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Complex regional pain syndrome type I: incidence and prevalence in Olmsted county, a population-based study.

The objective of this study is to undertake a population based study on the incidence, prevalence, natural history, and response to treatment of complex regional pain syndrome (CRPS). All Mayo Clinic and Olmsted Medical Group medical records with codes for reflex sympathetic dystrophy (RSD), CRPS, and compatible diagnoses in the period 1989-1999 were reviewed as part of the Rochester Epidemiology Project. We used IASP criteria for CRPS. The study population was in the Olmsted County, Minnesota (1990 population, 106,470). The main outcome measures were CRPS I incidence, prevalence, and outcome. Seventy-four cases of CRPS I were identified, resulting in an incidence rate of 5.46 per 100,000 person years at risk, and a period prevalence of 20.57 per 100,000. Female:male ratio was 4:1, with a median age of 46 years at onset. Upper limb was affected twice as commonly as lower limb. All cases reported an antecedent event and fracture was the most common trigger (46%). Excellent concordance was found between symptoms and signs and vasomotor symptoms were the most commonly present. Three phase bone scan and autonomic testing diagnosed the condition in >80% of cases. Seventy-four percent of patients underwent resolution, often spontaneously. CRPS I is of low prevalence, more commonly affects women than men, the upper more than the lower extremity, and three out of four cases undergo resolution. These results suggest that invasive treatment of CRPS may not be warranted in the majority of cases.

Adolescent↗

Efficacy of pamidronate in complex regional pain syndrome type I.

OBJECTIVES: Complex regional pain syndrome (CRPS), formerly known as reflex sympathetic dystrophy (RSD), is a painful, disabling disorder for which treatment is difficult. The aim of this study was to determine the efficacy of pamidronate in a double-blind randomized placebo-controlled trial. METHODS: Patients referred to our regional multidisciplinary pain management center who fulfilled the International Association for the Study of Pain criteria for CRPS Type I were enrolled in the study over a 2-year period. Patients were administered, intravenously, either pamidronate, 60 mg as a single dose, or normal saline. Patients' pain scores, global assessment of disease severity scores, and functional assessment (SF-36) scores were documented at baseline and at 1 and 3 months. RESULTS: Twenty-seven patients (18 female, 9 male; average age 45 years) were recruited, of whom 14 received pamidronate and 13 received placebo. Overall improvements in pain score, patient's global assessment of disease severity score, and physical function (SF-36) score were noted in the pamidronate group at 3 months, and improvements in role physical (SF-36) score were noted at 1 and 3 months. There was variability in pamidronate response among individuals. CONCLUSIONS: Pamidronate may be a useful treatment option in the management of patients with CRPS Type I. Although treatment response was variable, the majority of patients improved. Early administration in tandem with other treatment measures is recommended.

Diphosphonates↗

Complex regional pain syndrome I in the upper extremity.

Complex regional pain syndrome (CRPS) I, formerly known as reflex sympathetic dystrophy (RSD), is a painful neuropathic condition that most commonly affects a traumatized extremity. It is characterized by pain that is out of proportion to the original injury, has a distal predominance, and is not attributable to a specific peripheral nerve injury. The name RSD has been changed to CRPS I reflecting the fact that although sympathetic dysfunction can maintain the painful state, it is not the essential pathophysiologic lesion. Successful treatment hinges on early recognition of suspected cases, prompt referral to pain specialists, and ultimately pain control and return of limb function. Treatments range from noninvasive medications and therapies to sympathetic ganglion blockade and sympathectomy. The sports medicine physician is in an ideal position to recognize CRPS I in its earliest stages postinjury, and is advised to make prompt referral to a pain specialist when suspected.

Diagnosis, Differential↗

[Sudeck's atrophy. 3 clinical cases].

