and a global clinical assessment (GCA) (resolved, improved, no change, worse) were obtained following each treatment. The final GCA was analyzed vs: pretreatment score; age; sex; pain duration; number of treatments; and precipitating event. Adverse effects were documented. RESULTS: Of 55 enrolled patients, 2 were lost to follow-up, and 2 returned 1 and 4 years later for repeat treatment. Therefore, 53 patients were evaluated for 55 treatments. Age: 38.2 +/- 14.8 (SD) (range 10-77) years. Sex: 11 males, 44 females. Average pain duration: 2.0 +/- 1.7 years (3 days-7 years). Final assessment occurred at 3.88 +/- 5.21 months (6 days-22/3 years). Effect on pain: resolution-9.1%; improved-14.5%; no change-61.8%; worsening-14.5%. No significant relationship was found between GCA and the factors evaluated. There was a significant positive linear association between pretreatment pain and post treatment GCA (P = 0.032). Fifty-six adverse effects occurred in 19 (34.5%) patients (nausea, vomiting, orthostatic hypotension, dizziness, diarrhea, weakness). CONCLUSIONS: IVRGBB does not provide long-term pain relief and is associated with adverse effects in over 1/3 of patients.

Adolescent↗

Regional intravenous guanethidine vs. stellate ganglion block in reflex sympathetic dystrophies: a randomized trial.

Regional intravenous guanethidine blocks and stellate ganglion blocks have been compared in a randomized trial. Nineteen patients, randomly allocated to two groups of therapy and exhibiting severe reflex sympathetic dystrophy following peripheral nerve lesions, have been treated. The performance of the intravenous guanethidine block is of longer duration and superior to stellate ganglion block, as regards some early pharmacological effects (skin temperatures and amplitude of plethysmographic waves recorded before blockade and 15 min, 60 min, 24 h, 48 h after institution of the block). In fact the intravenous guanethidine group shows a persistent and significant increase of the skin temperature and of the plethysmographic traces in the blocked side 24 h and 48 h after blockade in comparison with the patients treated with stellate ganglion block. Concerning the therapeutic effects (changes in pain scores and clinical signs--hyperpathia, allodynia, vasomotor disturbances, trophic changes, oedema and limited motion), recorded at the end of treatment and 1 month and 3 months follow-up, an intravenous guanethidine block carried out every 4 days up to a total of 4 blocks is comparable with a stellate ganglion block every day up to a total of 8 blocks. The results of this study show that regional sympathetic block with guanethidine is a good therapeutic tool in the treatment of reflex dystrophies, especially on account of its negligible risks and contraindications.

Adult↗