complex regional pain syndrome arthur r. smith, md january 13, 2009 arthur r. smith, md january 13,...
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COMPLEX REGIONAL COMPLEX REGIONAL PAIN SYNDROMEPAIN SYNDROME
COMPLEX REGIONAL COMPLEX REGIONAL PAIN SYNDROMEPAIN SYNDROME
Arthur R. Smith, MDArthur R. Smith, MD
January 13, 2009January 13, 2009
Arthur R. Smith, MDArthur R. Smith, MD
January 13, 2009January 13, 2009
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COMPLEX REGIONAL PAIN SYNDROME
COMPLEX REGIONAL PAIN SYNDROME
History Epidemiology Definition & Taxonomy Causes Clinical Presentation Diagnostic Tests Pathophysiology Treatments
History Epidemiology Definition & Taxonomy Causes Clinical Presentation Diagnostic Tests Pathophysiology Treatments
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1864 - Colonel Weir Mitchell, MD“severely painful dystrophic syndrome following ballistic
injuries” in Civil War soldiers: Causalgia
1864 - Colonel Weir Mitchell, MD“severely painful dystrophic syndrome following ballistic
injuries” in Civil War soldiers: Causalgia
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HistoryHistory
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
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
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Rene LericheRene Leriche
Sympathetic nervous system dysfunction as a cause of pain.
Therapeutic surgical sympathectomy
Sympathetic nervous system dysfunction as a cause of pain.
Therapeutic surgical sympathectomy
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John BonicaJohn Bonica
The syndrome much as we know it today Promoted the term RSD Described 3 stages
The syndrome much as we know it today Promoted the term RSD Described 3 stages
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EpidemiologyEpidemiology CRPS I: 21 per 100,000 CRPS II: 4 per 100,000
Female-to-Male ratio: 3:1 Any age, but middle age predominates
Median 42 years Onset 9 – 85 years of age CRPS occurs in about 1-2% of patients who have
had fractures and in approximately 2-5% of patients after peripheral nerve injuries
CRPS I: 21 per 100,000 CRPS II: 4 per 100,000
Female-to-Male ratio: 3:1 Any age, but middle age predominates
Median 42 years Onset 9 – 85 years of age CRPS occurs in about 1-2% of patients who have
had fractures and in approximately 2-5% of patients after peripheral nerve injuries
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Complex Regional Pain Syndrome:
a variety of painful conditions following injury which appears regionally having a distal predominance of abnormal findings, exceeding in both magnitude and duration the expected clinical course of the inciting event, often resulting in significant impairment of motor function, and showing variable progression over time.
Complex Regional Pain Syndrome:
a variety of painful conditions following injury which appears regionally having a distal predominance of abnormal findings, exceeding in both magnitude and duration the expected clinical course of the inciting event, often resulting in significant impairment of motor function, and showing variable progression over time.
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IASP NomenclatureIASP 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.
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.
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Clinical PresentationClinical Presentation Precipitating event:
CRPS I Minor trauma, contusion, sprain or strain Fracture (especially colles fx) Post surgical Immobilization Less frequently: CVA, spinal cord injury
CRPS II 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.)
Precipitating event: CRPS I
Minor trauma, contusion, sprain or strain Fracture (especially colles fx) Post surgical Immobilization Less frequently: CVA, spinal cord injury
CRPS II 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.)
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Clinical PresentationClinical Presentation
Usually an extremity(65%) , but any part of the body can be affected.
CRPS may progress and spread to other extremities over time.
Usually an extremity(65%) , but any part of the body can be affected.
CRPS may progress and spread to other extremities over time.
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Clinical PresentationClinical Presentation
PAIN, PAIN, PAIN Spontaneous, constant, burning, aching, throbbing Disproportionate to the injury and persists beyond normal
or expected recovery period Asymmetrical and not in the distribution of a peripheral
nerve. Worst distally. Severe mechanical and thermal allodynia, hyperalgesia,
and hyperpathia
PAIN, PAIN, PAIN Spontaneous, constant, burning, aching, throbbing Disproportionate to the injury and persists beyond normal
or expected recovery period Asymmetrical and not in the distribution of a peripheral
nerve. Worst distally. Severe mechanical and thermal allodynia, hyperalgesia,
and hyperpathia
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Clinical PresentationClinical Presentation
Autonomic (Sympathetic) Abnormalities Vascular
Hot, swollen, erythemetous Cold, blanched Mottled
Sudomotor Hyperhydrosis Hypohydorosis
Autonomic (Sympathetic) Abnormalities Vascular
Hot, swollen, erythemetous Cold, blanched Mottled
Sudomotor Hyperhydrosis Hypohydorosis
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Clinical PresentationClinical Presentation
Motor Diffuse weakness of the extremity, but normal
EMG/NCS until late in the course of the disease.
Tremor Dystonia occasionally
Motor Diffuse weakness of the extremity, but normal
EMG/NCS until late in the course of the disease.
