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Despite being different conditions, complex regional pain syndrome type 1, phantom limb pain and stroke share some potentially important similarities. This report examines experimental and clinical findings from each patient population. It identifies common aspects of symptomatic presentation, sensory phenomena and patterns of cortical reorganization. Based on these common findings, we argue that established principles of stroke rehabilitation are also applicable to rehabilitation of complex regional pain syndrome type 1 and phantom limb pain. In addition, we contend that promising treatment approaches for complex regional pain syndrome type 1 and phantom limb pain may be helpful in stroke rehabilitation. Examples of emerging supportive evidence for these hypotheses are provided and discussed.

Complex regional pain syndrome is characterised by an exaggerated response to injury in a limb with intense prolonged pain, vasomotor disturbance, delayed functional recovery and trophic changes. This review describes the current knowledge of the condition and outlines the methods of treatment available with particular emphasis on the knee.

Introduction: Complex regional pain syndrome type I is an agreed umbrella term used to replace other terms such as sympathetic-reflex dystrophy. This condition is a true neurological disease, which is completely different to the type II variation; both the central and the peripheral nervous systems are involved, the clinical presentation varies from patient to patient and it is diagnosed by means of a series of imprecise agreed criteria that are subject to debate., Case Reports: We report two cases of atypical presentation, both with peculiarities that have not previously appeared in the literature, in which a diagnostic suspicion and proper management favoured the patients' recovery. It is shown how treatment, especially in more severe cases, requires an early, tailored, multidisciplinary approach based on the patient's signs and symptoms as manifestations of the underlying physiopathogenic mechanisms., Conclusions: We review currently held knowledge about the pathophysiology of the condition and the therapeutic management that is employed, including the role played by the neurologist, who must work in cooperation with other health care professionals to reach a diagnosis, dissect the physiopathogenic mechanisms at play and plan the treatment of what we hold to be a neurological disease. Future work will need to be aimed at determining the risk factors, explaining the pain mechanisms involved, drawing up a better definition of the different clinical forms and at developing complementary tests, as well as better criteria for diagnosing the condition and enhanced therapeutic algorithms.

Background: The complex regional pain syndrome (CRPS) and fibromyalgia (FM) are chronic pain syndromes occurring in highly stressed individuals. Despite the known connection between the nervous system and immune cells, information on distribution of lymphocyte subsets under stress and pain conditions is limited., Methods: We performed a comparative study in 15 patients with CRPS type I, 22 patients with FM and 37 age- and sex-matched healthy controls and investigated the influence of pain and stress on lymphocyte number, subpopulations and the Th1/Th2 cytokine ratio in T lymphocytes., Results: Lymphocyte numbers did not differ between groups. Quantitative analyses of lymphocyte subpopulations showed a significant reduction of cytotoxic CD8+ lymphocytes in both CRPS (p < 0.01) and FM (p < 0.05) patients as compared with healthy controls. Additionally, CRPS patients were characterized by a lower percentage of IL-2-producing T cell subpopulations reflecting a diminished Th1 response in contrast to no changes in the Th2 cytokine profile., Conclusions: Future studies are warranted to answer whether such immunological changes play a pathogenetic role in CRPS and FM or merely reflect the consequences of a pain-induced neurohumoral stress response, and whether they contribute to immunosuppression in stressed chronic pain patients., ((c) 2008 S. Karger AG, Basel)

The effects of short-term dependency and immobility on skin temperature were assessed in two experiments. In the first study, ten volunteers hung their left arm dependent and motionless while using their right hand for light office work. After 30 minutes, the dependent left hands were cooler (mean=0.9 degrees C: 95% CI=0.7-1.1) than the active right hands and exhibited a deeper colour with significantly increased green intensity (P=0.03) as assessed on digital images. In the second study, seven volunteers submerged both their hands in water at 15 degrees C for 15 minutes. The left arm was then left dependent and motionless while the right hand was placed on the table and its fingers were flexed and extended every 10 seconds. The left hands remained cooler than the right and were a median of 3.6 (range 2.9-4.5) degrees C cooler after 60 minutes. These studies demonstrate that skin temperature and colour differences between the hands, which are sometimes used to support the diagnosis of CRPS-1, can be produced and maintained by short-term immobility and dependency of the hand.

Background: In complex regional pain syndrome type 1 (CRPS1) pro-inflammatory mediators and vascular changes play an important role in the sustained development and outcome of the disease. The aim of this study was to determine the involvement of vasoactive substances endothelin-1 (ET-1) and nitric oxide (NO) during early chronic CRPS1., Methods: Included were 29 patients with CRPS 1 who were diagnosed during the acute stage of their disease and observed during follow-up visits. Disease activity and impairment were determined and artificial suction blisters were made on the CRPS1 and the contralateral extremities for measurements of IL-6, TNF-alpha, ET-1 and nitrate/nitrite (NOx)., Results: The levels of IL-6, TNF-alpha and ET-1 in blister fluid in the CRPS1 extremity versus the contralateral extremity were significantly increased and correlated with each other, whereas NOx levels were decreased., Conclusion: The NOx/ET-1 ratio appears to be disturbed in the intermediate stage of CRPS, resulting in vasoconstriction and consequently in a diminished tissue blood distribution.

