CRPS / RSD

What is complex regional pain syndrome (CRPS / RSD)?

Complex regional pain syndrome (CRPS) is a chronic pain condition most often affecting one of the limbs (arms, legs, hands, or feet), usually after an injury or trauma to that limb. CRPS is believed to be caused by damage to, or malfunction of, the peripheral and central nervous systems. The central nervous system is composed of the brain and spinal cord, and the peripheral nervous system involves nerve signaling from the brain and spinal cord to the rest of the body. CRPS is characterized by prolonged or excessive pain and mild or dramatic changes in skin color, temperature, and/or swelling in the affected area.

There are two similar forms, called CRPS-I and CRPS-II, with the same symptoms and treatments. CRPS-II (previously called causalgia) is the term used for patients with confirmed nerve injuries. Individuals without confirmed nerve injury are classified as having CRPS-I (previously called reflex sympathetic dystrophy syndrome). Some research has identified evidence of nerve injury in CRPS-I, so the validity of the two different forms is being investigated.

CRPS symptoms vary in severity and duration. Studies of the incidence and prevalence of the disease show that most cases are mild and individuals recover gradually with time. In more severe cases, individuals may not recover and may have long-term disability.

What are the symptoms of CRPS?

The key symptom is prolonged pain that may be constant and, in some people, extremely uncomfortable or severe. The pain may feel like a burning or “pins and needles” sensation, or as if someone is squeezing the affected limb. The pain may spread to include the entire arm or leg, even though the precipitating injury might have been only to a finger or toe. Pain can sometimes even travel to the opposite extremity. There is often increased sensitivity in the affected area, such that even light touch or contact is painful (called allodynia).

People with CRPS also experience constant or intermittent changes in temperature, skin color, and swelling of the affected limb. This is due to abnormal microcirculation caused by damage to the nerves controlling blood flow and temperature. An affected arm or leg may feel warmer or cooler compared to the opposite limb. The skin on the affected limb may change color, becoming blotchy, blue, purple, pale, or red.

Other common features of CRPS include:

• changes in skin texture on the affected area; it may appear shiny and thin
• abnormal sweating pattern in the affected area or surrounding areas
• changes in nail and hair growth patterns
• stiffness in affected joints
• problems coordinating muscle movement, with decreased ability to move the affected body part, and
• abnormal movement in the affected limb, most often fixed abnormal posture (called dystonia) but also tremors in or jerking of the affected limb.

How is CRPS diagnosed?

Currently there is no single diagnostic test to confirm CRPS. Diagnosis is based on the affected individual’s medical history and signs and symptoms that match the definition. But because several other conditions can cause similar symptoms, careful examination is important. Since most people improve gradually over time, diagnosis may be more difficult later in the course of the disorder.

Testing also may be used to help rule out other conditions, such as arthritis syndromes, Lyme disease, generalized muscle diseases, a clotted vein, or small nerve fiber polyneuropathies (such as from diabetes), because these require different treatment. The distinguishing feature of CRPS is usually a history of earlier injury to the affected area, as most of these other conditions are not triggered by injury. Individuals without a history of injury should be carefully examined to make sure that another treatable diagnosis is not missed.

Magnetic resonance imaging or triple-phase bone scans sometimes identify CRPS-characteristic changes in the bone metabolism. CRPS is often associated with excess bone resorption, a process in which certain cells break down the bone and release calcium into the blood.

Spinal cord stimulation. Placing stimulating electrodes through a needle into the spine near the spinal cord provides a tingling sensation in the painful area. Typically the electrode is placed temporarily for a few days to assess whether stimulation will be helpful. Minor surgery is required to implant all the parts under the skin on the torso. Once implanted, the stimulator can be turned on and off, and adjusted using an external controller. Data shows that about one-fourth of individuals develop equipment problems that may require additional surgeries.

Peer-Reviewed Research

Kemler, M. A., et al. (2008). “Effect of spinal cord stimulation for chronic complex regional pain syndrome Type I: five-year final follow-up of patients in a randomized controlled trial.” J Neurosurg 108(2): 292-298.

