neurology
Content
Complex Regional Pain Syndrome
1864 - Colonel Weir Mitchell, MD “severely painful dystrophic syndrome following ballistic injuries” in Civil War soldiers: Causalgia
History
Paul Sudeck Suggested that the signs and symptoms of RSD may be caused by an exaggerated inflammatory response to injury or operation of an extremity. Sudeck’s Atrophy: Bone loss associated with RSD
Rene Leriche
Sympathetic nervous system dysfunction as a cause of pain. Therapeutic surgical sympathectomy
John Bonica
The syndrome much as we
know it today
Promoted the term RSD Described 3 stages
CRPS History
CRPS (complex regional pain syndrome) initially considered in
early 1800s by Claude Bernard et al. During the Civil War it was seen that soldiers who suffered from low-velocity, high-mass missile injuries developed a neuropathic pain that was termed "causalgia" by Silas Weir-Mitchell. In 1940s the term of reflex sympathetic dystrophy came into use relating the belief of an abnormal efferent reflex from the sympathetic nervous system to bodily injury. Since that time much study and frustration has come from this relatively rare condition of which the pathophysiology is still not fully understood.
Basics
Often seen after injury to a limb or related to
some inciting event. The patient complains of and can manifest skin color/ temperature/ appearance changes in the affected limb. Pain often excruciating – burning, tingling, electric-like, etc. are often symptoms that patients feel. The pain is often out of proportion to stimulus or the event.
Physical Appearance
Diagnostic Criteria
IASP (International Assoc for the Study of Pain) diagnostic criteria include 4 subjective and/ or objective findings: 1. The presence of an initiating event or a cause of immobilization – peripheral injury or central (stroke, etc)*. (Injury) 2. Continuing pain, allodynia, or hyperalgesia in which the pain is disproportiate to inciting event. (Sensory) 3. Evidence of edema, changes in skin blood flow, or abnormal sudomotor activity in region of pain. (Vasomotor) 4. Diagnosis is excluded by the existence of other conditions that would otherwise account for the degree of pain/ dysfunction. One symptom from each category (except #1 as 5% of pts lack known event) and at least one sign from 2 categories must be evident to diagnose CRPS, at least by research criteria. *Not always present or identifiable.
CRPS More Widely Diagnosed
Quickly becoming more recognized, and possibly overly diagnosed for several reasons: The standardization of diagnosis. The "discovery" by personal injury lawyers who use it as a tool for settlements. New treatments and research for chronic pain. Patient self-research and the internet.
Diagnostic Si/Sx
The most common pain finding is a "burning" and
"stinging” sensations that occur spontaneously. This is seen in as many as 87% of cases. 69% of patients report hyperesthesia with light touch (such as clothing laying on the skin or even draughts of blowing wind). Vasomotor dysfunction is manifested by asymmetrical edema in affected limb, skin color and temperature changes, abnormal sweating (either hyperhidrosis or anhidrosis), and skin/ nail changes. Patients may also complain of muscle jerking, myoclonus, or rigidity in affected limb including contractures of hands and feet.
RSD RENAMED
Reflex Sympathetic Dystrophy renamed as
Complex Regional Pain Syndrome in 1995 CRPS type 1 is RSD CRPS type 2 is Causalgia (nerve lesion) Current evidence suggests CRPS 1 is minute nerve injury in C fibers (Oaklander AL MD PhD et al Pain 2006)
CRPS II
The majority of patients seen are diagnosed with
CRPS type I – or reflex sympathetic dystrophy (RSD). This is the less painful, debilitating of the two. CRPS II (causalgia) is related to a known injury to a specific major nerve with neuropathic pain frequently following along the distribution of that nerve alone, though not always.
While the diagnostic criteria of the IASP may apply, these patients may also demonstrate hypoesthesia in the nerve distribution associated with electrical shocks and extreme allodynia. This is rarer than CRPS I and the patients are often more debilitated.
