RSD-Reflex Sympathetic Dystrophy

Reflex sympathetic dystrophy (RSD), also called complex regional pain syndrome (CRPS), is a chronic, painful, and progressive neurological condition that affects the skin, muscles, joints, and bones. The syndrome usually develops in an injured limb, such as a broken leg, or following surgery. However, many cases of RSD involve only a minor injury, such as a sprain. And in some cases, no precipitating event can be identified. RSD/CRPS is characterized by various degrees of burning pain, excessive sweating, swelling, and sensitivity to touch. Pain may begin in one area or limb and then spread to other limbs. In some cases, symptoms of RSD/CRPS diminish for a period of time and then reappear with a new injury.

Types

Two types of RSD/CRPS have been defined:

Type 1—without nerve injury

Type 2 (formerly called causalgia)—with nerve injury

Both types of RSD/CRPS share the same signs and symptoms.

Incidence and Prevalence

Millions of people in the United States may suffer from RSD/CRPS. This chronic pain syndrome affects both men and women, and also occurs in children. It can occur at any age, but usually affects people between the ages of 40 and 60 years. The National Institute of Neurological Disorders and Strokes (NINDS) reports that 2–5% of peripheral nerve injury patients and 12–21% of patients with paralysis on one side of the body (hemiplegia) develop reflex sympathetic dystrophy as a complication. The Reflex Sympathetic Dystrophy Syndrome Association of America (RSDSA) reports that the condition develops after 1–2% of bone fractures.

RSD/CRPS Causes and Risk Factors

RSD/CRPS appears to involve a complex interaction among the sensory, motor, and autonomic nervous systems, and the immune system. It is thought that brain and spinal cord (central nervous system) control over these various processes is somehow changed as a result of an injury. Causes and risk factors associated with the onset of RSD/CRPS include the following:

Cerebral lesions

Heart disease, heart attack

Infection

Paralysis on one side of the body (hemiplegia)

Radiation therapy

Repetitive motion disorder (e.g., carpal tunnel syndrome)

Spinal cord disorders

Surgery

Trauma (e.g., bone fracture, gunshot and shrapnel wounds)

In 10–20% of cases, no direct cause for RSD/CRPS can be found. Injury that precedes the onset of the condition may or may not be significant.

Treatment

The goals of RSD/CRPS treatment are to control pain and maintain as much mobilization of the affected limb as possible. Treatment involves an individualized treatment plan, which often combines medications, physical therapy, nerve blocks, and psychosocial support. Medication Treatment for RSD/CRPS involves medications to control pain. The type of medication prescribed is determined by the type of pain experienced by the patient. Constant pain caused by inflammation is treated using nonsteroidal anti-inflammatory drugs (NSAIDs; e.g., aspirin, ibuprofen, naproxen, indomethacin). Due to potenially severe gastrointestinal and cardiovascular side effects, NSAIDs should only be used as instructed. Constant pain not caused by inflammation may be treated with central acting agents, such as tramadol (Ultram®). Sharp pain and pain that disrupts sleep may be treated with antidepressants (e.g., amitriptyline, doxepin, nortriptyline, trazodone) or anticonvulsants (e.g., carbamazapine, pregabalin). In some cases, generalized, severe pain that does not respond to other medications may be treated with opioids (e.g., propoxyphine, codeine, morphine). Muscle cramps (spasms and dystonia) can be treated using clonazepam and baclofen. Localized pain related to nerve injury may be treated with Capsaicin® cream, but the effectiveness of this medication to treat RSD/CRPS has not been proven. Medications that affect the sympathetic nervous system such as clonidine (Catapres®) also may be useful in some cases. Muscle stiffness may be treated with muscle relaxants such as: Tizanidine (Zanaflex®) Baclofen Clonazepam (Klonopin®)

Physical Therapy

Physical therapy for RSD/CRPS involves daily range of motion exercises. Patients are advised to avoid activities that could accelerate osteoporosis or cause joint injury.

