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complex regional pain syndrome type 1; reflex sympathetic dystrophy; RSD; hand shoulder syndrome; algodystrophy; algoneurodystrophy; Sudeck's atrophy; reflex neurovascular dystrophy

Components: 1) continuous severe burning pain with indistinct boundaries 2) sympathetic nervous system dysfunction 3) edema 4) movement disorder 5) dystrophy/atrophy Etiology: 1) nerve injury a) large nerve b) nociceptive C-fibers c) A-delta pain fibers d) a completely severed nerve does not cause RSD 2) injuries associated with RSD a) brachial plexus injuries b) carpal tunnel injury c) arthrosopic knee surgery d) fractures associated with nerve injury e) radiculopathy & soft tissue injury f) wrist injury in the elderly [5] 3) damage to blood vessel walls & nerves from: a) electrical injury b) ergotamine toxicity 4) vein stripping 5) post stroke in the elderly [5] Pathology: 1) process is sympathetically driven in early stages but becomes independent of sympathetic influence in later stages 2) may spread to entire body 3) may involve visceral autonomic nervous system 4) may involve bone 5) generalized capillary dysfunction 6) peripheral alpha-1 adrenergic receptor-mediated pain pathways may be invovled Clinical manifestations: 1) pain a) out of proportion to injury b) burning pain c) deep ache d) allodynia e) hyperalgesia 2) some patients may be hypoesthetic 3) edema 4) autonomic dysfunction a) warm, red, dry skin b) cool, pale, hyperhidrotic, cyanotic skin c) generalized capillary dysfunction in all fingers & toes rather than in nerve or dermatomal distribution 5) movement disorder a) dystonia b) weakness c) spasm d) hyperactive reflexes e) tremor 6) dystrophy/atrophy a) dystrophic nails b) muscle atrophy c) hair growth, then hair loss d) shiny atrophic skin e) tendon & cartilage degeneration f) bone resorption 7) spontaneous remission are common 8) chronic or relapsing courses are more common Diagnotic criteria: - Budapet crtiteria - continuing pain disproportionate to any apparent cause - at least one symptom in >= 3 categories in table below - at least one sign >= 2 categories in table below - no other diagnosis explains symptoms Category Sign & Symptoms Sensory hyperalgesia allodynia Vasomotor temperature asymmetry skin-color changes skin-color asymmetry Sudomotor/Edema edema sweating changes sweating asymmetry Motor/Trophic diminished range of motion motor dysfunction (weakness, tremor, dystonia) trophic changes (hair, nail, skin) Special laboratory: 1) thermography demonstrates sympathetic & C-fiber abnormalities 2) pain relief for sympathetically driven RSD with: a) sympathetic blockade - stellate ganglion block for upper extremity RSD - lumbar sympathetic block for lower extremity RSD b) intravenous phentolamine test (alpha-1 adrenergic receptor antagonist) Radiology: 1) bone scan (3 phase): a) increased periarticular uptake in 30% of patients b) asymmetry in flow in all 3 phases [5] 2) bone changes on X-ray in late-stage RSD: a) periarticular b) cystic Differential diagnosis: 1) thrombophlebitis 2) underlying soft tissue injury or fracture 3) ergotism Management: 1) physical therapy & to a lesser extent occupational therapy [6] 2) local heat or cooling packs [5] 3) oral agents a) glucocorticoids: prednisolone, 83% response rate [6] - GRS11 cites falls in the elderly as reason for prednisolone vs gabapentin [6] b) amitriptyline (Elavil) for sleep c) non-steroidal anti-inflammatory drugs (NSAIDs) for pain in early stages - diflusinal (Dolobid) 250 mg TID d) carbamazepine (Tegretol) 400 mg TID e) sympathetic nervous system antagonists 1] clonidine (Catapres) 0.1-1.2 mg BID, start 0.1 mg BID 2] prazosin 3] phenoxybenzamine e) dilantin 300 mg PO QD f) piroxicam, 17% response rate [6] g) gabapentin treatment of choice according to [7] 4) sympathetic blockade not effective [6] a) upper extremity: 5 superior ganglion blocks b) lower extremity: paravertebral sympathetic blocks c) both lower extremities: 3-5 day epidural block with 0.25% Marcaine d) guanethidine not effective [6] 5) electrical stimulation of the spinal cord can reduce pain & improve health-related quality of life in selected patients [8] 6) sympathetic-independent pain a) dorsal column stimulator b) nerve & dorsal column stimulator in refractory RSD in a single extremity 7) generalized RSD: morphine pump (generally 35 mg/day) 8) prophylaxis: - vitamin C 500 mg QD may prevent RSD after uncomplicated fracture [4] 9) prognosis: - if left untreated, may progress to contracture, deformity & permanent disability

Specific

lumbosacral plexopathy

General

complex regional pain syndrome

References

  1. Harrison's Principles of Internal Medicine, 13th ed. Companion Handbook, Isselbacher et al (eds), McGraw-Hill Inc. NY, 1995, pg 2
  2. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 1048-49
  3. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1957-58
  4. Journal Watch 20(2):17, 2000 Zollinger PE, Tuinebreijer WE, Kreis RW, Breederveld RS. Effect of vitamin C on frequency of reflex sympathetic dystrophy in wrist fractures: a randomised trial. Lancet. 1999 Dec 11;354(9195):2025-8. PMID: 10636366
  5. UCLA Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 12-15, 2001
  6. Geriatric Review Syllabus, 7th edition Parada JT et al (eds) American Geriatrics Society, 2010 - Geriatric Review Syllabus, 11th edition (GRS11) Harper GM, Lyons WL, Potter JF (eds) American Geriatrics Society, 2022
  7. NEJM Knowledge+ Internal Medicine Board Review. https://myknowledgeplus.nejm.org/flow/flowjs.htm
  8. Kemler MA et al. Spinal cord stimulation in patients with chronic reflex sympathetic dystrophy. N Engl J Med 2000 Aug 31; 343:618. PMID: 10965008 https://www.nejm.org/doi/full/10.1056/NEJM200008313430904
  9. Stanton-Hicks M et al. Reflex sympathetic dystrophy: Changing concepts and taxonomy. Pain 1995 Oct; 63:127. PMID: 8577483
  10. NEJM Knowledge+ Question of the Week. Aug 11, 2020 https://knowledgeplus.nejm.org/question-of-week/756/
  11. National Institute of Neurological Disorders and Stroke (NINDS) NINDS Complex Regional Pain Syndrome Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Complex-Regional-Pain-Syndrome-Information-Page