Complex regional pain syndrome-type I: research relevance, practice realities.
Complex regional pain syndrome-type ! (CRPS I) was known as reflex sympathetic dystrophy syndrome (RSDS) prior to the International Association for the Study of Pain (IASP) conference in 1993. At that time an IASP special interest group, Pain and the Sympathetic Nervous System, restructured and proposed a new taxonomy to better define and classify manifestations previously diagnosed as RSDS and causalgia (CRPS II) to facilitate more accurate diagnosis and treatment. Indiscriminate and imprecise diagnosis of clients with chronic pain syndromes has contributed to confusion, misdiagnosis, and mistreatment. The IASP work group designed a comprehensive algorithm incorporating multidisciplinary services and interventions to be used for clients with a diagnosis of CRPS (Stanton-Hicks et al., 1998). This article reviews the most recent CRPS I research findings, examining their relevance to clinical practice.
CRPS I is an extremely painful condition usually occurring in an extremity following minor trauma. Although there are autonomic abnormalities, trophic changes, and mobility disturbances of various degrees, the outstanding characteristic of CRPS I is severe, deep, burning pain that is out of proportion to the injury and without discernible nerve injury. If there is nerve injury present, the condition is designated CRPS II. There is no consensus on the underlying pathophysiology of this condition or its treatment. There is wide variability in the course of the condition, which leads researchers to speculate about several possible variants or subgroups of CRPS I.
IASP in its consensus paper lists the following criteria for the diagnosis of CRPS I:
1. Follows a noxious event without an apparent nerve lesion
2. Spontaneous pain (hyperalgesia and allodynia) disproportionate to the inciting event
3. Presence of edema, skin temperature, and color changes in distal aspect of affected limb since the inciting event
4. Exclusion of comorbidity that accounts for the degree of pain and dysfunction (Boas, 1996).
There still is no definitive diagnostic test for CRPS I. Instead, clinicians must rely on their assessment skills and subjective client reports. All clients complain of a severe, deep, somatic, burning pain that seems exaggerated. Hyperalgesia and allodynia are frequently present or develop later in the course of illness.
Motor and mobility manifestations are present and include weakness, exaggerated deep tendon reflexes in the affected extremity, tremors, and muscle spasms. Schwartzman (2000) discussed a "movement disorder" of CRPS I that includes the following five components: the client's inability to initiate movement, tremors, weakness, muscle spasm, and dystonia. He reported that these changes may occur spontaneously and may precede pain sensation. All these responses, plus guarding of the affected part, interfere with range of motion and the individual's mobility.
Manifestations are attributed to increased sympathetic activity in the affected area and can include variable skin color, temperature changes, and hyperhidrosis. Trophic changes include increased hair and nail growth, followed by tissue atrophy, alopecia, and brittle nails.
Conventional wisdom of the past maintained that sympathetic nerve blocks are diagnostic as well as therapeutic. This thinking has changed in recent years. It is believed that blocks are diagnostic for sympathetically maintained pain. Because CRPS I pain is not always sympathetically maintained, the blocks are of limited value in diagnosis and treatment (Birklein & Handwerker, 2001).
X rays remain of questionable value. Spotty osteoporosis, seen in 40% of clients with CRPS I, does not appear until 4-8 weeks after injury (Birklein & Handwerker, 2001). Scintigraphy may be helpful but is not a reliable diagnostic tool.
Thermography can assist in diagnosis because it records differences in the body's radiant heat-emission patterns between the affected and contralateral sides. It can detect sensory nerve root irritation and objectively document clinically painful conditions, such as CRPS, that do not conform to a dermatome distribution of pain. The thermogram's asymmetric color variations are a reflection of abnormal sympathetic function and can be recorded within 72 hours after an injury.
Techniques such as thermometry, telethermometry, passive infrared methods, and laser Doppler flowmetry also may be used to test and compare skin temperatures, which are reflective of cutaneous blood flow changes seen in CRPS (Stanton-Hicks, 2000). The quantitative sudomotor axon reflex test, (a test to document abnormalities of resting and evoked sweat production), may prove useful to demonstrate disturbances in peripheral autonomic function.
The debate still continues about the pathophysiology of CRPS I (RSDS): Is it a peripheral or central event? Perhaps it is mixed. Research data reveal that, in most cases, this phenomenon begins as a localized neuropathy or inflammatory process, but if left untreated, it progresses to what appears to be a systemic disease process. Further, no one treatment modality provides a cure. Because of this, Greipp and Thomas (1990) suggested that CRPS I (RSDS) might be caused by more than one pathophysiologic mechanism and that there might be several possible variants.
