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Overview:

Complex Regional Pain Syndrome

CPRC manifests as a chronic pain condition that often occurs after trauma or surgery in the extremities of the body – usually the limbs.

Complex Regional Pain Syndrome, or CRPS, is known to affect approximately 26 people out of a 100,000 around the world. The pain caused form this syndrome is predominantly due to damage, abnormal swelling, or dysfunction of the nerves in the region. This condition often shows up predominantly more in women than men, and usually (although not always) starts to affect people in their mid-30s.

What are the causes of Complex Regional Pain Syndrome or CRPS?

It is estimated that approx. 2–5% of patients with peripheral nerve injury and 13–70% of patients with paralysis of one side of the body will develop CRPS. It is also estimated that about 7% of patients who have had CRPS manifested in one limb will develop it in the other as well.

Complex Regional Pain Syndrome usually starts as extreme pain in one limb, evolves to the skin and bones and leads to a decreased range of motion. It can, in some cases, start spreading to the rest of the body.

About 10% of patients report no specific cause for the manifestation of Complex Regional Pain Syndrome (indicating the possibility of an internal nerve injury), although CRPS usually develops after (or due to) any of the following health conditions:

  • Contusions
  • Fractures
  • Sprains/Strains
  • Nerve lesions
  • Stroke
  • Heart attack
  • Tumors
  • Poor nerve health
  • Autoimmune and inflammatory disorders
  • Genetic disorders impacting ability to recover from trauma or injury
  • Peripheral neuropathies
  • Arthritis
  • Cellulitis
  • Lymphatic obstruction

Although a person might experience one or more of these health conditions does not necessarily mean they will develop CRPS. Why certain patients develop CRPS and others do not are still questions the medical community is trying to answer.

What is known about CRPS is that it is a neurological condition predominantly caused by damage to the peripheral sensory neurons. The improper functioning of the peripheral c-fiber nerves impacts the ability of the brain and the spinal cord to send the appropriate pain signals to the rest of the body. Another characteristic of CRPS is the inflammation caused by the excessive firing of the neurons.

What are the types of Complex Regional Pain Syndrome or CRPS?

Complex Regional Pain Syndrome is a very broad-based term that is used to denote a neurological condition. CRPS is typically characterized by extreme pain and inflammation in the arm(s) or leg(s) and can cause chronic pain.

There are two basic types of classifications for this condition, and this differentiation is made based on one main factor – if there is evidence of nerve damage present in the region that is affected.

Type 1 — This type of Complex Regional Pain Syndrome is also known as Sudeck’s atrophy or Reflex Sympathetic Dystrophy (RSD). This type is characterized by the lack of evidence of nerve damage in the affected limb, and accounts for approximately 90% of all known cases.

Type 2 — Causalgia, or type 2 Complex Regional Pain Syndrome, is characterized by evidence of nerve damage in the affected region.

What are the signs and symptoms of Complex Regional Pain Syndrome or CRPS?

In the past, patients were classified with Type 1 CRPS whenever there was a lack of evidence of nerve damage. In these cases, without obvious signs of nerve damage being present, the symptoms were noticed to be more subtle than in CRPS type 2.

The symptoms for both Type 1 CRPS and Type 2 CRPS both come across as identical, with stronger symptoms manifested in Type 2.

Signs and symptoms include:

  • Constant or fluctuating pain that can often be unprovoked
  • Constant sense of burning in the affected regions
  • Hypersensitivity to cold and to touch (allodynia or hyperalgesia)
  • Swelling in the affected regions
  • Fluctuations in temperature of the affected part
  • Changes in skin color – from white to red or blue
  • Changes in skin texture
  • Changes in hair or nails
  • Stiffness in joints
  • Decreased range of motion
  • Wasting away of bone
  • Impaired muscle strength

Those diagnosed with Type 2 CRPS also have more extensive symptoms, which can include:

  • Muscle weakness and shrinkage
  • Loss of control over motor activities
  • Dystonia or abnormal fixed postures
  • Tremors or sudden jerking of the body

In more severe cases, the symptoms can include the inability to use limbs, regression of normal bodily functions such as hair growth, etc.

