RSD/ CRPS Reflex Sympathetic Dystrophy Syndrome (RSD) is also known as Complex Regional Pain Syndrome (CRPS). RSD/CRPS is a multi-symptom, multi-system, syndrome usually affecting one or more extremities, but may affect virtually any part of the body. Although it was clearly described 125 years ago by Drs. Mitchell, Moorehouse and Keen, RSD/CRPS remains poorly understood and is often unrecognized.
The best way to describe RSD/CRPS is in terms of an injury to a nerve or soft tissue (e.g. broken bone) that does not follow the normal healing path. The development of RSD/CRPS does not appear to depend on the magnitude of the injury (e.g. a sliver in the finger can trigger the disease). In fact, the injury may be so slight that the patient may not recall ever having received an injury.
For reasons that are not fully understood, the sympathetic nervous system seems to assume an abnormal function after an injury. There is no single laboratory test to diagnose RSD/CRPS.
The physician must assess and document both subjective complaints (medical history) and, if present, objective findings (physical examination), in order to support the diagnosis.
There is a natural tendency to rush to the diagnosis of RSD/CRPS with minimal objective findings because early diagnosis is critical.
If undiagnosed and untreated, RSD/CRPS can spread to all extremities, making the rehabilitation process a much more difficult one.
If one can demonstrate major nerve damage associated with the development of RSD/CRPS symptoms, the condition is called complex regional pain syndrome (CRPS) type II or causalgia. Generally, causalgia provides more objective evidence of disease due to neurological changes (numbness and weakness).
The terms complex regional pain syndrome (CRPS) type I and type II have been used since 1995, when the International Association for the Study of Pain (IASP) felt the respective names reflex sympathtic dystrophy and causalgia were inadequate to represent the full spectrum of signs and symptoms.
The term "Complex" was added to convey the reality that RSD and causalgia express varied signs and symptoms. Many publications, particularly older ones, still use the names RSD and causalgia. To facilitate communication and understanding the designation RSD/CRPS is generally used throughout these practice guidelines. The principles applicable to the diagnosis and management of RSD are similar to those principles applicable to the diagnosis and management of causalgia.
Often the physician needs to rule out other potentially life-threatening disorders that may have clinical features similar to RSD/CRPS, e.g. a blood clot in a leg vein or a breast tumor spreading to lymph glands can cause a swollen, painful extremity.
RSD/CRPS may be a component part of another disease, (e.g. a herniated disc of the spine, carpal tunnel syndrome of the hand, heart attack). Thus, treating RSD/CRPS will often be directed to treating clinical features rather than a well defined disease.
When RSD/CRPS spreads the diagnosis can be more complicated. For example, if it spreads to the opposite limb, it may be more difficult to establish a diagnosis because there is no normal side (control) to compare for objective findings.
On the other hand, the spreading of RSD/CRPS symptoms may actually facilitate the diagnosis of RSD/CRPS because spreading symptoms is a characteristic of the disorder.
The physician must aggressively seek and document objective findings. For example, about 80% of RSD/CRPS cases have differences in temperature in opposite sides that may be either colder or warmer. These temperature changes may be associated with changes in skin color. Furthermore, the temperature differences are not static. The skin temperature can undergo dynamic changes in a relatively short period of time (within minutes) depending critically on room temperature, local temperature of the skin and emotional stress.
In some cases, the differences in temperatures may fluctuate spontaneously even without any apparent provocation. Thus, the objective finding of differences in temperature and color of the skin can be missed by the physician if only a single physical examination is made. A useful and relatively inexpensive instrument to have available at the time of the physical examination is a portable infrared thermometer to measure differences in skin temperature. Changes in skin temperature and color are only two examples of several objective findings that should be sought in the patients with RSD/CRPS.
The diagnosis of RSD/CRPS can be made in the following context. A history of trauma to the affected area associated with pain that is disproportionate to the inciting event plus one or more of the following:
Abnormal function of the sympathetic nervous system.
Changes in tissue growth (dystrophy and atrophy).
Thus patients do not have to meet all of the clinical manifestations listed above to make the diagnosis of RSD/CRPS. The new CRPS classification system acknowledges this fact by stating that some patients with RSD/CRPS may have a third type of CRPS by categorizing it as "otherwise not specified".
There seems to be a small group of patients whose pain following trauma resolves over time, leaving the patient with a movement disorder.
