Rsd Reflex Sympathetic Dystrophy AKA CRPS or Complex Regional Pain Syndrome CRPS

History of RSD

The history and the discovery of RSD (Reflex Sympathetic Dystrophy) Syndrome and its symptoms have typically been associated with wars. While there is no doubt that RSD from physical stress and injury existed earlier, it was left up to war physicians to assign pathology to it. Silas Weir Mitchell, an army doctor during the Civil War, described the symptoms of "burning pain" left in soldiers long after the bullets have been removed. He attributed these residual and long lasting pains to major nerve injury. Weir was the first to call RSD causalgia (currently, specifically known as CRPS-2), which is Greek for "burning pain." He wrote that, "Under such torments, the temper changes, the most amiable grow irritable, the soldier becomes a coward, and the strongest man is scarcely less nervous than the most hysterical girl." Weir accurately reflected the symptoms. (PARC, 2004). Mitchell accurately described the symptoms associated with the disorder as feeling of heat in the afflicted area where the skin tone changed to a glossy, rash-like appearance. He also described, very accurately, the secondary, psychological symptoms of the disease.

World War I, army surgeon Rene Leriche first treated causalgia by administering numbing medication to the parasympathetic nerve endings associated with the region of pain. This was the first time the parasympathetic nervous system was implicated in the disorder. Later, William Livingstone, also working with the defense services described the symptoms of RSD with greater accuracy. He also identified, in injured soldiers, the spread of the symptoms -- mirror pains on the opposite side of the body. This proved that there the origins of the disorder are at higher centers in the brain.

The history and the discovery of RSD can also be traced, parallelly to other countries. Sudeck and Keinbock found and confirmed the symptoms of RSD and also showed that disuse of parts of the body due to pain could cause osteoporosis. In France, in 1890, the physician Charcot, attributed the symptoms of RSD to psychosomatic origins. We know now that this is not true. Nonetheless, Charcot contributed a great deal to the school of thought in accurately describing the symptoms of RSD. In 1947, Steinbrocker named RSD, "shoulder-hand" syndrome. (PARC, 2004).

Definition of RSD

While RSD Regional Sympathetic Dystrophy has been readily named after earlier attempts based on the symptoms of the condition, identifying a specific cause of the disease and treating it effectively has been very difficult. This is because the incidence of RSD is difficult to pinpoint. The symptoms vary in severity. The aftereffects are also largely varied. Though RSD is often caused by injuries from high velocity impact such as bullets and shrapnel, on occasion it arises from no known injury. Treatment is difficult because the symptoms are usually masked and misdiagnosis occurs often. (Schwartzman & McLellan, 1987).

RSD has been better defined by the acronym, CRPS (Complex Regional Pain Syndrome). There are two types of CRPS. CRPS Type 1 is also known as RSD. For the purpose of this, only CRPS Type 1 will be explored. CRPS Type 2 is known as causalgia. The two types should not be used interchangeably. Though the symptoms are most often the same within internal variations in severity and aftereffects, what distinguishes RSD (CRPS 1) from Causalgia or CRPS 2 is that the former is due to no identifiable nerve injury and the latter is due to a severe nerve injury as was identified in soldiers.

The precipitating causes of RSD are difficult to identify because even remote innervations to the sympathetic nervous system can cause symptoms. Causative factors are even more difficult to identify because the sympathetic nervous system coordinates and controls many of the involuntary functions necessary to sustain life. The most easily identifiable cause is trauma. The difficulty here is that a minor injury, which most patients ignore when the initial pain goes away, may also trigger symptoms of RSD. Heart disease and myocardial infarctions are other known causes, as are cervical spine- and other spinal cord disorders. Infections and trauma from surgery may also cause RSD. Cerebral lesions are difficult to identify externally, though they have been implicated. Repetitive motion disorders such as carpal tunnel syndrome can also cause symptoms of RSD. (Bonica, 1988).

Both types of CRPS are associated with cardinal and secondary symptoms. Very often, psychological factors such as depression are directly attributed to the disorder. (Ciccone, Bandilla, & Wu, 1997). To date however, there has been no identifiable psychological symptom for RSD. Researchers have concluded that depression that arises from RSD is due to the cardinal symptoms like pain and inherent skin conditions.