Since CRPS is considered a systemic issue, it can also affect any organ in the region where it presents itself.

Induced by trauma, injury, or surgery, the main characteristics of this syndrome are – severe pain, as well as an impairment to the sensory, motor, trophic, and autonomic functions of the body.

Impairment

Type 1 CRPS

Type 2 CRPS

Sensory
  • Allodynia and hyperalgesia
  • Hypersensitivity
  • Feeling of dislocation in the limbs
  • Allodynia and hyperalgesia
  • Hypersensitivity
  • Intolerance even to mild sensations on the skin
Motor
  • Muscle weakness
  • Stiffness
  • Tremors
  • Reduced range of motion
  • Inability to move the affected region
  • Muscle weakness
  • Stiffness
  • Tremors
  • Reduced range of motion
  • Inability to move the affected region
  • Dystonia
Trophic
  • Osteoporosis
  • Fibrosis
  • Changes in hair and/or nail growth
  • Changes in skin texture
  • Acute arthritis
  • Mottling of skin
Autonomic
  • Swelling
  • Edema
  • Changes in skin color
  • Abnormalities in blood flow
  • Changes in temperature and sweating
  • Distal extremity swelling
  • Changes in skin color
  • Abnormalities in blood flow
  • Changes in temperature and sweating

Type 1 CRPS Impairment

Sensory

  • Allodynia and hyperalgesia
  • Hypersensitivity
  • Feeling of dislocation in the limbs

Motor

  • Muscle weakness
  • Stiffness
  • Tremors
  • Reduced range of motion
  • Inability to move the affected region

Trophic

  • Osteoporosis
  • Fibrosis
  • Changes in hair and/or nail growth
  • Changes in skin texture

Autonomic

  • Swelling
  • Edema
  • Changes in skin color
  • Abnormalities in blood flow
  • Changes in temperature and sweating

Type 2 CRPS Impairment

Sensory

  • Allodynia and hyperalgesia
  • Hypersensitivity
  • Intolerance even to mild sensations on the skin

Motor

  • Muscle weakness
  • Stiffness
  • Tremors
  • Reduced range of motion
  • Inability to move the affected region
  • Dystonia

Trophic

  • Acute arthritis
  • Mottling of skin

Autonomic

  • Distal extremity swelling
  • Changes in skin color
  • Abnormalities in blood flow
  • Changes in temperature and sweating
What are the stages of Complex Regional Pain Syndrome or CRPS?

Symptoms vary from case to case and can change over time. In some cases, the symptoms are mild and abate on their own as the injured nerve regrows. In others, they may persist for years.

If the condition progresses without timely medical intervention, it can progress into higher stages of pain (although not always the case with children).

  • Stage 1 — Acute — Lasts between 10 days to about 6 months. It is characterized by severe pain, hyperalgesia, allodynia, hypersensitivity, swelling, mild temperature changes, hyperthermia, dryness of skin, and an increase/decrease in hair/nail growth.
  • Stage 2 — Dystrophic — Begins at the end of the acute stage and typically lasts for 6 months post that. There is an increase in pain, swelling and other symptoms, along with joint stiffness. Skin can get thin and sweaty, and nails become thin and hard. The onset of osteoporosis and bone erosions are noticed.
  • Stage 3 — Atrophic — This can last for years. Pain may be experienced all over the body, with stronger manifestations of other symptoms. The onset of ankylosis and demineralization is noticed. Fingertips and toes get atrophic.
What are the risk factors of Complex Regional Pain Syndrome or CRPS?

Trauma, injuries, surgeries, or infections are the usual causes of Complex Regional Pain Syndrome. However, there is no direct connection between the cause and the severity of the manifestation. There are some risk factors that are known to increase the incidence of Complex Regional Pain Syndrome.

These include:

  • Stressful lifestyle
  • History of psychological disorders
  • Occupations where high-impact trauma can occur
When should I see a doctor?