Objective: To explore the mechanism of post-stroke reflex sympathetic dystrophy (RSD) patients electromyographic abnormality and confirm its clinical value., Methods: Fifty patients with first-onset stroke, aged 33 - 78, including 30 with RSD and 20 without RSD, underwent needle electromyography (EMG) to test the nerve conduction velocity (NCV) and sensory nerve conduction velocity (SCV) of bilateral median nerves, and the number and position of spontaneous EMG activity of bilateral short abductor muscles of thumb and abductor muscles little finger., Results: The median nerve compound muscle action potential (CMAP) amplitude of the affected upper extremities of the RSD group was 8.6 mV +/- 2.9 mV, significantly lower than that of the non-RSD group (13.2 mV +/- 4.6 mV, P < 0.01). The incidence of spontaneous electrical potential of the RSD group was 100%; significantly higher than hat of the non-RSD group (65%, P < 0.001). The quantity of spontaneous EMG activity on the short abductor muscles of thumb and abductor muscles little finger was increased in the RSD group (P < 0.01). The motor nerve conduction velocity and electrophysiological presentation of sensory nerve of these 2 groups were all normal and without significant differences between them., Conclusion: Partial axonal degeneration occurs on the distal motor never fibers of the affected upper extremity of the RSD patients, which may be related to subsequent peripheral nerve injury after central nerve system impairment.

Background: Complex Regional Pain Syndrome type 1 (CRPS 1) is a potentially incapacitating complication in which pain seems to be the most disabling factor. We performed a late follow up study of a well-defined CRPS 1 population more than eight years after diagnosis. The relationships between early and late impairments were studied with a view to outcome prediction and to investigate possible differences in long-term impairments according to initial CRPS 1 subdiagnosis (i.e. "warm" or "cold", diagnosed according to skin temperature measured via infrared thermometer)., Methods: We again measured patients using the Impairment Level SumScore (ISS) (T8). These data were compared with earlier ISS measurements at CRPS diagnosis (T0) and after one year's treatment (T1). Correlations were determined between these measures., Results: Forty-five patients participated in the present study. Total median ISS improved by 55% (statistically/clinically significant) after one year's treatment (T1), and worsened (non-significantly) by 14% from T1 to T8 - without differences according to original subdiagnosis. ISS correlations were stronger for T1 vs. T8 than for T0 vs. T1 or T0 vs. T8, being strongest for the ISS factors related to pain., Conclusions: Considerable impairments, as measured by ISS, are still present over eight years after first CRPS 1 diagnosis. These do not greatly change between one and eight years post-diagnosis. ISS outcomes are similar for "cold" and "warm" CRPS 1 diagnostic subgroups. Component ISS scores associated with pain appear to possess greatest predictive power.

Introduction: The purpose of this study was to examine the efficacy of stellate ganglion blockade (SGB) in patients with complex regional pain syndromes (CRPS I) of their hands., Methods: After IRB approval and patient informed consent, 25 subjects, with a clinical diagnosis of CRPS I of one hand as defined by the International Association for the Study of Pain (IASP) criteria, had three SGB's performed at weekly intervals. Laser Doppler fluxmetric hand perfusion studies were performed on the normal and CRPS I hands pre- and post-SGB therapy. No patient was included in this study if they used tobacco products or any medication or substance that could affect sympathetic function. The appropriate parametric and nonparametric data analyses were performed and a p value <0.05 was used to reject the null hypothesis., Results: Symptom onset of CRPS I until the initiation of SGB therapy ranged between 3 to 34 weeks. Following the SGB series, patient pain relief was as follows: group I, 10/25 (40%) had complete symptom relief; group II, 9/25 (36%) had partial relief and group III, 6/25 (24%) had no relief. The duration of symptoms until SGB therapy was: group I, 4.6 +/- 1.8 weeks, group II, 11.9 +/- 1.6 weeks and group III, 35.8 +/- 27 weeks. Compared with the normal control hand, the skin perfusion in the CRPS I affected hand was greater in group I and decreased in groups II and III., Discussion: The results of our study demonstrate that an inverse relationship exists between hand perfusion and the duration of symptoms of CRPS I. On the other hand, a positive correlation exists between SGB efficacy and how soon SGB therapy is initiated. A duration of symptoms greater than 16 weeks before the initial SGB and/or a decrease in skin perfusion of 22% between the normal and affected hands adversely affects the efficacy of SGB therapy.

Ambulatory accelerometry is a technique that allows objective measurement of aspects of everyday human behavior. The aim of our research has been to develop, validate, and apply this technique, which recently resulted in an upper limb activity monitor (ULAM). The ULAM consists of body-mounted acceleration sensors connected to a waist-worn data recorder and allows valid and objective assessment of activity of both upper limbs during performance of also automatically detected mobility-related activities: lying, sitting, standing, walking, cycling, and general movement. The ULAM can be used to determine (limitations of) upper limb activity and mobility in freely moving subjects with upper limb disorders. This article provides a detailed description of its characteristics, summarizes the results of a feasibility study and four application studies in subjects having upper limb complex regional pain syndrome, discusses the most important practical, technical, and methodological issues that were encountered, and describes current and future research projects related to measuring (limitations of) upper limb activity.

