Complex Regional Pain Syndrome Complex Regional Pain Syndrome

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Complex Regional Pain Syndrome 神經內科 洪國華

Complex Regional Pain Syndrome 神經內科 洪國華

Complex Regional Pain Syndrome

Complex Regional Pain Syndrome

Background 4 Historical names – Minor Causalgia, posttraumatic spreading neuralgia, shoulder hand syndrome, Sudeck’s

Background 4 Historical names – Minor Causalgia, posttraumatic spreading neuralgia, shoulder hand syndrome, Sudeck’s atrophy, sympathalgia, etc 4 Renamed in 1993 by the International Association for the Study of Pain (IASP) – Regional sympathetic dystrophy syndrome (RSDS) as Complex Regional Pain Syndrome Type 1 – Causalgia as Complex Regional Pain Syndrome Type 2

Classification of CRPS 4 Type I – Previously known as reflex sympathetic dystrophy syndrome

Classification of CRPS 4 Type I – Previously known as reflex sympathetic dystrophy syndrome – Occurs after an illness or injury that didn't directly damage the nerves in affected limb 4 Type II – Once referred to as causalgia – Follows a distinct nerve injury

Epidemiology 4 Actual incidence is unknown, as CRPS is often misdiagnosed 4 Incidence of

Epidemiology 4 Actual incidence is unknown, as CRPS is often misdiagnosed 4 Incidence of causalgia (CRPS II) following injury to a peripheral nerve is 1 -5 4 Incidence of RSD (CRPS I) is 1 -2% after various fractures 4 Ratio of female: male is 3: 1 4 May appear in every age group 4 Less common in children aged less than 10 years

Etiology 4 Several causes linking to the disease – The most common is being

Etiology 4 Several causes linking to the disease – The most common is being trauma – No correlation between severity of insult to the severity of the CRPS – Other causes are venipuncture, infection, surgery, arthritis, coronary artery disease, Parkinson’s disease, head injury and stroke 4 Hard to find any cause in a large number of cases

Risks Factors 4 Immobilization 4 Smoking 4 Substance abuse 4 Genetic factor 4 Psychological

Risks Factors 4 Immobilization 4 Smoking 4 Substance abuse 4 Genetic factor 4 Psychological factor – premorbid history of depression and anxiety disorder – dependent personality

Pathophysiology 4 Exact pathophysiology is unknown – Dysfunction of peripheral autonomic nervous system –

Pathophysiology 4 Exact pathophysiology is unknown – Dysfunction of peripheral autonomic nervous system – Myofascial dysfunction – Abnormal changes in the spinal cord and brain – Aberrant healing response – Exaggerated inflammatory response – Protective disuse of the limb

The lateral (A) and medial (B) systems of transmission of Pain signals

The lateral (A) and medial (B) systems of transmission of Pain signals

Antinociceptive pathways

Antinociceptive pathways

Synapse between nociceptors and dorsal horn cells during acute and chronic pain

Synapse between nociceptors and dorsal horn cells during acute and chronic pain

Clinical Features 4 The common symptoms are pain, paresis, altered skin temperature, skin color

Clinical Features 4 The common symptoms are pain, paresis, altered skin temperature, skin color change, limited range of motion, edema, hyperesthesia, sweating changes, tremor, muscle atrophy, altered nail and hair growth and skin atrophy 4 Progression of CRPS has been classified into three stages

Stages I 4 Usually last between two to six weeks but can last up

Stages I 4 Usually last between two to six weeks but can last up to 4 4 4 six months Skin is warm, red & dry initially but eventually cyanotic, cold & sweaty Mottling of skin Change in skin temperature, hyperhydrosis, hyperesthesia and edema (usually non-pitting type) affecting involved limb Pain is usually not significant but there may be tenderness on palpation or movement and is exacerbated by emotional stimuli Hyperesthesia is reported to be in a stocking and glove distribution

Stages II 4 Occur after 2 to 6 weeks, even 3 and 7 months,

Stages II 4 Occur after 2 to 6 weeks, even 3 and 7 months, after the 4 4 4 4 initial injury Last for approximately 3 to 6 months Pain is continuous, burning and becomes more diffused Skin has cool, pale, mottled cyanotic and shiny appearance Hyperhydrosis is common Edema may become more extensive Other characteristics: stiffness of joints, atrophical changes in skin, nails and muscles X-rays may show patchy osteoporosis

Complex Regional Pain Syndrome

Complex Regional Pain Syndrome

Stage III 4 Occurs six to eight month after the initial injury 4 Characterized

Stage III 4 Occurs six to eight month after the initial injury 4 Characterized by atrophy of skin, muscles and fascia 4 4 (complete and irreversible) Skin temperature decreases & joints become weakened with limited range of motion Digits become thin with associated tendon contractures Pain is intractable and usually spreads to involve the entire limb in a proximal direction (contiguous) or elsewhere (mirror-image or independent) X-rays shows diffused bony demineralization