It is important to treat CRPS as early as possible. Once the symptoms progress, they can become irreversible. Also, if you are experiencing persistent and high-intensity pain and are unable to touch or move the affected limb, contact your physician at Advanced Pain Care immediately to schedule an appointment so they can determine the cause.

How is Complex Regional Pain Syndrome or CRPS diagnosed?

As the symptoms are so varied, diagnosis of CRPS usually depends on the clinical findings including evidence of sensory, vasomotor, sudomotor, and/or trophic changes.

A diagnosis involves a thorough clinical evaluation by a healthcare professional and involves the following evaluations:

  • Patient’s medical history including genetic predispositions
  • Trauma, surgery, or injury details
  • History of sensory, motor, autonomic, trophic disturbances
  • Assessment of swelling
  • Current signs and symptoms
  • Physical examinations

There are no tests that specifically identify the injured nerves. However, there are some basic criteria that are prescribed by the Budapest Criteria to determine the manifestation of CRPS. Clinical diagnostic criteria include the following checklist:

  • Distinct signs that symptoms are being developed in at least two other lists of characteristics
  • Clear indication that the symptoms displayed are not for any other illness or condition
  • Persistent, higher than normal pain
  • At least one symptom from the categories of motor, sensory, trophic, and autonomic list of characteristics

Apart from these criteria, a healthcare specialist may prescribe the following tests to determine characteristics and signs:

  1. Nerve conduction studies to detect nerve injuries
  2. Magnetic resonance neurography or imaging to check for bone/bone marrow
    abnormalities to identify the injured nerves
  3. Triple-phase bone scans to check for excess bone resorption
  4. Infrared thermography to determine asymmetries in temperature
  5. Sweat testing
  6. X-rays to check bone structure irregularities
  7. Bone densitometry to determine the state of the bone mineral
  8. Sympathetic blocks

In addition, the specialist will also check basic body functions including movements such as sitting and rising, walking up the stairs, foot movements, grip strength, etc. Additional measures include a neuropathic pain questionnaire, impairment sum score and a CRPS severity score (also used for self-monitoring).

How is Complex Regional Pain Syndrome or CRPS treated?

Treatment of Complex Regional Pain Syndrome can be effective immediately. At the onset, pain, inflammation, temperature changes, and hypersensitivity are common. When symptoms, such as skin and growth changes or muscle spasms, begin to occur then it is a sign that the condition has reached an irreversible stage and can start causing disabilities. In some cases, dystonia or abnormal fixed postures and tremors can also occur. These cases can only be treated by an Orthopedic surgeon.

Treatment of CRPS is usually multi-modal, with the pain care specialists at Advanced Pain Care prescribing different treatment plans that can include:

  1. Prescription pain medication, corticosteroids, and non-steroidal anti-inflammatory drugs
  2. Bisphosphonates to reduce bone changes
  3. Drugs for neuropathic pain conditions
  4. Topical ointments, sprays, and creams
  5. Rehabilitation and physical therapy
  6. Graded motor imagery
  7. Transcutaneous electrical nerve stimulation
  8. Spinal cord stimulation
  9. Sympathetic nerve block
When is surgery recommended for Complex Regional Pain Syndrome or CRPS?

Surgical procedures are only considered as an option in the most severe CRPS cases. For example, if the condition is a result of a compressed nerve caused by carpal tunnel syndrome, then the Neurosurgeon or Orthopedist may recommend surgery to release the pressure on the nerve. This may include a upper thoracic or lumbar surgical sympathectomy – In this procedure, a portion of the sympathetic nerve trunk is surgically destroyed (by cutting, burning, or clipping).

If CRPS is not diagnosed and treated in a timely manner, it can cause long-term disabilities and complications in the form of:

  • Atrophy or tissue wasting and deterioration of skin, muscles, and bones
  • Tightening of muscles to a point where the toes and fingers may take a fixed position
How can Complex Regional Pain Syndrome or CRPS be prevented – what precautions can you take?