As CRPS I frequently develops after tissue trauma, we proposed that an exaggerated inflammatory response to tissue trauma may underlie CRPS I. Therefore, we studied the vascular inflammatory, nociceptive and apoptotic sequelae of (i) soft tissue trauma and (ii) exaggerated soft tissue trauma in comparison to those of (iii) sciatic nerve chronic constriction injury, modeling CRPS II. Standardized soft tissue trauma (TR) was induced by means of a controlled impact injury technique in the hind limb of pentobarbital-anesthetized rats. Additional animals received soft tissue trauma and femoral arterial infusion of mediator-enriched supernatant achieved by homogenization and centrifugation of traumatized muscle tissue in order to provoke an exaggerated trauma response (ETR). Infusion of supernatant of non-traumatized muscle served as control intervention (STR, sham trauma response). Neuropathy was induced by chronic constriction injury of the sciatic nerve (CCI). Untreated animals served as controls (CO). Detailed nociceptive testing showed temporarily decreased mechanical pain thresholds in ETR animals that resolved within 14 days, while TR and STR animals, i.e. those with singular limb trauma, and controls remained free of pain. Neither cold- nor heat-evoked allodynia developed in post-traumatic animals, whereas CCI animals presented the well-known pattern of ongoing neuropathic pain. Using high-resolution in vivo multifluorescence microscopy, muscle tissue of traumatized animals revealed an enhanced inflammatory response that was found most pronounced in ETR animals. CCI of the sciatic nerve was not accompanied by tissue inflammation; however, induced myocyte apoptosis. Collectively, these data indicate that exaggeration of trauma response induces signs and symptoms of acute CRPS I. Pain perception displays differences to that in CRPS II. Apoptosis turns out to be a distinctive marker for CRPS, warranting further evaluation in clinical studies.

This study was performed to determine the utility of sympathetic skin response (SSR) in evaluating the sympathetic function and to follow up the effects of sympathetic blockade in reflex sympathetic dystrophy (RSD). Thirty patients having RSD with upper extremity involvement were randomly divided into two groups. Besides medical therapy and exercise, physical therapy agents were applied to both the groups. In addition to this treatment protocol, stellar ganglion blockade was done by diadynamic current in Group II. The normal sides of the patients were used for the control group. SSRs were measured in all the patients before and after the therapy. The amplitude was found to be increased and the latency was found to be decreased in the affected side in both the groups before the therapy. After the therapy, the amplitude was decreased and latency was increased in both the groups. But, the differences in amplitude (P = 0.001) and latency (P = 0.002) before and after the therapy were significantly higher in Group II. (Before the treatment, SSRs were significantly different between the normal and the affected sides in both the groups. The observed change in SSRs after the treatment was higher in Group II.) It was concluded that, SSR can be a useful and noninvasive method in diagnosing the sympathetic dysfunction in RSD and can be used for evaluating the response to sympathetic blockade and other treatment modalities.

Background: Complex Regional Pain Syndrome type 1 (CRPS1) is a clinical diagnosis based on criteria describing symptoms of the disease. The main aim of the present study was to compare the sensitivity and specificity of calculation methods used to assess thermographic images (infrared imaging) obtained during temperature provocation. The secondary objective was to obtain information about the involvement of the sympathetic system in CRPS1., Methods: We studied 12 patients in whom CRPS1 was diagnosed according to the criteria of Bruehl. High and low whole body cooling and warming induced and reduced sympathetic vasoconstrictor activity. The degree of vasoconstrictor activity in both hands was monitored using a videothermograph. The sensitivity and specificity of the calculation methods used to assess the thermographic images were calculated., Results: The temperature difference between the hands in the CRPS patients increases significantly when the sympathetic system is provoked. At both the maximum and minimum vasoconstriction no significant differences were found in fingertip temperatures between both hands., Conclusion: The majority of CRPS1 patients do not show maximal obtainable temperature differences between the involved and contralateral extremity at room temperature (static measurement). During cold and warm temperature challenges this temperature difference increases significantly. As a result a higher sensitivity and specificity could be achieved in the diagnosis of CRPS1. These findings suggest that the sympathetic efferent system is involved in CRPS1.

Algodystrophy (AD) of the lower limbs during pregnancy is rare and probably underdiagnosed. The physiopathologic mechanisms remain under discussion and seem multiple and complex. This report describes a retrospective survey of 6 patients seen between 1993 and 2004 who had algodystrophy of the lower limbs during pregnancy. Comparing the clinical, radiological and evolutionary results to the literature allows for identifying the main features of AD during pregnancy: disease progression during the second or third trimester, preferential localization of the left hip associated or not with other lower limb joint involvement and decalcification as seen on radiography. Magnetic resonance imaging (MRI), which is accurate, specific and non-invasive, is currently the exam of choice in early and differential diagnosis. The evolution is favourable in a few months, with general recovery without disability.

