Reflex Sympathetic Dystrophy

Reflex Sympathetic Dystrophy is a medical condition which is usually referred to as a debilitating disorder, characterized by chronic pain in a particular limb. This condition is also referred to as Complex Regional Pain Syndrome (CRPS) Type 1. This is one of two types of Complex Regional Pain Syndrome which is a post-traumatic disorder associated with severe, continuous and spontaneous pain in the affected body part. The other type is Complex Regional Pain Syndrome Type 2, which is a rarer form of the medical condition than Reflex Sympathetic Dystrophy. Both conditions have been found to develop after a major injury in the affected body part, however CRPS Type 2 (or Causalgia as it was previously known) is differentiated from its counterpart by significant nerve damage to the peripheral nerves.

Sensory, vasomotor, sudomotor and trophic disturbances to the patient’s body in the affected area are known to be associated with the Reflex Sympathetic Dystrophy disorder. These changes occur gradually and may eventually lead the patient to lose function of the affected body part. The severe pain from the Reflex Sympathetic Dystrophy disease may last for several months or longer, especially if the condition goes untreated past the early stages. Reflex Sympathetic Dystrophy usually affects a specific body part and originates after an injury, trauma or surgery on that part which is usually an arm, hand, leg or foot.

The International Statistical Classification of Diseases and Related health problems (ICD) identifies Reflex Sympathetic Dystrophy using several codes that specify which limb is affected. This helps in identifying the specific limb affected by the disorder when a diagnosis is made. Once a diagnosis has been made, some examples of Reflex Sympathetic Dystrophy ICD 10 codes that can be assigned are G90.51 for Reflex Sympathetic Dystrophy of the upper limb, G90.52  for Reflex Sympathetic Dystrophy of lower extremity, and G90.523 for Reflex Sympathetic Dystrophy of the lower limb, bilateral.

Reflex Sympathetic Dystrophy Causes

Now that there is an explanation of what is Reflex Sympathetic Dystrophy, understanding the medical condition is fairly easier. The first step towards understanding the Reflex Sympathetic Dystrophy disorder is knowing what causes it in the first place. The causes of Reflex Sympathetic Dystrophy have perplexed medical minds for years. Challenges in understanding the Reflex Sympathetic Dystrophy disease have caused most examiners to depend on inferences based on experimental models designed to determine how the medical condition develops in a patient.

Technically speaking, it is suspected that a malfunction in the sympathetic nervous system and immune system results in the Reflex Sympathetic Dystrophy disorder. This may be because of how these systems react to tissue damage from injury. This may affect nerves causing them to send disproportionate pain signals to the brain. These signals then result in the patient feeling the disproportionate and extreme pain which is related to the Reflex Sympathetic Dystrophy disease. Malfunctions in the sympathetic nervous system can be triggered by trauma such as fractures, as well as infections, sprains, amputation, radiation therapy, and surgical operations.

Scientists have proposed that after the causal trauma, pro- inflammatory neuropeptides and cytokines are released, causing neurogenic inflammation. This opens the pathway for excess microvascular permeability which then leads to intramedullary pressure which limits oxygen extraction and offsets cellular hypoxia. This can explain the worsening of the Reflex Sympathetic Dystrophy disorder with time. It worsens the disorder and produces metabolic tissue acidosis. From this, it is possible that the sympathetic nervous system contributes to the development of vasomotor disturbances when it malfunctions. There are some instances where Reflex Sympathetic Dystrophy develops in the absence of the triggers mentioned above.

Reflex Sympathetic Dystrophy stages

It has been observed that over time, the Reflex Sympathetic Dystrophy disorder changes such that it will exhibit different characteristics at different points in time. This led to the proposition that the Reflex Sympathetic Dystrophy disorder has stages. There are three clinical stages of Reflex Sympathetic Dystrophy; namely acute, subacute, and chronic and their progression is as follows.

Acute

After an inciting injury or without apparent cause, the patient begins to experience pain in the affected limb. This stage lasts approximately 3 months. The burning pain is usually the first symptom followed by puffy swelling, hyperhidrosis, redness, warmth, and demineralization of the underlying bony skeleton as a result of disuse of the limb which can be detected by radiographs. Vasomotor disturbances may occur with varying intensity and these may lead to changes in color and temperature of the skin of the affected limb.

