J Korean Med Assoc.  2006 Aug;49(8):688-700. 10.5124/jkma.2006.49.8.688.

Diagnosis and Treatment of Complex Regional Pain Syndrome

• Moon DE

• KMID: 2184698
• DOI: http://doi.org/10.5124/jkma.2006.49.8.688

Abstract

The complex regional pain syndromes (CRPS I and CRPS II), also known as reflex sympathetic dystrophy and causalgia, have been recognized for the past 2,500 years. Despite its long history, the diagnosis and treatment of CRPS are still challenging. These syndromes can be characterized by discrete sensory, motor, and autonomic findings, but many patients with CRPS continue to suffer for years without the diagnosis. Although much progress has been made in the understanding of CRPS, many questions still remain unanswered. CRPS is probably a disease of the central nervous system. Yet, peripheral inflammatory processes, abnormal sympathetic- afferent coupling, and adrenoreceptor pathology may also be part of the picture. A close multidisciplinary approach amongst the pain medicine consultants, psychologist, physical and occupational therapists, and neurologist is necessary to achieve the maximum treatment outcomes. If conventional treatment (e.g. pharmacotherapy) fails to show a significant response within 12 weeks, an interventional technique such as spinal cord stimulation (SCS) needs to be tried. The current concepts of CRPS could be replaced by a new mechanism-based term or group of terms in the near future leading to improved clinical guidelines. This article reviews the different aspects of CRPS including its definition, classification, epidemiology and natural history, clinical presentation, pathophysiology and management.

Keyword

Complex regional pain syndrome; Reflex sympathetic dystrophy; Causalgia; Multidisciplinary approach; Spinal cord stimulation

MeSH Terms

Causalgia
Central Nervous System
Classification
Complex Regional Pain Syndromes
Consultants
Diagnosis*
Epidemiology
Humans
Natural History
Pathology
Psychology
Reflex Sympathetic Dystrophy
Spinal Cord Stimulation

Figure

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  Figure 1 Treatment algorithm. Proposed algorithm for the treatment of peripheral neuropathic pain TCA, tricyclic antidepressants; noradrenaline reuptake inhibitors. *Pain relieving effect of topical lidocaine has been shown in patients with allodynia.

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  Figure 2 Treatment paradigm for CRPS. A paradigm should be layered (in-parallel) in a interdisciplinary approach with the goal being functional recovery.

Reference

1. Mitchell SW. Injuries of the Nerves and Their Consequences. 1872. Philadelphia: JB Lippincott.

2. Evans J. Reflex sympathetic dystrophy. Surg Clin North Am. 1946. 26:780.

3. Stanton-Hicks M, Janig W, Hassenbusch S, Haddox JD, Boas R, Wilson P. Reflex sympathetic dystrophy: changing concepts and taxonomy. Pain. 1995. 63:127–133.

4. Taylor RS. Epidemiology of refractory neuropathic pain. Pain Practice. 2006. 6:22–26.

5. Quisel A, Gill JM, Witherell P. Complex regional pain syndrome underdiagnosed. J Fam Pract. 2005. 54:524–532.

6. Harden RN, Bruehl SP. Diagnosis of complex regional pain syndrome: signs, symptoms, and new empirically derived diagnostic criteria. Clin J Pain. 2006. 22:415–419.

7. Intenzo CM, Kim SM, Capuzzi DM. The role of nuclear medicine in the evaluation of complex regional pain syndrome type I. Clin Nucl Med. 2005. 30:400–407.

8. Oaklander AL, Rissmiller JG, Gelman LB, Zheng L, Chang Y, Gott R. Evidence of focal small-fiber axonal degeneration in complex regional pain syndrome-I (reflex sympathetic dystrophy). Pain. 2006. 120:235–243.

9. Albrecht PJ, Hines S, Eisenberg E, Pud D, Finlay DR, Connolly MK, et al. Pathologic alterations of cutaneous innervation and vasculature in affected limbs from patients with complex regional pain syndrome. Pain. 2006. 120:244–266.

10. Galer BS, Bruehl S, Harden RN. IASP diagnostic criteria for complex regional pain syndrome: a preliminary empirical validation study. International Association for the Study of Pain. Clin J Pain. 1998. 14:48–54.

11. Bruehl S, Harden RN, Galer BS, Saltz S, Bertram M, Backonja M, et al. External validation of IASP diagnostic criteria for complex regional pain syndrome and proposed research diagnostic criteria. Pain. 1999. 81:147–154.