Previous studies have suggested that sympathetic sprouting in the periphery may contribute to the development and persistence of sympathetically maintained pain in animal models of neuropathic pain. In the present study, we examined changes in the cutaneous innervation in rats with a chronic constriction injury to the sciatic nerve. At several periods postinjury, hind paw skin was harvested and processed by using a monoclonal antibody against dopamine-beta-hydroxylase to detect sympathetic fibers and a polyclonal antibody against calcitonin gene-related peptide to identify peptidergic sensory fibers. We observed migration and branching of sympathetic fibers into the upper dermis of the hind paw skin, where they were normally absent. This migration was first detected at 2 weeks, peaked at 4-6 weeks, and lasted for at least 20 weeks postlesion. At 8 weeks postlesion, there was a dramatic increase in the density of peptidergic fibers in the upper dermis. Quantification revealed that densities of peptidergic fibers 8 weeks postlesion were significantly above levels in sham animals. The ectopic sympathetic fibers did not innervate blood vessels but formed a novel association and wrapped around sprouted peptidergic nociceptive fibers. Our data show a long-term sympathetic and sensory innervation change in the rat hind paw skin after the chronic constriction injury. This novel fiber arrangement after nerve lesion may play an important role in the development and persistence of sympathetically maintained neuropathic pain after partial nerve lesions.

In this paper the chronicity of pain in non-specific pain syndromes is discussed. Experts in the study of pain with several professional backgrounds in rehabilitation are the authors of this paper. Clinical experience and literature form the basis of the paper. Non-specific low back pain and Complex Regional Pain Syndrome type I (CRPS-I) are discussed in the light of chronic pain. Many definitions of chronic pain exist. Yellow flags are important factors to identify possible chronic pain. In the acute phase of a non-specific pain complaint one should try to identify possible psychosocial inciting risk factors. Behavioural and cognitive treatment seems to be effective for chronic pain patients.

Objectives: Complex regional pain syndromes (CRPS) can be relieved by sympathetic blockade. Different sympathetic efferent output channels innervate distinct effector organs (ie, cutaneous vasoconstrictor, muscle vasoconstrictor. and sudomotor neurons, as well as neurons innervating deep somatic tissues like bone, joints, and tendons). The aim of the present study was to elucidate in CRPS patients the sympathetically maintained pain (SMP) component that exclusively depends on cutaneous sympathetic activity compared with the SMP depending on the sympathetic innervation of deep somatic tissues., Methods: The sympathetic outflow to the painful skin was modulated selectively in awake humans. High and low cutaneous vasoconstrictor activity was produced in 12 CRPS type 1 patients by whole-body cooling and warming (thermal suit). Spontaneous pain was quantified during high and low cutaneous vasoconstrictor activity. By comparing the cutaneous SMP component with the change in pain that was achieved by modulation of the entire sympathetic outflow (sympathetic ganglion block), the SMP component originating in deep somatic structures was estimated., Results: The relief of spontaneous pain after sympathetic blockade was more pronounced than changes in spontaneous pain that could be induced by selective sympathetic cutaneous modulation. The entire SMP component (cutaneous and deep) changes considerably over time. It is most prominent in the acute stages of CRPS., Conclusions: Sympathetic afferent coupling takes place in the skin and in the deep somatic tissues, but especially in the acute stages of CRPS, the pain component that is influenced by the sympathetic innervation of deep somatic structures is more important than the cutaneous activation. The entire sympathetic maintained pain component is not constant in the course of the disease but decreases over time.

This feature presents information for patients in a question and answer format. It is written to simulate actual questions that many pain patients ask and to provide answers in a context and language that most pain patients will comprehend. Issues addressed in this issue are the role of the pain psychologist, trigger point injections, and reflex sympathetic dystrophy.

Objective: The motor cortical representation in patients with complex regional pain syndrome type I (CRPS I) was determined under the assumption that the motor cortex undergoes representational adaptations in the course of CRPS., Methods: A total of 14 patients with CRPS I and a group of healthy subjects without any known neurological symptoms participated in the study. The motor cortical representation, i.e. the size of representation (cm2), motor-evoked potentials (MEP), the calculated volume (cm2 mV), and the center of gravity (CoG) were measured by transcranial magnetic stimulation. Recordings were made of the long extensor muscles of the forearm of the affected and unaffected hand., Results: Analyses of the results revealed a significant asymmetry between the two hemispheres: the motor cortical representation corresponding to the unaffected hand was significantly larger. While the CoG data did not differ statistically between the two hemispheres, the CoG coordinates corresponding to the affected hand indicated a larger variability., Conclusions: The presence of pain and other CRPS symptoms may induce lasting changes in motor cortical plasticity, as it also does in the sensory cortex., Significance: This could be of importance in rehabilitative strategies for the sensory motor system in CRPS I patients.

