Hand Surgery Center of Brooklyn and Staten Island

Reflex Sympathetic Dystrophy

Reflex Sympathetic Dystrophy (Complex Regional Pain Syndrome 1)

Causalgia (Complex Regional Pain Syndrome 2) Back to home page

Complex regional pain syndrome is poorly understood by the patients, their families, and their healthcare and legal professionals. CRPS remains an enigma and is difficult to treat, often leaving the physician frustrated, the patient severely compromised and the lawyers exploring facts unknown to the doctors and research scientists.

What is complex regional pain syndrome?
What are the symptoms of CRPS?
What causes CRPS?
How is CRPS diagnosed?
What is the prognosis?
How is CRPS treated?
Is research currently being done on CRPS?
Where can I get more information?

What is complex regional pain syndrome?

Complex regional pain syndrome (CRPS) is a chronic pain condition that is believed to be the result of dysfunction in the central AND peripheral nervous systems. We now recognize that there are structural, chemical and functional changes in the brain and spinal cord that serve to maintain and amplify the experience of pain. Typical features include dramatic changes in the SKIN and JOINTS. There is change in color and temperature of the skin over the affected limb. There is intense burning pain and skin sensitivity. Sweating is increased or decreased. Joints are painful, swollen and stiff. CRPS I is frequently triggered by tissue injury; and the term describes all patients with the above symptoms but with no underlying nerve injury. Patients with CRPS II (causalgia) experience the same symptoms but they are clearly associated with a nerve injury.

Older terms used to describe CRPS are “reflex sympathetic dystrophy syndrome” and “causalgia.” Causalgia was a term first used during the Civil War to describe the intense, hot, burning pain felt by some male veterans after nerve injuries.

CRPS can strike at any age and affects both men and women, although most experts agree that today it is more common in young women.

What are the symptoms of CRPS?

Normally, after an injury, pain gradually subsides aver a few days or weeks. The key symptom of CRPS is continuous, intense pain out of proportion to the severity of the injury, which gets worse rather than better over time. CRPS most often affects one of the extremities (arms, legs, hands, or feet) and is also often accompanied by:

1. “burning” pain

2. increased skin sensitivity

3. changes in skin temperature: warmer or cooler compared to the opposite extremity

4. changes in skin color: often blotchy, purple, pale, or red

5. changes in skin texture: shiny and thin, and sometimes excessively sweaty

6. changes in nail and hair growth patterns

7. pain, swelling and stiffness in affected joints with decreased mobility

Often the pain spreads from the hand to include the entire arm (or leg), even though the initiating injury might have been only to a finger or toe. It is usually causes and is heightened by emotional stress.

The symptoms of CRPS vary in severity and length. Some experts believe there are three stages associated with CRPS, marked by progressive changes in the skin, muscles, joints, ligaments, and bones of the affected area, although this progression has not yet been validated by clinical research studies.

Stage one is thought to last from 1 to 3 months and is characterized by severe, burning pain, along with muscle spasm, joint stiffness, rapid hair growth, and alterations in the blood vessels that cause the skin to change color and temperature.

Stage two lasts from 3 to 6 months and is characterized by intensifying pain, swelling, decreased hair growth, cracked, brittle, grooved, or spotty nails, softened bones, stiff joints, and weak muscle tone.

In stage three the syndrome progresses to the point where changes in the skin and bone are no longer reversible. Pain becomes unyielding and may involve the entire limb or affected area. There may be marked muscle loss (atrophy), severely limited mobility, and involuntary contractions of the muscles and tendons that flex the joints. Limbs may become contorted.

What causes CRPS?

The most common cause of CRPS 1 (RSD) in hand surgery is a fracture. Wrist fracture is the most common fracture that causes RSD. It occurs after any type of injury including surgical trauma.

