Complex Regional Pain Syndrome
Complex Regional Pain Syndrome(CRPS), formerly Reflex Sympathetic Dystrophy or Causalgia, is a chronic progressive disease characterized by severe pain, swelling, and changes in the skin. It often affects an arm or a leg and may spread to another part of the body and is associated with dysregulation of the autonomic nervous system resulting in multiple functional loss, impairment, and disability. Though treatment is often unsatisfactory, early multimodal therapy can cause dramatic improvement or remission of the syndrome in some patients. The International Association for the Study of Pain has proposed dividing CRPS into two types based on the presence of nerve lesion following the injury.
Type 1: Formerly known as reflex sympathetic dystrophy (RSD), Sudeck's atrophy, reflex neurovascular dystrophy (RND), or algoneurodystrophy, does not have demonstrable nerve lesions.
Type 2: Formerly known as causalgia, has evidence of obvious nerve damage.
The cause of this syndrome is currently unknown. Precipitating factors include injury and surgery, although there are documented cases that have no demonstrable injury to the original site.
Symptoms
The symptoms of CRPS usually manifest near the site of an injury, which is usually minor. The most common symptoms overall are burning and electrical sensations, described to be like "shooting pain". The patient may also experience muscle spasms, local swelling, abnormally increased sweating, changes in skin temperature (usually hot but sometimes cold) and color (bright red or a reddish violet), softening and thinning of bones, joint tenderness or stiffness, and/or restricted or painful movement.
The pain of CRPS is continuous and may be heightened by emotional or physical stress. Moving or touching the limb is often intolerable. The symptoms of CRPS vary in severity and duration. There are three variants of CRPS, previously thought of as stages. It is now believed that patients with CRPS do not progress through these stages sequentially. These stages may not be time-constrained, and could possibly be event-related, such as ground-level falls or re-injuries in previous areas. It is important to remember that often the sympathetic nervous system[22] is involved with CRPS, and the autonomic nervous system can go haywire and cause a wide variety of odd complaints that are not mental in origin. Rather than a progression of CRPS from bad to worse, it is now thought, instead, patients are likely to have one of the three following types of disease progression:
1- Stage one is characterized by severe, burning pain at the site of the injury. Muscle spasm, joint stiffness, restricted mobility, rapid hair and nail growth, and vasospasm. The vasospasm is that which causes the changes in the color and temperature of the skin.
2- Stage two is characterized by more intense pain. Swelling spreads, hair growth diminishes, nails become cracked, brittle, grooved, and spotty, osteoporosis becomes severe and diffuse, joints thicken, and muscles atrophy.
3- Stage three is characterized by irreversible changes in the skin and bones, while the pain becomes unyielding and may involve the entire limb. There is marked muscle atrophy, severely limited mobility of the affected area, and flexor tendon contractions (contractions of the muscles and tendons that flex the joints). Occasionally the limb is displaced from its normal position, and marked bone softening and thinning is more dispersed.
treatment of disc-related pain.
Diagnosis
CRPS types I and II share the common diagnostic criteria shown below. Spontaneous pain or allodynia (pain resulting from a stimulus which would not normally provoke pain, such as a light touch of the skin) is not limited to the territory of a single peripheral nerve, and is disproportionate to the inciting event.
There is a history of edema, skin blood flow abnormality, or abnormal sweating in the region of the pain since the inciting event.
No other conditions can account for the degree of pain and dysfunction.
The two types differ only in the nature of the inciting event. Type I CRPS develops following an initiating noxious event that may or may not have been traumatic, while type II CRPS develops after a nerve injury.
No specific test is available for CRPS, which is diagnosed primarily through observation of the symptoms. However, thermography, sweat testing, x-rays, electrodiagnostics, and sympathetic blocks can be used to build up a picture of the disorder. Diagnosis is complicated by the fact that some patients improve without treatment. A delay in diagnosis and/or treatment for this syndrome can result in severe physical and psychological problems. Early recognition and prompt treatment provide the greatest opportunity for recovery.
The International Association for the Study of Pain (IASP) lists the diagnostic criteria for complex regional pain syndrome I (CRPS I) (RSDS) as follows:
1- The presence of an initiating noxious event or a cause of immobilization.
2- Continuing pain, allodynia (perception of pain from a nonpainful stimulus), or hyperalgesia (an exaggerated sense of pain)
disproportionate to the inciting event.
3- Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the area of pain
4- The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.
According to the IASP, CRPS II (causalgia) is diagnosed as follows:
1- The presence of continuing pain, allodynia, or hyperalgesia after a nerve injury, not necessarily limited to the distribution of the injured
nerve.
2- Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the region of pain.
3- The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.
The IASP criteria for CRPS I diagnosis has shown a sensitivity ranging from 98-100% and a specificity ranging from 36-55%. Per the IASP guidelines, interobserver reliability for CRPS I diagnosis is poor. Two other criteria used for CRPS I diagnosis are Bruehl's criteria and Veldman's criteria which have moderate to good interobserver reliability. In the absence of clear evidence supporting 1 set of criteria over the others, clinicians may use IASP, Bruehl�s, or Veldman�s clinical criteria for diagnosis. While the IASP criteria are nonspecific and possibly not as reproducible as Bruehl�s or Veldman�s criteria, they are cited more widely the literature including treatment trials.
