Complex Regional Pain Syndrome (CRPS) is a chronic progressive disease characterized by severe pain, swelling and changes in the skin. The International Association for the Study of Pain has divided CRPS into two types based on the presence of nerve lesion following the injury. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // M00-M99 - Diseases of the musculoskeletal system and connective tissue (M00-M25) Arthropathies (M00-M03) Infectious arthropathies (M00) Pyogenic arthritis (M01) Direct infections of joint in infectious and parasitic diseases classified elsewhere (M02) Reactive arthropathies (M023) Reiters disease (M03) Postinfective and reactive arthropathies in diseases classified elsewhere (M05-M14... // G00-G99 - Diseases of the nervous system (G00-G09) Inflammatory diseases of the central nervous system (G00) Bacterial meningitis, not elsewhere classified (G01) Meningitis in bacterial diseases classified elsewhere (G02) Meningitis in other infectious and parasitic diseases classified elsewhere (G03) Meningitis due to other and unspecified causes (G04) Encephalitis, myelitis... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ...

• 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.

The cause of this syndrome is currently unknown. Precipitating factors include illness, injury and surgery, although there are documented cases that have no documentable injury to the original site.

History and nomenclature

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] Silas Weir Mitchell Silas Weir Mitchell (b. ... Combatants United States of America (Union) Confederate States of America (Confederacy) Commanders Abraham Lincoln, Ulysses S. Grant Jefferson Davis, Robert E. Lee Strength 2,200,000 1,064,000 Casualties 110,000 killed in action, 360,000 total dead, 275,200 wounded 93,000 killed in action, 258,000 total...

severe CRPS of right arm

Image File history File links Severe_CRPS.jpg‎ severe CRPS I, the creator of this work, hereby grant the permission to copy, distribute and/or modify this document under the terms of the GNU Free Documentation License, Version 1. ... Image File history File links Severe_CRPS.jpg‎ severe CRPS I, the creator of this work, hereby grant the permission to copy, distribute and/or modify this document under the terms of the GNU Free Documentation License, Version 1. ...

Pathophysiology

The pathophysiology of CRPS remains unclear. However, recent research has suggested that oxidative damage (e.g. by free radicals) may play a role.^[3] Pathophysiology is the study of the disturbance of normal mechanical, physical, and biochemical functions, either caused by a disease, or resulting from a disease or abnormal syndrome or condition that may not qualify to be called a disease. ... In chemistry free radicals are uncharged atomic or molecular species with unpaired electrons or an otherwise open shell configuration. ...

Susceptibility

CRPS can strike at any age, but is more common between the ages of 40 and 60. ^{[citation needed]} 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. ^{[citation needed]}

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. ^{[citation needed]}

Research has demonstrated that the administration of Vitamin C after an injury may decrease one's susceptibility to developing CRPS.^[3] This article is about the nutrient. ...

Symptoms

The symptoms of CRPS usually manifest near the site of an injury, either major or minor, and usually [spread] beyond the original area. Symptoms may spread to involve the entire limb and, rarely, the opposite limb. The most common symptom is burning pain. The patient may also experience muscle spasms, local swelling, increased sweating, softening of bones, joint tenderness or stiffness, restricted or painful movement, and changes in the nails and skin.

The pain of CRPS is continuous and may be heightened by emotional stress. Moving or touching the limb is often intolerable. Eventually the joints become stiff from disuse, and the skin, muscles, and bone atrophy. The symptoms of CRPS vary in severity and duration. There are three variants of CRPS, previously thought of as stages. It is now believed that patients with CRPS do not progress through these stages sequentially and/or that these stages are not time-limited. Instead, patients are likely to have one of the three following types of disease progression: A top-down view of skeletal muscle Muscle (from Latin musculus little mouse [1]) is contractile tissue of the body and is derived from the mesodermal layer of embryonic germ cells. ...