12. Harden RN, Bruehl S, Galer BS, Saltz S, Bertram M, Backonja M, et al. Complex regional pain syndrome: Are the IASP diagnostic criteria valid and sufficiently comprehensive? Pain. 1999. 83:211–219.

13. Harden R, Bruehl S. Wilson PR, Stanton-Hicks M, Harden RN, editors. Diagnostic criteria: the statistical derivation of the four criterion factors. CRPS: Current Diagnosis and Therapy. 2005. Seattle: IASP Press;45–58.

14. Baron R, Janig W. Complex regional pain syndromes-how do we escape the diagnostic trap. Lancet. 2004. 364:1739–1741.

15. Janig W, Baron R. Complex regional pain syndrome: mystery explained? Lancet Neurol. 2003. 2:687–697.

16. Maihofner C, Handwerker HO, Birklein F. Functional imaging of allodynia in complex regional pain syndrome. Neurology. 2006. 66:711–717.

17. Raja SN, Grabow TS. Complex regional pain syndrome I (reflex sympathetic dystrophy). Anesthesiology. 2002. 96:1254–1260.

18. Bennett DS, Brookoff D. Complex regional pain syndromes (reflex sympathetic dystrophy and causalgia) and spinal cord stimulation. Pain Med. 2006. 7:S64–S96.

19. Maleki J, LeBel AA, Bennett GJ, Schwartzman RJ. Patterns of spread in complex regional pain syndrome type I (reflex sympathetic dystrophy). Pain. 2000. 88:259–266.

20. Stanton-Hicks MD, Burton AW, Bruehl SP, Carr DB, Harden RN, Hassenbusch SJ, et al. An updated interdisciplinary clinical pathway for CRPS: report of an expert panel. Pain Practice. 2002. 2:1–6.

21. Forouzanfar T, Koke AJ, van Kleef M, Weber WE. Treatment of complex regional pain syndrome type I. Eur J Pain. 2002. 6:105–122.

22. Kingery WS. A critical review of controlled clinical trials for peripheral neuropathic pain and complex regional pain syndromes. Pain. 1997. 73:123–139.

23. Rowbotham MC. Pharmacologic management of complex regional pain syndrome. Clin J Pain. 2006. 22:425–429.

24. Quisel A, Gill JM, Witherell P. Complex regional pain syndrome: which treatments show promise? J Fam Pract. 2005. 54:599–603.

25. Harden RN. Pharmacotherapy of complex regional pain syndrome. Am J Phys Med Rehabil. 2005. 84:S17–S28.

26. van de Vusse AC, Stomp-van den Berg SG, Kessels AH, Weber WE. Randomised controlled trial of gabapentin in Complex Regional Pain Syndrome type 1 [ISRCTN84121379]. BMC Neurol. 2004. 4:13.

27. Finnerup NB, Otto M, McQuay HJ, Jensen TS, Sindrup SH. Algorithm for neuropathic pain treatment: an evidence based proposal. Pain. 2005. 118:289–305.

28. Harden RN, Swan M, King A, Costa B, Barthel J. Treatment of complex regional pain syndrome: functional restoration. Clin J Pain. 2006. 22:420–424.

29. Bruehl S, Chung OY. Psychological and behavioral aspects of complex regional pain syndrome management. Clin J Pain. 2006. 22:430–437.

30. Bonica JJ. Bonica JJ, Loeser JD, Chapman CR, editors. Causalgia and other reflex sympathetic dystrophies. The Management of Pain. 1990. 1:2nd ed. Philadelphia: Lea & Febiger;220–243.

31. Nelson DV, Stacey BR. Interventional therapies in the management of complex regional pain syndrome. Clin J Pain. 2006. 22:438–442.

32. Kemler MA, Barendse GA, van Kleef M, de Vet HC, Rijks CP, Furnee CA, et al. Spinal cord stimulation in patients with chronic reflex sympathetic dystrophy. N Engl J Med. 2000. 343:618–624.

33. Kemler MA, De Vet HC, Barendse GA, Van Den Wildenberg FA, Van Kleef M. The effect of spinal cord stimulation in patients with chronic reflex sympathetic dystrophy: two years' follow-up of the randomized controlled trial. Ann Neurol. 2004. 55:13–18.

34. Stanton-Hicks M. Complex regional pain syndrome: manifestations and the role of neurostimulation in its management. J Pain Symptom Manage. 2006. 31:S20–S24.