The cardinal symptoms of CRPS Type 1 are led by pain. (Veldman et al., 1993). The pain felt is usually intense and burning. The area of affliction may also be at a higher temperature (or sometime at a lower temperature). The pain, with time, travels across the extremity. The pain occurs long after the wounds from the injury are healed. In case of minor injuries, the pain is often incommensurate with the severity of the injury. With RSD, hyperpathia and allodynia are found. The first is about pain felt long after the pain stimulus is removed. The second is pain at even the slightest touch. Pain from RSD increases with movement. It also increases in cold weather. Slight climatic changes in pressure can exacerbate the pain.

In addition to the pain, edema or swelling of afflicted area due to fluid retention also occurs. The skin takes on glossy tones and there is significant discoloration in hues ranging from red, blue, purple and gray. There is also muscle stiffness.

Due to the patient's reticence to move, which worsens the pain, osteoporosis and atrophy often occurs. There is a breakdown of skin tissue. If RSD afflicts the hand, there is thickening in the palmar fascia. Reddening of the skin occurs due to the dilation of capillaries (erythema). One of the secondary characteristics of RSD is hyperhidrosis or excessive perspiration. This often results in dehydration. (Lankford & Thompson, 1977).

CRPS is unique and complex. It can affect the blood vessels, bones, muscles and nerves with varying severity. The complexity of the disorder points to the fact that it might be associated with the sympathetic and parasympathetic nervous system. The mammalian nervous system consists of the central nervous system and the autonomic nervous system. The Central Nervous System consists of the brain and the spinal cord. The autonomic nervous system consists of sympathetic and parasympathetic nervous system. The autonomic nervous system consists of nerves that run from the hypothalamus (sympathetic) and the medulla oblongata (parasympathetic) and the nether regions of the body. These are sensory and motor nerves. The motor nerves control muscle function. The pre-ganglion motor neurons of the sympathetic nervous system arise from the spinal chord. They pass into the ganglia that are on either side of the spinal chord. These pre-ganglion motor neurons then synapse with post-ganglion neurons. These then pass into the spinal nerves, which innervate the extremities up to the outer dermis of the skin. This is the mechanism that is most closely associated with RSD. It explains some of the symptoms.

Additionally, (but not of any specific relevance here), the sympathetic nervous system is also associated with the involuntary functions such as the raising of the heartbeat and blood pressure, dilation of pupils, controlling peristalsis in the gastro intestinal tract, dilating the trachea, stimulation of the conversion of glycogen to glucose in the liver (and vice versa). In a sense therefore, the sympathetic nervous system trains the body to react. (Bakewell, 1995).

The parasympathetic nervous system works in conjunction with the sympathetic nervous symptoms. Briefly described, the parasympathetic nervous system returns the body to the normal state. For example, if the sympathetic nervous system raises the heart rate or dilates the pupils, the parasympathetic nervous system lowers the heart rate and constricts the pupils back to normal. RSD is thought to be a result of excitation of peripheral nerve elements with an abnormal and severe sympathetic response resulting in the pain and signs and symptoms of RSD. (Bakewell, 1995).

From a mechanistic standpoint, it is important to understand how the sympathetic nervous system plays a role the cause of RSDS. The sympathetic nervous system is often associated with the "fight or flight" aphorism. This means that it triggers an involuntary reaction mechanism to protect the body from violence or unwanted stimuli. Muscle vibrations cause shivering, which is a warming mechanism against cold. Or, firing of sympathetic nerves causes blood vessels in the skin to contract, forcing blood deep into muscle and enabling the victim to use his muscle to get up after an acute injury and escape from further danger. Also the decreased supply of blood to the skin reduces blood loss through superficial injuries that may occur on the surface of the body. The role of the sympathetic nervous system is therefore to function for a short period of time. The parasympathetic nervous system then takes over to bring the body back to normal. In RSD/CRPS, the sympathetic nervous system functions abnormally. It fires, with pain and swelling responses when the body does not reactively require it. In a sense therefore, one might also implicate the failure of the parasympathetic nervous system in bringing the body back to normal function. (Blumberg et al., 1997).

Progress of the Disease

CRPS Type 1 progresses in four stages. These are Dysfunction, Dystrophy, Atrophy, and Irreversible Failure of the Immune System. Because of the complexity and problems with misdiagnosis, many research panels have decided to avoid the process of staging. The disorder has been considered too complex to categorize. Incorrect categorization leads to significant problems for patients. Though RSD does not itself cause death, one has to be cautious of the problems with failure of the immune system and secondary effects.