Type 1: Formerly known as reflex sympathetic dystrophy (RSD), Sudeck's atrophy, reflex neurovascular dystrophy (RND), or algoneurodystrophy, does not have demonstrable nerve lesions.
Type 2: Formerly known as causalgia, has evidence of obvious nerve damage.
The cause of this syndrome is currently unknown. Precipitating factors include injury and surgery, although there are documented cases that have no demonstrable injury to the original site.
Symptoms
The symptoms of CRPS usually manifest near the site of an injury, which is usually minor. The most common symptoms overall are burning and electrical sensations, described to be like "shooting pain". The patient may also experience muscle spasms, local swelling, abnormally increased sweating, changes in skin temperature (usually hot but sometimes cold) and color (bright red or a reddish violet), softening and thinning of bones, joint tenderness or stiffness, and/or restricted or painful movement.
The pain of CRPS is continuous and may be heightened by emotional or physical stress. Moving or touching the limb is often intolerable. The symptoms of CRPS vary in severity and duration. There are three variants of CRPS, previously thought of as stages. It is now believed that patients with CRPS do not progress through these stages sequentially. These stages may not be time-constrained, and could possibly be event-related, such as ground-level falls or re-injuries in previous areas. It is important to remember that often the sympathetic nervous system[22] is involved with CRPS, and the autonomic nervous system can go haywire and cause a wide variety of odd complaints that are not mental in origin. Rather than a progression of CRPS from bad to worse, it is now thought, instead, patients are likely to have one of the three following types of disease progression:
1- Stage one is characterized by severe, burning pain at the site of the injury. Muscle spasm, joint stiffness, restricted mobility, rapid hair and nail growth, and vasospasm. The vasospasm is that which causes the changes in the color and temperature of the skin.
2- Stage two is characterized by more intense pain. Swelling spreads, hair growth diminishes, nails become cracked, brittle, grooved, and spotty, osteoporosis becomes severe and diffuse, joints thicken, and muscles atrophy.
3- Stage three is characterized by irreversible changes in the skin and bones, while the pain becomes unyielding and may involve the entire limb. There is marked muscle atrophy, severely limited mobility of the affected area, and flexor tendon contractions (contractions of the muscles and tendons that flex the joints). Occasionally the limb is displaced from its normal position, and marked bone softening and thinning is more dispersed.
treatment of disc-related pain.
Diagnosis
CRPS types I and II share the common diagnostic criteria shown below. Spontaneous pain or allodynia (pain resulting from a stimulus which would not normally provoke pain, such as a light touch of the skin) is not limited to the territory of a single peripheral nerve, and is disproportionate to the inciting event.
There is a history of edema, skin blood flow abnormality, or abnormal sweating in the region of the pain since the inciting event.
No other conditions can account for the degree of pain and dysfunction.
The two types differ only in the nature of the inciting event. Type I CRPS develops following an initiating noxious event that may or may not have been traumatic, while type II CRPS develops after a nerve injury.
No specific test is available for CRPS, which is diagnosed primarily through observation of the symptoms. However, thermography, sweat testing, x-rays, electrodiagnostics, and sympathetic blocks can be used to build up a picture of the disorder. Diagnosis is complicated by the fact that some patients improve without treatment. A delay in diagnosis and/or treatment for this syndrome can result in severe physical and psychological problems. Early recognition and prompt treatment provide the greatest opportunity for recovery.
The International Association for the Study of Pain (IASP) lists the diagnostic criteria for complex regional pain syndrome I (CRPS I) (RSDS) as follows:
1- The presence of an initiating noxious event or a cause of immobilization.
2- Continuing pain, allodynia (perception of pain from a nonpainful stimulus), or hyperalgesia (an exaggerated sense of pain)
disproportionate to the inciting event.
3- Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the area of pain
4- The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.
According to the IASP, CRPS II (causalgia) is diagnosed as follows:
1- The presence of continuing pain, allodynia, or hyperalgesia after a nerve injury, not necessarily limited to the distribution of the injured
nerve.
2- Evidence at some time of edema, changes in skin blood flow, or abnormal sudomotor activity in the region of pain.
3- The diagnosis is excluded by the existence of any condition that would otherwise account for the degree of pain and dysfunction.
The IASP criteria for CRPS I diagnosis has shown a sensitivity ranging from 98-100% and a specificity ranging from 36-55%. Per the IASP guidelines, interobserver reliability for CRPS I diagnosis is poor. Two other criteria used for CRPS I diagnosis are Bruehl's criteria and Veldman's criteria which have moderate to good interobserver reliability. In the absence of clear evidence supporting 1 set of criteria over the others, clinicians may use IASP, Bruehl�s, or Veldman�s clinical criteria for diagnosis. While the IASP criteria are nonspecific and possibly not as reproducible as Bruehl�s or Veldman�s criteria, they are cited more widely the literature including treatment trials.
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