Doctors aren’t sure what causes CRPS after injury. In some cases the sympathetic nervous system injury plays an important role in sustaining the pain. The most recent theories suggest that pain receptors in the affected part of the body become over responsive to a family of normally existing nervous system messengers known as catecholamines. Animal studies indicate that norepinephrine, a catecholamine released from sympathetic nerves, acquires the capacity to activate pain pathways after tissue or nerve injury. The incidence of sympathetically maintained pain in CRPS is not known. Some experts believe that the importance of the sympathetic nervous system is different at different stages of the disease.

Another theory is that after injury in CRPS II, there is triggering of the immune response, which leads to the characteristic inflammatory symptoms of redness, warmth, and swelling in the affected area. CRPS may represent a disruption of the nomal healing process. In all likelihood, CRPS does not have a single cause, but is rather the result of multiple causes that produce similar symptoms. It may be genetically related.

How is CRPS diagnosed?

CRPS is diagnosed primarily through observation of the signs and symptoms. Many other conditions have similar symptoms, and it can be difficult for doctors to make a firm diagnosis of CRPS early in the course of the disorder when symptoms are few or mild. Diagnosis is further complicated by the fact that some people will improve gradually over time without treatment.

There is no specific diagnostic test for CRPS. Some doctors use triple-phase bone scans to identify changes in the bone, but this does not change the treatment.

What is the prognosis?

The prognosis for CRPS varies from person to person. Spontaneous remission from symptoms occurs in certain people. Others can have unremitting pain and crippling, irreversible changes in spite of treatment. Some doctors believe that early treatment is helpful in limiting the disorder, but this belief has not yet been supported by evidence from clinical studies. More research is needed to understand the causes of CRPS, how it progresses, and the role of early treatment.

How is CRPS treated?

Because there is no known cause for CRPS, treatment is aimed at relieving painful symptoms.

1. Occupational and Physical therapy: A gradually increasing exercise program to keep the painful limb moving may help to relieve pain, restore some range of motion and improve function and is the mainstay of initial treatment program. I like patients to lift a broom of increasing weight as high as possible and hit it against the floor as hard as they can within pain tolerance. I tell the patient to be able to keep a smile on the lips and faith in providence while doing this. A happy smile ensures that this exercise or any other is done within patients pain tolerance and the exercise is not worse than her own pain and does not result in increase in pain at the end of an exercise session. It is important to adjust intensity and frequency of exercises based on patient’s pain tolerance. This is the mainstay of treatment in my practice, because this has resolves pain and improved joint motion in most patients. I refer all my patients to a certified hand therapist, Lynn Bassini that works next door to my office in Brooklyn. She uses passive treatments (massage, TENS, acupressure, contrast baths, edema control treatments) and active treatments ( desensitization techniques, active exercises, assisted active exercises, stress loading exercises like scrubbing and sponging, activities of daily living and finally functional activities). This allows me to closely follow progress of pain and mobility of joints and modify treatment as necessary. My direction of treatment is to teach self management techniques through compassion, motivation and hope.

2. Psychotherapy: CRPS often has profound psychological effects on people and their families. Those with CRPS may suffer from depression, anxiety, or post-traumatic stress disorder, all of which magnify the perception of pain and make rehabilitation efforts more difficult. Patients often think that they do not have psychological pain and they are right but psychotherapy may help them to deal with their pain and suffering. Cognitive/psychosocial/behavioral treatment approaches are part of multidisciplinary pain management teams.

3. Sympathetic nerve blocks: Some patients will get significant pain relief from sympathetic nerve blocks. Sympathetic blocks can be done in a variety of ways. One technique involves intravenous administration of phentolamine, a drug that blocks sympathetic receptors. Another technique involves injection of an anesthetic close to the spine and to directly block the sympathetic nerves.

4. Medications: Many different classes of medication are used to treat CRPS. Topical analgesic drugs like Bengay act locally on painful skin, nerves, ligaments, and muscles. Antiseizure drugs like neurontin and lyrica soothe nerves and relieve the pain in many patients. Anti depressant drugs have a very good analgesic property and many of my patients express awe and surprise at the pain relief. Some patients are benefited by a short course of corticosteroids like prednisone. I prescribe anti-inflammatory medications (Motrin) and opioids (codeine) in all patients at one time or another. However, no single drug or combination of drugs has produced consistent long-lasting improvement in symptoms in all patients.