Subacute

For the next 3 to 6 months, if treatment of Reflex Sympathetic Dystrophy does not begin in the acute phase, the condition may progress to the second stage. In this subacute stage, the patient develops persistent, severe pain in the affected limb and fixed edema. Loss of function increases because of the enhanced pain, as well as thickening and scarring of connective tissue in the joints as a result of chronic inflammation. Skin and subcutaneous tissues begin to atrophy and demineralization of the underlying bony skeleton that began in the acute stage becomes pronounced. Muscle wasting may also occur, as well as development of brawny skin. Bone scan graphs done in the second stage of the disease will show osteopenia.

Chronic

The chronic stage is the last stage that may develop approximately 1 year after the onset of the Reflex Sympathetic Dystrophy disease. This stage may last for several years or become permanent. During the chronic stage, the pain level varies and may subside or continue undiminished. Edema may subside over time and the skin will be dry, pale, cool, and shiny. This stage is mainly characterized by a more pronounced stiffness of the affected area and limitation of movement, the shoulder-hand syndrome, contractures of the digits, waxy trophic skin, and brittle ridged nails. A bone scan done in the chronic stage of the Reflex Sympathetic Dystrophy disease will indicate severe demineralization.

Clinical Features of Reflex Sympathetic Dystrophy

Of the many clinical features present in Reflex Sympathetic Dystrophy patients, most are influenced by the duration of the medical condition and the location. With regards to duration, most of the initial symptoms go away within a year and a half from the onset on their own or with treatment. The longer the symptoms last, the higher the chances of some symptoms enduring such as sensory abnormalities, while others are less likely to last such as abnormal sweating and swelling.

The symptoms can be located anywhere in the body but the typical sites for Reflex Sympathetic Dystrophy are the extremities. With time, the symptoms may actually spread from the original site of the affected part to other parts of the body; for example a frozen shoulder sensation is usually associated with Reflex Sympathetic Dystrophy in the hand. The clinical features that are used to identify Reflex Sympathetic Dystrophy are:

  • Spontaneous pain

Unlike most Reflex Sympathetic Dystrophy symptoms, pain is not limited to the affected region. The pain is usually constant, but it may be intermittent in some cases, and is often described as a burning pain that’s disproportionate to the inciting event. The pain can feel worse when the affected extremity is moved, touched, or exposed to any external stimuli such as running water or cold weather.

  • Reduced mobility of the affected extremity

Due to the severity of the Reflex Sympathetic Dystrophy pain, the patient usually avoids moving the limb so as not to aggravate the pain. Prolonged disuse results in difficulties or the inability to use the affected extremity. This can also be associated with neglect-like symptoms where the limb feels foreign and directed mental and visual attention is required to move it.

  • Skin temperature changes

The temperature between the affected and unaffected limbs differs in Reflex Sympathetic Dystrophy patients. In the initial stages of the Reflex Sympathetic Dystrophy syndrome, the affected limb is usually warmer and later gets colder as the disease evolves.

  • Impaired motor function

This affects most Reflex Sympathetic Dystrophy patients at some point of the disease.

  • Limited range of motion

Results from pain and the affected limb tremors in later stages of the Reflex Sympathetic Dystrophy syndrome.

  • Muscle spasms

Present in Reflex Sympathetic Dystrophy patients who have had the medical condition for a long time.

  • Allodynia

Increased sensitivity to non-painful stimuli such as touch and noise.

  • Hyperpathia

Exaggerated response to painful stimuli.

  • Abnormal sweating

This is a sign of autonomic disturbances and for most patients, the condition can be hyperhidrosis where the affected part sweats more profusely than normal.

  • Skin colour changes

These are a result of vasomotor changes.

  • Atrophy

The soft tissues, muscles, and bones might atrophy as a result of trophic changes which may result from disuse of the affected extremity.

 

Reflex Sympathetic Dystrophy Diagnosis

The first clue pointing to a Reflex Sympathetic Dystrophy diagnosis is a lasting pain that is more severe than it should be for the inciting injury. Some medical conditions such as arthritis, Lyme disease, muscle diseases, blood clots in veins, and small fiber polyneuropathies may exhibit the same symptoms and must be checked for and ruled out before a Reflex Sympathetic Dystrophy diagnosis is given.  Some examples of related disorders that might have the same symptoms as Reflex Sympathetic Dystrophy include:

  • Infections of the skin, muscle, or bone which can have clinical features similar to Reflex Sympathetic Dystrophy such as redness, swelling, and pain.
  • Vascular diseases which are medical conditions that affect the blood vessels can produce redness, swelling, and pain in the affected extremity because of clotting of the veins in the legs and feet. The extremities can also become discoloured and cold to the touch due to reduced blood flow as a result of the clots.
  • Raynaud’s disease, which is a condition where the blood vessels in the hands and fingers are sensitive and highly responsive to cold temperature and emotional stress. As a result of these conditions, the skin colour of the hands changes to blue and/or white.
  • Rheumatoid arthritis—a chronic inflammatory disease which affects the joints, hence causing pain that might be mistaken for Reflex Sympathetic Dystrophy pain.