Pain The hallmark of RSD/CRPS is pain and mobility problems out of proportion to those expected from the initial injury. The first and primary complaint occurring in one or more extremities is described as severe, constant, burning and/or deep aching pain. All tactile stimulation of the skin (e.g. wearing clothing, a light breeze) may be perceived as painful (allodynia). Repetitive tactile stimulation (e.g. tapping on the skin) may cause increasing pain with each tap and when the repetitive stimulation stops, there may be a prolonged after-sensation of pain (hyperpathia). There may be diffuse tenderness or point-tender spots in the muscles of the affected region due to small muscle spasms called muscle trigger points (myofascial pain syndrome). There may be spontaneous sharp jabs of pain in the affected region that seem to come from nowhere (paroxysmal dysesthesias and lancinating pains).
Skin changes - skin may appear shiny (dystrophy-atrophy), dry or scaly. Hair may initially grow coarse and then thin. Nails in the affected extremity may be more brittle, grow faster and then slower. Faster growing nails is almost proof that the patient has RSD/CRPS. RSD/CRPS is associated with a variety of skin disorders including rashes, ulcers and pustules. Although extremely rare, some patients have required amputation of an extremity due to life-threatening reoccurring infections of the skin. Abnormal sympathetic (vasomotor changes) activity may be associated with skin that is either warm or cold to touch. The patient may perceive sensations of warmth or coolness in the affected limb without even touching it (vasomotor changes). The skin may show increased sweating (sudomotor changes) or increased chilling of the skin with goose flesh (pilomotor changes). Changes in skin color can range from a white mottled appearance to a red or blue appearance. Changes in skin color (and pain) can be triggered by changes in the room temperature, especially cold environments. However, many of these changes occur without any apparent provocation. Patients describe their disease as though it had a mind of its own.
Swelling - pitting or hard (brawny) edema is usually diffuse and localized to the painful and tender region. If the edema is sharply demarcated on the surface of the skin along a line, it is almost proof that the patient has RSD/CRPS. However, some patients will show a sharply demarcated edema because they tie a band around the extremity for comfort. Therefore, one has to be certain that the sharply demarcated edema is not due to a previously wrapped bandage around the extremity.
Movement Disorder - Patients with RSD/CRPS have difficulty moving because they hurt when they move. In addition, there seems to be a direct inhibitory effect of RSD/CRPS on muscle contraction. Patients describe difficulty in initiating movement, as though they have "stiff" joints. This phenomena of stiffness is most noticeable to some patients after a sympathetic nerve block when the stiffness may disappear. Decreased mobilization of extremities can lead to wasting of muscles (disuse atrophy). Some patients have little pain due to RSD/CRPS but instead they have a great deal of stiffness and difficulty initiating movement. Tremors and involuntary severe jerking of extremities may be present. Psychological stress may exacerbate these symptoms. Sudden onset of muscle cramps (spasms) can be severe and completely incapacitating. Some patients describe a slow "drawing up of muscles" in the extremity due to increased muscle tone leaving the hand-fingers or foot-toes in a fixed position (dystonia).
Spreading Symptoms - Initially, RSD/CRPS symptoms are generally localized to the site of injury. As time progresses, the pain and symptoms tend to become more diffuse. Typically, the disorder starts in an extremity. However, the pain may occur in the trunk or side of the face. On the other hand, the disorder may start in the distal extremity and spread to the trunk and face. At this stage of the disorder, an entire quadrant of the body may be involved. Maleki et. al. recently described three patterns of spreading symptoms in RSD/CRPS:
1. A "continuity type" of spread where the symptoms spread upward from the initial site, e.g. from the hand to the shoulder.
2. A "mirror-image type" where the spread was to the opposite limb.
3. An "independent type" where symptoms spread to a separate, distant region of the body. This type of spread may be related to a second trauma
Bone changes X-rays may show wasting of bone (patchy osteoporosis) or a bone scan may show increased or decreased uptake of a certain radioactive substance (technecium 99m) in bones after intravenous injection.
Duration of RSD/CRPS The duration of RSD/CRPS varies, in mild cases it may last for weeks followed by remission; in many cases the pain continues for years and in some cases, indefinitely. Some patients experience periods of remission and exacerbation. Periods of remission may last for weeks, months, or years.
RSD/CRPS may present in three stages:
The staging of RSD/CRPS is a concept that is dying. The course of the disease seems to be so unpredictable between various patients that staging is not helpful in the treatment of RSD/CRPS. Not all of the clinical features listed below for the various stages of RSD/CRPS may be present. The speed of progression varies greatly in different individuals.