Tremor Dystonia occasionally
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Clinical PresentationClinical Presentation
Trophic Changes Nail growth Loss of function: muscle, joint and tendon
atrophy, contractures and fibrosis Hair changes (coarse hair, loss of hair) Skin--thin and glossy, loss of elasticity,
ulceration. Osteoporosis
Trophic Changes Nail growth Loss of function: muscle, joint and tendon
atrophy, contractures and fibrosis Hair changes (coarse hair, loss of hair) Skin--thin and glossy, loss of elasticity,
ulceration. Osteoporosis
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Clinical PresentationTime Course
Clinical PresentationTime Course
Three stages: Stage 1 (acute) Stage 2 (dystrophic) Stage 3 (atrophic)
Three stages: Stage 1 (acute) Stage 2 (dystrophic) Stage 3 (atrophic)
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CRPS Stage 1 (Acute)Immediately after injury--3 months
MOST LIKELY TO BE REVERSED AND CURED
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
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
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Stage 1 (Acute)Stage 1 (Acute)
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Stage 1 (Acute)Stage 1 (Acute)
QuickTime™ and a decompressor
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CRPS Stage 2 (Dystrophic)CRPS Stage 2 (Dystrophic)
Pain remains SEVERE. Same characteristics as Stage 1.
Pain remains SEVERE. Same characteristics as Stage 1.
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CRPS Stage 2 (Dystrophic)6 weeks--1 year
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.
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.
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Stage 2 (Dystrophic)
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Stage 2 (Dystrophic)
QuickTime™ and a decompressor
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Stage 2 (Dystrophic)
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CRPS Stage 3 (atrophic)6 months--Forever?
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 Atrophy)
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 Atrophy)
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Atrophic Stage 3 Atrophic Stage 3
Severe MottlingSevere Mottling
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Atrophic Stage 3Atrophic Stage 3
ContracturesSkin UlcerationMigratory/progressive
ContracturesSkin UlcerationMigratory/progressive
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Atrophic Stage 3Atrophic Stage 3
ContracturesContracturesQuickTime™ and a
decompressorare needed to see this picture.
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Diagnostic TestsSympathetic Blockade
Diagnostic TestsSympathetic Blockade
Sympathetically Maintained Pain Previously synonymous with CRPS Sympathetic block was deemed
diagnostic for CRPS. Now considered a symptom of
underlying neuropathic pain sydromes, including, but not exclusively, CRPS.
Sympathetically Maintained Pain Previously synonymous with CRPS Sympathetic block was deemed
diagnostic for CRPS. Now considered a symptom of
underlying neuropathic pain sydromes, including, but not exclusively, CRPS.
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SYMPATHETICALLY MAINTAINED PAIN
SYMPATHETICALLY MAINTAINED PAIN
SMP
CRPS
PHN
PNP
PHANTOM LIMB
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Diagnostic Tests:Sympathetic Blockade
Diagnostic Tests:Sympathetic Blockade
Stellate Ganglion Block Lumbar Sympathetic Block
A successful block (increase in temperature, for example) that results in pain relief helps confirm a diagnosis of CRPS in the presence of other consistent clinical findings.
Stellate Ganglion Block Lumbar Sympathetic Block
A successful block (increase in temperature, for example) that results in pain relief helps confirm a diagnosis of CRPS in the presence of other consistent clinical findings.
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Diagnostic TestsDiagnostic Tests
Three Phase Bone Scintigraphy Only in acute stage Hyperperfusion Suggestive and supportive of the diagnosis of
CRPS, but not diagnostic
Three Phase Bone Scintigraphy Only in acute stage Hyperperfusion Suggestive and supportive of the diagnosis of
CRPS, but not diagnostic
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Diagnostic TestsDiagnostic Tests
Plain Radiographs Late findings only with atrophic stage showing
bone loss and patchy osteoporosis
Plain Radiographs Late findings only with atrophic stage showing
bone loss and patchy osteoporosis
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Diagnostic Tests Skin TemperatureDiagnostic Tests
Skin TemperatureThermography may show asymmetry. Affected limb is
warmer than normal in acute stage and later becomes cooler. Not a readily available procedure.
Thermography may show asymmetry. Affected limb is warmer than normal in acute stage and later becomes cooler. Not a readily available procedure.
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Diagnostic TestsDiagnostic Tests
Quantitative Sensory Testing: Rarely available and no specific profile for CRPS
QSART: Quantitative Sudomotor Axon Reflex Test of autonomic function. Rarely available
Quantitative Sensory Testing: Rarely available and no specific profile for CRPS
QSART: Quantitative Sudomotor Axon Reflex Test of autonomic function. Rarely available
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Diagnostic CriteriaDiagnostic Criteria
Diagnosis is based on clinical findings although tests may support either a positive or negative diagnosis.
Diagnosis is based on clinical findings although tests may support either a positive or negative diagnosis.
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CRPS I Diagnostic Criteria - IASPCRPS 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.
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.
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CRPS II (Causalgia) - IASPCRPS II (Causalgia) - IASP1. 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 CodesMerskey 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.
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 CodesMerskey 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.