Dysfunction or Stage I: This stage is also called early RSDS. Here, the pain is severe. But it is limited to the sight of injury. Patients often describe the pain to be of a burning and aching type. This pain often causes quality of life issues and emotional distress. A temperature reading often indicate that the local region where the pain is at either a higher or lower temperature than the rest of the body by up to one degree. Swelling is seen in the area of pain. Stage 1 is often characterized by abnormal hair or nail growth. Some changes in the bone mass or bone structure are also seen as a result of bone scans. This stage is also marked by muscle cramps. The skins color of the afflicted area changes from red to blue; the temperature changes from hot to cold. Stage 1 is marked by increases sweating. This is the best stage for treatment and complete recovery from RSDS. Unfortunately, depending upon the severity of the pain and associated symptoms, patients tend to ignore it or medical professional treat the pain as caused by other ailments, often prescribing incorrect treatment. It was mentioned earlier of the suggestion of eliminating suggestion. This is because of the variation in time associated with this stage. Typically, from the onset of the symptoms, the stage of dysfunction lasts for three months. In some however, it might last for shorter durations. In others, Stage 1 might last for several years. Research has shown that some patients never progress beyond this stage.

Dystrophy or Stage 2: This stage is also called the established stage. This stage is marked by increased severity of pain. The pain starts spreading at this stage, as does the swelling. The swelling also changes in character. The edematous tissue becomes indurate. In other words, it becomes hard to the touch, progressing from the soft swelling of the dysfunction stages. The excess growth of hair and nails is now replaced by falling hair and breakdown in nail-tissue giving rise to brittleness and cracks.

The dehydration from excess sweating causes dryness that it noticeable in the hands and palms. This is the stage when osteoporosis sets in. Bone scans or radiographs at this point are usually positive. Due to inactivity and reticence to move because of the pain, muscle tissue begins to waste away. The atrophy also extends to the skin and subcutaneous tissue. Once again, the severity and length of this stage are in dispute. In most patients however, the stage of dystrophy lasts between three months to a year.

Atrophy or Stage 3: The pain is still there and it is still severe. Occasionally, the pain diminishes but it is present. The pain also spreads to the entire limb. The spreading of the pain is also more significant. Studies have shown that the pain not only spreads proximally and laterally, there is a mirror affliction on the symmetrically opposite side of the body -- in some cases. (Maleki et al., 2000). The skin of the afflicted area becomes thin and shiny. This stage however, is marked by a lack of edema. Skin flare-ups and other problems occur spontaneously. Bone demineralization occurs. Muscle tissue is also often irreversibly damaged. Because of the progressive destruction of non-bony tissue such as muscles, ligaments and tendons, joint motion becomes severely restricted. Such joint motions are called contractures. In the atrophy stage, a small percentage of patients have generalized RSD. This means that it affects the entire body.

Stage Four: The fourth stage is when the disease becomes chronic enough and it is serious enough that the patient develops disturbance of immune system, hypertension, chest pain, stroke, and heart attack, and is at higher risk than the general population for high blood pressure and cancer. In the fourth stage, there is a high mortality due to suicide from secondary and psychosomatic effects. (Parillo, 2001).

Complications of the disease

Complications from RSD can be significant and widespread. Some of the complications have already been mentioned in the discussion on the stages of the progression of the disease. Like any other illness, patients with RSD/CRPS, often suffer from depression and anxiety. Skin, muscle, and bone atrophy (wasting) are possible complications of the syndrome. Atrophy may occur because of reduced function of the limb.

One of the problems in assigning a complication to RSDS as opposed to other factors is that it is hard to distinguish them. The sympathetic nervous system is so widespread in its functionality that a complication from RSD might be ascribed to another condition, and vice versa. A comprehensive study of over one thousand patients was undertaken to identify how predisposition to RSDS complications were different if the patient had no predisposition to RSDS. The data was collected over twelve years in the outpatient clinic.

The progress of the disorder in every patient was documented in detail. The researchers identified severe complications of chronic edema, impaired muscle function or dystonia, which are movement disorders in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures. Dystonias are involuntary and sometimes painful, may affect a single muscle; a group of muscles such as those in the arms, legs, or neck; or the entire body.

