Reflex Sympathetic Dystrophy . V1, I3
Reflex Sympathetic Dystrophy is characterized by severe pain, swelling and changes in the skin.
Reflex Sympathetic Dystrophy
Reflex Sympathetic Dystrophy is characterized by severe pain, swelling and changes in the skin. It is a condition formerly known as “causalgia” or “Sudek’s atrophy”. In today’s medical literature, it is referred to as Complex Regional Pain Syndrome (CRPS).
CRPS often worsens over time. It may initially affect an arm or leg and can spread throughout the body The International Association for the Study of Pain has proposed dividing CRPS into two types based on the presence of nerve lesion following an injury:
Type I does not demonstrate nerve lesions. Most of the patients diagnosed with CRPS have this type.
Type II, formerly known as “causalgia”, demonstrates evidence of obvious nerve damage. CRPS is associated with impaired social and occupational function. The pain of CRPS is continuous, and it is widely recognized that it can be exacerbated by emotional or physical stress.
Exact Cause Unknown
Complex regional pain syndrome’s exact cause is unknown. Possible causes include trauma, i.e. fracture, obvious injury to nerves, and surgery.
Nerve damage is considered to be the cause in many cases. CRPS can occur at any age with the average age at diagnosis being 42. It affects both men and women; however, CRPS is found more frequently in females than males.
It is estimated that between 1.5 and 6 million Americans suffer from the devastating chronic pain disease. It has also been reported that CRPS cases among adolescents and young adults is increasing.
The signs and symptoms are as follows:
Continuous and intense pain that is out of proportion to the severity of the injury. Sometimes there is no injury. The pain gets worse rather than better over time. It most often affects the arms, legs, hands or feet and is accompanied by complaints of “burning” pain, increased skin sensitivity, changes in skin temperature: warmer or cooler compared to the opposite extremity changes in skin color: often blotchy, purple, pale or red.
There are also changes in skin texture: shiny and thin, sometimes excessively sweaty (hyperhidrosis). In addition there are changes in nail and hair growth patterns. There is also softening and thinning of the bones affected as well as swelling and stiffness in affected joints. This in turn can lead to decreased or lack of mobility of the extremities.
RSD pain can be exacerbated by the slightest physical or emotional stimulation. For example, bright lights, vibrations, or a gentle breeze, etc. Many healthcare professionals mistakenly believe RSD is a psychological condition rather than a physical one. Misdiagnosis can occur.
Those who sufferer often develop severe psychological problems, probably due to their pain. RSD wreaks havoc on the lives of the patient, their loved ones and co-workers. Some of the associated problems include suicide, divorce, patient disability, unemployment, improper treatments, multiple surgeries, and diminished quality of life for the affected individuals and their spouses/significant others.
There is no single diagnostic test to confirm CRPS. Diagnosis is based on the affected individual’s medical history and physical examination. Careful attention is paid to the signs and symptoms and physical examination is important. Differential diagnosis is key because several other conditions can cause similar symptoms.
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 trauma/injury should be carefully examined to avoid misdiagnosis.
In addition to history and physical examination, x-rays, other diagnostic imaging, and EMG/NCV can be used to confirm the diagnosis. X-rays and magnetic resonance imaging (MRI) can be a useful diagnostic tool. Osteoporosis which may be due to disuse (the patient will protect the affected extremity) of the affected extremity, can be detected through X-rays as early as two weeks after the onset of CRPS. A bone scan of the affected limb can detect these changes even sooner. Bone densitometry can also be used to detect changes in bone mineral density.
Electromyography (EMG) and Nerve Conduction Studies (NCS) are important tests to perform for the differential diagnosis of CRPS because they are among the most reliable and reproducible methods of detecting nerve injury. This testing also helps to distinguish between CRPS I & II, which differ based on whether there is evidence of actual nerve damage.
EMG & NCS are also among the best tests for ruling in or out diagnoses. CRPS is a “diagnosis of exclusion”, which requires that there be no other diagnosis that can explain the patient’s symptoms. Strong emphasis is placed here because patients can be misdiagnosed.
