CRPS Type 2

Introduction: What Is CRPS Type 2?

CRPS Type 2, or Chronic Regional Pain Syndrome Type 2 in full, is a rare type of the medical condition known as Chronic Regional Pain Syndrome CRPS (or Complex Regional Pain Syndrome in some cases). Around the time when Chronic Regional Pain Syndrome was initially discovered, the disease was called Causalgia—a Greek word meaning “burning pain.” The CRPS medical condition is a disabling, progressive disease characterized by severe pain in one or more limbs—usually a hand or foot. The other more common type of CRPS is Chronic Regional Pain Syndrome Type 1 (CRPS Type 1). The difference between the two types is that CRPS Type 2 is caused by significant damage to the peripheral nerves while CRPS Type 1 shows no sign of such damage.

Peripheral nerves are nerves connecting the spine and brain to the body’s extremities. These nerves control the functions of sensation, movement, and motor coordination in the body.  There are three types of peripheral nerves which are motor, sensory, and autonomic and all of these are fragile and easily damaged. Certain conditions may damage all three types of peripheral nerves hence causing the sensory, motor, and autonomic disturbances that are typical in CRPS Type 2 patients. Due to the connection of peripheral nerves to the extremities, any significant damage to the nerves will be reflected in the body’s extremities such as the arms, legs, hands, and feet which are all sites commonly affected by CRPS Type 2.

Even though CRPS Type 2 is a commonly used medical term for the condition, there are other denotations used to identify it. These are known as ICD-10 codes, which are part of the International Statistical Classification of Diseases and Related Health Problems. This medical classification list was created by the World Health Organization (WHO) and it features a variety of diseases, signs and symptoms and a lot more, each with a dedicated code. For CRPS Type 2, the codes specify which limb is affected by the medical condition when a diagnosis is made on a patient. The CRPS Type 2 ICD-10 codes currently in use include:

  • 4 for CRPS Type 2 of upper limb
  • 7 for CRPS Type 2 of lower limb 
  • 70for CRPS Type 2 of unspecified lower limb 
  • 71 for CRPS Type 2of right lower limb 
  • 72 for CRPS Type 2 of left lower limb.

Causes of CRPS Type 2

It has already been established that CRPS Type 2 develops following significant damage to the peripheral nerves. But what exactly causes that kind of nerve damage in the first place? Peripheral nerve damage, or peripheral neuropathy as it is often called, can be a result of several health conditions, traumatic injuries, infections, metabolic problems, hereditary conditions, and exposure to toxins. Some examples of health conditions and other events that cause peripheral neuropathy include:

  • Diabetes
  • Infections such as HIV and Lyme disease
  • Tumors
  • Bone marrow disorders
  • Autoimmune diseases such as arthritis and other diseases such as kidney and liver disease.
  • Vitamin deficiencies
  • Alcoholism
  • Medications or medical procedures such as chemotherapy
  • Amputation

CRPS Type 2 Symptoms

Most CRPS Type 2 symptoms are similar to those of CRPS Type 1, although for the former the symptoms are localized to the area around the injured nerve; whereas for the latter the condition may spread to other areas of the affected limb and even to the rest of the body.  Some common CRPS Type 2 symptoms are:

  • Pain

The CRPS Type 2 pain is described as a prolonged, intense burning pain that the patient feels in the affected area. Pain is usually the first thing that a CRPS Type 2 patient experiences before all the other symptoms kick in. It may last for several months or longer and may even get worse on some days when the patient goes through flare ups, whereby the pain gets worse for some time usually because of emotional stress.

  • Sweating

Abnormal sweating occurs in CRPS Type 2 patients as a result of sudomotor disturbances in the body. The affected individual tends to get sweaty even while doing light activities.

  • Skin discolouration

The skin on the affected part changes to shades of red, purple, or grey as the disease progresses. Sometimes the colour changes are visible post-swelling when the swelling goes down.