Diagnosis 4 CRPS is a clinical diagnosis 4 In 1994 IASP established the diagnostic

Diagnosis 4 CRPS is a clinical diagnosis 4 In 1994 IASP established the diagnostic criteria – The presence of an initiating event – A cause of immobilization – Continuous pain, allodynia and/or hyperalgesia – Skin temperature changes more than 1. 1 o C difference from the homologous body part – Evidence at some time of edema, skin color changes and abnormal pseudomotor activity in the area of pain – No existence of other condition that would otherwise account for the degree of pain and dysfunction

Three Clinical Criteria 4 4 4 Criterion #1: Persistent pain, which is disproportionate to

Three Clinical Criteria 4 4 4 Criterion #1: Persistent pain, which is disproportionate to the original injury Criterion #2: During the subjective examination, the patient may report of: 1)Hyperesthesia (extra skin sensitivity) 2)Skin temperature and/or colour asymmetry 3)Swelling and or sweating of the effected limb 4)Decreased ROM and/or motor weakness Criterion #3: During the objective examination, the patient may present with: 1)Pain with light touch (Hyperalgesia and/or allodynia) 2)Swelling and/or sweating asymmetry 3)Decreased ROM and/or motor weakness 4)Trophic changes in hair, nail and/or skin Bruel S et al 1999 External validation of IASP diagnostic criteria for complex regional pain syndrome and proposed research diagnostic criteria. Pain 81; 147 -154

Imaging Studies 4 X-ray films may show patchy periarticular demineralization within 3 -6 weeks

Imaging Studies 4 X-ray films may show patchy periarticular demineralization within 3 -6 weeks – Osteoporotic Changes do not usually appear until the sixth week and can progress for about a year 4 Three phase bone scan demonstrates distinctive pattern of radiotracer uptake – Early stages shows increase in blood flow – Late stages shows total decrease in blood flow – The diagnostic sign is periarticular pooling specially in the late phase of the scan (only sensitive within the first 20 -26 weeks of the onset of CRPS)

CRPS after Colles’ Fracture

CRPS after Colles’ Fracture

Three-phase bone scan

Three-phase bone scan

Other Laboratory Tests 4 Thermographic imaging – A temperature difference of 0. 60 C

Other Laboratory Tests 4 Thermographic imaging – A temperature difference of 0. 60 C is considered significant 4 Diagnostic stellate ganglion block and lumbar sympathetic block for upper and lower extremity CRPS respectively – An optimal block increases the temperature of the skin of the affected part 4 Sudomotor function testing: – Quantative sudomotor axon reflex test (QSART) – Sympathetic skin response (SSR)

Thermographic imaging

Thermographic imaging

Treatment 4 Pharmacological Management 4 Physical therapy 4 Psychological therapies 4 Sympathetic Blocks (SB)

Treatment 4 Pharmacological Management 4 Physical therapy 4 Psychological therapies 4 Sympathetic Blocks (SB) 4 Sympathectomy 4 Spinal Cord Stimulation 4 Intrathecal drug delivery system

Pharmacological Management-1 4 Analgesics – Nonsteroidal anti-inflammatory drugs, corticosteroids (prednisone), tramadol (Ultram) 4 Antidepressants

Pharmacological Management-1 4 Analgesics – Nonsteroidal anti-inflammatory drugs, corticosteroids (prednisone), tramadol (Ultram) 4 Antidepressants – Amitriptyline (Elavil), doxepin (Sinequan), nortriptyline (Pamelor), trazodone (Desyrel) 4 Anticonvulsants – Carbamazepine (Tegretol), gabapentin (Neurontin), phenytoin (Dilantin) 4 Sympathomimetics – Clonidine patch (Catapres-TTS), phentolamine IV (Regitine), epidural blocks, guanethidine (not available in the United States), bretylium (Bretylol), calcium channel blockers, beta-blockers, alpha-blockers 4 Muscle relaxants – Clonazepam (Klonopin), baclofen (Lioresal)

Pharmacological Management-2 4 Antiarrhythmics – Mexiletine (Mexitil) 4 Calcitonin – Calcitonin injections (Calcimar) 4

Pharmacological Management-2 4 Antiarrhythmics – Mexiletine (Mexitil) 4 Calcitonin – Calcitonin injections (Calcimar) 4 Oral opioids (controversial) – Hydromorphone (Dilaudid), morphine, oxycodone (Percocet) 4 Topical analgesics – Capsaicin cream (Zostrix), lidocaine transdermal (Lidoderm) 5% patches

Physical therapy 4 Mobilization is the most important aspect of treatment particularly during the

Physical therapy 4 Mobilization is the most important aspect of treatment particularly during the acute phase 4 The goal of physical therapy is restoration of function by – – Eliminating guarding postures & substitute movements Restoration of normal ROM, strength & motor control Increasing total daily activity time Decrease pain responses to noxious stimuli 4 Forceful manipulation of the extremity should be avoided.