1. Type one is characterized by severe, burning pain at the site of the injury. Muscle spasm, joint stiffness, restricted mobility, rapid hair and nail growth, and vasospasm (a constriction of the blood vessels) that affects color and temperature of the skin can also occur.
2. Type two is characterized by more intense pain. Swelling spreads, hair growth diminishes, nails become cracked, brittle, grooved, and spotty, osteoporosis becomes severe and diffuse, joints thicken, and muscles atrophy.
3. Type three is characterized by irreversible changes in the skin and bones, while the pain becomes unyielding and may involve the entire limb. There is marked muscle atrophy, severely limited mobility of the affected area, and flexor tendon contractions (contractions of the muscles and tendons that flex the joints). Occasionally the limb is displaced from its normal position, and marked bone softening is more dispersed

For the musical, see Hair (musical). ... Anatomy In anatomy, a nail is a horn-like piece at the end of a humans or an animals finger or toe. ... Vasospasm refers to a condition in which blood vessels spasm, leading to constriction. ... f you all The blood vessels are part of the circulatory system and function to transport blood throughout the body. ... Osteoporosis is a disease of bone in which the bone mineral density (BMD) is reduced, bone microarchitecture is disrupted, and the amount and variety of non-collagenous proteins in bone is altered. ... A tendon (or sinew) is a tough band of fibrous connective tissue that connects muscle to bone and is built to withstand tension. ...

Diagnosis

CRPS types I and II share the common diagnostic criteria shown below.

1. Spontaneous pain or allodynia/hyperalgesia is not limited to the territory of a single peripheral nerve, and is disproportionate to the inciting event.
2. There is a history of edema, skin blood flow abnormality, or abnormal sweating in the region of the pain since the inciting event.
3. No other conditions can account for the degree of pain and dysfunction.

The two types differ only in the nature of the inciting event. Type I CRPS develops following an initiating noxious event that may or may not have been traumatic, while type II CRPS develops after a nerve injury. Allodynia, meaning other pain, is an exaggerated response to a non-noxious stimuli and can be either static or mechanical. ... Hyperalgesia is an extreme sensitivity to pain, which in one form is caused by damage to nociceptors in the bodys soft tissues. ... This page is about the condition called edema. ...

No specific test is available for CRPS, 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. Diagnosis is complicated by the fact that some patients improve without treatment. 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.

Thermography

Thermography is a diagnostic technique for measuring blood flow by determining the variations in heat emitted from the body. A color-coded "thermogram" of a person in pain often shows an altered blood supply to the painful area, appearing as a different shade (abnormally pale or violet) than the surrounding areas of the corresponding part on the other side of the body. A difference of 1.0°C between two symmetrical body parts is considered significant, especially if a large number of asymmetrical skin temperature sites are present. ^{[citation needed]} The affected limb may be warmer or cooler than the unaffected limb. This article is about the infrared imaging technique. ...

Sweat testing

Abnormal sweating can be detected by several tests. A powder that changes color when exposed to sweat can be applied to the limbs; however, this method does not allow for quantification of sweating. Two quantitative tests that may be used are the resting sweat output test and the quantitative sudomotor axon reflex test. These quantitative sweat tests have been shown to correlate with clinical signs of CRPS.^[4]

Radiography

Patchy osteoporosis, which may be due to disuse of the affected extremity, can be detected through X-ray imagery as early as two weeks after the onset of CRPS. A bone scan of the affected limb may detect these changes even sooner. Bone densitometry can also be used to detect changes in bone mineral density. It can also be used to monitor the results of treatment, as bone densitometry parameters improve with treatment. Drawing shows patient lying on a table that slides under the scanner, a technician operating the scanner, and a monitor that will show images made during the scan. ... Dual energy X-ray absorptiometry (DXA, previously DEXA) is a means of measuring bone mineral density (BMD). ...

Electrodiagnostic testing

The nerve injury that characterizes type II CRPS can be detected by electromyography. In contrast to peripheral mononeuropathy, the symptoms of type 2 CRPS extend beyond the distribution of the affected peripheral nerve. Electromyography (EMG) is a technique for evaluating and recording physiologic properties of muscles at rest and while contracting. ...

