Complex regional pain syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Complex Regional Pain Syndrome (CRPS) is a chronic progressive disease characterized by severe pain, swelling, and changes in the skin. Complex regional pain syndrome (CRPS) is a chronic pain condition that can affect any area of the body, but it often affects an arm or a leg.

It is important to understand that not all complex regional pain syndromes are the same. It is quite possible to have a post-phlebitic syndrome involving the leg that may present with skin color changes, excess pain, and swelling, but has nothing to do with RSD.

The cause of complex regional pain syndrome is currently unknown. Precipitating factors include underlying illnesses (especially vascular disease, an underlying rheumatic condition, a hidden infection or a coagulopathy in RSD cases), orthopedic injury, and surgery. Although there are documented cases that have no identifiable injury to the original site in almost all cases of RSD, there was an underlying event or injury.

The condition currently known as Complex regional pain syndrome was originally described by Silas Weir Mitchell during the American Civil War, who named the condition causalgia. In the 1940s, the term reflex sympathetic dystrophy came into use to describe this condition.

Complex regional pain syndrome has been classified as type I (reflex sympathetic dystrophy with no nerve lesions) and type II (causalgia with obvious nerve damage).

Recent research has suggested that oxidative damage (e.g. by free radicals) may play a role in pathophysiology of complex regional pain syndrome. The sympathetic nervous system may also play an important role in the pain. Another theory is that CRPS is caused by a triggering of the immune response

The cause of complex regional pain syndrome is currently unknown. Precipitating factors include underlying illness (especially vascular disease, an underlying rheumatic condition, a hidden infection or a coagulopathy in RSD cases), orthopedic injury and surgery.

Complex regional pain syndrome must be differentiated from shoulder-hand syndrome, Sudeck syndrome, Steinbrocker syndrome and Erythromelalgia.

Complex regional pain syndrome is more common between ages 40- 60, affects women more frequently.

Precipitating factors for developing complex regional pain syndrome include underlying illness (especially vascular disease, an underlying rheumatic condition, a hidden infection or a coagulopathy in RSD cases), orthopedic injury and surgery.

Good progress can be made in treating complex regional pain syndrome if treatment is begun early which may even result in remission. If treatment is delayed, however, the disorder can quickly spread to the entire limb and changes in bone and muscle may become irreversible.

Both type I and type II varieties of complex regional pain syndrome share common diagnostic criteria of having spontaneous onset of pain not limited to the distribution of a single nerve, a history of edema or abnormal sweating. The only difference lies in the nature of the inciting event.

The key symptom of complex regional pain syndrome is intense and burning pain which is much stronger than would be expected for the type of injury that occurred and it gets worse over time. Other symptoms include changes in skin, hair or nails. As disease progresses, there is more edema and there occur irreversible changes in skin and bones.

No specific test is available for complex regional pain syndrome, which is diagnosed primarily through observation of the symptoms. However, thermography, sweat testing, x-rays, electrodiagnostics, and sympathetic blocks can be used to build up a picture of the disorder. Three phase bones scans have been thought of as one of the most specific diagnostic studies for RSD, but they are not very sensitive and therefor delay recognition of cases. A delay in diagnosis and/or treatment for this syndrome can result in severe physical and psychological problems. Early recognition and prompt treatment provide the greatest opportunity for recovery.

Patchy osteoporosis can be detected through X-ray as early as two weeks after the onset of CRPS.

Bone scan can be used to detect osteoporosis sooner than X ray. Bone densitometry is also used to detect changes in bone density and monitor results of treatment.

Thermography is the only test that allows for vasomotor mapping and therefore also provides information as to both the distribution of that patient’s complex regional pain syndrome and its underlying generator. It is a diagnostic technique for measuring blood flow by determining the variations in heat emitted from the body. The nerve injury that characterizes type II CRPS can be detected by electromyography.

The medical therapy of complex regional pain syndrome involves use of a variety of drugs including antidepressants, anti-inflammatories such as corticosteroids and COX-inhibitors such as piroxicam, vasodilators, GABA analogs such gabapentin and pregabalin, and alpha– or beta-adrenergic-blocking compounds. Local anaesthetic like lidocaine is often the first step in treatment. Physical therapy is the most important part of treatment. Implantation of spinal cord stimulators, sympathectomy, EEG feedback, psychotherapy and ketamine are amongst the newer forms of treatment. Surgical, chemical, or radiofrequency sympathectomy can be used as a last resort in patients with impending tissue loss, edema, recurrent infection, or ischemic necrosis suffering from complex regional pain syndrome. There is no prevention at this time for complex regional pain syndrome. Vitamin C has been shown to reduce the prevalence.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

The condition currently known as complex regional pain syndrome was originally described by Silas Weir Mitchell during the American Civil War, who named the condition causalgia. In the 1940s, the term reflex sympathetic dystrophy came into use to describe this condition.

The condition currently known as CRPS was originally described by Silas Weir Mitchell during the American Civil War, who named the condition causalgia. In the 1940s, the term reflex sympathetic dystrophy came into use to describe this condition, based on the theory that sympathetic hyperactivity was involved in the pathophysiology.^[1] Misuse of the terms, as well as doubts about the underlying pathophysiology, led to calls for better nomenclature. In 1993, a special consensus workshop held in Orlando, Florida, provided the umbrella term “complex regional pain syndrome,” with causalgia and RSD as subtypes.^[2]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Complex regional pain syndrome has been classified as type I (reflex sympathetic dystrophy with no nerve lesions) and type II (causalgia with obvious nerve damage).