Some of the patients showed dystonias, others showed myoclonus (a brief, sudden, singular, shock-like muscle contraction), and others showed both. Typically, complications affect the extremities and in a large number of cases, the lower extremities. (van Hilten et al., 2001).

One of the salient conclusions of this work was that patients whose RSD symptoms showed decrease in local temperature (of the affliction) were at greater risk for developing severe complications or symptoms that were resistant to therapy.

Occasionally, complications arise from severe misdiagnosis or mistreatment or also the lack of knowledge. These include the beliefs that RSD is rare, or that it does not occur in children or that the pain being not related to an injury will go away in a few months if the patient merely guts it out.

Sometimes, the pain forces the doctor to use a cast, which further complicates the symptoms and hastens the onset of atrophy. All treatment modalities are not exhaustively used.

While physical therapy is often called for to treat pain and ensure that muscles are adequately used, aggressive physical therapy without proper medical supervisions often makes the symptoms worse.

Prevalence of the disease

No demographic is really immune from the diseases. RSD affects several million people annually with varying levels of severity.

Children also develop the disease and have the best chance of treatment working. Both men and women suffer from RSD, but there is a statistically significant greater chance of women complaining of the symptoms. (Mailis & Wade, 1994). People between the ages of forty and sixty are those most afflicted, though there are no defined age barriers.

According to the federal agency monitoring this disorder, National Institute of Neurological Disorders and Strokes (NINDS), between two to five percent of peripheral nerve injury patients complain of RSD symptoms. The prevalence of symptoms is greater in hemiplegics or those that are paralyzed on one side of the body with between twelve and twenty-one percent of the people presenting. The NINDS also indicates that between one and two percent of people with bone fractures report signs RSD either while healing or even after their fractures have completely healed. (NINDS, 2004).

A study by Allen and co-workers of 134 patients might be considered as fairly typical for this disease -- in terms of epidemiology.

The median age was slightly higher than forty-one years of age.

The age range of this cohort was from eighteen to seventy-one years. Also the average time before the evaluation was approximately thirty months. This means that most patients were well into the second stage of RSD. Seventy percent of the patients were female. A large percentage was Caucasian and fewer were of African-Americans, Asians, Hispanic and Middle Easter. With race and other demographics, of course, are confounding factors of injury and occupational stress. Sprains, post-surgical symptoms and fractures were the most common injuries. Crush injuries were relatively rate. Other injuries were associated with veni-punctures, lacerations and spinal cord injuries. (Allen, Galer, & Schwartz, 1999).

In terms of localization, most of the patients identified afflicted regions as the extremities. Lower extremity afflictions were more than those for upper extremities were. One patient in the study identified RSD localization in the jaw and about seven percent had afflictions in multiple extremities.

Symptoms were more frequent in the right side than the left. Sixteen percent of the cases showed symptoms that were bilateral.

Unfortunately, there are very few studies that rigorously identify the epidemiology of RSD. This is because the complications in diagnosis and confounding factors are too many. The results from the study by Allen et al. are far from being generalizable and extensible -- except for the age range and prevalence of symptoms among women.

Diagnosis

As has been mentioned previously in this work, diagnosing CRPS is very difficult because of the wide and varied nature of the symptoms. In believing that a patient has RSD, a potentially far more serious condition may be ignored. On the other hand, RSD symptoms may initially be ignored because the symptoms are not severe enough to warrant medication. Yet in some situations, the symptoms often correct themselves.

It is much easier to diagnose Type II CRPS because of the knowledge of occurrence of nerve damage from an identifiable injury. RSD is more difficult to detect; because of there is no known cause for the symptoms. (Aronoff et al., 2002).

The medical practitioner must adopt a more cautious approach in diagnosing RSD for the above-mentioned reasons. Practitioners aver that they should identify some symptoms more than other symptoms. These symptoms have been described earlier in detail. More often diagnosis can be easier if spreading occurs. On the other hand, in case of mirror afflictions, it is more difficult to identify the origin of the affliction, especially if the progress of the disorder has been slower.

The key aspects of diagnosis are first, pain that is not proportionate to the injury. Additionally, other abnormal manifestations of the sympathetic nervous system, movement disorder, dystrophy and atrophy of the soft and hard tissue as evidenced by X-rays or three-phase bone scans are also diagnostic tools. Since afflicted areas are characterized by difference in temperatures, thermograms are also used to identify RSD. Experts believe that identifying one symptom is sufficient to make an initial diagnosis of CRPS.