Nerve Block

Sympathetic nerve block interrupts the transmission of pain signals from a group of nerve cell bodies (called a ganglion). Nerve block should be performed by a physician who is familiar with the technique. This procedure involves inserting a needle into the appropriate location (e.g., alongside the windpipe [trachea], in the lower [lumbar] spine) and injecting an alpha adrenergic antagonist (type of anesthesia) into the ganglion. When treating an upper extremity, this treatment is called a stellate ganglion block. The effects of the nerve block are monitored over time.

Sympathectomy

RSD/CRPS patients who have a good but temporary response to nerve block may be candidates for a surgical procedure called sympathectomy. This procedure involves cutting and sealing (cauterizing) a portion of the sympathetic nerve, which runs down the spine. The goal of treatment is to suppress sympathetic nervous system activity in the affected area. The role of sympathectomy to treat RSD/CRPS is controversial and in some cases, the procedure worsens symptoms.

TENS Unit

A transcutaneous electrical nerve stimulation (TENS) unit may be used to treat the affected area in patients who have RSD/CRPS. In some cases, a spinal cord stimulator is permanently implanted. This device supplies low-intensity impulses to a location in the spinal cord to interrupt the pain signals that are being transmitted to the brain.

Psychosocial Support

RSD/CRPS patients often become depressed and anxious because of chronic pain and reduced physical ability. Counseling, support groups, and chronic pain center programs can help patients learn coping strategies and can provide emotional and psychological support.

Patient Information about RSD/CRPS

Reflex sympathetic dystrophy (RSD), also called complex regional pain syndrome (CRPS) or causalgia, is a chronic, progressive nerve disorder. Patients who have RSD/CRPS experience severe pain and other symptoms (e.g., changes in skin temperature, color, or sensitivity, swelling), often with no known cause. In some cases, the disorder develops following an injury. RSD/CRPS usually affects the arms, legs, hands, and feet, but it can occur in any part of the body.RSD/CRPS can be difficult to diagnose and the course of the disorder often is unpredictable. Symptoms of reflex sympathetic dystrophy/complex regional pain syndrome may worsen over time and can be disabling. It is important for patients with RSD/CRPS to work with their health care team to develop an effective treatment plan. Here are some questions to ask your doctor (e.g., neurologist, pain management specialist) about RSD/CRPS. Print this page, check off the questions you would like answered, and take it with you to your doctor appointment. The more knowledge you have about RSD/CRPS, the easier it is to make important decisions that can help reduce your pain and other symptoms.

Questions to Ask Your Doctor about RSD/CRPS

What is RSD/CRPS?

Why do you suspect that I have reflex sympathetic dystrophy/complex regional pain syndrome?

What other conditions might be causing my symptoms?

Which diagnostic tests will be performed to determine if I have RSD/CRPS and to rule out other causes for my symptoms?

What do these tests involve?

How should I prepare for these diagnostic tests and procedures?

Should I see a doctor who specializes in RSD/CRPS? Why or why not?

What type of RSD/CRPS do I have? Is my condition classified as type I or type II?

Might lifestyle changes help reduce my symptoms? If so, what modifications do you recommend?

What treatment(s) do you recommend to reduce my RSD/CRPS symptoms?

Why do you recommend these treatments?

What are the possible benefits, risks, and complications of treatment?

Do you recommend physical therapy? Why or why not?

Will medication be used to treat my RSD/CRPS? If so, which medications?

How will these medicines be administered?

What are the common side effects of these medications? What should I do if I develop severe side effects? Telephone number to call:

If my symptoms continue to worsen in spite of treatment, what other treatment options are available?

Might I require surgery to treat my RSD/CRPS? Why or why not?

What does surgery to treat reflex sympathetic dystrophy/complex regional pain syndrome involve?

Are there any clinical trials available for patients with RSD/CRPS? If so, do you recommend that I participate in a clinical trial? Why or why not?

Can you recommend a local or online support group for patients, family members, and caregivers?

Can you recommend additional resources for information about RSD/CRPS?

Signs and Symptoms

The symptoms of RSD/CRPS often progress in three stages—acute, dystrophic, and atrophic. The acute stage occurs during the first 1–3 months and may include burning pain, swelling, increased sensitivity to touch, increased hair and nail growth in the affected region, joint pain, and color and temperature changes. The dystrophic stage may involve constant pain and swelling. The affected limb may feel cool to the touch and appear bluish in color. Muscle stiffness, wasting of the muscles (atrophy), and early bone loss (osteoporosis) also may occur. This stage usually develops 3–6 months after onset of the disorder. During the atrophic stage, the skin becomes cool and shiny, increased muscle stiffness and weakness occur, and symptoms may spread to another limb.