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PathophysiologyPathophysiology
NOT KNOWN! What we do know:
Neurogenic Inflammation (acute stage)
NOT KNOWN! What we do know:
Neurogenic Inflammation (acute stage)
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PathophysiologyPathophysiology
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PathophysiologyPathophysiology
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PathophysiologyPathophysiology
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PathophysiologyPathophysiology
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Treatment GoalsTreatment Goals
Relief of pain Return of function Prevent or slow progression
Relief of pain Return of function Prevent or slow progression
IMPROVED OUTCOME=
EARLY TREATMENT
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Physical TherapyPhysical Therapy
In the acute stage PT is the most important factor in reversing the syndrome.
Later, it can improve pain & function and help prevent progression and migration.
Aggressive PT may only be possible with treatment of pain: pain meds, sympathetic and/or somatic blockade.
In the acute stage PT is the most important factor in reversing the syndrome.
Later, it can improve pain & function and help prevent progression and migration.
Aggressive PT may only be possible with treatment of pain: pain meds, sympathetic and/or somatic blockade.
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MedicationsMedications
NSAIDs-Mild to moderate pain Opioids-Effective for severe neuropathic pain.
Beware of all issues related to chronic opioid use.
Steroids-Proven effective in acute (inflammatory) stage.
Gabapentin and Pregabalin-Effective
NSAIDs-Mild to moderate pain Opioids-Effective for severe neuropathic pain.
Beware of all issues related to chronic opioid use.
Steroids-Proven effective in acute (inflammatory) stage.
Gabapentin and Pregabalin-Effective
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MedicationsMedications Tricyclics-Effective for a variety of neuropathies Sodium channel blockers-IV lidocaine, Lidoderm,
mexilitene, lamotrigine. Calcitonin (intranasal)-Effective in acute stage. How? NMDA blockers-Ketamine, dextromethorphan DMSO (topical)- Free radical scavenger.
Questionable benefit Topical Clonidine- 2-agonist: prevents release of
catecholamines? Maybe helpful.
Tricyclics-Effective for a variety of neuropathies Sodium channel blockers-IV lidocaine, Lidoderm,
mexilitene, lamotrigine. Calcitonin (intranasal)-Effective in acute stage. How? NMDA blockers-Ketamine, dextromethorphan DMSO (topical)- Free radical scavenger.
Questionable benefit Topical Clonidine- 2-agonist: prevents release of
catecholamines? Maybe helpful.
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Sympathetic BlockadeSympathetic Blockade
Lumbar sympathetic block Stellate ganglion block IV regional with guanethidine
If effective, sympathetic blockade often gives relief well past the duration of the block.
Repeated blocks can be reverse the course of the disease.
Very helpful in facilitating PT.
Lumbar sympathetic block Stellate ganglion block IV regional with guanethidine
If effective, sympathetic blockade often gives relief well past the duration of the block.
Repeated blocks can be reverse the course of the disease.
Very helpful in facilitating PT.
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Psychology (Psychiatry)Psychology (Psychiatry)
Earlier anxiety progresses to severe depression. (Pain, loss of work and self worth, financial loss, family breakdown, pain behavior, medication dependence and abuse)
Medical treatment of depression Counseling, set realistic goals and expectations,
behavioral & cognitive therapies, biofeedback, hypnosis.
Earlier anxiety progresses to severe depression. (Pain, loss of work and self worth, financial loss, family breakdown, pain behavior, medication dependence and abuse)
Medical treatment of depression Counseling, set realistic goals and expectations,
behavioral & cognitive therapies, biofeedback, hypnosis.
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Continuous InfusionContinuous Infusion
Tunneled epidural catheter. Patients who have a good but short duration response to sympathetic block or for sympathetic independent pain. May be left in place for several weeks. Titrate local anesthetic to sympathetic or
somatic block with minimal motor block. Physical therapy every waking moment!
Tunneled epidural catheter. Patients who have a good but short duration response to sympathetic block or for sympathetic independent pain. May be left in place for several weeks. Titrate local anesthetic to sympathetic or
somatic block with minimal motor block. Physical therapy every waking moment!
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Spinal Cord StimulationSpinal Cord Stimulation
Permanently implanted for control of chronic neuropathic pain
Tunneled percutaneous leads for several weeks in the acute stage for therapeutic reversal for the disease. More and more frequently used.
Permanently implanted for control of chronic neuropathic pain
Tunneled percutaneous leads for several weeks in the acute stage for therapeutic reversal for the disease. More and more frequently used.
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Spinal Cord StimulationSpinal Cord Stimulation
QuickTime™ and a decompressor
are needed to see this picture.
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KetamineKetamine
NMDA as a mediator of chronic pain Ketamine “coma” (Germany & Mexico)
NMDA as a mediator of chronic pain Ketamine “coma” (Germany & Mexico)
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Mirror TherapyMirror Therapy The brain wants congruence between motor
intention, peripheral sensory input and visual input. Mirror therapy “restores” this relationship.
The brain wants congruence between motor intention, peripheral sensory input and visual input. Mirror therapy “restores” this relationship.
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The FutureThe Future
Education for earlier detection and aggressive treatment.
Better understanding of the pathophysiology for development of specific, targeted therapies.
Education for earlier detection and aggressive treatment.
Better understanding of the pathophysiology for development of specific, targeted therapies.
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