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CRPS

Complex Regional Pain Syndrome

Complex Regional Pain Syndrome

Complex regional pain syndrome is a greater than normal reaction of the body to an injury. The symptoms of complex regional pain syndrome vary in severity and how long they last. The main symptom is pain in an arm or leg, which is often burning, sharp, stabbing or stinging. Early treatment is often effective in treating symptoms. Complex regional pain syndrome can sometimes cause severe problems and be difficult to treat.

Symptoms

The symptoms of CRPS vary in severity and how long they last. Symptoms usually begin within one month after an injury or after having to stay in bed for a long time. The main symptom is pain in the arm or leg, which is often burning, sharp, stabbing or stinging. There may also be tingling and numbness.

The symptoms are usually much worse than you would expect from the injury. CRPS pain continues after the original injury has healed. The symptoms are often severe and have a big effect on day-to-day activities. The pain may spread to other limbs. The pain is constant and can become worse rather than better with time. Staying in bed and not moving around makes the pain and stiffness worse.
Movement may be limited, both because of the pain and because joints can feel stiff. The muscles may become weak. Other muscle problems may occur and include sudden and severe spasms, tremors, severe jerking and other abnormal movements.
The pain and other symptoms often spread up the arm or leg from the site of the original injury. The symptoms may suddenly affect the opposite limb.

Many patients say that their limb ‘feels strange’. It can feel as if it does not belong to the rest of the body and as if it is not your own limb. Sometimes the limb feels bigger or smaller than normal.

As well as pain, the affected area may have other symptoms such as:

• Skin sensitivity: the skin may become oversensitive to light touch.
• Allodynia: this means that you feel pain even after just a gentle touch, such as clothes brushing your skin or even air blowing on your skin. This may be felt as severe pain.
• Swelling: may occur over the painful region.
• Temperature differences between opposite sides: the affected arm or leg may often be warmer or cooler and the temperature may keep changing.
• Abnormal sweating.
• Skin changes:
• Abnormal skin changes may occur, like ‘goosebumps’ and skin rashes.
• There may be changes in the skin colour of the affected limb.
• Skin infections can occur and can be very severe.
• Your skin may become shiny, dry or scaly.
• Hair changes: hair may become coarse but then become thin.
• Fingernail or toenail changes: nails in the affected area may become brittle (crumbly or break easily) and grow faster at the beginning and then slower.

Psychological symptoms

Psychological symptoms may include:

• Difficulty relaxing.
• Feeling less confident in yourself.
• Feeling unable to cope.
• Difficulty getting or accepting support from friends or family.
• Depression (this is common).

  • CAUSES OF CRPS

CRPS is rare but can follow any injury. CRPS Type I (RSD) follows an injury to the skin, muscle, ligaments, joints or bone at any site. The injury can be as a result of an accident or surgery. Most commonly it occurs after a bone is broken and immobilized with a splint or a sling, but can occur even after a minor sprain. CRPS Type II (Causalgia) follows partial damage to a nerve in the arm or leg, such as from a gunshot wound or crush injury. The cause of the prolonged pain and other symptoms is unknown. Changes in the way nerves send messages to the brain about pain may occur at the injury site. These changes may then lead to more changes in the nerves of the spinal cord and brain. All these changes are thought to play a role in causing and prolonging the condition. CRPS may be prevented by ensuring that plaster casts and bandages are not too tight and that limbs are used as early as possible after injury.
CRPS can also start after other problems such as a head injury, stroke or prolonged bed rest.

TREATMENT

Early detection will help in managing the condition. The primary aim of treatment is to restore full use to the painful limb, especially load bearing, e.g. standing and walking, despite the pain. Physiotherapy is a very effective treatment for CRPS. Intensive physiotherapy treatment and doing exercises taught by the physiotherapist at least twice a day are essential. Using the limb is very important. Desensitization of the skin can help to counter the skin hypersensitivity. The skin is rubbed with a series of cloths of increasing coarseness, e.g. from silk to toweling. In addition alternate immersion in hot and cold baths can help temperature sensitivity.