Prevention

Vitamin C has been shown to reduce the prevalence of complex regional pain syndrome after wrist fractures. A daily dose of 500 mg for fifty days is recommended^[5].

Treatment

Physicians use a variety of drugs to treat CRPS, 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. An antidepressant is a medication used primarily in the treatment of clinical depression. ... In physiology, corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. ... Cyclooxygenase (COX) is an enzyme (EC 1. ... Piroxicam (marketed in the U.S. under the trade name Feldene) is a non-steroidal anti-inflammatory drug used to relieve the symptoms of Rheumatoid and Osteoarthritis, primary dysmenorrhoea, Post Operative Pain; and act as an analgesic, especially where there is an inflammatory component. ... A vasodilator is a substance that causes blood vessels in the body to become wider by relaxing the smooth muscle in the vessel wall, or vasodilation. ... Gaba may refer to: Gabâ or gabaa (Philippines), the concept of negative karma of the Cebuano people GABA, the gamma-amino-butyric acid neurotransmitter GABA receptor, in biology, receptors with GABA as their endogenous ligand Gaba 1 to 1, an English conversational school in Japan Marianne Gaba, a US model... Gabapentin (brand name: Neurontin®) was initially synthesized to mimic the structure of GABA for the treatment of epilepsy. ... Pregabalin (brand name: Lyrica®) is a new anticonvulsant drug indicated as an add on therapy for partial onset seizures and for certain types of neuropathic pain. ... Alpha blockers (also called alpha-adrenergic blocking agents) constitute a variety of drugs which block alpha-adrenergic receptors in arteries and smooth muscles. ... Beta blockers or beta-adrenergic blocking agents are a class of drugs used to treat a variety of cardiovascular conditions and some other diseases. ...

Elevation of the extremity and physical therapy are also used to treat CRPS.

Injection of a local anesthetic such as lidocaine is often the first step in treatment. Injections are repeated as needed. However, early intervention with non-invasive management may be preferred to repeated nerve blockade. The use of topical lidocaine patches has been shown to be of use in the treatment of CRPS-1 and -2 ^[6]. ^[7]. TENS (transcutaneous electrical nerve stimulation), a procedure in which brief pulses of electricity are applied to nerve endings under the skin, has helped some patients in relieving chronic pain. Anesthesia (AE), also anaesthesia (BE), is the process of blocking the perception of pain and other sensations. ... Lidocaine (INN) (IPA: ) or lignocaine (former BAN) (IPA: ) is a common local anesthetic and antiarrhythmic drug. ... TENS (Transcutaneous Electrical Nerve Stimulator) is considered a method of pain relief, and has a wide following for use in obstetric care particularly labour. ...

Neurostimulation (spinal cord stimulators) may also be surgically implanted to reduce the pain by directly stimulating the spinal cord. These devices place electrodes either in the epidural space (space above the spinal cord) or directly over nerves located outside the central nervous system. Implantable drug pumps may also be used to deliver pain medication directly to the cerebrospinal fluid which allows powerful opioids to be used in a much smaller dose than when taken orally. Prednisolone (a corticosteroid) has been shown to be superior to piroxicam in the treatment of reflex sympathetic dystrophy.^[8] An opioid is a chemical substance that has a morphine-like action in the body. ... Prednisolone is the active metabolite of prednisone. ... Piroxicam (marketed in the U.S. under the trade name Feldene) is a non-steroidal anti-inflammatory drug used to relieve the symptoms of Rheumatoid and Osteoarthritis, primary dysmenorrhoea, Post Operative Pain; and act as an analgesic, especially where there is an inflammatory component. ...

Surgical, chemical, or radiofrequency sympathectomy — interruption of the affected portion of the sympathetic nervous system — can be used as a last resort in patients with impending tissue loss, edema, recurrent infection, or ischemic necrosis.^[9] However, there is little evidence that these permanent interventions alter the pain symptoms of the affected patients.