The Budapest Criteria is the guideline used for Reflex Sympathetic Dystrophy  diagnosis. It features two main categories for signs and symptoms, each with four subsections for sensory, vasomotor, motor, and trophic changes. The Reflex Sympathetic Dystrophy symptoms and signs mentioned above are listed under those subsections.  Those listed clinical features are compared to the signs and symptoms in the patient during an examination. If they fit the requirements of the Budapest Criteria, then a Reflex Sympathetic Dystrophy diagnosis is given. Another condition for a Reflex Sympathetic Dystrophy diagnosis to be made on the basis of these clinical features is that no other diagnosis should fit said signs and symptoms.

 Some medical tests can be done in addition to the use of the Budapest Criteria to help with this process of elimination and confirming certain Reflex Sympathetic Dystrophy signs and symptoms. These include:

1.        Imaging studies

These include tests that show images within the body, highlighting changes caused by Reflex Sympathetic Dystrophy.

  • Radiographs

Radiographic films such as X-rays can show demineralization due to bone loss within 3 to 6 weeks from the onset of the Reflex Sympathetic Dystrophy disease.

  • Three-phase bone scan

This type of scan can help reveal bone loss typical of Reflex Sympathetic Dystrophy.

2.        Other tests

  • Skin temperature tests

Variations in skin temperatures are one of the key features of Reflex Sympathetic Dystrophy. There are typically two phases of vascular changes in Reflex Sympathetic Dystrophy. The first phase is called the hyperemic phase where the temperature of the skin increases and it is often seen in the early stages of the medical condition. The second phase which usually occurs later is the cold limb phase where the temperature of the skin decreases significantly. The skin temperature differences can be measured using thermography tests which quantitatively highlight differences in limb temperature.

  • Sudomotor function testing

This consists of tests for sudomotor changes such as the sweat test, quantitative sudomotor axon reflex test (QSART), chemical sweat test, and testing the sweat output.

  • Quantitative sensory testing

These tests are used to quantify perception thresholds using precise, reproducible stimuli. Quantitative sensory tests provide information on the severity and progression of the sensory disturbances. Heat and cold pain thresholds are also checked for during these tests.

  • Laser doppler imaging

This is a method for studying autonomic functions by testing the skin’s autonomic reflexes. The images also quantify vasoconstrictor reflexes.

Reflex Sympathetic Dystrophy Treatment and Management

One thing that most people suffering from the disorder wonder is can Reflex Sympathetic Dystrophy be cured? Unfortunately there is no cure for the medical condition, but several treatments are available to help in managing the pain. Early diagnosis and treatment of the Reflex Sympathetic Dystrophy disorder help to avoid secondary physical problems that may arise from disuse of the affected limb, as well as the psychological consequences of living with a chronic pain. The primary objectives of Reflex Sympathetic Dystrophy treatment are pain management, preserving or restoring limb function, and helping the affected individual manage their condition and improve their quality of life. Reflex Sympathetic Dystrophy treatment incorporates patient education, pain relief, physical rehabilitation, and psychological intervention. Reflex Sympathetic Dystrophy treatment can be tailored for each stage as the diseases evolves as follows:

  • Pain management for acute and chronic stages

Medical pain therapy for these stages may include a variety of drugs used together for the best results. The types commonly used to treat Reflex Sympathetic Dystrophy include tri-cyclic antidepressants, analgesics, opioids, and intravenous ketamine. If the medical therapy approach fails to provide significant pain relief, some alternative non-medical pain therapies can be considered such as spinal cord stimulation.

  • Anti-inflammatory therapy for the acute stage

Most Reflex Sympathetic Dystrophy patients experience swelling in the affected limb, especially in the initial phase of the disease. This is why anti-inflammatory therapy should be more focused in the acute phase. Corticosteroids are a class of drugs that have anti-inflammatory properties and can be prescribed to help reduce inflammation in a Reflex Sympathetic Dystrophy patient. The dosage might have to be increased over time. Bisphosphonates such as Neridronate also inhibit post traumatic inflammation in addition to relieving other symptoms. Neridronate is actually one of the most effective treatments for Reflex Sympathetic Dystrophy, especially in the early stages. It is authorized for Reflex Sympathetic dystrophy treatment in Italy so anyone who wants to try that treatment has to travel there.