IASP Nomenclature
CRPS I = Reflex Sympathetic Dystrophy CRPS II = Causalgia The only difference between the two is the
inciting event: minor trauma (I) versus major peripheral nerve injury (II). Algodystrophy, Sudeck’s atrophy, sympathetically maintained pain, shoulder/hand syndrome, transient osteoporosis, and acute atrophy of bone.
Clinical Presentation
Precipitating event: CRPS I
Minor trauma, contusion, sprain or strain Fracture (especially colles fx) Post surgical Immobilization Less frequently: CVA, spinal cord injury Documented peripheral nerve injury and concordant focal deficits (but the signs and symptoms of CRPS are not limited to the same distribution as the affected nerve.)
CRPS II
Clinical Presentation Time Course
Three stages:
Stage
1 (acute) Stage 2 (dystrophic) Stage 3 (atrophic)
CRPS Stage 1
(Acute)
Immediately after injury--3 months
MOST LIKELY TO BE REVERSED AND CURED
SKIN: Red, warm, swollen, dry, inflamed. Later color may
change to mottled and colder with marked hyperhydrosis. Changes back and forth especially with painful use. DISTRIBUTION: Pain is not compatible with a single peripheral nerve, trunk, or root lesion. SYMPATHETIC:
VASOMOTOR: Disturbances occur with variable intensity, producing altered color and temperature. Hyperemic Mottled SUDOMOTOR: Dry Hyperhydrosis
MOTOR: Decreased ROM, weakness X-RAYS: Normal BONE SCAN: Increased uptake
Stage 1 (Acute)
Stage 1 (Acute)
Qui ck me™ and a Ti decompress r o are ne ede d to see thi s pi cture.
CRPS Stage 2 (Dystrophic)
Pain remains SEVERE. Same characteristics
as Stage 1.
CRPS Stage 2 (Dystrophic) 6 weeks--1 year
SKIN: Cool, moist, tight/shiny, swelling,
coarse/sparse hair, brittle nails, discolored, edema SYMPATHETIC:
VASOMOTOR: Mottled/cyanotic SUDOMOTOR: Hyperhydrosis
MOTOR: Weakness, decreased ROM BONE SCAN: No longer helpful.
Stage 2 (Dystrophic)
Stage 2 (Dystrophic)
QuickTime™ and a decompress or are needed to see this picture.
Stage 2 (Dystrophic)
CRPS Stage 3 (atrophic) 6 months--Forever?
Pain is somewhat decreased (but still debilitating) less at rest, worse with passive motion Changes are irreversible, poor outcomes,
permanent disability SKIN: Atrophy, “waxy”, very thin, ulcerations, brittle nails SYMPATHETIC:
VASOMOTOR: Cold, intermittently cyanotic/mottled
MOTOR: Decreased ROM, weakness, muscle &
tendon atrophy, contractures, dystonia, tremor. Nonfunctional limb. X-RAYS: Diffuse patchy osteoporosis (Sudeck’s
Atrophic Stage 3
Severe Mottling
Atrophic Stage 3
Contractures Skin Ulceration Migratory/progressive
CRPS I Diagnostic Criteria - IASP
1. The presence of an initiating noxious event
or a cause of immobilization. 2. Continuing pain, allodynia or hyperalgesia with which the pain is disproportionate to the inciting event. 3. Evidence at some time of edema, changes in skin blood flow or abnormal sudomotor activity in the painful region. 4. The diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction.
note: Criteria 2,3 and 4 are necessary for a diagnosis of complex regional pain syndrome.
International Association for the Study of Pain: Diagnostic Criteria for Complex Regional Pain Syndrome with 1997 ICD Codes Merskey H, Bodguk N, eds. Classification of chronic pain, descriptions of chronic pain syndromes and definitions of pain terms. Id ed. Seattle: IASP Press, 1994:40-3.
CRPS II (Causalgia) - IASP
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 the pain. 3. The diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction. note: All three criteria must be satisfied.