Physical therapy is the most important part of treatment, though it should be noted that many patients are incapable of participating in physical therapy due to muscular and bone problems. People struggling with CRPS often develop guarding behaviors where they avoid using or touching the affected limb. Unfortunately, inactivity can exacerbate the disease and perpetuate the pain cycle. Physical therapy works best for most patients, especially goal-directed therapy, where the patient begins from an initial point, regardless of how minimal, and then endeavors to increase activity each week. Therapy should be directed at facilitating the patient to engage in physical therapy, movement and stimulation of the affected areas.

Some treating physicians have even initiated physical therapy under light general anesthesia, in an attempt to remobilize the extremity. While the unpredictability of this illness often causes a frustrating pattern of progress and regress, it is essential to continue to try to increase and normalize physical activity.

A study in 2007 indicated that Collateral Meridian Therapy^[10] was effective in lowering CRPS patient's VAS pain score.

EEG Biofeedback^[11], various forms of psychotherapy^[12], relaxation techniques and hypnosis ^[13] are adjunctive treatments which assist coping. There are very few or no other articles that link to this one. ... Psychotherapy is an interpersonal, relational intervention used by trained psychotherapists to aid clients in problems of living. ... Relaxation techniques are used by people who wish to relax, for a wide variety of reasons. ... Professor Charcot was well-known for showing, during his lessons at the Salpêtrière hospital, hysterical woman patients – here, his favorite patient, Blanche (Marie) Wittman, supported by Joseph Babiński. ...

Ketamine Therapy

Ketamine, a potent anesthetic, is being used as an experimental and controversial treatment for Complex Regional Pain Syndrome. The theory of ketamine use in CRPS/RPS is primarily advanced by neurologist Dr Robert J. Schwartzman of Drexel University College of Medicine in Philadelphia, and researchers at the University of Tübingen in Germany. The hypothesis is that ketamine manipulates NMDA receptors which might reboot aberrant brain activity. Ketamine is a dissociative anesthetic for use in human and veterinary medicine. ... Neurology is the branch of medicine that deals with the nervous system and disorders affecting it. ... Drexel University is an institution of higher learning and research located in Philadelphia, Pennsylvania. ... Eberhard Karls University of Tübingen (German: Eberhard-Karls-Universität Tübingen) is a state-supported university located on the Neckar river, in the city of Tübingen, Baden-Württemberg, Germany. ... NMDA (N-methyl-D-aspartic acid) is an amino acid derivative acting as a specific agonist at the NMDA receptor, and therefore mimics the action of the neurotransmitter glutamate on that receptor. ...

There are two treatment modalities; the first consist of a low dose ketamine infusion of between 25-90 mg per day, delivered over five days either in hospital or as an outpatient. This is called the awake technique.

Open label, prospective, pain journal evaluation of a 10-day infusion of intravenous ketamine (awake technique) in the CRPS patient concluded that "A four-hour ketamine infusion escalated from 40-80 mg over a 10-day period can result in a significant reduction of pain with increased mobility and a tendency to decreased autonomic dysregulation".^[14]

The second treatment modality consists of putting the patient into a medically-induced coma, then administering an extremely high dosage of ketamine; typically between 600-900 mg.^[15] This version, currently not allowed in the United States, is most commonly done in Germany but some treatments are now also taking place in Monterrey, Mexico. In medicine, a coma (from the Greek koma, meaning deep sleep) is a profound state of unconsciousness. ... This article is about the Mexican city; for other uses, see Monterrey (disambiguation). ...

According to Dr Schwartzman, 14 cases out of 41 patients in the induced-coma ketamine experiments were completely cured. "We haven't cured the original injury," he says, "but we have cured the RSD or kept it in remission. The RSD pain is gone."

"No one ever cured it before," he adds. "In 40 years, I have never seen anything like it. These are people who were disabled and in horrible pain. Most were completely incapacitated. They go back to work, back to school, and are doing everything they used to do. Most are on no medications at all. I have taken morphine pumps out of people. You turn off the pain and reset the whole system." ^[16]

No trials have been done for the coma induced method to date.