Care for CRPS aims to give education, pain relief, physical rehabilitation and psychological support. There is a lack of high quality evidence for the effectiveness of most individual treatments and further research is needed.

However, early treatment that looks at the whole problem is often effective. Treatment includes helping you to understand the condition and what you can do to help yourself. Other treatments may reduce pain, or be physical treatments like physiotherapy, or be support and treatment for any psychological difficulties. The right treatment for CRPS is different for each person but the aim of all treatments is to allow you to use your affected arm or leg as normally as you can. The success of some treatments may depend on how much effort you are able to put into them.

Do I need to see a specialist?

Your GP will usually refer you to see a specialist so that:
• The diagnosis of CRPS can be confirmed.
• Other causes for your symptoms can be ruled out.
• You can be helped with a treatment plan to control the difficult symptoms, including pain and distress.
• Treatment to keep you as active as possible can be provided, including physiotherapy.
If your symptoms are more severe and don’t improve with treatment then you may need to be referred to a Pain Clinic or another specialised unit for further treatments. Because of the different symptoms with CRPS, you may need to see other teams of doctors too, such as:
• A bone specialist (orthopaedic specialist).

• A bone and joint specialist (rheumatologist).
• A nerve specialist (neurologist).
• A skin specialist (dermatologist).

Rehabilitation
Rehabilitation should be considered for anyone with CRPS and should be started early. Support and treatment from a physiotherapist and an occupational therapist are incredibly important. Although exercise may make the pain worse, not exercising also makes the pain worse and the therapist will help you to find the ‘happy medium’.
Rehabilitation includes helping you to use the limb with gentle exercises and helping to make the limb less sensitive. Other treatments help you to use the limb more normally and reduce any swelling (oedema).
Specialised units may also use more specialised treatments such as mirror visual feedback and graded motor imagery.
Mirror visual feedback involves you describing your affected and your unaffected arm or leg with your eyes closed. Then you imagine moving your affected arm or leg and then do this while looking at it in a mirror.
Graded motor imagery involves you imagining moving your affected arm or leg and also watching your normal limb in a mirror while you move it.

Medication treatment
Medicines can be used to help reduce the pain and help you to sleep. Simple medicines for pain relief such as non-steroidal anti-inflammatory drugs (NSAIDs) are used first. Then the strength and doses of the medicines are increased until the pain is controlled well enough for you to be able to use your arm or leg a little.

Some patients may benefit from treatment with strong pain killers such as morphine. Tramadol is a milder drug, similar to morphine, which may sometimes be useful. Antidepressant drugs such as amitriptyline or imipramine, originally developed to treat depression, can sometimes be useful for nerve pain (as in CRPS ). They may cause side effects such as dry mouth, drowsiness, or constipation. It is often possible to get the right balance between side effects and pain relief so that they are of benefit. Anticonvulsant drugs used for epilepsy treatment can also relieve nerve pain. Gabapentin or pregabalin (Lyrica®) are very useful drugs. Carbamazepine (Tegretol®) may help. You may have side effects, such as tiredness and weight gain. These antidepressant and anticonvulsant drugs must be taken regularly for them to work and not just when the pain is bad. They can take up to 3 weeks to have an effect. They will probably need to be taken for a long time. You may need to take more than one kind of drug. Your doctor will try to find the best combination for you.
Pamidronate is a bisphosphonate medicine that is given by slow injection into a vein. It may be given as a one-off treatment if you have had CRPS for less than six months but it isn’t getting any better.

Psychological support and treatment
Sometimes psychological treatment can help to reduce distress. A psychological assessment by an expert in chronic pain may help to identify problems that can be helped. Psychological interventions (including cognitive behavioural therapy and relaxation techniques) can help the emotional difficulties associated with CRPS.

What are specialised treatments for CRPS?
There are two particular specialised CRPS treatments:

Pain management programme (PMP)
This is a programme to help you to improve your quality of life and manage your pain better. PMP is given to a group of people with CRPS and includes help from different health professionals, including doctors, physiotherapists, occupational therapists and psychologists.

