Segmental reflex sympathetic dystrophy: clinical and scintigraphic criteria

Clinical and scintigraphic criteria are proposed for the diagnosis of segmental reflex sympathetic dystrophy. Eight patients met previously described clinical criteria for reflex sympathetic dystrophy with involvement limited to only a portion of the hand. The delayed phase of the three-phase radionuclide bone scan was found to be highly sensitive (100%) for this small group of patients. Consecutive bone scans (n = 127) performed during a 6-month period for a variety of upper extremity problems were reviewed, and a segmentally diffuse pattern of tracer uptake was found to be highly specific (98%) for segmental reflex sympathetic dystrophy. Recognition and documentation of a more localized form of reflex sympathetic dystrophy will allow earlier recognition and treatment, which is an important factor in a successful outcome for managing pain dysfunction disorders.     

Bisphosphonate therapy of reflex sympathetic dystrophy syndrome

Four cases of post-operative ulnar nerve mononeuropathy are reported. In all the cases a severe sensory and motor loss was strictly limited to ulnar nerve territory. The electrophysiological examination: needle examination, motor and sensory nerve conduction studies and even more somatosensory potential evoked from ulnar nerve after stimulation above elbow allowed to eliminate a lesion at the elbow and to asses the lesion at wrist, arm, axilla or plexus. Full recovery occurred once and partial recovery twice. We considered that these ulnar lesions are neuralgic amyotrophies of Parsonage and Turner according to the epidemiological, clinical, evolutive and electrophysiological data.     

Calcitonin treatment in reflex sympathetic dystrophy: a preliminary study

Reflex sympathetic dystrophy is one of the important complications effecting the rehabilitation programmes of hemiplegic patients in a negative manner by causing pain and function loss. In this study, the aim was to investigate the effects of salmon calcitonin treatment in reflex sympathetic dystrophy that develops in hemiplegia. Forty-one patients with hemiplegia resulting from cerebrovascular events and stage 1-2 reflex sympathetic dystrophy were included in the study. Salmon calcitonin, 1 x 100 IU/day intramuscularly for 4 weeks, was administered to 25 of these patients (calcitonin group) to the other 16 patients physiological saline, 1 ml/day intramuscularly for 4 weeks, was administered (control group). At the end of the fourth week of treatment the pain score of the calcitonin group was significantly lower than that of the control group. Shoulder abduction and external rotation, wrist flexion and metacarpophalangeal extension of the calcitonin group were found to be significantly better than those of the control group. In the calcitonin group the significant decrease in pain and tenderness resulted in improvement of range of motion and motor functions.     

Role of neuropeptides in pathogenesis of reflex sympathetic dystrophy

In 1993, a study was undertaken at the Hand Clinics of Loyola University Medical Center in Chicago to investigate the role of the neuropeptides in the pathogenesis of Reflex Sympathetic Dystrophy. All of the patients had recurrent or continuous pain, swelling, and stiffness of one or both extremities following either acute trauma or surgical intervention. All of the patients showed a markedly increased level of bradykinin as well as calcitonin gene-related peptide. The levels of bradykinin were four times as high as the controls. A few showed increased levels of the other neuropeptides. With these results, we agree with Veldman, Goris and others who consider Reflex Sympathetic Dystrophy to be an exaggerated regional inflammatory disorder.     

Validation of thermography in the diagnosis of reflex sympathetic dystrophy

OBJECTIVES: To examine the validity of several thermogram-derived indices of autonomic functioning in the diagnosis of reflex sympathetic dystrophy (RSD). DESIGN: A series of chronic pain patients were classified diagnostically based on thermogram results using discriminant function analysis, and validity measures (e.g., sensitivity, specificity) were used to determine the accuracy of computerized thermographic pixel analysis in discriminating RSD from other pathology. SETTING: The study was conducted at the Rush Pain Center, a multidisciplinary outpatient pain clinic. PATIENTS: A series of 46 chronic pain patients referred for suspected sympathetically mediated pain. INTERVENTIONS: All patients underwent computerized thermographic examination under a baseline condition after acclimating to a climate-controlled room, immediately after a cold challenge was applied to the contralateral uninvolved extremity (4 degrees C for 90 s) and 20 min after the cold challenge. OUTCOME MEASURES: Temperature during the three experimental periods, degree of temperature asymmetry between affected and nonaffected limbs during the three periods, response to cold challenge, and recovery following cold challenge were measured. RESULTS: Temperature asymmetry accurately discriminated between RSD and non-RSD patients, with the most accurate asymmetry measures obtained at baseline. Responses to cold challenge and actual temperature values did not discriminate between RSD and non-RSD pain patients. CONCLUSIONS: Thermography can be a useful component of RSD diagnosis. In situations where sensitivity and specificity are equally important, an asymmetry cutoff of 0.6 degree C appears optimal. If specificity (i.e., accurately ruling out non-RSD cases) is more important, a cutoff of 0.8 degree C or 1.0 degree C may be considered as well.     