Three patients fulfilling criteria for Sudeck's atrophy (reflex sympathetic dystrophy syndrome--RSDS) are described and etiological, pathogenetic and clinical features of the disease are reviewed. RSDS is associated with a wide variety of precipitating factors, each of whom, often in concomitance with metabolic diseases and psychiatric disturbances, may cause the same clinical syndrome, which continues in a "vicious circle" of feed-back mechanisms, correlated with sympathetic hyperactivity. The symptoms may begin gradually and the disorder progresses in stages lasting from weeks to months. The management has not yet been established. Generally, the earlier the syndrome is recognized, the better the results of treatment will be. Analgesics, salmon calcitonin and physiokinesitherapy are recommended. Psychological support is advisable. In more severe patients sympathetic blockade and surgical sympathectomy may be necessary. The effects of hyperbaric oxygen treatment must still be assessed.

Adult↗

[Semi-quantitative analysis of 3-phase bone scintigraphy data in algodystrophy of the limbs].

Three-phase bone scanning of the extremities (foot or hand) was performed in 40 normal subjects and in 56 patients with an unequivocal clinical diagnosis of reflex sympathetic dystrophy. Ten patients were in the "cold" or atrophic stage of the disease process, whereas 46 were in the "hot" or acute phase. The scintigraphic parameters studied were the ratios of tracer activity in the affected side over the healthy side established for blood flow (BF), blood pool (BP), early vasculo-tissular fixation (EF), and late bone fixation (LF). In the controls, blood flow, blood pool, and early fixation showed considerable interindividual variation and only the variation of late fixation remained within narrow limits. Among the patients, those at the hot stage of the disease had significantly higher values for all four parameters than those at the cold stage. The group at the cold stage did not differ from the controls except for a significantly higher late fixation value. Furthermore, among hot stage patients, 15% to 25% had normal or diminished blood flow, blood pool and early fixation values. At the cold stage of the disease, radionuclide parameters were similar in affected feet and hands, whereas at the hot stage values at the feet were double those at the hands. Finally, statistical analysis revealed that late fixation was most closely correlated with early fixation, which in turn was most close correlated with blood pool. The clinical and pathophysiological significance of these data is discussed.

Adult↗

Alterations of the three-phase bone scan after sympathectomy.

OBJECTIVE: To evaluate alterations of the three-phase bone scan (TPBS) in patients with the clinical diagnosis of reflex sympathetic dystrophy (RSD) before and after surgical or chemical sympathectomy. DESIGN: The TPBS was done after i.v. administration of 99technetium dimethylphosphonate. Scans were read by one blinded and one nonblinded reviewer (as to site of symptoms and procedure), using a visual grading system devised in our institution. Since there were no statistically significant differences between the two examiners' scores for blood flow, pool, and delayed phases, all scores were averaged and used for comparison before and after sympathectomy. SUBJECTS: Ten women and four men with RSD were observed with 17 TPBSs before and 30 TPBSs after surgical (n = 6) or chemical (n = 8) sympathectomy. RESULTS: All three phases of TPBS were significantly enhanced after sympathectomy in 11 of 14 patients in a pattern indistinguishable from that very commonly reported in florid RSD. The increased uptake occurred within 24 h after the procedure, normalized as early as 3 months in some scans, and remained abnormal in some patients in the 12-month follow-up period. In total, only 33% of all sympathectomies performed led to long-term symptom relief. CONCLUSIONS: The data demonstrated that alterations in TPBS after sympathectomy are identical to those reported in early RSD and these alterations bear no relationship to the success of sympathectomy regarding pain relief. The mechanisms underlying alterations of TPBS as well as the potential mechanisms of sympathectomy failures are discussed.

Adolescent↗

Randomised controlled trial of gabapentin in Complex Regional Pain Syndrome type 1 [ISRCTN84121379].