EMG and NCS involve some measure of discomfort to the patient. EMG involves the use of a tiny needle that is inserted into specific muscles to test muscle and nerve function. It can be performed on the upper extremities or the lower extremities. The testing involves using a small current. In normal patients, this would not provoke an exaggerated response. However; in patients who experience severe allodynia, they may wish not to have the test performed. In any event, informed consent needs to be obtained prior to this procedure.
The general approach in CRPS treatment is often multi-disciplinary. This includes the specialties of neurology, pain management, physical therapy, occupational therapy, psychology/psychiatry, and the use of medications. The desired goal is to improve mobility, flexibility, strength, perception of pain, and function.
It is important to have a supervised exercise program designed to keep the painful limb or body part moving.
This can improve blood flow to the affected extremity. In addition, pain alters the way we move our bodies. It should also be noted that rehabilitating the affected limb can help to prevent or reverse the secondary brain changes that are associated with chronic pain. Occupational therapy can also help the individual re-educate their body with new ways to work and perform daily tasks.
Now let’s move on to psychotherapy. CRPS and other painful and disabling conditions have been associated with profound psychological changes for affected individuals and their families. People with CRPS may develop depression, anxiety, or PTSD. It is important to address the physical issues as well as the secondary emotional issues.
There are several medications which have been shown to be effective for the treatment of CRPS, especially when used early in the course of the disease. There is no drug which is approved by the U.S. FDA specifically for CRPS.
Drugs to treat CRPS include:
A. NSAIDS: Ibuprofen, Naproxen, Aspirin.
B. Corticosteroids which are used to treat inflammation/swelling and edema. For instance, prednisolone and methylprednisolone.
C. Drugs shown to be effective for neuropathic pain, such as gabapentin (Neurontin), pregabalin (Lyrica), amitriptyline (Elavil), nortriptyline (Pamelor), and duloxetine (Cymbalta)
D. Botox injections
E. Opioids such as oxycontin, morphine, hydrocodone, fentanyl, and vicodin
F. N-methyl-D-aspartate (NMDA) receptor antagonists such as dextromethorphan and ketamine
G. nasal calcitonin, especially for deep bone pain
H. topical local anesthetic creams and patches such as lidocaine.
I. Bisphosphonates (Boniva, Fosamax)
When pharmacological intervention fails or does not produce the desired relief, CRPS patients may try surgical avenues. A sympathetic nerve block. Some individuals report temporary pain relief from sympathetic nerve blocks. There is no published evidence of long-term benefit. Sympathetic blocks involve injecting an anesthetic next to the spine to directly block the activity of sympathetic nerves and improve blood flow.
Another surgical procedure is surgical sympathectomy. This procedure destroys some of the nerves and is controversial at best. Some experts think it is unwarranted and others report a favorable outcome. Sympathectomy should only be used in individuals whose pain is substantially relieved by sympathetic nerve blocks. It also can reduce excess sweating. Other types of surgical procedures include spinal cord stimulation and neural stimulation.
There has been ongoing research in regards to treatment of CRPS. This includes: Intravenous immunoglobulin (IVIG), Ketamine, and Hyperbaric oxygen. In addition, there are several alternative therapies have been used to treat CRPS. These include psychotherapy, acupuncture, behavioral and relaxation techniques, and chiropractic treatment.
CRPS is a debilitating condition. It worsens over time. It is important to employ a multidisciplinary approach in order to address the complexities of this condition.
There is help out there. Refer to the Resources for the contact information for organizations which can help you and educate you about this condition.
P.O. Box 5801
Bethesda, MD 20824
American RSD Hope Organization
P.O. Box 875
Harrison, ME 04040-0875
International Research Foundation for RSD/CRPS
1910 East Busch Boulevard
Tampa, FL 33612
Fax: (813) 830-7446
Reflex Sympathetic Dystrophy Syndrome Association (RSDSA)
P.O. Box 502
99 Cherry Street
Milford, CT 06460
Fax: (203) 882-8362
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