Pain, negative mood, and perceived support in chronic pain patients: A daily diary study of people with reflex sympathetic dystrophy syndrome.

Chronic pain patients show substantial psychological distress, including depressed mood, anxiety, and anger. Nevertheless, the causal role of negative mood in the course of chronic pain conditions remains unclear. This study prospectively investigated the relationship between daily pain, negative mood, and social support in 109 people with reflex sympathetic dystrophy syndrome. Participants completed 28 daily diaries that included questions about pain, mood, and perceived support. Time-lagged within-subject analyses indicated that pain led to increases in depressed, anxious, and angry mood. Depressed mood, but not anxiety or anger, contributed to increases in pain. Perceived support had both main and buffering (interaction) effects on negative mood and a main effect on pain. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Reflex sympathetic dystrophy: changing concepts and taxonomy

We present a revised taxonomic system for disorders previously called reflex sympathetic dystrophy (RSD) and causalgia. The system resulted from a special consensus conference that was convened on this topic and is based upon the patient's history, presenting symptoms, and findings at the time of diagnosis. The disorders are grouped under the umbrella term CRPS: complex regional pain syndrome. This overall term, CRPS, requires the presence of regional pain and sensory changes following a noxious event. Further, the pain is associated with findings such as abnormal skin color, temperature change, abnormal sudomotor activity, or edema. The combination of these findings exceeds their expected magnitude in response to known physical damage during and following the inciting event. Two types of CRPS have been recognized: type I, corresponds to RSD and occurs without a definable nerve lesion, and type II, formerly called causalgia refers to cases where a definable nerve lesion is present. The term sympathetically maintained pain (SMP) was also evaluated and considered to be a variable phenomenon associated with a variety of disorders, including CRPS types I and II. These revised categories have been included in the 2nd edition of the IASP Classification of Chronic Pain Syndromes.     

Severe hyponatraemia: complications and treatment

To observe the incidence of complications in severely hyponatraemic hospitalized patients and relate outcome to rate of correction, all patients admitted to a tertiary referral hospital in New York City, USA or a group of hospitals in Oxford, UK with a sodium < or = 120 mmol/l were studied. Review of the notes and prospective evaluation were used to ascertain cause of hyponatraemia, method of management and outcome. There were 84 episodes in New York and 100 in Oxford, over 9.5 months and one year, respectively; 79% had chronic hyponatraemia ( > 3 days duration). During hyponatraemia, 76% of patients had clouding of consciousness with 11% in coma. Other hyponatraemic complications included long track signs (including hemiparesis) (6.0%), seizures (3.3%), hallucinations (0.5%), tremor (1.0%), intellectual impairment without clouding of consciousness (0.5%), and acute psychosis (0.5%). 4.3% died as a direct result of their electrolyte disturbance. After correction, central pontine myelinolysis (0.5%), post-correction seizures (1.0%), intellectual impairment (2.2%), tremor (0.5%), paraesthesiae (0.5%), and striatal syndrome (0.5%) were observed. Correction of hyponatraemia was started in 158 patients, and the mean maximum rate of correction in 24 h was 8.4 mmol/l (SD 5.6, range 2-42). The maximum rate of correction was higher in those who developed neurological sequelae (12.1 mmol/l/24 h vs. 8.2 mmol/l/24 h; p = 0.0125, t-test, separate variance, two-tail). Neurological sequelae were associated with faster rates of correction, and correction of chronic severe hyponatraemia should be < 10 mmol/l in 24 h.     