Stage I and II symptoms begin to appear within a year. Some patients do not progress to Stage III. Furthermore, some of the early symptoms (Stage I and II) may fade as the disease progresses to Stage III.
Onset of severe, pain limited to the site of injury
Increased sensitivity of skin to touch and light pressure (hyperasthesia).
Stiffness and limited mobility
At onset, skin is usually warm, red and dry and then it may change to a blue (cyanotic) in appearance and become cold and sweaty.
Increased sweating (hyperhydrosis).
In mild cases this stage lasts a few weeks, then subsides spontaneously or responds rapidly to treatment
Pain becomes even more severe and more diffuse
Swelling tends to spread and it may change from a soft to hard (brawny) type
Hair may become coarse then scant, nails may grow faster then grow slower and become brittle, cracked and heavily grooved
Spotty wasting of bone (osteoporosis) occurs early but may become severe and diffuse
Muscle wasting begins
Marked wasting of tissue (atrophic) eventually become irreversible
For many patients the pain becomes intractable and may involve the entire limb.
A small percentage of patients have developed generalized RSD affecting the entire body.
A number of precipitating factors have been associated with RSD/CRPS including:
Trauma (often minor) ranks as the leading provocative event
Ischemic heart disease and myocardial infarction
Cervical spine or spinal cord disorders
Repetitive motion disorder or cumulative trauma, causing conditions such as carpal tunnel.
However, in some patients a definite precipitating event can not be identified.
There is no laboratory test that can stand alone as proof of RSD/CRPS. However, there are a couple of tests (thermogram and bone scan) which can be useful in providing evidence for RSD/CRPS.
Thermogram - A thermogram is a noninvasive means of measuring heat emission from the body surface using a special infrared video camera. It is one of the most widely used tests in suspected cases of RSD/CRPS. As noted, detecting an abnormal change in skin temperature in RSD/CRPS depends on many factors. A normal thermogram does not necessarily mean the patient does not have RSD/CRPS. An abnormal thermogram may be helpful before a jury in a court of law when there are minimal objective findings for RSD/CRPS documented in the medical record. Furthermore, certain patterns of abnormal heat emission from the body (e.g. circumferential versus dermatomal changes) are more indicative of the existence of RSD/CRPS than others. The thermogram should be performed at a reputable medical facility. The quality of the test may vary among providers.
Three phase radionuclide bone scanning the role of the 3 phase bone scan in the diagnosis of RSD/CRPS has been debated and is controversial.
Sympathetic blocks - can be diagnostic and may show prognosis.
X-rays, EMG, Nerve Conduction Studies, CAT scan and MRI studies All of these tests may be normal in RSD/CRPS. These studies may help to identify other possible causes of pain; for example, RSD/CRPS plus a carpal tunnel syndrome.
Medications commonly used to treat RSD/CRPS based on the type of pain include:
For constant pain associated with inflammation: Nonsteroidal anti-inflammatory agents (e.g. aspirin, ibuprofen, naproxen, indomethacin, etc).
For constant pain not caused by inflammation: Agents acting on the central nervous system by an atypical mechanism (e.g. tramadol)
For constant pain or spontaneous (paroxysmal) jabs and sleep disturbances; Anti-depressants (e.g. amitriptyline, doxepin, nortriptyline, trazodone, etc) Oral lidocaine (mexilitine - some what experimental)
For spontaneous (paroxysmal) jabs Anti-convulsants (e.g. carbamazepine, gabapentin may relieve constant pain as well)
For widespread, severe RSD/CRPS pain, refractory to less aggressive therapies: Oral opioid. The use of opioids (e.g. narcotics with names such as Darvon, Vicodin, Loratab, Percocet, morphine, codeine, etc) to treat RSD/CRPS is debated and there are potential hazards.
Sympathetic Blocks: There are three reasons to consider sympathetic blockade to facilitate the management of RSD/CRPS. First, the sympathetic block may provide a permanent cure or partial remission of RSD/CRPS. Second, by selectively blocking the sympathetic nervous system the patient (and physician) will gain further diagnostic information about what is causing the pain. The sympathetic block helps determine what portion of the patients pain is being caused by malfunction of their sympathetic nervous system. Third, the patients response to a sympathetic block provides prognostic information about the potential merits of other treatments.
