Reflex Sympathetic Dystrophy Syndrome: Sometimes Misdiagnosed, Often Misunderstood

Reflex sympathetic dystrophy syndrome (RSD) is not a new condition. Reports demonstrate that the first cases of RSD date back to the days of the Civil War. RSD has also been referred to as Sudeck's atrophy; minor and major causalgia; posttraumatic neuralgia; posttraumatic dystrophy; shoulder hand syndrome; and reflex neurovascular dystrophy. As of 1998, reports suggest that the name will change to complex regional pain syndrome (CRPS).¹

RSD is a multisymptom, multisystem syndrome usually affecting one or more extremities, but may affect any part of the body. Although it was clearly described more than 25 years ago by Drs. Mitchell, Moorehouse and Keen, RSD remains poorly understood and is often unrecognized.^2,3

RSD is a disabling disease with simultaneous involvement of nerve, skin, muscle, blood vessels and bones. The sympathetic nervous system affects all tissue levels: skin, subcutaneous, fascia, muscle, synovium and bone. The only common denominator in all patients is pain. It should be noted that RSD is a condition that not only affects adults but also children.^4,5

Causes of RSD

The etiology of RSD is varied. The largest precipitating factor believed by most practitioners and researchers is trauma. Trauma does not have to be severe to cause the condition to develop. Many believe minor trauma to a joint, tendon or muscle may be the largest provoking factor in causing this disabling condition. Other causes may be a result of ischemic heart disease and myocardial infarction, cerebral lesions, infections, surgery and repetitive motion disorders or cumulative trauma (such as carpal tunnel). There is also a case reported in the literature regarding RSD resulting from surgical breast reduction.⁶

It has been suggested that cigarette smoking may be linked to RSD or be involved in the pathogenesis by increasing sympathetic activity, vasoconstriction, or by some unknown mechanism.^7,8,9 An untreated peripheral nerve entrapment is another condition which can result in RSD.¹⁰ Burns have also been implicated in precipitating causes to RSD.¹¹

In many patients, an exact precipitating cause can not be identified. Other possible hypotheses to the etiology of RSD include: failure of normal sympathetic arcs to shut down; a reverberating circuit mechanism in the spinal internuncial pool, generating abnormal volleys of nerve impulses up, down and across the spinal cord; and injury to a peripheral nerve which causes hypersensitivity to circulating norepinephrine by pressure and movement. It is thought that artificial synapses cause a shortþcircuit effect in the periphery. And lastly, a nociceptor response associated with trauma can produce long-term sensitization of wide dynamic range (WDR) in the dorsal horn causing a high rate of impulse firing.^12,13

A recent hypothesis is that there may be a genetic aberration of a recessive gene as it relates to the opiate hormonal system. It has been discovered that a recessive gene in a specialized breed of rat may have an abnormality which causes a neuropathic type of pain. This abnormality may cause some type of reflex neurogenic inflammation which causes neurotransmitters to be released into the peripheral nerves causing vasospasms, vasoedema and pain.

Signs and Symptoms

The first symptom associated with RSD is pain. Pain may start out in one or more extremities as mild and burning. It is usually constant. It tends to progress into severe pain and has been described as boring. Swelling usually accompanies the pain and can be pitting or nonpitting edema. It is usually localized and tender to touch.

There may be diminished motor function. This may include a decrease in the normal range of motion and/or a diminished strength and atrophy of the muscle associated with the joint/extremity. There may be tremors or difficulty initiating movement. There may be muscle spasms and focal dystonia.

There are skin changes. The skin may appear atrophied; there may be dryness, scaling increased hair growth or loss, nail changes and changes in temperature of one limb as compared to the other. There will be vasomotor instability: Raynaud's phenomenon, cool-pallid, mottles, warm erythematous and hyperhidrosis, and increased sweating. There are bone changes, such as patchy osteoporosis, increased vascularity (noted on bone scan) and prominent osteoclastic activity. The condition may be bilateral. It is reported that between 18-50% of cases are bilateral. Synovial fluid may be abnormal. High levels of lymphocytes, perivascular infiltration and fibrosis of the subsynovium have been reported.