BACKGROUND: Complex Regional Pain Syndrome type one (CRPS I) or formerly Reflex Sympathetic Dystrophy (RSD) is a disabling syndrome, in which a painful limb is accompanied by varying symptoms. Neuropathic pain is a prominent feature of CRPS I, and is often refractory to treatment. Since gabapentin is an anticonvulsant with a proven analgesic effect in various neuropathic pain syndromes, we sought to study the efficacy of the anticonvulsant gabapentin as treatment for pain in patients with CRPS I. METHODS: We did a randomized double blind placebo controlled crossover study with two three-weeks treatment periods with gabapentin and placebo separated by a two-weeks washout period. Patients started at random with gabapentin or placebo, which was administered in identical capsules three times daily. We included 58 patients with CRPS type 1. RESULTS: Patients reported significant pain relief in favor of gabapentin in the first period. Therapy effect in the second period was less; finally resulting in no significant effect combining results of both periods. The CRPS patients had sensory deficits at baseline. We found that this sensory deficit was significantly reversed in gabapentin users in comparison to placebo users. CONCLUSIONS: Gabapentin had a mild effect on pain in CRPS I. It significantly reduced the sensory deficit in the affected limb. A subpopulation of CRPS patients may benefit from gabapentin.

Adult↗

Complex regional pain syndrome type I: use of the International Association for the Study of Pain diagnostic criteria defined in 1994.

OBJECTIVES: The objective was to assess the reported use in recent publications of the diagnostic criteria for complex regional pain syndrome type I (CRPS I) proposed by the International Association for the Study of Pain (IASP) in 1994. METHODS: A literature search of MEDLINE (January 1996 to July 2000) was performed with use of the medical subject heading "reflex sympathetic dystrophy" and the free texts words "complex," "regional," "pain," and "syndrome." Publications in English, German, and Dutch were analyzed. From the search, 65 original publications were selected. Another 27 publications (referenced publications) that were referenced in the 65 original publications for the description of diagnostic criteria for CRPS I also were included. A standard form was used to assess a total of 92 publications. A sensitivity analysis was performed by means of analyzing three scenarios in which the diagnostic criteria were used as proposed and two combinations of less stringent criteria. RESULTS: Use of the diagnostic criterion pain was reported in 35 (38%) of the analyzed publications. None of the original publications satisfied the proposed IASP diagnostic criteria. Four (15%) of the referenced publications satisfied the proposed IASP diagnostic criteria. Ten (15%) of the original publications referred correctly to the referenced publications. With the less strict criteria used in scenarios 2 and 3, 2 (3%) and 3 (5%), respectively, of the original publications fulfilled these criteria. CONCLUSIONS: If the diagnostic criteria for CRPS I are not used uniformly, the populations in clinical studies may not be uniform either. Whether different authors are describing the same syndrome and whether their findings can be compared is open to question. On the basis of the results of this study, it is concluded that the IASP criteria for CRPS I are poorly used in clinical studies.

Diagnosis, Differential↗

Technique and early results of videoscopic lumbar sympathectomy.

The authors describe their technique of videoscopic (VS) lumbar sympathectomy (LSE), compared to the open LSE. From 1992 to 1994, 21 open and 19 VS LSE were performed. The indication was reflex sympathetic dystrophy in 17 and arterial insufficiency in 23 patients. In the open LSE the mean duration of anaesthesia was 80 min (55-115) and of surgery 37 min (25-65). The length of the chain removed varied from 1 to 3 ganglia (6-7 cm). Complications were noted in 5 patients: 1 pneumonia, 2 superficial wound problems and 2 cases of postsympathectomy neuralgia. Hospital stay of patients with RSD varied from 2 to 5 days. Of the 19 attempts to perform a VS LSE 4 had to be converted to the open technique. The duration of anesthesia was 150 min (90-280) and of surgery 92 min (45-240). Lengths of chain removed varied from 1 to several ganglia (6-7 cm). A pneumoperitoneum was present in 10 procedures, but a Veress needle was placed in only 4 of these. Complications were present in 9 patients: 1 important subcutaneous emphysema, 1 severe costal pain, 2 neuralgia, 1 temporary psoas dysfunction, 1 haemorrhage from a lumbar vein with conversion to the open technique and 3 minor superficial wound problems. The hospital stay ranged from 2 to 5 days. This study suggests that the VS LSE has no benefit over the open technique as far as the operative and early results are concerned. Whether this technique avoids some of the late disadvantages of a lumbotomy remains to be seen.