A case of juvenile onset ataxia with dystonia, myoclonus, sensorineural hearing loss and mental retardation

A 35-year-old female was reported who presented early onset and slowly progressive ataxia and retrocollis which appeared at the age of nine. On admission, neurological examination revealed cerebellar ataxia, dystonia of the neck and the right arm, myoclonus of the neck and the shoulder, slight mental retardation, supranuclear upper gaze palsy, and sensorineural hearing loss. Laboratory examination showed high serum CK activity. Electromyography and muscle biopsy findings suggested slight muscular involvement. CSF level of HVA and 5-HIAA were reduced. MRI demonstrated marked cerebellar atrophy and slight atrophy of the brain stem. To our knowledge, the characteristic combination of the neurological sign in this case has not been reported. This case was compared with EOCA (early onset cerebellar ataxia with retained tendon reflexes) and other juvenile onset cerebellar ataxia and dystonia.     

Reflex sympathetic dystrophy: result of autonomic denervation?

1. To investigate the nature of sympathetic dysfunction in the pathogenesis of reflex sympathetic dystrophy, the microcirculatory vasoconstrictive responses to dependency were investigated in the skin of the hand of 76 reflex sympathetic dystrophy patients with unilateral disease by means of laser Doppler flowmetry (in perfusion units) and capillary microscopy. The patients were divided into three stages according to their perception of skin temperature (stage I in the case of a stationary warmth sensation, stage II in the case of an intermittent warmth and cold sensation, and stage III in the case of a stationary cold sensation). The vasoconstrictive responses were induced by lowering of the affected hand. 2. As compared to controls, the mainly sympathetically mediated vasoconstrictive response at thermoregulatory level of the skin microcirculation, as measured by laser Doppler flowmetry, was attenuated at stage I (1.82 versus 1.41, P < 0.05), stage II (1.82 versus 1.09, P < 0.0001) and stage III (1.82 versus 1.14, P < 0.01), suggesting the involvement of sympathetic denervation at all stages of the reflex sympathetic dystrophy syndrome. This sympathetic denervation may also account for the observed increase in thermoregulatory skin blood flow at stage I as compared to controls (152 versus 81, P < 0.01). 3. Since sympathetic denervation has been reported to cause increased sensitivity of vascular structures to catecholamines, the decrease in thermoregulatory skin blood flow at stages II (54 versus 81, P < 0.05) and III (31 versus 81, P < 0.05), both as compared to controls, may result from hypersensitivity to catecholamines of skin microvessels. 4. The sympathetically independent vasoconstrictive response at the nutritive level of skin microcirculation, as measured by capillary microscopy, was impaired only at stage III as compared to controls (1.04 versus 2.06, P < 0.05). This divergence in microvascular reactivity upon dependency of the nutritive and thermoregulatory subsystems also supports the hypothesis of sympathetic dysfunction.(ABSTRACT TRUNCATED AT 250 WORDS)     

The reflex sympathetic dystrophy syndrome of the lower extremities in patients after kidney transplantation--another complication of cyclosporin A therapy?

The involvement of the immune system in changes in luteal function was evaluated in rabbits. Pseudopregnancy was induced in 10 females and the spleens (considered to be the source of macrophages) of five were excised on day 7 of pseudopregnancy, while the five controls had sham splenectomies. Subsequent changes in serum progesterone concentrations were measured as an indicator of luteal function and luteolysis. A second pseudopregnancy was induced 31 days after splenectomy. The first pseudopregnancy was prolonged and during the second pseudopregnancy the serum progesterone concentrations on days 3 and 7 were much lower in the splenectomy group than in the control group. On day 14 of the second pseudopregnancy, the serum progesterone concentration markedly decreased in the control group while it remained almost at the level of day 7 in the splenectomy group. These results suggest that splenectomy suppresses the expression of luteal function and delays luteolysis in rabbits.     

Reflex sympathetic dystrophy due to phenobarbital: a frequent and treatable condition

Reflex sympathetic dystrophy (RSD) complicating barbiturates therapy is not well acknowledged in the neurologic literature in spite of the fact that barbiturates are present in 17% of the cases of RSD. Two new cases detected during one year in a general neurology clinic are described. One of them did not suffer from any previous neurologic disease. A review of the literature showed that the association of RSD and barbiturates affects predominantly upper limbs and is often accompanied by other fibrosing musculoskeletal disorders. Apart from barbiturate withdrawal, corticosteroid therapy may be necessary to prevent irreversible tissue damage.     