International Association for the Study of Pain: Diagnostic Criteria for Complex Regional Pain Syndrome with 1997 ICD Codes Merskey H, Bodguk N, eds. Classification of chronic pain, descriptions of chronic pain syndromes and definitions of pain terms. Id ed. Seattle: IASP Press, 1994:40-3.
Mirror Therapy
The brain wants congruence between motor intention, peripheral sensory input and visual input. Mirror therapy “restores” this relationship.
Treatment
Treatment usually consists of several objectives: Functional restoration of affected limb - often should be considered first before other treatments Sympathetic and/or motor blocks Cognitive behavioral techniques Psychotherapy Pharmacotherapy Occupational and physical therapy
Tx – Functional Restoration
Functional restoration involves steady progression
from gentle movements to gentle, weight bearing movement. Results in more active load bearing with expected gradual desensitization and increased functionality of limb. Examples include moving from silk stimulation to other cloths and textures, the scrub and carry technique, and contrast baths that widen the temperature range that the patient can tolerate. If limitations occur then addition of blocks, pharmacotherapy, etc. can help increase the patient's tolerance and improvement.
Tx - Psychotherapy
Psychotherapy is critical to improvement in patients with CRPS.
There is a high incidence of depression and anxiety noted in many CRPS patients. Unknown at this time if antecedent psychological factors prior to injury are common. CRPS patients also develop a type of PTSD termed "kinesophobia" or fear of movement related to prior pain or initial injury. The patient develops "negative reinforcements" through fear of initial movements that caused the injury of prior movements that resulted in extreme pain in the past. Fear of movement often results in contractures and reduced functionality. Cognitive behavioral therapy is the most beneficial psychotherapy to help patients with these concerns, though other interventions including family therapy are also beneficial.
Tx - Pharmacotherapy
Pharmacotherapy is often on a trial and error basis and is very patient specific. Drugs are considered based on neuropathic pain treatments and then used for CRPS and have yet to be shown effective in RCTs. - initial drug(s) to consider include gabapentin and pregabalin (both used for neuropathic pain) and are approved for these conditions - TCAs often used for patients with sleep disturbances, but are hindered by their numerous side effects and drug interactions; not currently approved for pain treatment
Tx - Pharmacotherapy
- SSRIs and SNRIs like fluoxetine and duloxetine,
resp, are often used with the latter being approved for neuropathic pain conditions. - Opioids should be avoided as much as possible as their effectiveness is not well proved and dependence/ addiction are serious concerns in CRPS patients. - Intrathecal baclofen, IV steroids, IVIG, and anticonvulsant medications are all treatments that have been considered and used with variable success, though studies are still lacking.
Tx – Interventional Approaches
Considering the nature of the sympathetic
involvement in the patient, blocking the sympathetic nervous system seems inherent towards improvement. Cervical and lumbar sympathetic blocks are frequently performed as outpatient procedures with patients receiving a few weeks or months of relief. Often done to help with movement therapies. Beir blocks with local anesthetics, guanethidine, or other neurolytic agents have been performed with varying success. SCS, pump implantation, and thermocoagulation have also been used to treat patients with CRPS with variable success rates.
Despite the belief that blocking the sympathetic nervous system will result in reduced pain, many patients do not gain substantial benefit from these procedures for any substantial length of time and the procedures themselves may actually exacerbate the patient's pain.
CRPS Prognosis
Overall the prognosis for patients with CRPS is
relatively low. Effective treatments are very patient specific and patient satisfaction, mental state, willingness to be involved in their treatment all contribute to their pain reduction. Many patients report extreme lack of satisfaction with their pain control and are usually disabled. More research is required into chronic pain and developing better methods to treat chronic autonomic dysfunctional pain.
References
Harden RN, Complex Regional Pain
Syndrome, British Journal of Anesthesia, 2001, 87, pp. 99-106. Schott GD, Complex? Regional? Pain? Syndrome? Practical Neurology. 2007, 7, pp. 145-157. Forouzanfar T, et al. Treatment of Complex Regional Pain Syndrome Type I. European Journal of Pain. Vol 6, issue 2. Apr 2002. pp. 105-122.
