Complex Regional Pain Syndrome (a.k.a. RSD)
Title: Reflex sympathetic dystrophy: changing concepts and taxonomy.
Author: Stanton-Hicks M; Jänig W; Hassenbusch S; Haddox JD; Boas R; Wilson P
Address: Pain Management Center, Cleveland Clinic Foundation, OH 44195, USA.
Source: Pain, 63: 1, 1995 Oct, 127-33
Abstract: 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.
MedLine Unique Identifier: 96124033
Reflex Sympathetic Dystrophy Syndrome
by: The Joint Program in Nuclear Medicine May 1990
John A. Moriarty, MD
David E. Drum, MD, PhD
Presentation: A 58 year-old white male was referred for evaluation of right foot and ankle pain of four months duration associated with swelling. There was no history of trauma, surgery or infection. He had been placed in a plaster cast for several weeks for pain of unclear etiology. Erythrocyte sedimentation rate was 29. Alkaline phosphatase was normal.
Imaging Findings: The bone scintigram showed diffusely increased deposition of Tc-99m MDP in the ankle, subtalar, tarsal and metatarso-phalangeal joints of the right foot. Slightly increased radiotracer was deposited in the region of the tarso-matatarsal joints of the left foot. An X-ray of the right foot showed peri-articular osteopenia. A diagnosis of reflex sympathetic dystrophy syndrome was made. The patient markedly improved on conservative treatment.
Discussion
Clinical:
RSDS has been considered as a distinct entity since the description by Mitchell:
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(1). He used the term, 'Causalgia'. However, this was later recognized as part of a spectrum of conditions which have in common non-specific findings such as hyperalgesia, vasomotor abnormalities and variable skin changes. Edema is often present. DeTakats, Steinbrocker and Evans unified this miscellany under the title, 'Reflex Sympathetic Dystrophy Syndrome'. This is appropriate because of the apparent involvement of the autonomic nervous system. The most frequently observed varieties are Sudeck's atrophy and the Shoulder Hand Syndrome. Kozin
(2) demonstrated that the syndrome is often bilateral and provided a clinical classifications. Severity ranges from minor, which includes Sudeck's atrophy, to major causalgia, which is usually associated with major nerve damage. In such cases pain usually corresponds to the cutaneous distribution of the injured nerve. There is a slight female preponderance.
The Should Hand Syndrome is a variant of RSDS often related to myocardial infarction, cervical disk herniation and degenerative disease of the cervical spine. Other correlates of RSDS include sepsis, systemic viral infections and spinal cord concussion. RSDS may occur
in many hospitalized stroke patients. Kozin reports a figure of 14%% and Tepperman 25%%. Rarely RSDS may be associated with underlying carcinoma (bladder, lung and esophagus), herpes zoster and isoniazid therapy.
Local sympathetic blockade is used in therapy and diagnosis.
Radiological Evaluation:
Patchy osteoporosis is the primary roentgenologic manifestation of RSDS. This non-specific finding is usually periarticular and associated with soft tissue swelling. Erosions of the subchrondral bone may also be found.
There has been a great deal of controversy about the precise findings on scintigraphy. Although in adults increased activity is usually observed in the affected regions, Matter studied 32 patients and concluded that the pattern of decreased activity described in children is occasionally encountered in adults. This variability may be related to the interval between onset of symptoms and imaging. Demangeat (3) studied 181 patients with RSDS of the hand providing quantitative measurements of blood flow (BF), blood pool (BP) and delayed images (DI). He divided the studies into three stages based on interval from onset of symptoms.
Stage . . .Weeks . . . BF . . . BP . . . DI
1. . . . . . . 0-20 . . . . .INC . . INC . . INC
2 . . . . . 20-60 . . . . . N . . . INC . . . INC
3 . . . . . 60-100 . . . DEC . . N . . . . . N
INC=increased, DEC=decreased, N=normal
The reduced flow in advanced RSDS may be due in part to amyotrophia caused by disuse. Intenzo (7) recently found that 28%% of 32 patients with RSDS of the lower extremities had normal scans, 59%% showed increased and 13%% showed decreased activity; overall sensitivity was 72%%. Values for the sensitivity and specificity of the one scan for RSDS vary amongst authors, however. Werner (6) reported a sensitivity of 50%% and a specificity of 92%% based on the delayed image. When blood flow and blood pool images were considered these values were not altered. MacKinnon and Holder (4) reported significantly higher values: sensitivity=96%%; specificity=98%%.
