Commonly referred to as CRPS, complex regional pain syndrome is a chronic pain condition characterized by severe pain, swelling and skin changes. It can affect any body part, but usually affects the arms or legs.
Dawn Carlson, PTA, explained that CRPS involves dysregulation of the autonomic nervous system, which results in multiple function loss, impairment and disability.
"Therapy can help to decrease pain in the affected limbs and increase range of motion and strength," said Carlson, a physical therapist assistant at Arthritis and Sports Orthopedics and Physical Therapy in Sterling, VA. "One of the most important roles that therapy plays in the management of CRPS is desensitization of the affected body part."
The earlier that CRPS is diagnosed, the more effective therapeutic interventions will be.
Symptoms & Diagnosis
One of the key symptoms of CRPS is intense, burning pain that progressively gets worse over time, said Carlson. Patients also may exhibit changes in blood flow, skin temperature, sweating and nail/skin/hair appearance; reduction in joint motion and muscle length; and possibly myofascial restrictions and joint hypomobility.
"Patients will be hesitant and may outright refuse to move the joint, and will be ultra-protective, often not letting clothing, or even a bed sheet touch the local area of pain due to sensitivity," said Jill Murphy, DPT, LAT, CSCS. "In further stages, the bone may weaken and become osteoporotic, the muscles will atrophy, joints thicken and further stiffen, and tendons display contractures that are difficult to reverse."
Though pain can begin at the point of injury, it can spread to the whole limb. Carlson noted that there are three stages of CRPS; however, the disease does not always follow this pattern:
Stage 1 lasts from 1 to 3 months, and is characterized by changes in skin temperature, severe burning pain, muscle spasm, joint stiffness and rapid hair growth.
Stage 2 typically lasts from 3 to 6 months and is characterized by increasing pain and swelling, decreased hair growth, stiff joints and weak muscle tone.
Stage 3 brings about changes in skin and bone that are no longer reversible. There may be marked muscle atrophy, severe limitations in joint mobility and involuntary contractions of muscle.
It should be noted that two types of CRPS exist: CRPS I (formerly known as RSD) and CRPS II. Type I is a nerve disorder in which there are no demonstrable nerve lesions, whereas Type II (formerly known as causalgia) has evidence of obvious damage to the nerve.
"Type I and Type II also differ in the nature of the inciting event," said Carlson. "Type I develops after an initiating noxious event that may or may not have been traumatic. Type II develops following a nerve injury."
Murphy, owner of MotionWorks Physical Therapy in Neenah, WI, added that there is actual nerve damage in CRPS II, with the precipitating event being damage to a local, peripheral nerve. However, the location of CRPS II may extend beyond the distribution of the peripheral nerve.
"CRPS I and CRPS II are typically diagnosed by the presence of the typical symptoms, with absence of any other explanation for these symptoms," she said.
Numerous tests can contribute to the diagnosis of CRPS, noted Carlson.
"Bone scans and X-rays can detect bony changes. An MRI can detect soft-tissue changes. Nerve conduction studies show injury to the nerve. Sympathetic nervous system tests can detect disturbances in the sympathetic nervous system," she explained.
EMG or nerve-conduction-velocity studies can help determine whether the patient is suffering from CRPS I or CRPS II, mentioned Murphy.
"In later stages, radiological studies may show osteoporotic changes, and sweat studies may help reinforce the diagnosis," she added.
Since pain by nature is a subjectively based phenomenon, diagnosis is difficult, noted Jeffrey M. Gontarski, PT, DPT, director of physical therapy at Advanced Wellness Center, Marlboro, NJ.
"Neurological testing can be performed to assist in diagnosis of a nerve-based issue to start. From this point, subjective data and a lack of solid findings seem to place a patient in the CRPS category."
Decreasing Pain Through Therapy
Therapeutic treatment can assist in decreasing sensitivity to the injured area through deep-tissue massage (as tolerated), scar mobilization, stretching, strengthening and energy-conservation techniques to assist the patient in activities of daily living, said Dr. Gontarski.
Therapists can apply many helpful treatment techniques for patients suffering with CRPS, including easing patients into joint range of motion, gentle joint mobilization, edema massage and soft-tissue mobilization with a very gentle, superficial approach, and muscle flexibility and strengthening exercises, said Murphy.
"Aerobic exercise is very helpful to reduce sensitization of the central nervous system, so starting the patient on some low-grade exercise as early as possible should be a prominent goal of therapy," she said. "Patient education is key to address guarding postures, and to allow gentle stimulation from a variety of sources in the affected area. Stress-relaxation techniques, diaphragmatic breathing and other activities to assist in nervous system relaxation can be helpful."
Therapy goals are focused on movement and function, not pain, as the typical CRPS patient is already quite focused on pain, noted Murphy.
"The prognosis will be slow, especially in later stages of the disease, and depending on the co-morbidities the patient may have. It is important to fully explain to the patient what is happening, so he or she can understand the disease process and become more compliant with the home exercise program and suggested activities," she stated.
Typical therapy goals for this population include "increased range of motion, decreased pain, increased strength, becoming independent with activities of daily living and home exercise programs," listed Gontarski.
Physicians may also prescribe medications to assist in desensitizing the nervous system, such as Gabapentin, anti-depressants, NSAIDs, corticosteroids and pain medications, which may be helpful in easing the transition to more movement in early physical therapy visits, said Dr. Murphy.
Early Recognition and Intervention
The cause of CRPS is still unknown, noted Carlson.
"It is thought to be the result of damage to the nervous system, which may be precipitated by surgery or injury. However, there are some documented cases in which there was no injury involved," she said.
A current patient at Advanced Wellness Center suffers from CRPS after a severe dog bite to the calf. Gontarski remarked that the patient has a great deal of scarring, continues to have pain after four years and is currently scheduled for a sympathetic nerve block. Recently, she asked if it was possible for the syndrome to have a psychological component. She was advised to see the outcome of the nerve block and may be referred to psychological services in the future.
Carlson has treated a few patients that developed CRPS in their wrists/hands, but mostly has seen it in the foot/ankle joints. In her experience, only patients diagnosed with CRPS in the beginning stages of the disease benefited from therapy.
"The patients that have not responded well to therapy intervention were those who had already progressed into Stage 3 of the disease. They made no progress after the initial first few weeks of therapy," she said. "Some patients ultimately had nerve blocks installed by their doctors in hopes of alleviating the pain."
Murphy agreed that early therapeutic intervention is vital to success, noting that the only unsuccessful case was one involving a patient who had a pending lawsuit as well as several psychological co-morbidities that made it challenging to understand the patient's varying responses to standard physical therapy treatments.
"When patients understand the disease process, they can better understand the treatment approach," she said.
Ultimately, Murphy believes that the best treatment for CRPS is prevention.
"Educating surgeons and primary providers about CRPS and the importance of early recognition and intervention with therapy is the key to addressing this diagnosis," she concluded.
Beth Puliti is a frequent contributor to ADVANCE.