The maximum sustained benefit from a series of sympathetic blocks is usually apparent after a series of 3-6 blocks. Even if the original site is unresponsive to sympathetic blockade, future exacerbation of RSD/CRPS symptoms at the same site or at a distant site may be responsive to 1-3 sympathetic blocks.
Since there is a hyperalgesia component the spinal levels can be identified with an allodynia skin rolling test or a diagnostic electrotherapy test using the AC or DC (depending on the actuality of the patient )current to stimulate the lowered threshold of the dorsal horn.
Spinal mobilization is also used grade (1-3)
**In both cases what we are looking for are referrals of pain, warmth, vibration,
The goal of any of the treatment is to restore sympathetic function to the area involved. The only way to do that manually is to get a somatic sympathetic reaction to reset the the sympathetic tone, and the dorsal and lateral horns.
Somato-sympathetic Reflexes (Sato & Schmidt 1973)
Single afferent volleys in muscle or cutaneous nerves elicit sympathetic reflex discharges followed by a PED (Post Excitatory Depression) of the spontaneous activity. This is when parasympathetic activity is increased.
The somato-sympathetic reflex induced by group II and III afferent volleys consists of components that take different central reflex pathways. On the basis of latency differences and various other experimental evidence three magor components have been recognized
the early spinal,
the late medullary and
the very late suprapontine component
The early spinal component is restricted to the segments near the level of afferent input. This reflex pathway possibly forms the basis for local sympathetic reflex actions.
Amount of PED
Treatment of RSDS & Segmental Impairments
CTM (Local, Segmental and Reaction points)
Massage (Local, Segmental)
Massage Deep Tissue (Rolfing, Friction, German) (Local, Segmental, Limbic)
Spinal Mobilization (Segmental, Limbic), grade I-IV
Manipulation (Segmental, Limbic)
TENS current/ AC current (Local, Segmental, Limbic)
DC current (Local, Segmental, Limbic)
***Joint Mobilization and stretching as well as nerve mobilization****
To tolerance may be very painful in later stages so may need a sympathetic block
The lab work that best identifies RSDS: 1)CBC DIFF 2)ANA Profile 3)RA factor 4)None
Causalgia is a form of Complex Regional Pain Syndrome (CRPS). T or F
What type of method with a thermometer should be used to diagnose Complex Regional Pain Syndrome (CRPS): 1) Rectally 2)Orally 3)from the ear 4)On Skin
What gives you the longest (time wise) PED Post Excitatory Depression stimulation to the sympathetic nervous system: 1) Spinal Mobilization with DC current 2) Manipulation 3) TENS 4) US
A Sympathetic block may be diagnostic and a permanent cure of RSDS, and the body will always react to the first injection. T or F
Stanton-Hicks M, Janig W, Hassenbursch S, et al. Reflex sympathetic dystrophy: changing concepts and taxonomy. Pain 1995 63:127-33.
Merskey H, Bogduk N, eds. Classification of chronic pain: pain syndromes and definition of pain terms. Second Edition. Seattle: IASP Press. 1994.
Janig W, Stanton-Hicks M, eds. Reflex sympathetic dystrophy: A reappraisal. Progress in Pain Research and Management. Volume 6. Seattle: IASP Press. 1996.
Stanton-Hicks M, Baron R, et al. Consensus Report: Complex regional pain syndromes: Guidelines for therapy. Clin J Pain 1998 14: 155-66.
Schwartzman R., McLellan T.: Reflex sympathetic dystrophy, A Review. Archives Neurology 1987 44:555-61.
Bonica J.J., et al: Causalgia and other reflex sympathetic dystrophies, Management of pain, 2nd Ed. Lea & Febiger, Philadelphia, Chapter 11, pp 234-235.
Low P. Clinical Autonomic Disorders, Evaluation and Management, 2nd Ed. Lippincott&Raven, Philadelphia (1997): Pg 211.
"Autonomic Neuropathies." Low, PA; McLeod, JG. In Clinical Autonomic Disorders: Evaluation and Management2nd ed., Low, PA, editor. Philadelphia: Lippincott-Raven Publishers (1997): chapter 36, 463-486.
Hoogland Rolf, Electrostimulation on efferent nerves, Hogeschool van Amsterdam text for physiotherapy 1995.
Van Koop Joost, Overview of CTM Massage, Hogeschool van Amsterdam text for physiotherapy 1995.