Complex Regional Pain Syndrome (CRPS)

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Original Editors - Katelyn Koeninger & Kristen Storrie  from Bellarmine University's Pathophysiology of Complex Patient Problems project.

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Definition/Description[edit | edit source]

Complex Regional Pain Syndrome (CRPS) is also known as reflex sympathetic dystrophy, causalgia, Sudeck's atrophy, algoneurodystrophy, among other names. It is a disease causing severe pain, disproportional to the expected amount of pain from a stimulus.[1] It is typically confined in one limb, but may spread to other limbs or even to the entire body. A person with CRPS will experience sensory, motor, autonomic, and skin/bone changes.[2]
There are two types of CRPS. CRPS type I occurs after any type of trauma. CRPS type II may also occur after trauma, but has neuronal involvement. CRPS most commonly occurs after surgery (including arthroscopies), upper and lower motor neuron injuries, traumatic brain injury, cerebrovascular accident, central nervous system lesion, neuropathies, or nerve entrapments.[1]

Prevalence[edit | edit source]

CPRS affects approximately 26 out of every 100,000 people. It is more common in females than males, with a ratio of 3.5:1.[2] CRPS can affect people of all ages, including children as young as 3 years old and adults as old as 75 years old, but typically is most prevalent beginning in the mid-thirties. CRPS type I occurs after 5% of all traumatic injuries.[1] 91% of all CRPS cases occur after surgery.[3]

Characteristics/Clinical Presentation[edit | edit source]

Signs and Symptoms of CRPS:
• pain, not present in 7%[1] [2]
• swelling[1][2]
• Tremor[1]
• trouble initiating movements[1]
• muscle spasms[1]
• muscle atrophy[1]
• temperature changes[1][2]
• color changes (red, blue)[1]
• thick, brittle, or rigid nails[1]
• weakness[1][2]
• thin, shiny, clammy skin[2]
• stiffness or decreased joint motion[2]
• painful or decreased sensation on skin (some patients report intolerance to air moving over skin)[2]
• strange, disfigured, or dislocated feelings in limbs[2]


Characteristics[1]:
• sensory impairments
• movement disorders, typically in contralateral extremity
• ANS dysfunction
• Dystrophy
• Atrophy
• Spreads proximally, to other extremities, and possibly the entire body
• Similar presentation to osteoporosis on radiographic images

Associated Co-morbidities[edit | edit source]

CRPS may also be associated with:
• Depression/Anxiety
• Migraines[2]
• Osteoporosis[2]
• Asthma[2]
• ACE-inhibitor therapy[2]

Medications[edit | edit source]

Possible treatments for CRPS include:

  • A stellate ganglion block, or sympathectomy, blocks the nerve pathways causing pain. This may be most beneficial in the early stages of CRPS.[1][2]
  • Oral pain-relieving medications including corticosteroids and NSAIDs, as well as acupuncture provide effective pain relief in approximately 20% of those with CRPS, but this is supported by weak evidence.[1]
  • Treatments may be geared to helping patients manage symptoms. Amitriptyline relieves depression and acts as a sleeping aid. Calcium channel blockers can help to improve circulation through SNS effect. Intrathecal baclofen, among other measures, improves motor dystonia.[1]
  • Pain intensity and perception of pain is sometimes relieved through use of an implanted transcutaneous electrical nerve stimulation (TENS) unit.[1]
  • A randomized double dummy controlled, double blind trial compared the effectiveness of Dimethylsulfoxide 50% (DMSO) and N-acetylcysteine (NAC) in treating CRPS type I. There were no significant differences between the two treatments, but are both successful treating CRPS type I. This study showed that DMSO-treatment is more favorable for warm CRPS whereas NAC is more favorable for cold CRPS[4]
  • Low doses of ketamine infusion has been shown to decrease pain in patients with CRPS type I who had been unsuccessful with other conservative methods of management. Ketamine blocks central sensitization by effecting the N-methyl-D-aspartate receptor which has been shown to be effected in CRPS[5]

Diagnostic Tests/Lab Tests/Lab Values[edit | edit source]

Diagnosis of CRPS is based solely on examination and patient history.[1] A triple-phase bone scan is the best method to rule out type I CPRS.[6] According to Cappello, the triple-phase bone scan has the best sensitivity, NPV, and PPV compared to MRI and plain film radiographs.[6] Radiographic examinations, laser Doppler flowmetry, and thermographic studies may be utilized to assess the secondary issues and symptoms of CRPS.[1]