  • Physiotherapy and occupational therapy

Loss of function is a common characteristic of the Reflex Sympathetic Dystrophy disease. It is believed that physiotherapy can help a patient overcome their movement limitations and regain function in the affected limb. Of course any kind of physical activity in the affected extremity is likely to aggravate the pain and other symptoms, but long-term this activity is believed to provide some benefits such as better pain tolerance and an increased pain threshold. Keeping the affected limb also helps to prevent contractures and irreversible damage.

Physiotherapy involves a range of gentle exercises and mental exercises such as mirror therapy where the patient learns to treat and move the affected limb as if it were the healthy one. Such therapies are believed to reduce pain by retraining the brain, and also improve movement. Mirror therapy is more effective with acute RSD and post-stroke RSD. Graded motor imagery is another type of physical therapy that can be used in the treatment of Reflex Sympathetic Dystrophy. This therapy involves identifying left and right extremities then imagining moving the affected extremity, followed by mirror therapy.

Occupational therapy involves developing, recovering, or maintaining certain activities that make up the patient’s daily home or work life so they can return to their normal routine, albeit in new or modified ways. Some examples of occupational therapy tasks and skills are self-care routines such as getting dressed, writing, and holding objects.

  • Psychotherapy and sociotherapy

Reflex Sympathetic Dystrophy patients typically have reduced range of motion and limited mobility often resulting from pain-related fear of moving their affected limbs. In addition to these feelings of fear, having to endure chronic pain constantly or on a regular basis causes other emotions such as stress, anxiety, and depression. Other negative emotions associated with Reflex Sympathetic Dystrophy are posttraumatic stress disorders (PTSD), feelings of injustice, and even financial worries on account of the expenses of getting adequate treatments. In fact, Reflex Sympathetic Dystrophy is nicknamed the “Suicide Disease” because it negatively impacts the mental state of the affected individual to the point that they would wish for death, rather than to deal with the pain any longer. Unfortunately, some patients actually go through with suicide to end the pain.

The emotional stress also aggravates the Reflex Sympathetic Dystrophy pain. This makes it necessary for psychotherapy to be part of the treatment plan. An example of psychotherapy treatments is Graded Exposure (GEXP) treatment. During this treatment, a psychologist identifies situations that trigger fear such as certain activities that aggravate the pain. After identifying and classifying these situations, a physiotherapist gradually exposes the patient to these fear triggering situations to encouraging adapting to them. GEXP is believed to reduce pain and improve function as a result.

  • Emerging treatments:
  • Immunomodulation: The use of anti-cancer drugs such as lenalidomide and thalidomide helps with Reflex Sympathetic Dystrophy treatment because they have anti-inflammatory and immunomodulatory effects.
  • Hyperbaric oxygen therapy: From some studies using hyperbaric oxygen therapy, Reflex Sympathetic Dystrophy patients have had rapid pain relief due to the anti-nociceptive effect of the therapy.
  • Boyulinum toxin-A (BTX-A): This chemical has been found to provide pain relief for neuropathic pain. Further studies need to be done on the efficacy of this treatment before it is recommended for Reflex Sympathetic Dystrophy treatment.
  • Plasma exchange: Some studies of the use of plasma exchange therapy have demonstrated that it is beneficial for autoimmune disorders, in particular maintaining pain relief.
  • Alternative treatments

For some cases of Reflex Sympathetic Dystrophy, the conventional treatments don’t work as well as they do in other cases or at all really. This is where alternative treatments come in. Most of these aren’t widely used due to the lack of evidence of their efficacy and also the disagreements surrounding their use in the first place. Alternative Reflex Sympathetic Dystrophy treatments include sympathetic nerve blocks which suppress the abnormal activity of the sympathetic nervous system which is a key feature of the disease.

Other alternatives include surgical sympathectomy, amputation, and spinal cord stimulation. Amputation is the least recommended treatment because it is actually one of the triggers for Reflex Sympathetic Dystrophy so the chances of developing the disease again after treatment are high. Surgical sympathectomy is only recommended for patients who get significant pain relief from sympathetic nerve blocks because surgery is a Reflex Sympathetic Dystrophy risk factor and it is not necessary to take that risk if simple nerve blocks don’t provide significant pain relief. Spinal cord stimulation can actually aggravate the pain so it’s also left as a last resort.