Goldsmith (5) evaluated the three phase bone scan in children and the lower extremity was affected much more frequently than in adults (12/15). Only one radiograph showed mild diffuse osteopenia, but the bone scan showed decreased uptake in 12/15. Increased uptake was the pattern in two patients. Blood flow and blood pool images did not increase the sensitivity.
Scintigraphy appears to be a useful guide to prognosis. 90%% of patients with positive scintigrams experienced a favorable response to steroids as opposed to 34%% of those with negative scintigrams (2).
Conclusions:
Changes on the roentgenogram are not specific. Findings on the bone scan depend to a great extent on the stage of the disease. Blood flow and blood pool images did not increase the sensitivity. The static delayed image appears most useful.
References:
1) Mitchell SN, et al. Gunshot wounds and other injuries of nerves. Lippincott, Philadelphia, 1964.
2) Kozin F, et al. RSDS. AJM 1976; 60:321 and 1981; 70:23-30.
3) Demangeant JL, et al. Three-phase bone scanning in RSDS of the hand. JNM 1988; 29:26-31.
4) MacKinnon SE, Holder LE. The use of three-phase bone scanning in the diagnosis of RSDS. J Hand Surg 1984; 9A:556-563.
5) Goldsmith DP, et al. Nuclear imaging and clinical features of childhood RSDS: comparison with adults. Arthr Rheum 1989; 32:480.
6) Werner R, et al. Factors affecting the sensitivity and specificity of the three-phase bone scan in the diagnosis of RSDS in the upper extremity.
7) Intenzo C, et al. Scintigraphic patterns of RSDS of the lower extremities. Clin Nucl Med 1989; 14(9):657-661.
Does sympathetic dysfunction originate from peripheral neuropathy?
Title: Reflex sympathetic dystrophy: does sympathetic dysfunction originate from peripheral neuropathy?
Author: Kurvers HA; Hofstra L; Jacobs MJ; Daemen MA; van den Wildenberg FA; Kitslaar PJ; Slaaf DW; Reneman RS
Address: Department of Surgery, Maastricht, The Netherlands.
Source: Surgery, 119: 3, 1996 Mar, 288-96
BACKGROUND: Sympathetic dysfunction in reflex sympathetic dystrophy (RSD) has been purported to consist of an afferently-induced increase in efferent sympathetic nerve impulses (somato-sympathetic reflex) and/or denervation-induced supersensitivity to catecholamines. In addition, both the central and peripheral nervous systems have been claimed to be involved. It was the aim of this study to obtain more insights into these underlying mechanisms.
METHODS: In the affected extremeties of 42 patients with RSD we investigated as indirect measures of sympathetic (dys)function: (1) skin blood flow and the vasoconstrictive response to dependency of skin microvessels by means of laser Doppler flowmetry (distal to the site of trauma), (2) relative distention of the brachial artery and changes in relative distention consequent to a cold pressor test by means of ultrasonic vessel wall tracking (proximal to the site of trauma), and (3) arterial blood pressures by means of the Finapres technique. Both provocation tests induce a sympathetically mediated response. Patients were divided into three categories according to their perception of skin temperature in their injured limb (stage I, stationary warmth sensation; stage II, intermittent warmth and cold sensation; or stage III, stationary cold sensation).
RESULTS: Distal to the site of trauma, when compared with controls, skin blood flow was increased at stage I and decreased at stages II and III, whereas the vasoconstrictive response to dependency was impaired at all three stages. Proximally, when compared with controls, relative distention of the brachial artery and its response to the cold pressor test were decreased at all three stages. No differences were observed in pulse pressure between patient groups and controls.
CONCLUSIONS: These results suggest that sympathetic dysfunction in extremities of patients with RSD distal to the site of trauma consists of hypersensitivity to catecholamines at stages II and III as a result of autonomic denervation at stage I, whereas proximal to the site of trauma sympathetic nerve impulses may be increased at all three stages.
Language of Publication: English
MEDLINE Unique Identifier: 96202549
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