The stretch reflex in the Eaton Lambert syndrome, myasthenia gravis and myotonic dystrophy

The stretch reflex at rest and after muscle work was studied in three cases of Eaton Lambert syndrome. After muscle work a potentiation of the stretch reflex was demonstrated clinically and electrophysiologically. The presence of muscle stretch reflexes and their potentiation after muscle work was correlated with the clinical stage of the disease. The enhancement of the stretch reflex after voluntary effort was absent in normal subjects, myasthenia gravis and myotonic dystrophy.     

Ozone-induced stimulation of pulmonary sympathetic fibers: a protective mechanism against edema

Tropospheric ozone exerts well-described toxic effects on the respiratory tract. Less documented, by contrast, is the ability of ozone to induce protective mechanisms against agents that are toxic to the lungs. In particular, interactions between ozone and the sympathetic nervous system have never been considered. Using a model of permeability edema in isolated perfused rabbit lungs, we report here that, immediately after exposure of rabbits to 0.4 ppm ozone for 4 hr, the pulmonary microvascular responses to acetylcholine and substance P are completely blocked. Several lines of evidence, including partial inhibition of the ozone-induced protective effect by several drugs (alpha2- and beta-adrenergic antagonists, neuropeptide Y antagonist, guanethidine), measured levels of released catecholamines in blood and urine and the in vitro response of isolated lungs exposed to 0.4 ppm ozone all seem to suggest that ozone can stimulate pulmonary adrenergic fibers and induce the local release of catecholamines and neuropeptide Y, this resulting in transient protection against pulmonary edema. We also showed that, 48 hr after the exposure, ozone increased the baseline microvascular permeability and the response to low concentrations of acetylcholine.     

Neck afferents and muscle sympathetic activity in humans: implications for the vestibulosympathetic reflex

We have shown previously that head-down neck flexion (HDNF) in humans elicits increases in muscle sympathetic nerve activity (MSNA). The purpose of this study was to determine the effect of neck muscle afferents on MSNA. We studied this question by measuring MSNA before and after head rotation that would activate neck muscle afferents but not the vestibular system (i.e., no stimulation of the otolith organs or semicircular canals). After a 3-min baseline period with the head in the normal erect position, subjects rotated their head to the side (approximately 90%) and maintained this position for 3 min. Head rotation was performed by the subjects in both the prone (n = 5) and sitting (n = 6) positions. Head rotation did not elicit changes in MSNA. Average MSNA, expressed as burst frequency and total activity, was 13 +/- 1 and 13 +/- 1 bursts/min and 146 +/-34 and 132 +/- 27 units/min during baseline and head rotation, respectively. There were no significant changes in calf blood flow (2.6 +/- 0.3 to 2.5 +/- 0.3 ml.100 ml-1.min-1, n = 8) and calf vascular resistance (39 +/- 4 to 41 +/- 4 units; n = 8). Heart rate (64 +/- 3 to 66 +/- 3 beats/min; P = 0.058) and mean arterial pressure (90 +/- 3 to 93 +/- 3; P < 0.05) increased slightly during head rotation. Additional neck flexion studies were performed with subjects lying on their side (n = 5), MSNA, heart rate, and mean arterial pressure were unchanged during this maneuver, which also does not engage the vestibular system. HDNF was tested in 9 of the 13 subjects. MSNA was significantly increased by 79 +/- 12% (P < 0.001) during HDNF. These findings indicate that neck afferents activated by horizontal neck rotation or flexion in the absence of significant force development do not elicit changes in MSNA. These findings support the concept that HDNF increases MSNA by the activation of the vestibular system.     

Reflex sympathetic dystrophy in hemiplegia--two case reports and review of the literature

Reflex sympathetic dystrophy (RSD) can be precipitated by a variety of events. We report two cases of RSD in hemiplegic patients detected within three months of the cerebrovascular accidents. Diagnosis of RSD was based upon clinical and scintigraphic findings. Management included elevation, range of movement of the affected joints and analgesics. With treatment, the pain and swelling subsided, range of motion of joints improved and the patients were able to participate in the rehabilitation programme. A high index of suspicion, early diagnosis and aggressive treatment were found to be essential for the successful treatment of RSD.