Background: An upper limb-activity monitor (ULAM) has been developed to determine activity limitations in complex regional pain syndrome type 1 (CRPS1). The ULAM is based on 24h ambulatory monitoring of body segment accelerations and enables valid and objective quantification of mobility and upper limb activity in transversal studies., Aims: To explore upper limb activity over time in acute upper limb CRPS1 as measured with the ULAM in a longitudinal study, and to compare this to time courses of other outcome measures for activity limitations and impairments., Methods: Four subjects were measured four times during a treatment protocol. Several ULAM outcome measures related to upper limb usage and mobility, three questionnaires (RASQ, DASH, RAND36), and six impairment outcome indicators (VAS-momentary pain, VAS-pain resulting from effort, volume, temperature, active range of motion, strength) were used., Results: Objectively measured upper limb activity frequently improved; improvements of >5% were found for 63% of the ULAM outcome measures at final assessment. The ULAM outcome measures had a time course more similar to the body-part and CRPS1 specific questionnaire RASQ than the other questionnaires. The time course of impaired temperature was most often in accordance with the ULAM, and both VAS scores showed least accordance., Conclusions: Clear changes in upper limb activity over time were frequently found as objectively measured with the ULAM, and relationships among the time courses of the ULAM and other outcome measures were largely explainable. The ULAM can validly assess upper limb activity over time in CRPS1, but between-measurement variability needs careful consideration.

A 10-year-old boy presented with pain and swelling of short duration in his right hand. A traumatic cause was ruled out. He had clinical features suggestive of sympathetic overactivity in the form of hyperesthesia, edema and dry skin. The results of a few baseline investigations performed at this stage were all within normal limits. A provisional diagnosis of sympathetically maintained pain syndrome (SMPS) was made. He responded dramatically to a stellate ganglion block. His symptoms recurred within 3 weeks, though now in the lower limb. Again the clinical features were consistent with SMPS. His response to a lumbar sympathetic block was again dramatic. This case illustrates that the diagnosis of SMPS is essentially clinical and that prompt diagnosis and treatment with sympathetic blocks can be very rewarding.

Reflex sympathetic dystrophy (RSD) is a relative common sequel after hemiplegia. The diagnosis of RSD in hemiplegic patients presents difficult clinical problems, as the symptoms and signs of RSD are not specific and RSD may be due to reasons other than hemiplegia. Bone scintigraphy has been routinely used for the diagnosis of RSD; however, the optimal acquisition protocols, diagnostic patterns and the utility of quantitation are controversial. This prospective study was conducted to demonstrate the higher predictive value of an additional late blood pool image to the three-phase bone scan compared to the regular three-phase bone scans in RSD patients associated with hemiplegia. Thirty-four RSD patients were enrolled into the study. Bone scans according to the new protocol were obtained for all patients. Those patients with either negative or positive bone scans with no evidence of RSD were followed for 6 months. The patients had positive bone scan findings and were symptomatic at the time of the study. Of these, seven patients (58.3%) subsequently became symptomatic and five patients (41.7%) remained asymptomatic at 6 months. None of the patients with negative bone scans had symptoms of RSD on presentation except one case. We conclude that the addition of a late blood pool image increases the predictive value and has an impact on initiating early treatment in asymptomatic patients.

Complex regional pain syndrome (CRPS) types I and II are neuropathic pain disorders that develop as an exaggerated response to a traumatic lesion or nerve damage, that generally affects the extremities, or as the consequence of a distant process such as a stroke, spinal lesion or myocardial infarction. It rarely appears without an apparent cause. CRPS of upper limbs after stroke is frequently today called shoulder-hand syndrome (SHS). The onset and severity of SHS appears to be related with the aetiology of the stroke, the severity and recovery of motor deficit, spasticity and sensory disturbances. Another important aetiological factor is glenohumeral subluxation. The physiopathology of the disease is still not known. In CRPS, there is an exaggerated inflammatory response and some chemical mediators have been identified and are present in the inflammatory soup around the primary afferent fibres that, through different processes, can induce hyper-excitability of the afferent fibres (peripheral sensitization). It is hypothesized that a localized neurogenic inflammation is at the basis of oedema, vasodilation and hyperhidrosis that are present in the initial phases of CRPS. The repeated discharge of the C fibres causes an increased medullary excitability (central sensitization). Another important factor is the reorganisation of the central nervous system, and in particular this appears to affect the primary somatosensory cortex. The central role of the sympathetic nerve is presently in doubt. However, it is thought that a sub-group of CRPS patients exists in whom a predominant factor is the hyper-activity of the sympathetic nervous system, and that it responds positively to sympathetic block. Diagnosis is clinical and there are no specific tests, nor pathognomic symptoms to identify this disease with certainty. Diagnosis of CRPS after stroke appears more complex than in other pathological situations: the paretic upper arm frequently appears painful, oedematose, with altered heat and tactile sensations and slightly dystrophic skin within a non-use syndrome. Some investigations can aid differential diagnosis with other diseases. Treatment may be non-pharmacological, pharmacological, with psychotherapy, regional anaesthesia, neuromodulation and sympathectomy. In any case there is little evidence that supports the efficacy of the interventions normally used to treat or prevent CRPS-SHS. The key to effective treatment undoubtedly lies in a an expert multidisciplinary team that is co-ordinated and motivated and that treats the disorder with individualised therapy.