Adolescent↗

[Treatment of algodystrophies. The point of view of an anesthetist].

Algodystrophy is a complex and heterogenous syndrome, better defined as a subtype of Reflex Sympathetic Dystrophy syndrome (RSD). The pathophysiological theory of RSD has been supported by basic studies and clinical efficacy of sympathetic blocks. Results may be good, but some of RSD are not responsive to sympathetic blocks, and distinction between sympathetic dependent and independent pain has been proposed as subtypes of RSD. Physiotherapy is an essential part of RSD treatment. Sympathetic blocks and other analgesic therapies are performed in order to permit painless action of the physiotherapy against sick limbs stiffness. The rule must be: no painful physiotherapy. Use of other kind of drugs has yet to be defined. Calcitonin is potentially beneficial, but must be given in combination with blocks. Some new treatments rise interesting fundamental questions and some of them are under evaluation. Psychological support is the third part of treatment of these chronic pain patients. Multidisciplinary organisation, as offered by pain centers, can help to understand this syndrome and to elaborate guidelines for diagnosis, treatment and research programs.

Autonomic Nerve Block↗

Acupuncture in the treatment of posttraumatic pain syndrome.

To test the hypothesis that classical Chinese acupuncture provides an additional benefit subjectively as well as objectively in patients suffering from reflex sympathetic dystrophy, a double-blind, placebo-controlled prospective trial was performed. Fourteen patients suffering clinically and scintigraphically from acute CRPS of the upper limb lasting of more than one but less than 6 months were studied. Patients were randomly assigned to either the classical acupuncture (group A) or sham acupuncture (group S), which was applied five times a week for three weeks and required 30 minutes. Both groups received the same defined standard treatment. The current state of pain was assessed by means of a visual analogue scale. Subjective success of treatment was rated by the patients by means of a rating scale. Each patient underwent a clinical examination and was investigated by 5-phase bone scan in order to confirm the diagnosis. The current state of pain as well as clinical parameters were almost identical in patients of group A and of group S at the beginning. During therapy clinical parameters as well as pain improved in both groups and reached nearly normal levels after 6 months. Owing to the small number of patients in our study, no differences between sham and treatment group could be recognized. For a definitive statement the treatment of further patients in both groups is planned. Determinations of the effect of acupuncture on clinical parameters, based on long-term follow-ups are projected.

Acupuncture Therapy↗

[Role of densitometry and scintigraphy in post-traumatic algodystrophy of the arms. Initial diagnosis and monitoring during treatment with carbocalcitonin].

In 24 patients affected with post-traumatic reflex sympathetic dystrophy syndrome (RSDS) with upper limb involvement following humeral fractures, bone mineral density (BMD, mg/cm2) was measured by means of dual-photon absorptiometry in the distal radius of both the affected and the normal contralateral limbs. Subsequently, all patients underwent dynamic and static scintigraphic exams after i.v. injection of 99mTc-MDP (20 mCi), with gamma camera collimator centered in both limbs. BMD values were significantly lower in the affected sides than in the normal contralateral ones. Time-activity curves with MDP showed increased flow in the involved limbs. Significant increase in blood pool and in bone uptake was also observed. After carbocalcitonin treatment (80 U/q.d. i.m. in 12 cases and 40 U/q.d. i.m. in the other 12 cases for a month) all the patients presented improved clinical symptoms and significant increase in BMD, that was restored to normal values in 7 of the patients who had a longer treatment (40 U/q.d. i.m. for 2 months). Both local blood flow and bone uptake in the affected side significantly decreased after carbocalcitonin therapy while bone avidity index increased in those patients in whom this parameter had been measured. Our results confirm the usefulness of radioisotopic procedures in post-traumatic RSDS for both diagnosis (by demonstrating increased local blood flow and early bone demineralization) and monitoring response to treatment with carbocalcitonin, which seems to play an important role in this condition.

