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Reflex Sympathetic Dystrophy Syndrome

Definition :
Complex regional pain syndrome (CRPS) is an uncommon, chronic condition that usually affects your arm or leg. Rarely, the disease can affect other parts of your body. You may experience intense burning or aching pain along with swelling, skin discoloration, altered temperature, abnormal sweating and hypersensitivity in the affected area.

The nature of complex regional pain syndrome is puzzling, and the cause isn't clearly understood.

Women are more likely to be affected by complex regional pain syndrome than men are. Although complex regional pain syndrome is most common in people between the ages of 40 and 60, it can occur at any age. Treatment for complex regional pain syndrome is most effective when started early in the course of the syndrome.

Causes:
Complex regional pain syndrome occurs in two types with similar signs and symptoms, but different Causes:
  • Type I. Previously known as reflex sympathetic dystrophy syndrome, this type occurs after an illness or injury that didn't directly damage the nerves in your affected limb. About 90 percent of people withcomplex regional pain syndrome have type I.
  • Type II. Once referred to as causalgia, this type follows a distinct nerve injury.

Many cases of complex regional pain syndrome occur after a forceful trauma to an arm or a leg, such as a gunshot wound or shrapnel blast. Other major and minor traumas — surgery, heart attacks, infections, fractures and even sprained ankles — also can lead to complex regional pain syndrome. It's not well understood why these injuries sometimes trigger complex regional pain syndrome.

The syndrome was first described after the U.S. Civil War when soldiers continued to report severe pain after their wounds had healed. It was often referred to as "hot pain" during that period.

When to seek medical advice :
If you experience constant, severe pain that affects a single limb and makes touching or movement of that limb seem intolerable, see your doctor to determine the cause. It's important to treat complex regional pain syndrome early.

Symptoms :
The main symptom of complex regional pain syndrome is intense pain, often described as "burning." Additional signs and symptoms include :
  • Skin sensitivity.
  • Changes in skin temperature, color and texture. At times your skin may be sweaty; at other times it may be cold. Skin color can range from white and mottled to red or blue. Skin may become tender, thin or shiny in the affected area.
  • Changes in hair and nail growth.
  • Joint stiffness, swelling and damage.
  • Muscle spasms, weakness and loss (atrophy).
  • Decreased ability to move the affected body part.

Complex regional pain syndrome typically has three stages, though not everyone progresses through these phases at the same pace :

  • Stage 1. Severe pain develops in one of your limbs. Swelling, sensitivity to touch or to cold, and skin changes, such as drying or thinning, begin to appear. This stage usually lasts one to three months.
  • Stage 2. Changes to the color and texture of your skin become increasingly obvious, and the swelling spreads. You may begin to feel stiffness in your muscles and joints. This stage may last three to six months.
  • Stage 3. Severe damage is evident, such as limited movement in your affected limb, irreversible skin damage, muscle atrophy and contractures in nearby digits.

Diagnosis:
Complex regional pain syndrome typically has three stages, though not everyone progresses through these phases at the same pace :

  • Stage 1. Severe pain develops in one of your limbs. Swelling, sensitivity to touch or to cold, and skin changes, such as drying or thinning, begin to appear. This stage usually lasts one to three months.
  • Stage 2. Changes to the color and texture of your skin become increasingly obvious, and the swelling spreads. You may begin to feel stiffness in your muscles and joints. This stage may last three to six months.
  • Stage 3. Severe damage is evident, such as limited movement in your affected limb, irreversible skin damage, muscle atrophy and contractures in nearby digits.
  • Bone scan. A radioactive substance injected into one of your veins permits viewing of your bones with a special camera. This procedure may show increased circulation to the joints in the affected area.
  • Sympathetic nervous system tests. These tests look for disturbances in your sympathetic nervous system. For example, thermography measures the skin temperature and blood flow of your affected and unaffected limbs. Other tests can measure the amount of sweat on both limbs. Dissimilar results can indicate complex regional pain syndrome.
  • X-rays. Loss of minerals from your bones may show up on an X-ray in later stages of the disease.
  • Magnetic resonance imaging (MRI). Images captured by an MRI device may show a number of tissue changes, such as skin thinning and muscle atrophy, that may help your doctor determine the stage of your disease.

Complications :
If complex regional pain syndrome isn't diagnosed and treated at an early stage, the disease may progress to more disabling signs and symptoms. If you avoid moving an arm or a leg because of pain, or if you have trouble moving a limb because of stiffness, your skin and muscles may begin wasting (atrophy). You may also experience tightening of your muscles. This may lead to a condition in which your hand and fingers or your foot and toes contract into a fixed position.

The illness may also spread from its source to elsewhere in your body in these patterns :

  • Continuity type. The symptoms may migrate from the initial site of the pain — for example, from your hand to your shoulder, trunk and face — affecting a quadrant of your body.
  • Mirror-image type. The symptoms may spread from one limb to the opposite limb.
  • Independent type. Sometimes, the symptoms may leap to a distant part of your body.

Treatment:
Dramatic improvement and even remission of complex regional pain syndrome is possible if treatment begins within a few months of your first symptoms. Treatment options include :

  • Medications. Doctors use various medications to treat the symptoms of complex regional pain syndrome. Over-the-counter nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin, ibuprofen (Advil, Motrin, others) and naproxen sodium (Aleve), may ease pain and inflammation. In some cases, doctors may recommend prescription medications. For example, antidepressants such as amitriptyline and anticonvulsants such as gabapentin (Neurontin) are used to treat pain that originates from a damaged nerve (neuropathic pain). Corticosteroids, such as prednisone, may reduce inflammation.

    Your doctor may suggest bone-loss medications, such as alendronate (Fosamax) and calcitonin (Miacalcin). Opioid medications may be another option. Taken in appropriate doses, they may provide acceptable control of pain. However, they may not be appropriate if you have a history of substance abuse or lung disease.

    Some pain medications, such as COX-2 inhibitors (Celebrex), may increase your risk of heart attack and stroke. It's wise to discuss your individual risk profile with your doctor.

  • Applying heat and cold. Applying cold may relieve swelling and sweating. If the affected area is cool, applying heat may offer relief.
  • Capsaicin. This cream, made from the seeds of hot chili peppers, may relieve pain caused by nerve damage in early-stage complex regional pain syndrome. Your doctor may recommend applying the cream to the affected area several times daily. Capsaicin cream can be very irritating if rubbed on nonaffected parts of your body. Follow the application instructions carefully. You should be able to tell within a week whether the treatment is effective and tolerable.
  • Physical therapy. Gentle, guided exercising of the affected limbs may improve range of motion and strength. The earlier the disease is diagnosed, the more effective exercises may be.
  • Sympathetic nerve-blocking medication. Injection of an anesthetic to block pain fibers in your affected nerves may relieve pain in some people.
  • Transcutaneous electrical nerve stimulation (TENS). Chronic pain is sometimes eased by applying electrical impulses to nerve endings.
  • Biofeedback. In some cases, learning biofeedback techniques may help. In biofeedback, you learn to become more aware of your body so that you can relax your body and relieve pain.
  • Spinal cord stimulation. Your doctor inserts tiny electrodes along your spinal cord. A small electrical current delivered to the spinal cord sometimes results in pain relief.
 
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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