Etiology/Causes[edit | edit source]

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The cycle of CRPS

Systemic Involvement[edit | edit source]

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Medical Management (current best evidence)[edit | edit source]

  • Spinal cord stimulation was more effective than conventional medical management in reducing pain in patients with CRPS type I[7]
  • Spinal cord stimulation was shown to be effective in completely eliminating pain in adolescent females 2-6 weeks after stimulation[8]
  • Use of surgical and chemical sympathectomy show moderate improvement in pain scores in patients with CRPS. There were no significant differences found between the surgical and chemical groups when comparing pain scores from day one to four months. More high quality research needs to be done before recommending this as a first line of defense.[9]
  • High frequency repetitive transcranial magnetic stimulation on the motor cortex in addition to pharmacological management was effective in reducing pain. This was demonstrated by the scores on the McGill Pain Questionnaire and Short Form-36 which include different aspects of pain such as sensory-discriminative and emotional-affective.[10]

Physical Therapy Management (current best evidence)[edit | edit source]

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Alternative/Holistic Management (current best evidence)[edit | edit source]

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Differential Diagnosis[edit | edit source]

CRPS needs to be differentiated from the following diagnosis[3]:

• Bony or soft tissue injury
• Neuropathic pain
• Arthritis
• Infection
• Compartment syndrome
• Arterial insufficiency
• Raynaud’s Disease
• Lymphatic or venous obstruction
• Thoracic outlet syndrome
• Gardner-Diamond Syndrome
• Erythromelalgia
• Self-harm or malingering

  Case Reports/ Case Studies[edit | edit source]

[11]




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Resources
[edit | edit source]

• Reflex Sympathetic Dystrophy Syndrome Association

• American Chronic Pain Association

• Mayo Clinic

• National Institute of Neurological Disorders and Stroke

Recent Related Research (from Pubmed)[edit | edit source]

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References[edit | edit source]

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  1. ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 1.14 1.15 1.16 1.17 1.18 1.19 Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. Saint Louis: Saunders Elsevier, 2009.
  2. ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 2.13 2.14 Goebel A. Complex regional pain syndrome in adults. Rheumatology. 2011;50;288-6.
  3. ↑ 3.0 3.1 Turner-Stokes L, Goebel A. Complex regional pain syndrome in adults: concise guidance. Clinical Med 2011; 11(6):596-600.
  4. ↑ Perez R, Zuurmond W, Bezemer P, Kuik D, vanLoenen A, deLange J, et al. The treatment of complex regional pain syndrome type I with free radical scavengers: a randomized controlled study. Pain 2003;102(3):297-307.
  5. ↑ Goldberg M, Domsky R, Scaringe D, Hirsh R, Dotson J, Sharaf I, et al. Multi-Day Low Dose Ketamine Infusion for the Treatment of Complex Regional Pain Syndrome. Pain Physician 2005;8:175-179.
  6. ↑ 6.0 6.1 Cappello Z, Kasdan M, Louis D. Meta-analysis of imaging techniques for the diagnosis of complex regional pain syndrome type I. JHS 2012;37A:288-296.
  7. ↑ Simpson E, Duenas A, Holmes M, Papaloannou D, Chilcott J. Spinal cord stimulation for chronic pain of neuropathic or ischaemic origin: systematic review and economic evaluation. Health Technology Assessment 2009;13(17):1-179.
  8. ↑ Olson GL, Meyerson BA, Linderoth B. Spinal cord stimulation in adolescents with complex regional pain syndrome type I. EUR J PAIN 2008;12(1):53-59.
  9. ↑ Straube S, Derry S, Moore RA, McQuay HJ. Cervico-thoracic or lumbar sympathectomy for neuropathic pain and complex regional pain syndrome. Cochrane Database of Systematic Reviews 2010;7:1-14.
  10. ↑ Picarelli H, Teixeira M, deAndrade D, Myzkowski M, Luvisotto T, Yeng L, et al. Repetitive Transcranial Magnetic Stimulation Is Efficacious as an Add-On to Pharmacological Therapy in Complex Regional Pain Syndrome Type I. J Pain 2010;11(11):1203-10.
  11. ↑ CNN. CNN Report on Reflex Sympathetic Dystrophy. Available from: http://www.youtube.com/watch?v=jaTlI6bfF64 [last accessed 3/28/12]
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