Extensive research in the past decade attempted to sort out the underlying pathogenic mechanisms of CRPS I (Apkarian, Grachev, Krauss, & Szeverenyi, 2001; Boas, 1996; Rommel & Thimineur, 2001; Sieweke, Birklein, Riedl, Neundorder, & Handwerker, 1999). Although each of the research studies presented evidence in support of study hypotheses, the exact pathologic progression of the syndrome remains unclear. This lack of knowledge continues to burden clinicians in the assessment, diagnosis, and treatment planning for these individuals.
Butler (2001) conducted one of the more interesting recent studies about the pathophysiology of CRPS. Based on animal research findings, he proposed that disuse alone could produce CRPS manifestations in healthy subjects. The study sample consisted of 23 healthy volunteers who agreed to wear fiberglass arm casts for 4 weeks. Butler's team was looking for evidence of peripheral and central nervous system changes attributable to immobility. Upon cast removal, participants underwent extensive physical assessments and quantitative sensory testing (QST) of casted and noncasted hands and completed questionnaires about perceived subjective abnormal symptoms.
The study team reported changes in the participants after 4 weeks of cast immobilization, which demonstrated involvement of brain centers associated with sensory processing, pain, and motor function (Butler, 2001). These findings must be considered when discussing the merits of various physical therapy (PT) approaches for CRPS clients, because so many physicians will not begin PT until the client is pain free.
Greipp, Thomas, and Renkun (1988) reported their research findings with a sample of 27 children and young adults with CRPS I (RSDS), 6-19 years of age. Their findings were contradictory to most of what could be found about pediatric RSDS in the medical literature at that time (Blau, 1984; Doolan & Brown, 1984; Ruggeri et al., 1982). The findings suggested that CRPS I (RSDS) is not rare in children and young adults within the overall RSDS population and it is not easily treated and cured in this cohort. They found that the RSDS manifestations in their study participants were as severe as those found in adults and were extremely difficult to treat.
Their data further suggested that CRPS I (RSDS) clients were rarely cured, but rather were subject to erratic patterns of remissions and exacerbations. In 96% of the participants, the pathology began regionally from minor trauma, and in a majority it progressed to a systemic problem (Greipp et al., 1988).
An acknowledged limitation of this study was the biased sample (from the RSDS Association database). However, it was the only published study documenting a significant occurrence in young people at the time and the only published study of such severe presentations of the syndrome (Greipp et al., 1988). Other publications reported isolated success stories with no longitudinal follow up.
Some researchers still hold to the belief that CRPS I (RSDS) is rare in children and very self-limiting when it does occur (Sherry, Wallace, Kelley, Kidder, & Sapp, 1999; Tong & Nelson, 2001). Others agree that CRPS I (RSDS) does occur in children and can be as severe and resistant to treatment as in adults (Murray, Cohen, Perkins, Davidson, & Sills, 2000; Stanton-Hicks et al., 1998; Wilder, 1996). Wilder reported that many pediatric patients are successfully treated with conservative therapy but that a larger subset has pain and manifestations resistant to all forms of therapy. He further reported that only about half of his patient population experienced complete resolution and that the rest had some degree of residual difficulty that could be classified as mild to severe.
There is almost universal agreement in the literature that CRPS I (RSDS) is not a psychogenic condition but that stress and emotional upset are known to exacerbate painful manifestations. Lankford (1984) proposed an RSDS diathesis or predisposition to the malady and identified the following two categories: "hypersympathetic reactor" is related to a genetic predisposition with the person exhibiting increased sympathetic activity such as hyperhidrosis and increased skin temperature variation; the second category is related to individual psychological factors along with exhibiting dependent personality traits. He postulated that persons with CRPS I share certain personality characteristics, including insecurity, fearfulness, suspiciousness, and low pain thresholds. Lankford further stated that he rarely encountered a client with RSDS he would describe as "stoic" or a "Spartan."
Greipp et al. (1988), in light of Lankford's proposed diathesis, studied the data on the 27 participants in the context of birth-order distribution and the personality characteristics associated with birth order. They ranked the birth order of the participants (oldest, middle, and youngest) and used a one-sample chi-square to determine whether the numbers in each category were significant. Three participants were the oldest children in the family--two of whom were the only child in the family. Eight participants were middle children (not the oldest or youngest), and 15 participants were the youngest child (one was a twin) in the family. Birth order of one subject was unknown. Results were significant at the .05 level (Greipp et al.). The majority of study participants were youngest children who are usually characterized as more dependent and indulged than older siblings. Perhaps this finding was relative to Lankford's diathesis.