The International Association for the Study of Pain has divided CRPS into two types based on the presence of nerve lesion following the injury.

• Type I, also known as reflex sympathetic dystrophy (RSD), Sudeck’s atrophy, Reflex neurovascular dystrophy (RND) or algoneurodystrophy, does not have demonstrable nerve lesions.
• Type II, also known as causalgia, has evidence of obvious nerve damage.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Recent research has suggested that oxidative damage (e.g. by free radicals) may play a role in the pathophysiology of complex regional pain syndrome. The sympathetic nervous system may also play an important role in the pain aspect of the condition. Another theory is that CRPS is caused by a triggering of the immune response.

The pathophysiology of CRPS remains unclear. However, recent research has suggested that oxidative damage (e.g. by free radicals) associated with one of the precipitating factors described earlier may play a role.^[1]

Doctors aren’t sure what causes CRPS. In some cases, the sympathetic nervous system plays an important role in the pain. Another theory is that CRPS is caused by a triggering of the immune response, which leads to the inflammatory symptoms of redness, warmth, and swelling in the affected area.

CRPS is thought to result from damage to the nervous system, including the nerves that control the blood vessels and sweat glands.

The damaged nerves are no longer able to properly control blood flow, feeling (sensation), and temperature to the affected area. This leads to medical problems in the:

• Blood vessels
• Bones
• Muscles
• Nerves
• Skin

CRPS can strike at any age, but is more common between the ages of 40 and 60. It affects both men and women, but is more frequently seen in women. The number of reported CRPS cases among adolescents and young adults is increasing.

Investigators estimate that two to five percent of those with peripheral nerve injury and 12 to 21 percent of those with hemiplegia (paralysis of one side of the body) will suffer from CRPS.

Research has demonstrated that the administration of Vitamin C after an injury may decrease one’s susceptibility to developing CRPS.^[1]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

The cause of complex regional pain syndrome is currently unknown. Precipitating factors include underlying illnesses (especially vascular disease, an underlying rheumatic condition, a hidden infection or a coagulopathy in RSD cases), orthopedic injury, and surgery.

The cause of this syndrome is currently unknown. Precipitating factors include underlying illness (especially vascular disease, an underlying rheumatic condition, a hidden infection or a coagulopathy in RSD cases), orthopedic injury and surgery. Although there are documented cases that have no identifiable injury to the original site in almost all cases of RSD there was an underlying event or injury.

Possible causes of CRPS:

• Injury directly to a nerve
• Injury or infection in an arm or leg

Rarely, sudden illnesses such as a heart attack or stroke can cause CSPS. The condition can sometimes appear without obvious injury to the affected limb.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Complex regional pain syndrome must be differentiated from conditions such as Sudeck syndrome, Steinbrocker syndrome, and erythromelalgia.

CRPS has characteristics similar to those of other disorders, such as shoulder-hand syndrome, which sometimes occurs after a heart attack and is marked by pain and stiffness in the arm and shoulder; Sudeck syndrome, which is prevalent in older people and women and is characterized by bone changes and muscular atrophy, but is not always associated with trauma; and Steinbrocker syndrome, which includes symptoms such as gradual stiffness, discomfort, and weakness in the shoulder and hand. Erythromelalgia also shares many components of CRPS (burning pain, redness, temperature hypersensitivity, autonomic dysfunction, vasospasm)they both involve small fiber sensory neurosympathetic components. Interestingly Erythromelalgia involves a lack of sweating, whereas CRPS often involves increased sweating. Subvariations of both exist.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Complex regional pain syndrome is more common between the ages of 40 and 60, and it affects women more frequently.

CRPS can strike at any age, but is more common between the ages of 40 and 60.The number of reported CRPS cases among adolescents and young adults is increasing.

It affects both men and women, but is more frequently seen in women.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Precipitating factors include underlying illnesses (especially vascular disease, an underlying rheumatic condition, a hidden infection or a coagulopathy in RSD cases), orthopedic injury, and surgery.

Precipitating factors include underlying illness (especially vascular disease, an underlying rheumatic condition, a hidden infection or a coagulopathy in RSD cases), orthopedic injury and surgery. Although there are documented cases that have no identifiable injury to the original site in almost all cases of RSD there was an underlying event or injury.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Good progress can be made in treating complex regional pain syndrome if treatment is started early on. Beginning treatment early may even result in remission. If treatment is delayed, however, the disorder can quickly spread to the entire limb, and changes in the bone and muscle may become irreversible.

• Loss of muscle size or strength in the affected limb
• Spread of the disease to another part of the body
• Worsening of the affected limb

Complications can also occur with some of the nerve and surgical treatments.

Good progress can be made in treating CRPS if treatment is begun early, ideally within 3 months of the first symptoms. Early treatment may even result in remission. Detailed assessment for an underlying perpetrator and addressing those factors that impact blood flow and never recovery can have a tremendous clinical impact. If treatment is delayed, however, the disorder can quickly spread to the entire limb and changes in bone and muscle may become irreversible. In 50 percent of CRPS cases, pain persists longer than 6 months and sometimes for years. In teens and younger patients with CRPS, the prognosis is excellent. Even without invasive therapy, upwards of 75% of children have full recovery with virtually 100% of the patients having marked improvement. The prognosis is not always good. The limb, or limbs, can experience muscle atrophy, loss of use and functionally useless parameters that require amputation.

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