Reflex sympathetic dystrophy syndrome has been known to present in three stages:

Stage 1

1. onset of severe, burning pain closely limited to site of injury;
2. hyperesthesia;
3. localized edema;
4. muscle spasm;
5. stiffness and limited mobility;
6. vasospasms; at onset, skin is usually warm, red and dry and then changes to cyanotic, cold and sweaty
7. average duration of stage is 1-3 months; in mild cases this stage lasts a few weeks, then subsides spontaneously or responds to treatment;
8. hyperhidrosis.

1. pain becomes even more severe and more diffuse;
2. edema spreads and changes from a soft to a brawny type;
3. hair becomes scant, nails become brittle, cracked and heavily grooved;
4. spotty osteoporosis occurs early, but may become severe and diffuse;
5. increased thickness of the joint;
6. muscle wasting;
7. stage two may last from 3-6 months.

1. marked trophic changes eventually become irreversible;
2. for many patients, the pain becomes intractable and may involve the entire limb;
3. atrophy of the muscles;
4. interphalangeal and other joints of the foot or hand have become extremely weak, have limited motion and may finally become ankylosed;
5. contraction of flexor tendons occurs and occasionally subluxations are produced;
6. bone deossification has now become marked and diffuse.

The diagnosis for RSD is concluded from objective documentation and from signs and symptoms directly from the patient. The diagnosis is sometimes obscure and requires a complete history, physical and psychological evaluation.¹⁴

Diagnostic testing consists of triphasic radionuclide bone scanning, thermography, galvanic skin test measurement, histological evaluation of synovial fluid, use of sympathetic nerve block (diagnostic and therapeutic), plain film radiology and use of EMG, MRI and CT scans.¹⁵

Numerous neurophysiological tools are helpful in establishing and quantifying neuropathic disturbances that may be associated with pain. Some of these tests include: quantitative sensory tests (QSTs); autonomic tests (ATs); microneurography (MCNG); and laser evoked potentials (LEPs). Quantitative sensory testing of the nociceptive system includes the thermal stimulation (TST) and current perception threshold (CPT) tests. The quantitative sudomotor axon reflex test (QSART), resting sweat output (RSO) and the sympathetic skin response (SSR) are the tests for sudomotor involvement. The vasomotor system is tested by measuring skin temperature using thermography.¹⁴

Positive results from bone scans will demonstrate increased uptake. Thermography results will show thermal asymmetries greater than .5 C, and sympathetic blocks (if positive) will relieve the patient's pain. Plain film radiographs in later stages will demonstrate osteoporosis. EMG, MRI and CT scans will usually be negative.

Treatment

Because this condition is a complex one, the treatment for it should be comprehensive and multidisciplinary. There are many treatment modalities, depending on the severity of the case.

Physical therapy and rehabilitation to relieve pain and increase function and range of motion to a joint should be utilized throughout any stage of this condition. In one study, ultrasound showed some effectiveness in relieving symptoms.¹⁶ Chiropractic spinal manipulation was shown to increase peripheral blood flow.¹⁷ Medications such as analgesics, muscle relaxers and narcotics are utilized. Sympathetic nerve blocks, used diagnostically as well, help to relieve and eliminate pain in some cases. Morphine infusion pumps have also been used to alleviate pain. Dorsal column stimulators which are implanted under the skin of the spine send out electrical signals to block pain receptors. Lastly, psychological counseling is important in the management of cases such as these.

RSD is a complex problem. It requires a team of experts to render appropriate care and perform necessary tests to diagnose this problem early. Chiropractors are uniquely positioned to have an important role in the comanaging of these patients. We may be the first physician to recognize that the patient has this condition. Because this condition appears to be sympathetically related, chiropractors should be involved in delivering treatment to patients who are suffering from this condition.

The important consideration of this condition is early detection. Left unrecognized and untreated, this condition could progress to a life of long-term disability. This condition can lead to severe unremitting pain, muscle atrophy/weakness, joint contractures and decreased range of motion. There have been cases of limb amputation from severe unrelenting pain.¹⁸

References

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