1864 - Colonel Weir Mitchell, MD “severely painful dystrophic syndrome following ballistic injuries” in Civil War soldiers: Causalgia
History
Paul Sudeck Suggested that the signs and symptoms of RSD may be caused by an exaggerated inflammatory response to injury or operation of an extremity. Sudeck’s Atrophy: Bone loss associated with RSD
Rene Leriche
Sympathetic nervous system dysfunction as a cause of pain. Therapeutic surgical sympathectomy
John Bonica
The syndrome much as we
know it today
Promoted the term RSD Described 3 stages
CRPS History
CRPS (complex regional pain syndrome) initially considered in
early 1800s by Claude Bernard et al. During the Civil War it was seen that soldiers who suffered from low-velocity, high-mass missile injuries developed a neuropathic pain that was termed "causalgia" by Silas Weir-Mitchell. In 1940s the term of reflex sympathetic dystrophy came into use relating the belief of an abnormal efferent reflex from the sympathetic nervous system to bodily injury. Since that time much study and frustration has come from this relatively rare condition of which the pathophysiology is still not fully understood.
Basics
Often seen after injury to a limb or related to
some inciting event. The patient complains of and can manifest skin color/ temperature/ appearance changes in the affected limb. Pain often excruciating – burning, tingling, electric-like, etc. are often symptoms that patients feel. The pain is often out of proportion to stimulus or the event.
Physical Appearance
Diagnostic Criteria
IASP (International Assoc for the Study of Pain) diagnostic criteria include 4 subjective and/ or objective findings: 1. The presence of an initiating event or a cause of immobilization – peripheral injury or central (stroke, etc)*. (Injury) 2. Continuing pain, allodynia, or hyperalgesia in which the pain is disproportiate to inciting event. (Sensory) 3. Evidence of edema, changes in skin blood flow, or abnormal sudomotor activity in region of pain. (Vasomotor) 4. Diagnosis is excluded by the existence of other conditions that would otherwise account for the degree of pain/ dysfunction. One symptom from each category (except #1 as 5% of pts lack known event) and at least one sign from 2 categories must be evident to diagnose CRPS, at least by research criteria. *Not always present or identifiable.
CRPS More Widely Diagnosed
Quickly becoming more recognized, and possibly overly diagnosed for several reasons: The standardization of diagnosis. The "discovery" by personal injury lawyers who use it as a tool for settlements. New treatments and research for chronic pain. Patient self-research and the internet.
Diagnostic Si/Sx
The most common pain finding is a "burning" and
"stinging” sensations that occur spontaneously. This is seen in as many as 87% of cases. 69% of patients report hyperesthesia with light touch (such as clothing laying on the skin or even draughts of blowing wind). Vasomotor dysfunction is manifested by asymmetrical edema in affected limb, skin color and temperature changes, abnormal sweating (either hyperhidrosis or anhidrosis), and skin/ nail changes. Patients may also complain of muscle jerking, myoclonus, or rigidity in affected limb including contractures of hands and feet.
RSD RENAMED
Reflex Sympathetic Dystrophy renamed as
Complex Regional Pain Syndrome in 1995 CRPS type 1 is RSD CRPS type 2 is Causalgia (nerve lesion) Current evidence suggests CRPS 1 is minute nerve injury in C fibers (Oaklander AL MD PhD et al Pain 2006)
CRPS II
The majority of patients seen are diagnosed with
CRPS type I – or reflex sympathetic dystrophy (RSD). This is the less painful, debilitating of the two. CRPS II (causalgia) is related to a known injury to a specific major nerve with neuropathic pain frequently following along the distribution of that nerve alone, though not always.
While the diagnostic criteria of the IASP may apply, these patients may also demonstrate hypoesthesia in the nerve distribution associated with electrical shocks and extreme allodynia. This is rarer than CRPS I and the patients are often more debilitated.