OTHER TREATMENTS:

Transcutaneous electrical nerve stimulation (TENS) may help some patients. This treatment, using electrodes placed on the painful area, causes a tingling sensation, which may reduce the pain. Nerve blocks. There is no evidence that these injections to the limb are an effective cure for CRPS. However, they may help enough so that physiotherapy can be done.
Spinal cord stimulation (SCS) can be an effective treatment for CRPS in a few suitable people. An electrical stimulator is implanted under the skin and an electrode is placed next to the spinal cord. This treatment is only available in a few specialist centres, for suitable patients in whom all other treatments have been ineffective.

What is the outcome (prognosis)?
Most people with complex regional pain syndrome (CRPS) get better. CRPS is very variable both in how severe the symptoms are and how long the symptoms last. CRPS may be mild and get better by itself or with treatment. In some people, CRPS can cause severe symptoms and last for many years. There is no way of knowing whether your CRPS will be the type to get better or when. Even if you have CRPS for several years, the rest of your body will continue to work as normal.
Some people with CRPS have times when the symptoms are bad (exacerbations) and other periods of time when the symptoms are much fewer or even disappear (remissions). The periods of exacerbation may last for weeks, months or years.
If it is not diagnosed and treated, CRPS can spread and affect other limbs. This makes CRPS much harder to treat.

Frequently Asked Questions

Q: Can complex regional pain syndrome (CRPS) be prevented?

A:

People experiencing fractures should try to move the part of their limb that they can move as much as possible. They are also advised to have a good intake of vitamin C but there is no proven way that CRPS can be prevented.

 

Q: What is the outcome (prognosis)?

A:

Most people with complex regional pain syndrome (CRPS) get better. CRPS is very variable both in how severe the symptoms are and how long the symptoms last. CRPS may be mild and get better by itself or with treatment. In some people, CRPS can cause severe symptoms and last for many years. There is no way of knowing whether your CRPS will be the type to get better or when. Even if you have CRPS for several years, the rest of your body will continue to work as normal.

Some people with CRPS have times when the symptoms are bad (exacerbations) and other periods of time when the symptoms are much fewer or even disappear (remissions). The periods of exacerbation may last for weeks, months or years.

If it is not diagnosed and treated, CRPS can spread and affect other limbs. This makes CRPS much harder to treat.

Q: What are specialised treatments for CRPS?

A:

There are two particular specialised CRPS treatments:

Pain management programme (PMP)

This is a programme to help you to improve your quality of life and manage your pain better. PMP is given to a group of people with CRPS and includes help from different health professionals, including doctors, physiotherapists, occupational therapists and psychologists.

OTHER TREATMENTS:

 

Transcutaneous electrical nerve stimulation (TENS) may help some patients. This treatment, using electrodes placed on the painful area, causes a tingling sensation, which may reduce the pain. Nerve blocks. There is no evidence that these injections to the limb are an effective cure for CRPS. However, they may help enough so that physiotherapy can be done.

Spinal cord stimulation (SCS) can be an effective treatment for CRPS in a few suitable people. An electrical stimulator is implanted under the skin and an electrode is placed next to the spinal cord. This treatment is only available in a few specialist centres, for suitable patients in whom all other treatments have been ineffective.

Q: Do I need to see a specialist?

A:

Your GP will usually refer you to see a specialist so that:

  • The diagnosis of CRPS can be confirmed.
  • Other causes for your symptoms can be ruled out.
  • You can be helped with a treatment plan to control the difficult symptoms, including pain and distress.
  • Treatment to keep you as active as possible can be provided, including physiotherapy.

If your symptoms are more severe and don’t improve with treatment then you may need to be referred to a Pain Clinic or another specialised unit for further treatments. Because of the different symptoms with CRPS, you may need to see other teams of doctors too, such as:

  • A bone specialist (orthopaedic specialist).
  • A bone and joint specialist (rheumatologist).
  • A nerve specialist (neurologist).
  • A skin specialist (dermatologist).