Objective: To improve a self-administered questionnaire that includes 42 dichotomous items and measures activity limitations in rising and sitting down (R&S) in patients with lower-extremity disorders who live at home., Design: Cross-sectional study., Setting: Outpatient clinics of secondary and tertiary care centers., Participants: Patients (N=759; 47% men; mean age +/- standard deviation, 60.7+/-15.2y) living at home, with lower-extremity disorders resulting from stroke, poliomyelitis, osteoarthritis, amputation, and complex regional pain syndrome type I., Interventions: Not applicable., Main Outcome Measures: (1) Unidimensionality, indicating that items assess only a single construct; (2) fit with the one-parameter logistic model (OPLM), yielding information about patient and item location parameters; (3) intratest reliability, indicating consistency of patients' item scores; and (4) content validity, indicating completeness with which the items cover the important aspects of the construct that they are attempting to represent., Results: Thirty-nine of 42 items: (1) loaded on 1 component (variance explained, 59%; item component loadings, >or=.51), (2) showed good fit with the OPLM (P=.15), (3) had a good intratest reliability (Cronbach alpha=.96), and (4) had a good content validity (all important aspects represented)., Conclusions: A unidimensional scale that fits with the OPLM has been developed for measuring activity limitations in R&S in patients with lower-extremity disorders who live at home.

Unlabelled: We describe a 12-year-old patient with severe, protracted complex regional pain syndrome type I. His pain did not respond to gabapentin, amitriptyline, physical therapy, opioids, or nonsteroidal drugs. Sympathetic or regional block was not attempted because of persistent bacteremia and severe local sepsis. His pain responded dramatically to the addition of oxcarbazepine, with rapid improvement in his symptoms and functional status. We suggest that oxcarbazepine might be a useful adjunct in the treatment of gabapentin-resistant complex regional pain syndrome type I in children and should be considered., Perspective: Oxcarbazepine's antinociceptive effect is mediated via sodium channel inhibition in neuropathic models and by inhibition of substance P and prostaglandins in anti-inflammatory models. The efficacy of oxcarbazepine in this patient might be attributable to these mechanisms or possibly to synergism with either gabapentin or the anti-inflammatory effects produced by amitriptyline.

Complex regional pain syndrome type I (CRPS-I) is a complex disorder characterised by pain, autonomic dysfunction, and decreased range of motion. The syndrome was believed as a well-recognized disorder in adults but, less commonly recognized in children. CRPS-I after vaccination has been rarely reported. We reported an 11-year-old young girl with CRPS-I due to rubella vaccine.

People with complex regional pain syndrome type 1 (CRPS1) watched a reflected image of their unaffected limb being touched and felt pain or paresthesia at the corresponding site on the affected limb. The authors suggest that allodynia and paresthesia can be mediated by the brain and that dysynchiria has implications for the understanding and management of CRPS1.

Reflex sympathetic dystrophy (RSD) may be a misdiagnosis or at least not descriptive enough in patients with atypical hand posture and atypical edema. Seven patients with the previous diagnosis of RSD were investigated further because of inconsistent clinical picture with the underlying pathology and bizarre course of the disease. Four patients had clenched fist and three had factitious edema. These seven patients underwent psychological examination, and MMPI was applied to all. In two of these no psychological disorder was obtained according to DSM-IV. One patient could not adapt to MMPI. In two anxiety disorders, in one depression, and in one patient conversion disorder was diagnosed. We suggest that these patients are not motivated enough to improve their conditions and expectations of such patients may show some differences depending on the environment.

Background and Purpose: In this prospective trial we assessed the long-term effect of spinal cord stimulation (SCS) on the improvement of functional status in complex regional pain syndrome type I (CRPS I)., Methods: A prerequisite for eligibility to SCS treatment was the responsiveness of patients to sympathetic nerve block. In 29 patients with chronic sympathetically maintained CRPS I, the efficacy of SCS on deep pain, allodynia and functional disability was determined. Pain intensity was estimated during SCS free intervals of 45 min (inactivation test) every 3 months and compared with that under SCS treatment., Results: On SCS treatment, both deep pain and allodynia could be permanently reduced from 10 to 0-2 on a 10 cm visual analogue scale (VAS) (p<0.01). During the inactivation tests, reoccurrence of pain up to 8 VAS (quartiles 6-8) was measured. Considerable impairments in daily living activities, objectified by the pain disability index, were also restored (p<0.01). After a follow-up period of 35.6+/-21 months, 12 of 16 patients with affected upper limb showed significant increase of the fist grip strength from 0 to 0.35 (quartiles 0.1-0.5) kg compared with 0.9 (quartiles 0.7-1.1) kg on the unaffected side (p<0.01). Eight of ten patients with lower limb disability resumed walking without crutches. Previous pain medication could be significantly reduced (p<0.01)., Conclusions: As a result of permanent pain relief under long-term SCS combined with physiotherapy, the functional status and the quality of life could be significantly improved in sympathetically maintained CRPS I.

Objectives: We tested whether repetitive magnetic stimulation (rMS) induces an afferent input to the spinocerebral tract in patients with complex regional pain syndrome (CRPS)., Methods: Cortical and spinal motor evoked potentials (cMEP and sMEP), as well as the contra- and ipsilateral silent period (cCSP and iCSP), were recorded in patients with CRPS type I before and after conditioning rMS, applied at cervical nerve roots innervating affected muscles. Patients were compared with a group of healthy subjects., Results: In the group of patients we found that cMEP amplitudes were always significantly smaller for both hemispheres. In the group of healthy subjects we found a significant prolongation of the cCSP and iCSP after rMS. This was absent in patients. SMEP were always unchanged in both groups., Discussion: This led us to the explanation that the afferent input to the motor cortical system in CRPS patients is less effective than in healthy subjects.