  • Hyperalgesia

Due to a dysfunctional nervous system, the affected individual’s perception of pain is altered, such that an injury that would normally only be slightly painful feels excruciatingly severe. This is especially bad in the event that a patient already diagnosed with CRPS Type 2 gets an injury post-diagnosis.

  • Abnormal nail and hair growth rate

The rate at which the nails and hair grow is altered and unsteady in CRPS Type 2 patients, possibly because of deficiency in certain crucial nutrients that results from changes caused by the medical condition.

  • Spasms in the joints

Because of the severe pain in the limb, CRPS Type 2 patients tend to avoid using that limb so that they don’t aggravate the pain. Continued disuse of the affected limb causes gradual loss of function and motor issues such as tremors and muscle spasms. If the symptoms aren’t addressed as early as possible, the limb may waste away and the condition may become irreversible.

  • Swelling

Swelling is one of the symptoms that doesn’t affect every CRPS Type 2 patient. It often results from fluid retention and causes the affected area to have a shiny appearance. The swelling usually goes down after some time, even though other symptoms may persist.

These signs and symptoms are used to make a clinical diagnosis of CRPS Type 2 through diagnostic guidelines such as the Budapest Criteria. Certain medical tests can also be used in conjunction with the Budapest Criteria diagnostic tool to confirm a CRPS Type 2 diagnosis and also rule out any other medical conditions that show similar signs and symptoms.

CRPS Type 2 Treatment

Similar to CRPS Type 1, there are a variety of treatment options for CRPS Type 2. These treatments span across different categories; including medications, physical therapy, and psychological therapy. Early treatment is the most effective and should begin as soon as a CRPS Type 2 diagnosis is made. Due to the diversity of CRPS Type 2 treatment options, the patient’s treatment plan may involve a number of different specialists such as neurologists and physical therapists. The different treatment options employed for CRPS Type 2 treatment are given in more detail below:

  1. Physical therapy

Regular, gentle exercises can help improve the patient’s blood flow so they can regain functionality in the affected limb and have better tolerance for pain. Keeping the limb in motion can help prevent muscle wastage and contortion of bones that may result from prolonged disuse.

  1. Psychotherapy

Living with a chronic condition like CRPS Type 2 can take its toll on the mental health of the affected individual. This is usually because of the daunting knowledge that there is no cure for the severe pain they experience on a daily basis. Another reason is the fact that the patient can no longer do most of the things they were able to before the onset of CRPS Type 2 due to the pain they feel every time they move their limb. As a result, the patient tends to feel depressed and anxious, hence the need for psychotherapy to address these negative emotions.

  1. Medication

A patient may have to take several types of drugs to effectively treat CRPS Type 2 and manage their pain and other symptoms. The various drugs often used for CRPS Type 2 treatment include the following:

  • Non-steroidal anti-inflammatory drugs (NSAIDS)

NSAIDs such as ibuprofen are used in the treatment of acute muscular and bone pain which is why they are useful in the treatment of the chronic pain of CRPS Type 2. The advantage of these is that some types of NSAIDs are obtainable over the counter so they are easily accessible for patients who need to manage their pain.

  • Anticonvulsants

The primary function of anticonvulsants is for the treatment of epileptic seizures, but for CRPS Type 2 patients these drugs are effective in the treatment of nerve pain.

  • Topical creams and patches

The creams and patches offer pain relief for some patients and can be obtained over the counter at some pharmacies. Some examples of topical creams and patches that can be used to reduce hypersensitivity are capsaicin cream and lidocaine patches.

  • Corticosteroids

Corticosteroids have an anti-inflammatory effect and are more effective when used in the early stages of the medical condition.

  • Bisphosphonates

Bisphosphonates such as Neridronate are highly effective in treating nerve pain. So far, Neridronate has been found to be effective in the treatment of CRPS Type 1. There aren’t many reports for its use in CRPS Type 2 treatment but so far it’s the next best thing to a cure for CRPS.

  • Botulinum toxin (botox) injections

Botulinum toxin injections act as sympathetic blocks to treat CRPS Type 2. They offer a prolonged analgesic effect hence providing long lasting pain relief, although it’s not permanent.