Psychological therapies 4 The goal of treatment – to help patient fight against fear

Psychological therapies 4 The goal of treatment – to help patient fight against fear of reinjury, and worsening pain – overcome anxiety, depression and other psychological co-morbidities 4 Common psychological strategies – Cognitive-behavioral psychotherapy – Group psychotherapy – Symptom specific psychological treatments such as biofeedback & hypnosis

Sympathetic Blocks (SB) 4 When diagnosed in the first three months nerve blocks may

Sympathetic Blocks (SB) 4 When diagnosed in the first three months nerve blocks may be effective 4 Techniques utilizing for SB – Selective sympathetic ganglion block, stellate ganglion block, lumbar sympathetic block, intravenous regional sympathetic block, and epidural clonidine injection 4 Current data does not support the long-term effectiveness of sympathetic block for the treatment of CRPS

Sympathectomy 4 Performed on those patients who consistently report transient pain relief after a

Sympathectomy 4 Performed on those patients who consistently report transient pain relief after a series of nonablative sympathetic blocks 4 the success rate of sympathectomy varies from 12% to 97% 4 Methods – Radiofrequency neurolysis (RFN) – chemical and surgical sympathectomy

Spinal Cord Stimulation 4 The possible mechanisms of actions – Activation of nerves in

Spinal Cord Stimulation 4 The possible mechanisms of actions – Activation of nerves in the dorsal column – Direct blockage of nociceptive input – Sympathetic inhibition 4 A retrospective case study reported excellent pain relief in 8 patients out of 12, with 41 -month follow-up (Kumar K et al: Spinal cord stimulation is effective in the management of reflex sympathetic dystrophy. Neurosurgery; 40: 503 -508, 1997 )

Spinal Cord Stimulation

Spinal Cord Stimulation

Intrathecal drug delivery system 4 This system besides morphine can also delivers other drugs

Intrathecal drug delivery system 4 This system besides morphine can also delivers other drugs such as Fentanyl, Sufentanil, Bupivicaine, Baclofen, Clonidine, Ziconotide and others 4 Some small-uncontrolled studies report the moderate improvement of CRPS pain with intrathecal opioid therapy 4 Reserved for those patients who have failed to response to other medical therapies

Conclusion 4 CRPS is a very complicated neuropathic pain syndrome 4 Early diagnosis is

Conclusion 4 CRPS is a very complicated neuropathic pain syndrome 4 Early diagnosis is crucial 4 The best recommended treatment is a multidisciplinary approach with the goal of symptom control and restoration of functionality 4 Aggressive medical and physical therapies should be administered as soon as possible under proper supervision 4 Psychological therapies should also initiate at the same time 4 Ablative sympathectomy and placement of spinal cord stimulator should be reserved for those patients who have failed conservative therapies

1. Which one of the following is not included in the criteria for the

1. Which one of the following is not included in the criteria for the diagnosis of CRPS? a. Pain that develops after an initial painful b. c. d. e. event that may or may not have been traumatic Distribution of the painful area is limited to the distribution of a single peripheral nerve History of edema, skin blood flow abnormalities, or sudomotor abnormalities in the painful region No other concomitant conditions account for the pain Allodynia, hyperalgesia, or spontaneous pain is present

2. Which one of the following is the cornerstone and first-line treatment of CRPS?

2. Which one of the following is the cornerstone and first-line treatment of CRPS? a. Gabapentin b. Tricyclic antidepressants c. Opioids d. Corticosteroids e. Physical therapy

3. Which one of the following statements is false? a. The sympathetic nervous system

3. Which one of the following statements is false? a. The sympathetic nervous system is always b. c. d. e. involved in the mechanism of pain in CRPS Sympathetic nerve blockade can be helpful in treating CRPS Somatic nerve blockade can be helpful in treating CRPS Chronic painful conditions are often accompanied by depression and anxiety Physical therapy should always be part of the treatment plan for patients with CRPS

4. Which one of the following is not a useful test in supporting the

4. Which one of the following is not a useful test in supporting the diagnosis of CRPS? a. Quantitative sudomotor axonal reflex b. c. d. e. test Resting sweat output Resting skin temperature Muscle biopsy Three-phase bone scan

5. Which one of the following statements is true? a. Negative response to a

5. Which one of the following statements is true? a. Negative response to a sympathetic block excludes b. c. d. e. the diagnosis of CRPS Negative response to a somatic block excludes the diagnosis of CRPS Absence of findings consistent with sympathetic dysfunction during physical examination excludes the diagnosis of CRPS Normal findings on electromyography of the affected extremity exclude the diagnosis of CRPS Properly performed sympathetic block spares sensory and motor function