There are also some less traditional Reflex Sympathetic treatments that are natural and can be done at home. Some examples of these are:

  • Acupuncture

This is a form of treatment that involves inserting very thin needles through the skin at specific points of the body. Acupuncture is believed to help with various types of pain such as low back pain, neck pain, osteoarthritis, knee pain, headaches and migraines. It is also believed to have some benefits for medical conditions such as fribromyalgia, spine pain and neuralgia. It addition to pain control, acupuncture has the advantage of having very few side effects, is safe for patients who can’t take pain medications, and it can be combined with other treatments. For RSD, acupuncture may help with the chronic pain and there’s no negative effect of trying it.

  • Meditation

 Meditation involves the use of techniques such as mindfulness or focusing on positive thoughts to train attention and awareness and help the patient relax and reach an emotionally stable state. There are different types of meditation such as mindfulness meditation, breath awareness meditation, and zen meditation. In general, meditation provides a relaxation technique. With Reflex Sympathetic Dystrophy patients, relaxing can be a great distraction from pain and also emotional stress associated with the disease.

  • Nutritional

There isn’t really a specific diet for Reflex Sympathetic Dystrophy patients but it does help to avoid certain foods that aggravate the medical condition such as inflammatory foods. In addition to following an anti-inflammatory diet, Reflex Sympathetic dystrophy patients can benefit from avoiding carbohydrates, caffeine, red meats, and sugars. Some even go on to outline four F’s to include in the diet and five C’s to avoid and these are:

  • Four F’s: fresh fruit, fresh vegetables, fish (baked or broiled), and fowl (skinned, baked, roasted, or grilled and not fried).
  • Five C’s: cookies, cake, chocolate, cocktails, and candy

Other lifestyle features that contribute to recovery from Reflex Sympathetic Dystrophy include:

  • Sleep

An adequate amount of sleep is good for the body, especially for Reflex Sympathetic Dystrophy patients. It can be hard for affected individuals to sleep due to pain so engaging in some relaxing techniques before bed can help to get the person comfortable for sleep. Avoiding interrupting their sleep is also important.

  • Diaphragmatic breathing

This is a relaxation technique that also improves blood flow and oxygenation within the body.

Reflex Sympathetic Dystrophy Outlook

Early identification and treatment of Reflex Sympathetic Dystrophy improves the likelihood for a positive prognosis. Most of the treatments available for Reflex Sympathetic Dystrophy recommend that treatment begins in the early stages of the medical condition, that is, the acute phase which is within the first three months of onset of the disease. However within that time, some of the defining clinical features of the disease will not yet be visible so it can be very easy to miss the Reflex Sympathetic Dystrophy diagnosis. Pain is usually the first symptom and even though it is often disproportionate to the inciting injury, in the absence of other symptoms it can easily be dismissed. Such a delay in treatment can mean a very bad outcome for the disease.

The purpose of treatment is to provide the most achievable level of pain relief and also restore limb function and motor skills lost. It also helps to prevent the effects of the Reflex Sympathetic Dystrophy syndrome from progressing even further throughout the body and causing more damage. If the medical condition is left untreated for too long, the consequences may be irreversible. The disease spreads to the rest of the limb, leading to bone degradation and nerve damage as the condition progresses. There might also be structural damage to the muscles and tendons. As a result the motor dysfunctions increase until the patient can no longer move the limb at all.

On a less grimmer note, early diagnosis and treatment can improve the chances for the Reflex Sympathetic Dystrophy disease to go into remission. Remission doesn’t mean the condition is cured. There is no cure for Reflex Sympathetic Dystrophy. With treatment, a patient may get as much as five years of remission or more before the effects of the condition return. In fact, studies on the effects of Neridronate for Reflex Sympathetic Treatment have provided a lot of evidence that the disease goes into remission. In Reflex Sympathetic Dystrophy remission, the patient may not be completely pain free but the symptoms may be suppressed so that they don’t feel as bad as they usually do. There is a high likelihood that the disease won’t stay in remission forever and the symptoms might return after some time.

Conclusion

Reflex Sympathetic Dystrophy is not the end of the world. It’s a difficult medical condition, but life can still go on regardless, with the help of medical treatments, physiotherapy, and psychotherapy. It’s important for the patient to understand their limitations so that they don’t worsen their condition by taking on more activity than they can handle and causing pain they can’t bear. With the right treatments and techniques, a Reflex Sympathetic Dystrophy patient can live a normal life.

 

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