OBJECT: Chronic complex regional pain syndrome-Type I (CRPS-I) is a painful, disabling disorder for which no treatment with proven effect is available. In the present randomized controlled trial, the authors assessed the effectiveness of spinal cord stimulation (SCS) in reducing pain due to CRPS-I at the 5-year follow-up. METHODS: The authors performed a randomized trial in a 2:1 ratio in which 36 patients with CRPS-I were allocated to receive SCS and physical therapy (PT) and 18 patients to receive PT alone. Twenty-four patients who received SCS+PT also underwent placement of a permanent spinal cord stimulator after successful test stimulation; the remaining 12 patients did not receive a permanent stimulator. The authors assessed pain intensity, global perceived effect, treatment satisfaction, and health-related quality of life. Patients were examined before randomization, before implantation, and every year until 5 years thereafter. Ten patients were excluded from the final analysis. RESULTS: At 5 years posttreatment, SCS+PT produced results similar to those following PT for pain relief and all other measured variables. In a subgroup analysis, the results with regard to global perceived effect (p=0.02) and pain relief (p=0.06) in 20 patients with an implant exceeded those in 13 patients who received PT. CONCLUSIONS: Despite the diminishing effectiveness of SCS over time, 95% of patients with an implant would repeat the treatment for the same result.

Kemler, M. A., et al. (2000). “Spinal cord stimulation in patients with chronic reflex sympathetic dystrophy.” N Engl J Med 343(9): 618-624.

BACKGROUND: Chronic reflex sympathetic dystrophy (also called the complex regional pain syndrome) is a painful, disabling disorder for which there is no proven treatment. In observational studies, spinal cord stimulation has reduced the pain associated with the disorder. METHODS: We performed a randomized trial involving patients who had had reflex sympathetic dystrophy for at least six months. Thirty-six patients were assigned to receive treatment with spinal cord stimulation plus physical therapy, and 18 were assigned to receive physical therapy alone. The spinal cord stimulator was implanted only if a test stimulation was successful. We assessed the intensity of pain (on a visual-analogue scale from 0 cm [no pain] to 10 cm [very severe pain]), the global perceived effect (on a scale from 1 [worst ever] to 7 [best ever]), functional status, and the health-related quality of life. RESULTS: The test stimulation of the spinal cord was successful in 24 patients; the other 12 patients did not receive implanted stimulators. In an intention-to-treat analysis, the group assigned to receive spinal cord stimulation plus physical therapy had a mean reduction of 2.4 cm in the intensity of pain at six months, as compared with an increase of 0.2 cm in the group assigned to receive physical therapy alone (P<0.001 for the comparison between the two groups). In addition, the proportion of patients with a score of 6 (“much improved”) for the global perceived effect was much higher in the spinal cord stimulation group than in the control group (39 percent vs. 6 percent, P=0.01). There was no clinically important improvement in functional status. The health-related quality of life improved only in the 24 patients who actually underwent implantation of a spinal cord stimulator. Six of the 24 patients had complications that required additional procedures, including removal of the device in 1 patient. CONCLUSIONS: In carefully selected patients with chronic reflex sympathetic dystrophy, electrical stimulation of the spinal cord can reduce pain and improve the health-related quality of life.

Harke, H., et al. (2005). “Spinal cord stimulation in sympathetically maintained complex regional pain syndrome type I with severe disability. A prospective clinical study.” Eur J Pain 9(4): 363-373.

BACKGROUND AND PURPOSE: In this prospective trial we assessed the long-term effect of spinal cord stimulation (SCS) on the improvement of functional status in complex regional pain syndrome type I (CRPS I). METHODS: A prerequisite for eligibility to SCS treatment was the responsiveness of patients to sympathetic nerve block. In 29 patients with chronic sympathetically maintained CRPS I, the efficacy of SCS on deep pain, allodynia and functional disability was determined. Pain intensity was estimated during SCS free intervals of 45 min (inactivation test) every 3 months and compared with that under SCS treatment. RESULTS: On SCS treatment, both deep pain and allodynia could be permanently reduced from 10 to 0-2 on a 10 cm visual analogue scale (VAS) (p<0.01). During the inactivation tests, reoccurrence of pain up to 8 VAS (quartiles 6-8) was measured. Considerable impairments in daily living activities, objectified by the pain disability index, were also restored (p<0.01). After a follow-up period of 35.6+/-21 months, 12 of 16 patients with affected upper limb showed significant increase of the fist grip strength from 0 to 0.35 (quartiles 0.1-0.5) kg compared with 0.9 (quartiles 0.7-1.1) kg on the unaffected side (p<0.01). Eight of ten patients with lower limb disability resumed walking without crutches. Previous pain medication could be significantly reduced (p<0.01). CONCLUSIONS: As a result of permanent pain relief under long-term SCS combined with physiotherapy, the functional status and the quality of life could be significantly improved in sympathetically maintained CRPS I.