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Reflex sympathetic dystrophy syndrome (RSDS) — also known as complex regional pain syndrome (CPRS)— is a chronic condition characterized by severe burning pain, pathological changes in bone and skin, excessive sweating, tissue swelling, and extreme sensitivity to touch. The syndrome, which is a variant of a condition known as causalgia, is a nerve disorder that occurs at the site of an injury (most often to the arms or legs). It occurs especially after injuries from high-velocity impacts such as those from bullets or shrapnel. However, it may occur without apparent injury. Causalgia was first documented in the 19th century by physicians concerned about pain Civil War veterans continued to experience after their wounds had healed. Doctors often called it "hot pain," after its primary symptom. Over the years, the syndrome was classified as one of the peripheral neuropathies, and later, as a chronic pain syndrome. RSDS is currently classified as a variant of causalgia, not necessarily caused by trauma.
In ICD-10, it is listed as "Sympathetic reflex dystrophy", a form of algoneurodystrophy (M89.0), which has a distinct classification from causalgia (G56.4).
Symptoms
The symptoms of RSDS usually occur near the site of an injury, either major or minor, and include: burning pain, muscle spasms, local swelling, increased sweating, softening of bones, joint tenderness or stiffness, restricted or painful movement, and changes in the nails and skin. One visible sign of RSDS near the site of injury is warm, shiny red skin that later becomes cool and bluish.
The pain that patients report is out of proportion to the severity of the injury and gets worse, rather than better, over time. It is frequently characterized as a burning, aching, searing pain, which may initially be localized to the site of injury or the area covered by an injured nerve but spreads over time, often involving an entire limb. It can sometimes even involve the opposite extremity. Pain is continuous and may be heightened by emotional stress. Moving or touching the limb is often intolerable. Eventually the joints become stiff from disuse, and the skin, muscles, and bone atrophy. The symptoms of RSDS vary in severity and duration. There are three variants of RSDS, previously thought of as stages. It is now believed that patients with RSDS do not progress through these stages sequentially and/or that these stages are not time limited. Instead, patients are likely to have one of the three following types of disease progression:
- Type one is characterized by severe, burning pain at the site of the injury. Muscle spasm, joint stiffness, restricted mobility, rapid hair and nail growth, and vasospasm (a constriction of the blood vessels) that affects color and temperature of the skin can also occur.
- Type two is characterized by more intense pain. Swelling spreads, hair growth diminishes, nails become cracked, brittle, grooved, and spotty, osteoporosis becomes severe and diffuse, joints thicken, and muscles atrophy.
- Type three is characterized by irreversible changes in the skin and bones, while the pain becomes unyielding and may involve the entire limb. There is marked muscle atrophy, severely limited mobility of the affected area, and flexor tendon contractions (contractions of the muscles and tendons that flex the joints). Occasionally the limb is displaced from its normal position, and marked bone softening is more dispersed.
Cause
The cause of RSDS is unknown. The syndrome is thought to be the result of damaged nerves of the sympathetic nervous system — the part of the nervous system responsible for controlling the diameter of blood vessels. These damaged nerves send inappropriate signals to the brain, interfering with normal information about sensations, temperature, and blood flow. Since RSDS is most often caused by trauma to the extremities, other conditions that can bring about RSDS include sprains, fractures, surgery, damage to blood vessels or nerves, and cerebral lesions. The disorder is unique in that it simultaneously affects the nerves, skin, muscles, blood vessels, and bones.
Susceptibility
RSDS can strike at any age, but is more common between the ages of 40 and 60. It affects both men and women, but is most frequently seen in women. Although it can occur at any age, the number of RSDS cases among adolescents and young adults is increasing. Investigators estimate that two to five percent of those with peripheral nerve injury and 12 to 21 percent of those with hemiplegia (paralysis of one side of the body) will suffer from RSDS.
Similar disorders
RSDS has many of the same features as causalgia, such as severe burning pain that is aggravated by physical or emotional stimuli. However, causalgia usually affects the lower limbs, the palm of the hand or the sole of the foot; RSDS may strike any part of the body.
RSDS also has characteristics similar to those of other disorders, such as shoulder-hand syndrome, which sometimes occurs after a heart attack and is marked by pain and stiffness in the arm and shoulder; Sudeck's syndrome, which is prevalent in older people and in women and is characterized by bone changes and muscular atrophy, but is not always associated with trauma; and Steinbrocker's syndrome, which affects both sexes but is slightly more prevalent in females, and includes such symptoms as gradual stiffness, discomfort, and weakness in the shoulder and hand.
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