On the other hand, after extensive study, Covington (1996) stated definitively that there is no RSDS personality. Monti, Herring, and Schwartzman (1998) concluded that personality distortions seen in patients with CRPS I may represent an exaggeration of maladaptive traits and coping styles in response to chronic, intense pain.
There is nothing reported in the sports medicine literature about epidemiology in athletes, especially professional athletes. CRPS is a condition usually precipitated by minor (sprains and soft tissue injuries) or major injuries. Can the absence of CRPS I in athletes be related to the more aggressive personalities of athletes or to the fact that they are always involved in intense physical exercise, adhering to a philosophy of "no pain, no gain?" Or, according to Lankford's work, can it be related to the more stoic or Spartan personality characteristics of athletes? These questions remain unanswered.
The literature during the last two decades has been consistent in describing three classic stages of CRPS I (RSDS), the first stage constituting localized manifestations and experienced for the shortest time. However, the literature does not consistently report that all clients with CRPS I (RSDS) progress through all three stages. There has been so much variability reported in the severity of manifestations, duration, and responses to treatment modalities that one has to question whether all reported cases are truly the same entity. Some researchers have questioned the possibility of subtypes or variants (Bruehl et al., 2002; Greipp & Thomas, 1990).
Bruehl et al. (2002) studied 113 CRPS I clients, focusing on the four domains of pain/sensory abnormalities, vasomotor disturbances, edema/sudomotor disturbances, and motor/trophic manifestations. The researchers were searching for evidence of homogeneous CRPS I subgroups that would be consistent with the classic three stages defined in the literature. Their findings offer only limited support for the traditional three stages. Instead they propose the following three subtypes of CRPS: (a) a relatively limited syndrome primarily with vasomotor manifestations, (b) a relatively mild syndrome in which the neuropathic pain/sensory manifestations are significant, and (c) a full-blown, florid syndrome likened to classic RSDS (CRPS I) (Bruehl et al.). The imprecision of Bruehl's subgroups does not particularly assist in diagnostic classification or in the selection of treatment protocols. It does validate the need to further investigate the basic entity and the possibility of existing variants or subtypes.
A number of recent references have alluded to the possibility of a genetic component or predisposition to CRPS I (Baron & Janig, 2001; Birklein & Handwerker, 2001; Mailis & Wade, 2001; Schwartzman, 2000). One citation reported three families in which two or more members were diagnosed with the syndrome (Greipp & Thomas, 1991).
Mailis and Wade (2001) conducted a study of 15 women with CRPS to identify any associations of CRPS with genes of the major histocompatibility complex (MHC). They concluded that one or more genes affecting susceptibility to the onset or progression of CRPS may be present near the MHC region of the short arm of chromosome 6. Their small sample size prevented a more in-depth statistical analysis. They suggested that genetic susceptibility could explain spreading, migration, recurrences, and rare familial occurrences of CRPS; variable treatment outcomes; and the epidemiological differences in gender and ethnic groups (Mailis & Wade).
Recurrences/Quality of Life
A number of researchers have discussed recurrences of CRPS I (RSDS) since the 1980s, which calls the question, How does one know for certain that a "cure" is not just a remission? Veldman and Goris (1996) found recurrences to be problematic in a study of 1,183 CRPS I (RSDS) patients who presented over a 10-year period. In 53% of their patients with known recurrences, the recurrences were spontaneous and not in response to an identifiable precipitating event. These researchers concluded that there was no way to predict or prevent CRPS recurrences. They did observe that recurrences are more frequent in younger clients and manifest in the contralateral extremity. A recent case study documented an 11-year-old girl with a spontaneous onset of RSDS who went on to exhibit a pattern of recurrent and migratory pathophysiology consistent with other pediatric reports in the literature (Greipp et al., 1988; Murray et al., 2000; Tong & Nelson, 2001; Wilder, 1996).
Only two citations could be found that explored quality-of-life findings in CRPS I clients (Galer, Henderson, Perander, & Jensen, 2000; Greipp, 2000). Although both study sample sizes were small, findings were similar. The majority of study participants reported ongoing, moderate to severe pain along with physical disabilities that interfered with their activities of daily living.