  • Opioids

For patients who feel severe pain that can’t be effectively treated with the other drugs, opioids such as codeine and morphine can be more effective in treating pain.

  • N-methyl-D-aspartate (NMDA) receptor antagonists

This group of drugs is a class of anaesthetics that inhibit the N-methyl-d-aspartate receptor which allows for the transfer of electric signals between neurons in the brain and spinal column. An example of N-methyl-D-aspartate (NMDA) receptor antagonists used for CRPS Type 2 treatments is ketamine.

In the event that the above medications don’t help much in improving the patient’s symptoms, some alternative treatments are the next best option. Some of these are only used as last resorts when all else has failed and they remain surrounded by controversy. These alternative treatments include:

  • Sympathetic nerve-blocking

This treatment targets the sympathetic nervous system which is a series of nerves connecting the spine to the body. As mentioned before, CRPS Type 2 affects the sympathetic nervous system and disturbs the involuntary body functions that the system controls, such as sweating, blood flow, and digestion. The nerve-blocking injection used often has an anaesthetic effect and blocks transmission of pain signals, hence providing pain relief.

  • Spinal cord stimulation

Spinal cord stimulation involves the use of low-level electrical signals delivered to the spinal cord or to specific nerves to block the transmission of pain signals to the brain. The electrical signals are administered through a device that is implanted in the body through a needle that’s placed in the back near the spinal cord. The device gives the patient full control over their pain, allowing them to turn the electrical current on and off and even adjust the intensity of the signals accordingly.

  • Repetitive Transcranial Magnetic Stimulation (rTMS)

This therapy targets brain stimulation and is used to treat depression and anxiety. It works hand-in-hand with CRPS Type 2 medical intervention. An electromagnetic coil  is placed near the head, close to the area of the brain that regulates mood. This coil passes magnetic pulses to the targeted area hence introducing electrical currents that stimulate brain cells and reduce depression.

 

Sometimes additional treatments are necessary to ensure maximum comfort for the patient and improved efficacy of the treatments. Complimentary therapies that can be done in addition to all the above treatments include:

  • Acupuncture
  • Relaxation techniques
  • Chiropractic therapy
  • Heat and cold therapy
  • Transcutaneous electrical nerve stimulation (TENS)

Experimental therapies available for CRPS Type 2 treatment that can be tried include:

  • Intravenous immunoglobulin
  • Amputation

Conclusion

While CRPS Type 2 is quite rare, the few individuals diagnosed with this form of the medical condition can try the above-mentioned treatments to manage their condition. As with most diseases, the sooner treatment begins, the better the chances for effectiveness. There isn’t a lot of information available with regards to CRPS Type 2 and its treatment on account of how rare it is.  The best practice is to evaluate the treatments to see what’s working and what’s not, and try any of the alternatives that are accessible.

 

 

Medications

  1. Steroids

In the early stages of the medical condition, inflammation is common. Pulsed doses of steroids such as corticosteroids have been found to be beneficial in reducing inflammation in CRPS Type 2 patients. They are especially helpful when used in the early stages of the disease and are most beneficial in patients exhibiting redness, swelling, and heat due to inflammation. There are some risks to long-term use that may outweigh potential benefits such as osteoporosis, adrenal insufficiency, and immunosuppression.

  1. Antidepressants

Antidepressants such as tri-cyclic antidepressants (TCAs) were originally designed to treat depression, but were also recognised for their effectiveness in treating nerve pain. The most widely used TCAs for treating CRPS Type 2 are amitriptyline and nortiptyline. TCAs must only be taken in the doses prescribed by the physician, which is usually lower than the doses needed to treat depression. Possible side effects of TCAs are a dry mouth, blurred vision, constipation, heart palpitations, and difficulty urinating. The patient may also experience withdrawal effects if they suddenly stop taking TCAs. A general practitioner will arrange for the dose to be slowly reduced over a period of at least 4 weeks if the patient feels they no longer need to take them. TCAs are not recommended for patients with certain types of glaucoma.

