What is complex regional pain syndrome (CRPS)?

What is complex regional pain syndrome (CRPS)?

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Complex regional pain syndrome (CRPS), also known as reflex sympathetic dystrophy (RSD), is a chronic pain condition that typically affects one limb, such as an arm, leg, hand, or foot. It is characterized by intense and disproportionate pain, along with various sensory, autonomic, and motor abnormalities. It includes disorders of the sympathetic nervous system (“fight or flight” response of the body) with or without known trauma.

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CRPS has its origin in a variety of conditions: it can follow medical procedures, including surgery, such as arthroscopy (surgical procedure for joint problems); it can occur after traumatic brain injury, cerebrovascular accidents (stroke), or destructive lesion in the central nervous system (brain and spinal cord); or it can occur after nerve injuries, entrapment, neuropathies (nerve damage). Any event that causes traction of the brachial plexus or nerve roots resulting in constant nerve irritation may result in this condition.

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Classifications and Clinical Features of Complex regional pain syndromes:

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1. CRPS type I (formerly known as Reflex Sympathetic Dystrophy): It is the label used to describe this syndrome when there is an absence of known traumatic nerve injury; however, there is usually an initiating noxious event. 
2. CRPS type II (formerly known as Causalgia): Refers to a similar condition associated with known traumatic nerve injury. 
3. CRPS type III (known as CRPS-NOS) (Not Otherwise Specified): It only partially meets CRPS criteria and cannot be explained by any other condition.

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Common symptoms of CRPS

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The symptoms of CRPS can vary in severity and may include:

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1. Persistent and severe pain that is disproportionate to the original injury or trauma. The overall pain intensity increases with disease duration. It spreads from localized to a regional distribution.
2. Sensory abnormalities such as hypersensitivity to touch, temperature changes, or even light pressure are most often the hallmark of CRPS.
3. Swelling and changes in skin temperature, color, or texture.
4. Motor dysfunction, including weakness, tremors, or limited range of motion may precede the appearance of other impairments by weeks or months.
5. Changes in hair and nail growth patterns.
6. Autonomic dysregulation, leading to abnormal sweating, temperature changes, or changes in blood flow.

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Complex regional pain syndrome typically progresses through three stages.

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• Stage I: Acute/Reversible Stage. Typically, symptoms begin within several days after injury or insidiously over several weeks. This stage of vasodilation (widening of blood vessels) lasts 3 weeks to 6 months. Pain, the predominant feature, is usually out of proportion to the severity of the injury. There is hyperhidrosis (excessive sweating), warmth, redness, rapid nail growth, and edema in the extremity.
• Stage II: Dystrophic or Vasoconstriction (narrowing of blood vessels) Stage. This stage typically begins around 3 months after the initial injury (although this varies) that lasts 3 to 6 months. It is characterized by sympathetic (fight or flight response of the body) hyperactivity, burning pain and hyperesthesia (increased sensitivity to stimulation) exacerbated by cold weather. There is mottling (spots) and coldness, brittle nails, and osteoporosis (a condition that weakens bone).
• Stage III: Atrophic (decrease in size) Stage. This begins 6 months to 1 year after injury, and is characterized by pain either decreasing or becoming worse and by severe osteoporosis. Muscle wasting (thinning) and contractures may occur. The condition can last for months or years, but spontaneous recovery often occurs within 18 to 24 months.

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In the absence of specific biomarkers, CRPS is usually diagnosed based on a combination of clinical symptoms, physical examination and medical history. There is no specific test to confirm the condition, so doctors rely on ruling out other possible causes of the symptoms. Because of the evolutionary nature of CRPS, a correct diagnosis may be delayed, especially in children.

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What causes complex regional pain syndrome?

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The exact cause of CRPS is not well understood, but it often occurs following an injury or trauma to the affected limb. It is believed that the condition arises due to a malfunction of the peripheral (nerves that branch out to the brain and spinal cord) and central (brain and spinal cord) nervous systems, resulting in an abnormal pain response.

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How can physical therapy help? 

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Physical therapy can play a significant role in the management and treatment of Complex Regional Pain Syndrome (CRPS). The primary goals of physical therapy for CRPS are to alleviate pain, improve physical function, and enhance quality of life. Here are some ways in which physical therapy can help individuals with CRPS:

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1. Pain and swelling management: Physical therapists can employ various techniques and modalities to help reduce pain, such as manual therapy, ultrasound, vibration, heat or cold therapy and transcutaneous electrical nerve stimulation (TENS). Elevation of the affected limb and use of elastic compression may also be prescribed to help reduce swelling.
2. Mobility exercise: In the early stages, gentle and active range of motion exercises are used to manage the increasing stiffness. As the patient improves, joint mobilization, nerve mobilization and stretching techniques are initiated to address tissues limiting mobility.  
3. Desensitization techniques: CRPS can cause increased sensitivity to touch. Physical therapists utilize desensitization techniques such as; having the patient work in various textures and tap or vibrate over the sensitive area to help decrease or normalize the affected area's response to particular sensations. 
4. Mirror therapy: Mirror therapy involves using a mirror to create a visual illusion that the affected limb is moving normally. This technique has been proven to be very effective in treating CRPS. It can help reduce pain and improve motor function by retraining the brain's perception of the affected limb.
5. Strengthening exercises: Initially, the physical therapists will facilitate active muscle contractions. It involves the use of both dynamic and isometric (static) exercise and alternating controlled compressive loading activities for neuromuscular control. This will help provide tissue stress with minimal joint motion. Once the patient improves, progressive strengthening exercises are prescribed to increase muscle strength, endurance and performance.
6. Functional training: A final component of an individualized rehabilitation program involves practicing activities that simulate the physical demands of a patient’s daily activities. Simulated activities and drills enable a patient to practice under supervised conditions and receive feedback on correct and safe mechanics. Physical therapists may also provide adaptive strategies and assistive devices to facilitate participation in meaningful activities.
7. Aerobic conditioning: Aerobic conditioning exercises provide many benefits for patients with CRPS. The activity not only improves cardiopulmonary endurance but stimulates feelings of well-being and relief of symptoms.
8. Education and self-management: Physical therapists play a crucial role in educating individuals  about CRPS, its symptoms, stages and possible causes as well as the role and importance of the rehabilitation program in managing their condition. They also teach patients about pain management strategies, lifestyle modifications, activity pacing, energy conservation techniques, and stress management.
9. Home Exercise Program: Physical therapists provide home exercise programs for each patient and teach them how to safely and effectively incorporate it into their daily routine to reinforce the interventions and promote optimal recovery.

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CRPS is a complex syndrome with varying severity and disability. The treatment for this condition tends to be multifactorial and prolonged. Successful treatments depend on early diagnosis, treatment of the underlying cause, and aggressive and sustained physical therapy.

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It's important to note that physical therapy for CRPS should be tailored to the individual's specific needs and may require a multidisciplinary approach involving other healthcare professionals, such as pain specialists and occupational therapists. Treatment plans should be individualized and adjusted based on the individual's response and progress.

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