Q: Are there treatments for complex regional pain syndrome (CRPS)?

A:

Early detection will help in managing the condition. The primary aim of treatment is to restore full use to the painful limb, especially load bearing, e.g. standing and walking, despite the pain. Physiotherapy is a very effective treatment for CRPS. Intensive physiotherapy treatment and doing exercises taught by the physiotherapist at least twice a day are essential. Using the limb is very important. Desensitization of the skin can help to counter the skin hypersensitivity. The skin is rubbed with a series of cloths of increasing coarseness, e.g. from silk to toweling. In addition alternate immersion in hot and cold baths can help temperature sensitivity.

Care for CRPS aims to give education, pain relief, physical rehabilitation and psychological support. There is a lack of high quality evidence for the effectiveness of most individual treatments and further research is needed.

However, early treatment that looks at the whole problem is often effective. Treatment includes helping you to understand the condition and what you can do to help yourself. Other treatments may reduce pain, or be physical treatments like physiotherapy, or be support and treatment for any psychological difficulties. The right treatment for CRPS is different for each person but the aim of all treatments is to allow you to use your affected arm or leg as normally as you can. The success of some treatments may depend on how much effort you are able to put into them.

Q: Are there any tests for complex regional pain syndrome (CRPS)?

A:

The diagnosis is usually made by an examination by a specialist. The diagnosis may be difficult, especially in the early stages. It is very important to diagnose CRPS as early as possible because early treatment helps you to recover more quickly.

CRPS is not an easy condition to diagnose and often referral to a Pain Clinic is necessary for an accurate diagnosis. Other possible causes of pain need to be excluded first. There is usually an event such as an injury to a limb which causes damage or immobilization. There is continuous pain out of proportion to the original injury. Not everyone with CRPS has all the symptoms but they will have some of them. 70% have increased sensitivity to pain, 80-85% have abnormal changes in temperature (feelings of hot and cold), abnormal changes in skin colour, swelling or reduced movement, 50% have abnormal sweating, and 20% have weakness, tremor, increased muscle tone or changes in hair, nail or skin growth.

There are no specific blood tests or other investigations. However, investigations may be important to rule out any other cause for your symptoms.

Q: What are the symptoms of complex regional pain syndrome (CRPS)?

A:

The symptoms of CRPS vary in severity and how long they last. Symptoms usually begin within one month after an injury or after having to stay in bed for a long time. The main symptom is pain in the arm or leg, which is often burning, sharp, stabbing or stinging. There may also be tingling and numbness.

The symptoms are usually much worse than you would expect from the injury. CRPS pain continues after the original injury has healed. The symptoms are often severe and have a big effect on day-to-day activities. The pain may spread to other limbs. The pain is constant and can become worse rather than better with time. Staying in bed and not moving around makes the pain and stiffness worse.

Movement may be limited, both because of the pain and because joints can feel stiff. The muscles may become weak. Other muscle problems may occur and include sudden and severe spasms, tremors, severe jerking and other abnormal movements.

The pain and other symptoms often spread up the arm or leg from the site of the original injury. The symptoms may suddenly affect the opposite limb.

Many patients say that their limb ‘feels strange’. It can feel as if it does not belong to the rest of the body and as if it is not your own limb. Sometimes the limb feels bigger or smaller than normal.

As well as pain, the affected area may have other symptoms such as:

  • Skin sensitivity: the skin may become oversensitive to light touch.
  • Allodynia: this means that you feel pain even after just a gentle touch, such as clothes brushing your skin or even air blowing on your skin. This may be felt as severe pain.
  • Swelling: may occur over the painful region.
  • Temperature differences between opposite sides: the affected arm or leg may often be warmer or cooler and the temperature may keep changing.
  • Abnormal sweating.
  • Skin changes:
    • Abnormal skin changes may occur, like ‘goosebumps’ and skin rashes.
    • There may be changes in the skin colour of the affected limb.
    • Skin infections can occur and can be very severe.
    • Your skin may become shiny, dry or scaly.
  • Hair changes: hair may become coarse but then become thin.
  • Fingernail or toenail changes: nails in the affected area may become brittle (crumbly or break easily) and grow faster at the beginning and then slower.