  1. Anticonvulsants

Anticonvulsants such as Neurontin have pain relieving effects that make them useful in CRPS Type 2 treatment. They are also known as antiepileptic/antiseizure drugs used in the treatment of epileptic seizures. Anticonvulsants help in treating nerve pain, such as the burning, shooting pain experienced by individuals with CRPS Type 2. It is recommended that these medications are taken every day, whether or not the patient feels pain. Some common side effects of anticonvulsants that the patient may experience are drowsiness, dizziness, and weight gain. If the patient has certain medical conditions such as kidney stones or glaucoma, the physician should be notified as there are some types of anticonvulsants that are not recommended with those conditions.

  1. Nerve block

A series of localized anaesthetic injections are used to block a group of nerves that cause pain to a specific organ or part of the body. The nerve-numbing procedure has been known to bring lasting relief to 18-25% of patients.

  1. Opioids

Many opioid-only and opioid combination drugs are used to treat chronic pain when other pain relief medications such as ibuprofen or acetaminophen aren’t strong enough. Opioid products are available in many forms and differ in how they are taken, how long they take to start working once taken, and how long they keep working. The doctor will have to consider many factors before selecting the best opioid for the patient’s treatment regimen, such as the severity of the pain, pain treatment history, other drugs or conditions the patient takes or has, age, health insurance coverage and whether there is a history of substance use disorders.

 

Surgery

Medical therapy is sometimes ineffective in the treatment of CRPS Type 2. In that case, surgery may be the next best option and is usually reserved for the most severe cases. The surgery comprises of cutting the affected nerves to prevent painful impulses from reaching the brain. Some of the surgical procedures used in CRPS Type 2 are described briefly down below:

  1. Spinal cord stimulators

Spinal cord stimulation masks pain signals before they are transmitted to the brain through the use of a small, pacemaker-like device that delivers electrical pulses to the spinal cord. This procedure has seen to be effective in the management of CRPS by improving pain and quality of life. The amount of pain relief with spinal cord simulation devices varies for each person.

  1. Sympathectomy

Surgical, chemical, or radiofrequency sympathectomy is used to interrupt or block the affected potion of the sympathetic nervous system. Sympathectomy for CRPS treatment can be used as a last resort in patients with impending tissue loss, edema, or recurrent infection. The procedure has been seen to bring lasting relief to over 80% of patients and relief to 90%. In addition to normal surgical risks such as bleeding and infection, sympathectomy has other specific risks such as adverse changes in how nerves function.

  1. Amputation

In the event that the above-mentioned treatments have failed, amputation is sometimes considered the next best option. On average, 50% of patients who opt for this have been reported to have resolution of their pain while the other half develop phantom limb pain and/or pain at the site of the amputation. It is recommended that amputation be left as a last resort, although it should be noted that late amputation may not work as well as expected.

Physical therapy

Physical therapy is a key part of CRPS treatment as it helps in sustaining or improving the patient’s range of motion in painful limbs. A physical therapist working with a CRPS patient may try the following:

  1. Exercise

An exercise plan for a type 2 of Crps patient may include gentle exercises such as stretches, hydrotherapy, or weight-bearing exercises. It is necessary for this programme to be supported by a therapist with experience of the condition to ensure that the patient doesn’t do too much or too vigorous exercise which may result in aggravating the condition and doing more harm than good.

  1. Transcutaneous Electrical Nerve Stimulation (TENS)

A TENS unit is used to send electrical impulses through fibers in the body to targeted parts in order to block pain signals. TENS therapy subdues hyperalgesia (high sensitivity to pain) and the electric currents sent are used to relieve pain.

What is the CRPS Type 2 Prognosis?

Various specialists may be needed for effective treatment of CRPS Type 2 such as experts in orthopedics, pain management, and even psychiatry. The effectiveness of CRPS Type 2 treatments is greatly improved the sooner the diseases is diagnosed and treated. Early diagnosis and treatment give the patient better chances for a positive outcome.+

Share This