The pain from Complex Regional Pain Syndrome is often even more severe than the pain of amputation, childbirth, or cancer. Due to the condition being so broad-based and with so many characteristic symptoms, there are not many effective ways to prevent or cure it. However, taking some basic precautions can help reduce the chance of developing CRPS:

Reducing the possibilities of occupational trauma

Early mobilization after a stroke

Right treatment protocol post surgeries

Taking precautions in sports and high-impact activities

Right treatment protocol for fractures

Regular exercise and healthy lifestyle to improve blood circulation

Decreasing smoking

Paying attention to mental health

For more information on Complex Regional Pain Syndrome, call 512-244-4272 to schedule an appointment with one of our Neurosurgeons OR Integrated Pain Management specialists at Advanced Pain Care. You can also schedule an online appointment at https://austinpaindoctor.com/locations

Frequently Asked Questions

Q: Is CRPS the most painful disease?

A: Complex Regional Pain Syndrome is a rare neurological disorder. It is rated as an extremely painful condition, almost on par with the pain of amputation, childbirth, or cancer, and there are very few effective treatments available. This condition has also been nicknamed the “suicide disease”.

Q: Is Complex Regional Pain Syndrome real?

A: The range of signs and symptoms with Complex Regional Pain Syndrome are varied. One sign is the intensity of pain in response to even the slightest sensations. Also, there is no direct link between the actual trauma, surgery, or injury and the severity of the symptoms, so the role of psychological factors in this condition are not completely ruled out.

Q: Is CRPS a permanent disability?

A: If CRPS is not diagnosed and treated in a timely manner, it can cause long-term disabilities and complications in the form of:

  • Atrophy or tissue wasting and deterioration of skin, muscles, and bones
  • Tightening of muscles to a point where the toes and fingers may take a fixed position

If you have CRPS and expect that you cannot resume work for at least a year, disability benefits can be claimed. Symptoms such as chronic fatigue, weakness, light-headedness, dizziness, drowsiness, or confusion will help qualify the claim.

Q: Can Complex Regional Pain Syndrome go away?

A: It has been observed that some of the signs and symptoms of CRPS can abate on their own (perhaps after the nerve has repaired itself), however, there is no known cure for the syndrome. A combination of medicine, physical treatments, and psychological interventions can help on a case-by-case basis. The best way to treat CRPS is to begin treatment at the onset of the condition.

Q: What helps Complex Regional Pain Syndrome?

A: Treatment of CRPS is usually multi-modal and can include:

  • Prescription pain medication, corticosteroids, and non-steroidal anti-inflammatory drugs
  • Bisphosphonates to reduce bone changes
  • Drugs for neuropathic pain conditions
  • Topical ointments, sprays, and creams
  • Intravenous immunoglobulin
  • Rehabilitation and physical therapy
  • Graded motor imagery
  • Transcutaneous electrical nerve stimulation
  • Spinal cord stimulation
  • Non-invasive brain stimulation
  • Transcranial magnetic stimulation
  • Spinal-fluid drug pumps
  • Sympathetic nerve block

Q: What causes Complex Regional Pain Syndrome?

A: Complex Regional Pain Syndrome is said to develop after a stroke, heart attack, trauma, or surgery. Though the underlying causes are not always clear, it is said to develop due to the following health conditions:

  • Contusions
  • Fractures
  • Sprains/Strains
  • Nerve lesions
  • Stroke
  • Heart attack
  • Surgeries
  • Any forceful trauma
  • Infections
  • Limb immobilization
  • Cuts or burns
  • Poor blood circulation
  • Tumors
  • Poor nerve health
  • Autoimmune and inflammatory disorders
  • Genetic disorders impacting ability to recover from trauma or injury
  • Peripheral neuropathies
  • Arthritis
CRPC Treatment

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Are You Experiencing Pain?

If you are experiencing any back pain or stiffness, call Advanced Pain Care at 512-244-4272 to schedule an appointment with one of our pain care and management specialists.

We have a range of pain management treatment options available right from surgical interventions to non-invasive and minimally invasive treatment options. Our team will work with you to develop a pain management plan based on the severity of your condition.