5. Intrathecal drug pumps: These devices administer drugs directly to the spinal fluid, so that opioids and local anesthetic agents can be delivered to pain-signaling targets in the spinal cord at doses far lower than those required for oral administration. This technique decreases side effects and increases drug effectiveness.

6. Spinal cord stimulation: The placement of stimulating electrodes next to the spinal cord provides a pleasant tingling sensation in the painful area just as does topical analgesic drugs and transcutaneous nerve stimulation. This technique appears to help some but not too many patients with their pain. I have yet to recommend this treatment in one of my patients.

7. Surgical sympathectomy: The use of surgical sympathectomy, a technique that destroys the nerves involved in CRPS, is controversial. I have not used it because many experts think it is unwarranted and makes CRPS worse; others report a favorable outcome. Sympathectomy may be used as a last resort, and only in patients whose pain is dramatically relieved by selective sympathetic blocks. I have yet to recommend this treatment in my patients.

Is research currently being done on CRPS?

The National Institute of Neurological Disorders and Stroke (NINDS), a component of the National Institutes of Health (NIH), supports and conducts research on the brain and central nervous system. Some studies are conducted at the Institute’s laboratories and clinics on the NIH campus in Bethesda, Maryland. Others are funded through grants to major medical institutions across the country. NINDS-supported scientists are studying new approaches to treat CRPS and intervene more aggressively after traumatic injury to lower the chances of developing the disorder. Other studies to overcome chronic pain syndromes are discussed in the NINDS pamphlet, “Pain: Hope through Research.”

Where can I get more information?

For more information on neurological disorders and research programs funded by the National Institute of Neurological Disorders and Stroke, contact NIH at the Institute’s Brain Resources and Information Network (BRAIN) at:

BRAIN
P.O. Box 5801
Bethesda, MD 20824
(800) 352-9424
http://www.ninds.nih.gov

Information also is available from the following organizations:

American Chronic Pain Association (ACPA)
P.O. Box 850
Rocklin, CA 95677-0850
ACPA@pacbell.net
http://www.theacpa.org
Tel: 916-632-0922
800-533-3231
Fax: 916-632-3208

Reflex Sympathetic Dystrophy Syndrome Association (RSDSA)
P.O. Box 502
99 Cherry Street
Milford, CT 06460
info@rsds.org
http://www.rsds.org
Tel: 203-877-3790 877-662-7737
Fax: 203-882-8362

American RSDHope Organization
P.O. Box 875
Harrison, ME 04040-0875
rsdhope@roadrunner.com
http://www.rsdhope.org
Tel: 207-583-4589

National Foundation for the Treatment of Pain
P.O. Box 70045
Houston, TX 77270
NFTPain@cwo.com
http://www.paincare.org
Tel: 713-862-9332
Fax: 713-862-9346

American Pain Foundation
201 North Charles Street
Suite 710
Baltimore, MD 21201-4111
info@painfoundation.org
http://www.painfoundation.org
Tel: 888-615-PAIN (7246)
Fax: 410-385-1832

International Research Foundation for RSD/CRPS
USF Medical Clinics c/o Dr. A. Kirkpatrick
12901 Bruce Downs Blvd., MDC59
Tampa, FL 33612
info@rsdfoundation.org
http://www.rsdfoundation.org
Tel: 813-907-2312
Fax: 813-830-7446

Mayday Fund [For Pain Research]
c/o SPG
136 West 21st Street, 6th Floor
New York, NY 10011
mayday@maydayfund.org
http://www.painandhealth.org
Tel: 212-366-6970
Fax: 212-366-6979
http://familydoctor.org/online/famdocen/home/common/pain/disorders/551.html
http://www.chronicpainsupport.org/
http://www.medicinenet.com/neuropathic_pain/article.htm

http://en.wikipedia.org/wiki/Chronic_pain

Modified from document prepared by:

Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892

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