This method gained attention in pop culture through season 3 of the FOX television drama House, M.D., which opens a few months after the title character, Gregory House, was placed into a ketamine coma to treat ongoing neuropathy in his right leg. House is shown to have recovered significant use of his right leg (he even goes running regularly), but the treatment eventually wears off and House is once more left in pain and significantly disabled. House, M.D. (commonly promoted as just House) is an American television series aired by the Fox Broadcasting Company. ... Dr. Gregory House, M.D., is a fictional character and protagonist of the Fox medical drama House. ...

Hyperbaric Oxygen Therapy

Hyperbaric Oxygen Therapy has proven itself to be a very viable option to treat this disorder. Numerous articles in the medical literature have shown the reduction in pain and swelling with return of function using hyperbaric oxygen. Best of all, this is accomplished without the need for painful injections, narcotics, or surgical implants for pain management. Most people find that following the first few treatments they are able to achieve a full nights sleep for the first time in years. They then notice the decreased need for narcotics, improved thinking, with a diminished depression.The swelling of the effected arm or leg diminishes and they are able to start physical therapy with marked improvement in muscle strength. Many people have then been able to return to a happier and more productive life, frequently returning to their prior occupation.

Prognosis

Good progress can be made in treating CRPS if treatment is begun early, ideally within 3 months of the first symptoms. Early treatment often results 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. In 50 percent of CRPS cases, pain persists longer than 6 months and sometimes for years. ^{[citation needed]} 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.

Similar disorders

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. Atrophy is the partial or complete wasting away of a part of the body. ... Erythromelalgia, also known as Mitchells disease (after Silas Weir Mitchell) and red neuralgia(also referred to as man on fire), is a rare disorder in which blood vessels, usually in the extremities and especially in the feet, are blocked and inflamed, causing a painful burning and throbbing sensation and...

Current research

The National Institute of Neurological Disorders and Stroke (NINDS), a part of the National Institutes of Health (NIH), supports and conducts research on the brain and central nervous system, including research relevant to RSDS, through grants to major medical institutions across the country. NINDS-supported scientists are working to develop effective treatments for neurological conditions and, ultimately, to find ways of preventing them.Investigators are studying new approaches to treat RSDS and intervene more aggressively after traumatic injury to lower the patient's chances of developing the disorder. In addition, NINDS-supported scientists are studying how signals of the sympathetic nervous system cause pain in RSDS patients. Using a technique called microneurography, these investigators are able to record and measure neural activity in single nerve fibers of affected patients. By testing various hypotheses, these researchers hope to discover the unique mechanism that causes the spontaneous pain of RSDS and that discovery may lead to new ways of blocking pain.Other studies to overcome chronic pain syndromes are discussed in the pamphlet "Chronic Pain: Hope Through Research," published by the NINDS. The National Institute of Neurological Disorders and Stroke is a part of the U.S. National Institutes of Health. ... Human Microneurography or simply microneurography involves the insertion of metal microelectrodes into nerve fascicles. ... An axon, or nerve fiber, is a long slender projection of a nerve cell, or neuron, which conducts electrical impulses away from the neurons cell body or soma. ...

Research into treating the condition with Mirror Visual Feedback is being undertaken at the Royal National Hospital for Rheumatic Disease in Bath. Patients are taught how to desensitize in the most affective way then progress on to using mirrors to rewrite the faulty signals in the brain that appear responsible for this condition.

CRPS in animals

CRPS has also been described in non-human animals.^[17]

External links

• RSDSA
• ForGrace.org
• RSDhope.org
• RSDS Fact Sheet at NINDS
• Complex Regional at NINDS
• Canadian RSD
• RSD UK, National charity helping those with and treating the condition
• Celebrity movement for RSD
• RSD Therapy

The National Institute of Neurological Disorders and Stroke is a part of the U.S. National Institutes of Health. ... The National Institute of Neurological Disorders and Stroke is a part of the U.S. National Institutes of Health. ...

References