Adult↗

Epidemiology of complex regional pain syndrome: a retrospective chart review of 134 patients.

Complex regional pain syndrome (CRPS) remains a poorly understood chronic pain disorder. Little data has been published assessing the epidemiology of CRPS (and reflex sympathetic dystrophy, RSD). This study assessed epidemiological variables in 134 CRPS patients evaluated at a tertiary chronic pain clinic in the US, including demographic, health care utilization and legal/workman's compensation measures. In addition, the frequency of physician-imposed immobilization of the CRPS limb was assessed, as was physical examination evidence of myofascial dysfunction. This study found that these patients had seen on average 4.8 different physicians before referral to the pain center and had received an average of five different kinds of treatments both prior to and during pain clinic treatment. The mean duration of CRPS symptoms prior to pain center evaluation was 30 months. Seventeen percent had a lawsuit and 54% had a worker compensation claim related to the CRPS. Fifty-one patients received a bone scan, but only 53% of which were interpreted as consistent with the diagnosis of RSD/CRPS. Forty-seven percent had a history of physician-imposed immobilization, and 56% had a myofascial component present at evaluation. The duration of CRPS symptoms and the involvement of the upper extremity was significantly associated with the presence of myofascial dysfunction. Thus, this study found that most CRPS patients are referred to a pain specialty clinic after several years of symptoms and many failed therapies. The data also suggest the lack of utility of a diagnostic bone scan and highlight the prominence of myofascial dysfunction in a majority of CRPS patients.

Adolescent↗

Complex regional pain syndrome of the breast in a patient after breast reduction.

Complex regional pain syndrome (CRPS) is characterized by devastating pain, swelling, and cutaneous discoloration that result from vasomotor dysfunction caused by an abnormally accelerating sympathetic loop reflex after trauma or surgery. Although in the extremities CRPS is well documented as reflex sympathetic dystrophy, it only has been reported anecdotally in the breast after modified radical mastectomy and never reported after breast reduction. We report CRPS in the right breast of a 27-year-old woman after revision breast reduction surgery. The patient had signs of CRPS and symptoms of pain, swelling, epidermal scaling, and cutaneous temperature changes lasting more than 1 year. Liquid crystal thermographic scanning revealed a persistent, clinically significant hypothermic region in the affected breast. Intravenous phentolamine temporarily relieved the symptoms. Subsequent sympathetic blockade of the stellate ganglion alleviated chronic CRPS symptoms. Surgeons should be alert that CRPS may need to be considered in the differential diagnosis of chronic disproportionate pain after breast surgery. Early identification and treatment will help alleviate persistent CRPS symptoms and avoid soft-tissue changes.

Adult↗

Complex regional pain syndrome type I (RSD): pathology of skeletal muscle and peripheral nerve.

BACKGROUND: Reflex sympathetic dystrophy (RSD) (recently reclassified as complex regional pain syndrome type I) is a syndrome occurring in extremities and, when chronic, results in severe disability and untractable pain. RSD may be accompanied by neurologic symptoms even when there is no previous neurologic lesion. There is no consensus as to the pathogenic mechanism involved in RSD. To gain insight into the pathophysiology of RSD, we studied histopathology of skeletal muscle and peripheral nerve from patients with chronic RSD in a lower extremity. METHODS: In eight patients with chronic RSD, an above-the-knee amputation was performed because of a nonfunctional limb. Specimens of sural nerves, tibial nerves, common peroneal nerves, gastrocnemius muscles, and soleus muscles were obtained from the amputated legs and analyzed by light and electron microscopy. RESULTS: In all patients, the affected leg showed similar neurologic symptoms such as spontaneous pain, hyperpathy, allodynia, paresis, and anesthesia dolorosa. The nerves showed no consistent abnormalities of myelinated fibers. In four patients, the C-fibers showed electron microscopic pathology. In all patients, the gastrocnemius and soleus muscle specimens showed a decrease of type I fibers, an increase of lipofuscin pigment, atrophic fibers, and severely thickened basal membrane layers of the capillaries. CONCLUSION: In chronic RSD, efferent nerve fibers were histologically unaffected; from afferent fibers, only C-fibers showed histopathologic abnormalities. Skeletal muscle showed a variety of histopathologic findings, which are similar to the histologic abnormalities found in muscles of patients with diabetes.