Characteristic signs and symptoms of sympathetic nervous system involvement include the following:

Burning pain Extreme sensitivity to touch Skin color changes (red or bluish) Skin temperature changes (hot or cold) Pain caused by RSD/CRPS usually in not proportionate to the degree of injury. It can be triggered by disuse of the affected limb or by stress and can be spontaneous or constant.

Symptoms associated with an immune reaction include:

Joint pain Redness Swelling (edema) Frequent infections

Signs of motor system dysfunction include the following:

Difficulty starting movement Increased muscle tone, stiffness Muscle spasm Tremor Weakness

Other symptoms of RSD/CPRS include the following:

Dermatitis, eczema (inflammation of the skin) Excessive sweating Fatigue Migraine headache

Complications

Patients with any chronic illness, including RSD/CRPS, often suffer from depression and anxiety. Skin, muscle, and bone atrophy (wasting) are possible complications of this syndrome. Atrophy may occur because of reduced function of the affected limb.

RSD/CRPS Diagnosis

RSD/CRPS can be difficult to diagnose. Diagnosis often involves taking a thorough medical history and performing a neurological examination and requires excluding other conditions that produce similar symptoms (called differential diagnosis). During the neuro exam, the clinician may notice that the response to mild sensory stimuli produces severe pain. Physical examination involves observing the skin color and temperature, any swelling, vascular reactivity, overgrown and grooved nails, swollen and stiff joints, and muscle weakness and atrophy. Other conditions are ruled out with appropriate testing, such as triple phase bone scan, magnetic resonance imaging (MRI scan), a full laboratory panel, electrophysiological studies of the nerves and muscles (e.g., EMG, NCV), and a thermogram (test that uses an infrared video camera to measure the emission of heat from the affected limb). EMG and NCV tests can be used in combination and are often referred to as EMG/NCV studies.

Pinched Nerve

Differential diagnosis for RSD/CRPS sometimes involves ruling out a pinched nerve. Pinched nerves occur when surrounding tissues, such as bones, muscles, or tendons, place too much pressure on a nerve, resulting in nerve compression or nerve entrapment. Conditions like diabetes, osteoarthritis, and obesity, as well as repetitive motions and overuse, can increase the risk for nerve disorders (neuropathies), including pinched nerves. Nerve compression can affect any nerve in the body and cause symptoms similar to those caused by RSD/CRPS. Compression of one of the nerves of the spine (called spinal nerves) can cause pain that radiates (spreads) from the neck, upper back, or lower back to the arm(s) or leg(s); decreased sensation; numbness and tingling; and muscle weakness. Compression of a spinal nerve can result from a herniated disc, arthritis of the spine, spinal stenosis (narrowing of the spinal canal), or bone spurs (bony protrusions in the spine). Conditions that involve nerve compression in other areas of the body include carpal tunnel syndrome (caused by a compressed nerve in the wrist), Morton’s neuroma (caused by nerve compression in the foot), and cubital tunnel syndrome (caused by a compressed nerve in the elbow). Diagnostic tests that may be used to detect a pinched nerve include electromyography (EMG), nerve conduction velocity (NCV) studies, and imaging tests (e.g., computed tomography, magnetic resonance imaging). EMG and NCV help evaluate nerve and muscle function. CT scan and MRI scan are used to create detailed images of internal tissues to help locate areas of nerve compression. Treatment for a pinched nerve depends on the cause and severity of the condition. In many cases, conservative treatment, such as resting the affected, applying ice, and taking medications (e.g., nonsteroidal anti-inflammatory drugs [NSAIDs], muscle relaxants) helps to reduce pinched nerve symptoms. Physical therapy, stress management techniques, chiropractic adjustments, and cortisone injections also may be helpful. In severe cases, surgery to relieve pressure on the nerve may be necessary. The type of surgery performed depends on the location of the pinched nerve.