IASP Nomenclature
CRPS I = Reflex Sympathetic Dystrophy CRPS II = Causalgia The only difference between the two is the
inciting event: minor trauma (I) versus major peripheral nerve injury (II). Algodystrophy, Sudeck’s atrophy, sympathetically maintained pain, shoulder/hand syndrome, transient osteoporosis, and acute atrophy of bone.
Clinical Presentation
Precipitating event: CRPS I
Minor trauma, contusion, sprain or strain Fracture (especially colles fx) Post surgical Immobilization Less frequently: CVA, spinal cord injury Documented peripheral nerve injury and concordant focal deficits (but the signs and symptoms of CRPS are not limited to the same distribution as the affected nerve.)
CRPS II
Clinical Presentation Time Course
Three stages:
Stage
1 (acute) Stage 2 (dystrophic) Stage 3 (atrophic)
CRPS Stage 1
(Acute)
Immediately after injury--3 months
MOST LIKELY TO BE REVERSED AND CURED
SKIN: Red, warm, swollen, dry, inflamed. Later color may
change to mottled and colder with marked hyperhydrosis. Changes back and forth especially with painful use. DISTRIBUTION: Pain is not compatible with a single peripheral nerve, trunk, or root lesion. SYMPATHETIC:
VASOMOTOR: Disturbances occur with variable intensity, producing altered color and temperature. Hyperemic Mottled SUDOMOTOR: Dry Hyperhydrosis
MOTOR: Decreased ROM, weakness X-RAYS: Normal BONE SCAN: Increased uptake
Stage 1 (Acute)
Stage 1 (Acute)
Qui ck me™ and a Ti decompress r o are ne ede d to see thi s pi cture.
CRPS Stage 2 (Dystrophic)
Pain remains SEVERE. Same characteristics
as Stage 1.
CRPS Stage 2 (Dystrophic) 6 weeks--1 year
SKIN: Cool, moist, tight/shiny, swelling,
coarse/sparse hair, brittle nails, discolored, edema SYMPATHETIC:
VASOMOTOR: Mottled/cyanotic SUDOMOTOR: Hyperhydrosis
MOTOR: Weakness, decreased ROM BONE SCAN: No longer helpful.
Stage 2 (Dystrophic)
Stage 2 (Dystrophic)
QuickTime™ and a decompress or are needed to see this picture.
Stage 2 (Dystrophic)
CRPS Stage 3 (atrophic) 6 months--Forever?
Pain is somewhat decreased (but still debilitating) less at rest, worse with passive motion Changes are irreversible, poor outcomes,
permanent disability SKIN: Atrophy, “waxy”, very thin, ulcerations, brittle nails SYMPATHETIC:
VASOMOTOR: Cold, intermittently cyanotic/mottled
MOTOR: Decreased ROM, weakness, muscle &
tendon atrophy, contractures, dystonia, tremor. Nonfunctional limb. X-RAYS: Diffuse patchy osteoporosis (Sudeck’s
Atrophic Stage 3
Severe Mottling
Atrophic Stage 3
Contractures Skin Ulceration Migratory/progressive
CRPS I Diagnostic Criteria - IASP
1. The presence of an initiating noxious event
or a cause of immobilization. 2. Continuing pain, allodynia or hyperalgesia with which the pain is disproportionate to the inciting event. 3. Evidence at some time of edema, changes in skin blood flow or abnormal sudomotor activity in the painful region. 4. The diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction.
note: Criteria 2,3 and 4 are necessary for a diagnosis of complex regional pain syndrome.
International Association for the Study of Pain: Diagnostic Criteria for Complex Regional Pain Syndrome with 1997 ICD Codes Merskey H, Bodguk N, eds. Classification of chronic pain, descriptions of chronic pain syndromes and definitions of pain terms. Id ed. Seattle: IASP Press, 1994:40-3.
CRPS II (Causalgia) - IASP
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 the pain. 3. The diagnosis is excluded by the existence of conditions that would otherwise account for the degree of pain and dysfunction. note: All three criteria must be satisfied.