Adult↗

A hypothesis on the physiological basis for causalgia and related pains.

A hypothesis is presented concerning the neuronal mechanisms which subserve the sympathetically maintained pains such as causalgia and reflex sympathetic dystrophy. The hypothesis rests on two assumptions: that a high rate of firing in spinal wide-dynamic-range (WDR) or multireceptive neurons results in painful sensations; and that nociceptor responses associated with trauma can produce long-term sensitization of WDR neurons. The hypothesis states that chronic sympathetically maintained pains are mediated by activity in low-threshold, myelinated mechanoreceptors, that this afferent activity results from sympathetic efferent actions upon the receptors or upon afferent fibers ending in a neuroma and that these afferent fibers evoke sufficient activity in sensitized spinal WDR neurons to produce a painful sensation. This hypothesis is based on known characteristics of these neuronal populations studied in experimental animals and on the observed sensory disturbances reported in patients successfully treated with sympathetic blocks. This hypothesis does not require nerve injury or dystrophic tissue. It explains both the continuous pain and the allodynia that are common to these syndromes and their abolition by sympathetic block. Specific changes are proposed in the diagnosis and treatment of post-traumatic pains.

Animals↗

Syringomyelia and complex regional pain syndrome as complications of multiple sclerosis.

OBJECTIVE: To describe a patient from Southeast Asia with the optic-spinal phenotype of multiple sclerosis who developed syringomyelia and resultant complex regional pain syndrome (formerly named reflex sympathetic dystrophy). DESIGN: Case report. SETTING: Department of neurology at a tertiary care hospital in the Republic of Singapore. PATIENT: A 53-year-old Chinese woman with a history of optic neuritis developed an episode of left hemiparesis leading to a diagnosis of multiple sclerosis. Serial neuroimaging studies revealed an active demyelinating plaque in the cervical area that later progressed into a syrinx. Over a period of 1 year she also developed signs of sympathetic dysfunction including Horner syndrome of the left eye and complex regional pain syndrome in the left hand. CONCLUSIONS: A case of the optic-spinal phenotype of multiple sclerosis that is commonly observed in Southeast Asia is described. This characteristically tissue-destructive form of multiple sclerosis resulted in syringomyelia complicated by a complex regional pain syndrome. Possible pathogenic mechanisms for these associations are discussed.

Cervical Vertebrae↗

[Inflammation theory for etiopathogenesis of algodystrophy].

Algodystrophy (reflex sympathetic dystrophy, Sudeckłs atrophy) is the condition of still unclear pathogenesis. Paul Sudeck, who described the syndrome was convinced of its inflammatory nature; later research established the inducing role of the sympathetic nervous system for many years. This view has been questioned in the last decade. This paper presents some evidence of inflammatory explanation of the disorder: increased uptake of the immunoglobulin IgM labeled In111 in affected area, followed by increased vascular permeability for macromolecules, and impaired metabolism of the high energy phosphates following the impaired oxygen extraction in the affected extremity. Free oxygen and hydroxyl radicals injurious role in the course of algodystrophy is also established by beneficial treatment with the use of free radicals scavengers (mannitol, dimethyl sulfoxide, N-acetylcysteine) and by pathologic ultrastructural changes in muscle cells due to oxidative stress. The view of the inflammatory nature of acute stage of algodystrophy does not preclude a role of sympathetic nervous system but it better explains some clinical aspects of this phase of the condition and increases recognition of its complicated nature.

Free Radicals↗