Tonic dystonia is an underrecognized complication of reflex sympathetic dystrophy syndrome (RSDS) characterized by an increase in muscle tone at the site of injury. Case-reports.- We describe five cases of tonic dystonia complicating RSDS of the lower extremity. There were four women and one man, with a mean age of 52 years. In addition to the typical features of RSDS, the patients had fixed equinovarus of the foot with hyperextension or hyperflexion of the great toe. In two patients, examination after spinal anesthesia showed that the deformity was reducible. Spontaneous resolution of the dystonia occurred in one patient. Another patient failed to experience meaningful improvement after a motor block followed by botulinic toxin injections. In two patients, the same treatment was followed by a slight improvement. Treatment options are still being evaluated in the last patient. Discussion.- Tonic dystonia is an underrecognized complication of RSDS that often develops after a minor injury yet causes prolonged pain and disability. Spread of the dystonia to other sites is not infrequent. The underlying mechanisms remain unclear but may involve dysfunction of the central or peripheral nervous system or psychogenic factors. Suggested treatments include motor block, intrathecal baclofen, sympathetic block, and sympathectomy. However, none of these treatments has been proved effective. Conclusion.- The five cases described here provide useful information on RSDS-associated tonic dystonia, a condition that runs a protracted course and remains difficult to manage.

Objective: To assess the validity of physician's judgements of symptoms associated with Complex Regional Pain Syndrome Type 1., Methods: The validity of physicians' judgments was assessed using measurements with regard to presence and severity of pain, temperature and volume asymmetry, and reduction in active range of motion in 66 Complex Regional Pain Syndrome Type 1 outpatients. Measurements were performed using Visual Analog Scales and McGill (number of words chosen total) for pain, infrared thermography for temperature differences, water displacement volumeters for volume differences, and hand-held goniometers for active range of motion. Physicians were blind to the outcomes of the measurements., Results: In general, physicians were capable of determining presence or absence of measured symptoms and indicate the direction of the symptom asymmetry. Establishing presence of temperature and volume asymmetries was, however, inadequate. Poor to moderate correspondence was found for the severity of individual symptoms between physicians' judgments and measurements. For the total number of assessments, correlation coefficients ranged from 0.39 for Volume to 0.68 for Pain. In general, lower correlations and percentages of association for Volume and Temperature were found. Monitoring changes between consecutive patient assessments showed poor correspondence between both assessment methods, with correlation coefficients ranging from 0.25 for Volume to 0.37 for Pain., Conclusions: We conclude that establishing the presence of Complex Regional Pain Syndrome Type 1 symptoms, except for temperature and volume asymmetries, and monitoring of disease progression based on these symptoms can be performed by clinical judgment. The severity of the individual symptoms evaluated in this study should be measured with reliable and valid measurement instruments.

Objective: To determine the place of a novel Upper Limb Activity Monitor in the field of instruments measuring functioning and health in upper limb complex regional pain syndrome type I, by exploring the correlation between the Upper Limb Activity Monitor and 4 questionnaires., Method: Subjects (n = 30) were measured at home and correlations were calculated between the Upper Limb Activity Monitor and 4 questionnaires; Sickness Impact Profile, RAND-36 Health Survey, Disabilities of Arm Shoulder Hand Questionnaire and Radboud Skills Questionnaire., Results: Of the inter-questionnaire correlations 83% were significant, whereas 46% of the correlations between the Upper Limb Activity Monitor and the questionnaires were significant. The number and strength of the correlations between the Upper Limb Activity Monitor and questionnaires was dependent on the degree to which similar aspects of functioning were measured., Conclusion: The Upper Limb Activity Monitor has some correlation with other instruments related to functioning and health, but generally it does not measure the same areas.

Patients with complex regional pain syndrome (CRPS) and intractable pain showed a shrinkage of cortical maps on primary (SI) and secondary somatosensory cortex (SII) contralateral to the affected limb. This was paralleled by an impairment of the two-point discrimination thresholds. Behavioral treatment over 1 to 6 months consisting of graded sensorimotor retuning led to a persistent decrease in pain intensity, which was accompanied by a restoration of the impaired tactile discrimination and regaining of cortical map size in contralateral SI and SII. This suggests that the reversal of tactile impairment and cortical reorganization in CRPS is associated with a decrease in pain.

The validity with respect to presence or absence of CRPS I according to Veldman's criteria was assessed for measured pain, temperature, volume differences and limitations in range of motion. Evaluated were 155 assessments of 66 outpatients, initially diagnosed with CRPS I, but many of them not so on follow up visits. Pain was measured with VAS and McGill, temperature by infrared thermometry, volume differences by water displacement volumeters and limitations in range of motion by universal goniometers. Sensitivity, specificity, positive and negative predictive value of the measurement instruments at different cut-off points was calculated. Combined symptom scores were evaluated in a similar fashion. High sensitivity was found for the VAS, McGill, and range of motion. The specificity was overall lower, but highest values were obtained for volume differences. The positive predictive value was good for all measurement instruments. Negative predictive value was lower, especially for measurement of temperature and volume asymmetries. If sensitivity and specificity are equally important, VAS>3 cm, McGill>6 words, temperature difference>or=0.4 degrees C, volume difference>6.5% and ROM limitation>15% provide the best results. Using these cut off values, the highest value of sensitivity and of sensitivity and specificity combined, was found for a combination of VAS, McGill and ROM. The highest value of specificity was found for different combinations of 3, 4 and 5 instruments, all containing the VAS. We conclude that the measured pain, temperature, volume and range of motion can be used as diagnostic indicators for establishing presence or absence of CRPS I.