Psychological symptoms

Psychological symptoms may include:

  • Difficulty relaxing.
  • Feeling less confident in yourself.
  • Feeling unable to cope.
  • Difficulty getting or accepting support from friends or family.

Depression (this is common).

Q: How common is complex regional pain syndrome (CRPS)?

A:

CRPS affects about 1 in 4,000 people each year. CRPS can affect any age but is more common between the ages of 40 and 60 years. It can also affect children, although is very rare in children aged younger than 6. The number of CRPS cases among teenagers and young adults is increasing. Although CRPS can start after a minor injury, it is more common after an injury to a nerve or after a fracture.

Q: What causes complex regional pain syndrome (CRPS)?

A:

CRPS is rare but can follow any injury. CRPS Type I (RSD) follows an injury to the skin, muscle, ligaments, joints or bone at any site. The injury can be as a result of an accident or surgery. Most commonly it occurs after a bone is broken and immobilized with a splint or a sling, but can occur even after a minor sprain. CRPS Type II (Causalgia) follows partial damage to a nerve in the arm or leg, such as from a gunshot wound or crush injury. The cause of the prolonged pain and other symptoms is unknown. Changes in the way nerves send messages to the brain about pain may occur at the injury site. These changes may then lead to more changes in the nerves of the spinal cord and brain. All these changes are thought to play a role in causing and prolonging the condition. CRPS may be prevented by ensuring that plaster casts and bandages are not too tight and that limbs are used as early as possible after injury.

CRPS can also start after other problems such as a head injury, stroke or prolonged bed rest.

Q: What is complex regional pain syndrome (CRPS)?

A:

Complex Regional Pain Syndrome Type I (CRPS I) is also known as Reflex Sympathetic Dystrophy (RSD) and Complex Regional Pain Syndrome Type II (CRPS II) is also known as Causalgia.

The pain usually develops after an injury to an arm or leg. Only rarely are other areas affected. CRPS I follows an injury to a limb such as a broken bone or even a minor sprain and CRPS II follows partial damage to a nerve in the limb. The symptoms are very similar. However, CRPS II is very rare. ¨ The main symptom is pain in the arm or leg. The pain is often burning, sharp, stabbing or stinging, with tingling and numbness. In addition there is a range of other symptoms which can vary and change over time. Increased skin sensitivity (allodynia), increased sensitivity to pain (hyperalgesia), skin discolouration, swelling, stiffness, feelings of hot or cold, excessive or reduced sweating and changes to the hair, skin or nails. The pain and other symptoms usually spread beyond the site of the original injury.

Pain continues long after the original injury has healed. It is often severe and may get progressively worse. In mild cases the pain can last for weeks or months but in severe cases, when the limb is not used, it can last for years.

The skin may become over sensitive to light touch. Clothes brushing the skin, or a slight draught on the skin, is felt as severe pain. This is called allodynia and is common in CRPS.

Often there is difficulty moving the limb, together with weakness and sometimes tremors or jerking. In severe cases the limb can be fixed in one position.

In very severe cases there may be bone softening resulting in breaks. This is called osteopaenia. There can also be muscle atrophy (wasting) and in extreme cases muscle contracture.

CRPS is a stronger than normal reaction of the body to an injury. The cause of CRPS is not known. The nerves in the affected limb are much more sensitive than other nerves and this causes pain and tenderness in the affected area. The brain is also involved. The way the brain communicates with your affected arm or leg changes.

The pain usually starts after an injury but may occur without an injury. It usually affects an arm or a leg but can affect another part of the body. The injury may be severe, such as a broken bone or a damaged nerve, or may be a minor injury.

CRPS is not in your imagination or in your mind. However, some emotional factors, like fear, worry or feeling depressed, can make the pain worse than it already is.

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