International Association for the Study of Pain: Diagnostic Criteria for Complex Regional Pain Syndrome with 1997 ICD Codes Merskey H, Bodguk N, eds. Classification of chronic pain, descriptions of chronic pain syndromes and definitions of pain terms. Id ed. Seattle: IASP Press, 1994:40-3.
Mirror Therapy
The brain wants congruence between motor intention, peripheral sensory input and visual input. Mirror therapy “restores” this relationship.
Treatment
Treatment usually consists of several objectives: Functional restoration of affected limb - often should be considered first before other treatments Sympathetic and/or motor blocks Cognitive behavioral techniques Psychotherapy Pharmacotherapy Occupational and physical therapy
Tx – Functional Restoration
Functional restoration involves steady progression
from gentle movements to gentle, weight bearing movement. Results in more active load bearing with expected gradual desensitization and increased functionality of limb. Examples include moving from silk stimulation to other cloths and textures, the scrub and carry technique, and contrast baths that widen the temperature range that the patient can tolerate. If limitations occur then addition of blocks, pharmacotherapy, etc. can help increase the patient's tolerance and improvement.
Tx - Psychotherapy
Psychotherapy is critical to improvement in patients with CRPS.
There is a high incidence of depression and anxiety noted in many CRPS patients. Unknown at this time if antecedent psychological factors prior to injury are common. CRPS patients also develop a type of PTSD termed "kinesophobia" or fear of movement related to prior pain or initial injury. The patient develops "negative reinforcements" through fear of initial movements that caused the injury of prior movements that resulted in extreme pain in the past. Fear of movement often results in contractures and reduced functionality. Cognitive behavioral therapy is the most beneficial psychotherapy to help patients with these concerns, though other interventions including family therapy are also beneficial.
Tx - Pharmacotherapy
Pharmacotherapy is often on a trial and error basis and is very patient specific. Drugs are considered based on neuropathic pain treatments and then used for CRPS and have yet to be shown effective in RCTs. - initial drug(s) to consider include gabapentin and pregabalin (both used for neuropathic pain) and are approved for these conditions - TCAs often used for patients with sleep disturbances, but are hindered by their numerous side effects and drug interactions; not currently approved for pain treatment
Tx - Pharmacotherapy
- SSRIs and SNRIs like fluoxetine and duloxetine,
resp, are often used with the latter being approved for neuropathic pain conditions. - Opioids should be avoided as much as possible as their effectiveness is not well proved and dependence/ addiction are serious concerns in CRPS patients. - Intrathecal baclofen, IV steroids, IVIG, and anticonvulsant medications are all treatments that have been considered and used with variable success, though studies are still lacking.
Tx – Interventional Approaches
Considering the nature of the sympathetic
involvement in the patient, blocking the sympathetic nervous system seems inherent towards improvement. Cervical and lumbar sympathetic blocks are frequently performed as outpatient procedures with patients receiving a few weeks or months of relief. Often done to help with movement therapies. Beir blocks with local anesthetics, guanethidine, or other neurolytic agents have been performed with varying success. SCS, pump implantation, and thermocoagulation have also been used to treat patients with CRPS with variable success rates.
Despite the belief that blocking the sympathetic nervous system will result in reduced pain, many patients do not gain substantial benefit from these procedures for any substantial length of time and the procedures themselves may actually exacerbate the patient's pain.
CRPS Prognosis
Overall the prognosis for patients with CRPS is
relatively low. Effective treatments are very patient specific and patient satisfaction, mental state, willingness to be involved in their treatment all contribute to their pain reduction. Many patients report extreme lack of satisfaction with their pain control and are usually disabled. More research is required into chronic pain and developing better methods to treat chronic autonomic dysfunctional pain.
References
Harden RN, Complex Regional Pain
Syndrome, British Journal of Anesthesia, 2001, 87, pp. 99-106. Schott GD, Complex? Regional? Pain? Syndrome? Practical Neurology. 2007, 7, pp. 145-157. Forouzanfar T, et al. Treatment of Complex Regional Pain Syndrome Type I. European Journal of Pain. Vol 6, issue 2. Apr 2002. pp. 105-122.