Objective: Sympathetic testing was carried out in patients in the acute phase of "complex regional pain syndrome type I" (CRPS I) shortly after trauma to the upper limb. Repeated measurements were used to detect changes in peripheral sympathetic function during the course of the disease., Material and Methods: In a busy trauma center, 10 consecutive patients who developed CRPS I following trauma or surgery of the upper limb were diagnosed according to the 1999 modified IASP diagnostic criteria for CRPS I. Clinical signs and symptoms and bilateral hand temperature (infrared thermometry) were recorded. Vasoconstrictor response to sympathetic provocation (inspiratory gasp, contralateral cooling) at the tip of the middle finger of both hands was measured employing laser Doppler flowmetry (LDF). Sympathetic reaction was quantified by the magnitude of blood flow decrease after provocation (SRF parameter)., Results: The diagnosis CRPS I could be established 63 days (46-72 days) post-injury. The mean follow-up time after diagnosis was 83+/-15 days. Pain measured by a visual analog scale (VAS 0-10) showed an average of 5.0+/-2.0 at the time of diagnosis and decreased to 1.7+/-1.9 at the last examination. Edema and active range of motion improved substantially during the follow-up period. On the ipsilateral hand marked sympathetic dysfunction was seen early after the onset of CRPS I (mean SRF parameter: 0.14+/-0.01), slowly returning to normal sympathetic reaction three months after the onset of symptoms (mean SRF parameter: 0.42+/-0.21). Diminished sympathetic function was seen even on the contralateral hand., Conclusions: Sympathetic dysfunction is regularly seen at the onset of CRPS I and normalizes during the course of the disease. This temporary phenomenon suggests a posttraumatic sympathetic deficit playing a decisive role in the genesis of CRPS I.

Functional alterations in noxious, sensory and motor circuits within the central nervous system may play an important role in the pathophysiology of complex regional pain syndrome (CRPS). The aim of the present study was to search for further evidence of hyperexcitability in the hemisphere contralateral to the affected limb in patients with CRPS by employing both psychophysical and transcranial magnetic stimulation (TMS) methods. Twelve patients with CRPS type I, confined to the distal part of a limb (six in an upper-limb and six in a lower-limb), were enrolled in the study. The quantitative thermal, mechanical and 'wind-up' like pain testing was performed at the most painful site in the affected limb and in the ipsilateral limb. Results were then compared to those found at mirror sites in the contralateral limbs. TMS was used to assess the inter-hemispheric difference in parameters of corticospinal excitability, intracortical inhibition, and intracortical facilitation. The quantitative thermal and mechanical testing showed significant differences in cold, heat and mechanical pain thresholds, as well as in the first and last 'wind-up' stimuli between the affected and the contralateral limbs of the CRPS patients. No significant differences between the ipsilateral unaffected limbs and their contralateral pair limbs were found. A significant reduction in the short intracortical inhibition associated with a significant increase of the I-wave facilitation was found in the hemisphere contralateral to the affected side in the upper-limb CRPS group. No significant inter-hemispheric asymmetry between the affected and the non-affected sides was revealed in the lower-limb CRPS group. Taken together, these results suggest that in patients with well-localized CRPS, there is evidence for sensory and motor CNS hyperexcitability, though it seems to involve only corresponding regions within the CNS rather than the entire hemisphere.

Altered pain appreciation and autonomic function are hallmarks of Cardiac syndrome X, Irritable bowel syndrome and Reflex sympathetic dystrophy. Both pain appreciation and autonomic function are controlled by the lateral medulla. This hypothesis proposes that lateral medullary ischaemia at a microvascular level is responsible for these syndromes and could also be linked to other conditions where autonomic dysfunction is a major feature such as late-onset asthma, type 2 diabetes and essential hypertension. Autonomic function is controlled by the nucleus tractus solitarius, which acts as the main viscero-afferent nucleus in the brain stem regulating vagal tone. It is particularly susceptible to ischaemia since it is highly metabolically active and lies in a medullary arterial watershed zone. The anatomical route of the vertebral artery through cervical vertebra makes it vulnerable to injury from whiplash with or without any genetic predisposition to atheroma formation. This could make microvascular occlusion commonplace and a plausible explanation for the above syndromes. Ischaemia rather than infarction occurs because of the excellent collateral blood supply in the brainstem. In support of this hypothesis, a new Transcranial doppler ultrasonography arterial signal has been described called small vessel knock, the ultrasound signal of small vessel occlusion. Recent evidence has shown that ultrasound targeting of this signal in the vertebral artery improves clinical symptoms in these syndromes which supports this hypothesis. Two such cases are discussed.