After reviewing all the recent literature and research findings available, I would like to focus on new developments and their relevance to nursing practice. Initially, most of these clients are in the community, having experienced only minor injuries as the exaggerated sympathetic response becomes apparent. Nurses come into contact with them as family members, friends, neighbors, coworkers, and acquaintances or in physicians' offices. In many cases the nurse may be the first one to see and recognize the clinical presentation and make the necessary early referral for CRPS I.
In recent years there has been a notable decrease in the use of nerve blocks and sympathectomies for CRPS clients. Because of new insights into the mechanics of sympathetically maintained pain and sympathetically independent pain with exhibited CRPS pathology, physicians are more circumspect about prescribing nerve blocks for diagnosis and treatment (Birklein & Handwerker, 2001). This is especially true in the early stages. If the pain is not sympathetically maintained, the invasive blocks will be of limited value in diagnosis and treatment. There seems to be more adherence to the IASP multidisciplinary algorithm, which emphasizes exhaustion of more conservative therapies before invasive therapeutic modalities are tried.
PT has always been recommended for CRPS I clients, but there seems to be increased emphasis on its early use in the last decade (Harden, 2000; Kemler, Coen, Rijks, & deVet, 2001; Oerlemans, Oostendorp, deBoo, & Goris, 1999; Stanton-Hicks et al., 2001). In contrast, Harden warned that PT should not be encouraged while the client is in an insensate state. This is contrary to past practice, which suggested the use of PT after analgesics and/or nerve blocks had been administered. Birklein and Handwerker (2001) stressed the importance of PT while the client is without pain. For nurses and other clinicians working with CRPS I clients, this is an almost impossible task. The continuous and intense hyperalgesia and allodynia experienced by these individuals, and their guarding of the involved extremities, contributes to their immobility and resistance to PT.
Stanton-Hicks et al. (1998) discussed this problem and the IASP's physical therapeutic algorithm, whose purpose is to help the client regain functional restoration as early as possible. As can be expected, PT is to be started slowly and gently while the client builds trust in the clinicians. Desensitization to increased sensory stimuli, a keystone of the early program, can be done by using mild nonnociceptive, controlled interventions such as massage, heat, and very gentle movement. As the client's tolerance increases, activities are increased to include isometric strengthening and stress-loading exercises. All of this needs to be carefully monitored while the client's responses and progress are documented.
The most encouraging news in recent years is the multidisciplinary nature of the approaches to treating these individuals with CRPS I. For years, people were shuttled from one practitioner to another, from one specialist to another, with no one coordinating the treatment plan. Now there is earlier recognition of CRPS I, which allows the person to get referred into a program or center where there is a comprehensive plan to treat CRPS I with coordination of multiple interdisciplinary services for better client outcomes. Emphasis continues to be on pain relief, psychological support, restored functional mobility, and assistance with developing a life plan.
The frustrating aspects for CRPS researchers include the difficulty in finding answers to the unknowns. Prognosis for the individual diagnosed with CRPS I is questionable at best and should be approached with caution. Complex patterns of spread, migration, remissions, and exacerbations continue to be reported and leave questions about the certainty of a cure. Are these various clinical presentations of CRPS I really the same entity? Is there a genetic factor or predisposition to developing CRPS I? Based on its absence from the sports medicine literature, I question why college and professional athletes do not seem to get CRPS I. Could this be related to the ongoing, intense physical training these athletes endure on good days and bad days? Or could it be related to personality factors, with athletes being more aggressive and willing to push through the CRPS I pain and the pain from intense physical therapy during acute stages of the syndrome? I am not condoning pushing through CRPS I pain, but the questions about the success of early and intense physical therapy with this cohort of athletes must be examined.
It is encouraging to see so much progress during the last decade, with early recognition of the malady and more collaborative and coordinated efforts to treat CRPS I. The difficult fact remains that, for some clients, no matter how early they are diagnosed or what treatments are utilized, nothing works. These individuals are left with lifetimes of chronic, intense pain and major disabilities. More prospective, longitudinal research is needed to find answers to the unresolved questions and to contribute to the increased quality of life needed by those with CRPS I.
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Questions or comments about this article may be directed to: Mary E. Greipp, EdD RN FAAN, by phone at 856/225-6226 or by e-mail at firstname.lastname@example.org. She is a professor and the chair of the department of nursing at Rutgers the State University of New Jersey, Camden College of Arts and Sciences, in Camden, NJ.
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|Title Annotation:||neuroscience nursing research|
|Author:||Greipp, Mary E.|
|Publication:||Journal of Neuroscience Nursing|
|Date:||Feb 1, 2003|
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