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Pathology
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TERMINOLOGY
Abbreviations
- Complex regional pain syndrome (CRPS)
Definitions
- Painful disorder of extremities
- Spontaneous &/or evoked regional pain, usually disproportionate in degree to usual course of any trauma or other lesions
- May initially affect one limb and then spread throughout body
- Usually has distal predominance of abnormal sensory, motor, sudomotor, vasomotor, &/or trophic findings
- CRPS shows variable progression over time
- ~ 35% of affected patients report symptoms in whole body
- International Association for the Study of Pain (IASP) divides CRPS into 2 types
- CRPS type 1
- Formerly called reflex sympathetic dystrophy (RSD), Sudeck atrophy
- No detectable nerve damage in affected limb
- Secondary to injury/trauma
- Accounts for ~ 90% of CRPS
- Exact mechanisms unknown
- CRPS type 2
- Formerly called causalgia
- Detectable nerve damage in affected limb
- Exact mechanisms unknown
- Having both subtypes is possible
- CRPS type 1
- Another subtype classification
- Warm or hot CRPS
- ~ 70% of patients have this type
- Acute form
- Increased skin temperature at onset of symptoms, suggesting inflammatory cause
- Cold CRPS
- Indicative of more chronic CRPS
- Differentiated by decreased skin temperature
- Poor prognosis and poor clinical outcome
- Warm or hot CRPS
- Previously, 3 sequential clinical stages of CRPS were reported
- No longer widely used due to lack of evidence to support definite progression through these stages
- Stage 1: Pain, cold/touch intolerance, swelling
- Stage 2: Muscle wasting, soft tissue edema, brawny skin, ↑ pain
- Stage 3: ↓ range of motion, digit/joint contracture, waxy skin; brittle, ridged nails; ↓ pain
- No longer widely used due to lack of evidence to support definite progression through these stages
- Painful disorder of extremities
IMAGING
General Features
Nuclear Medicine Findings
Radiographic Findings
CT Findings
MR Findings
Electromyography and Nerve Conduction Studies
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Prevention
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Gupta PK et al: Complex regional pain syndrome-A forgotten entity. J Family Med Prim Care. 8(5):1778-80, 2019
- Howard BA et al: Utility of radionuclide bonescintigraphy in complex regional pain syndrome. Curr Pain Headache Rep. 22(1):7, 2018
- Narimatsu H et al: Bone SPECT/CT localizes increased bone metabolism and subsequent bone resorption in reflex sympathetic dystrophy. Clin Nucl Med. 42(10):784-6, 2017
- AlSharif A et al: Is there a correlation between symptoms and bone scintigraphic findings in patients with complex regional pain syndrome? Ann Nucl Med. 26(8):665-9, 2012
- Cappello ZJ et al: Meta-analysis of imaging techniques for the diagnosis of complex regional pain syndrome type I. J Hand Surg Am. 37(2):288-96, 2012
- Hsu W et al: Radionuclide imaging in the diagnosis and management of orthopaedic disease. J Am Acad Orthop Surg. 20(3):151-9, 2012
- Moon JY et al: Analysis of patterns of three-phase bone scintigraphy for patients with complex regional pain syndrome diagnosed using the proposed research criteria (the 'Budapest Criteria'). Br J Anaesth. 108(4):655-61, 2012
- Ringer R et al: Concordance of qualitative bone scintigraphy results with presence of clinical complex regional pain syndrome 1: meta-analysis of test accuracy studies. Eur J Pain. 16(10):1347-56, 2012
- Wüppenhorst N et al: Sensitivity and specificity of 3-phase bone scintigraphy in the diagnosis of complex regional pain syndrome of the upper extremity. Clin J Pain. 26(3):182-9, 2010
- Park SA et al: Patterns of three-phase bone scintigraphy according to the time course of complex regional pain syndrome type I after a stroke or traumatic brain injury. Clin Nucl Med. 34(11):773-6, 2009
- Park SG et al: Quantitative evaluation of very acute stage of complex regional pain syndrome after stroke using three-phase bone scintigraphy. Nucl Med Commun. 28(10):766-70, 2007
- Harden RN et al: Diagnosis of complex regional pain syndrome: signs, symptoms, and new empirically derived diagnostic criteria. Clin J Pain. 22(5):415-9, 2006
- Lee E et al: Role of radionuclide imaging in the orthopedic patient. Orthop Clin North Am. 37(3):485-501, viii, 2006
- Shehab D et al: Impact of three-phase bone scintigraphy on the diagnosis and treatment of complex regional pain syndrome type I or reflex sympathetic dystrophy. Med Princ Pract. 15(1):46-51, 2006
- Wilder RT: Management of pediatric patients with complex regional pain syndrome. Clin J Pain. 22(5):443-8, 2006
- Intenzo CM et al: The role of nuclear medicine in the evaluation of complex regional pain syndrome type I. Clin Nucl Med. 30(6):400-7, 2005
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Scanning Tips
TERMINOLOGY
Abbreviations
- Complex regional pain syndrome (CRPS)
Definitions
- Painful disorder of extremities
- Spontaneous &/or evoked regional pain, usually disproportionate in degree to usual course of any trauma or other lesions
- May initially affect one limb and then spread throughout body
- Usually has distal predominance of abnormal sensory, motor, sudomotor, vasomotor, &/or trophic findings
- CRPS shows variable progression over time
- ~ 35% of affected patients report symptoms in whole body
- International Association for the Study of Pain (IASP) divides CRPS into 2 types
- CRPS type 1
- Formerly called reflex sympathetic dystrophy (RSD), Sudeck atrophy
- No detectable nerve damage in affected limb
- Secondary to injury/trauma
- Accounts for ~ 90% of CRPS
- Exact mechanisms unknown
- CRPS type 2
- Formerly called causalgia
- Detectable nerve damage in affected limb
- Exact mechanisms unknown
- Having both subtypes is possible
- CRPS type 1
- Another subtype classification
- Warm or hot CRPS
- ~ 70% of patients have this type
- Acute form
- Increased skin temperature at onset of symptoms, suggesting inflammatory cause
- Cold CRPS
- Indicative of more chronic CRPS
- Differentiated by decreased skin temperature
- Poor prognosis and poor clinical outcome
- Warm or hot CRPS
- Previously, 3 sequential clinical stages of CRPS were reported
- No longer widely used due to lack of evidence to support definite progression through these stages
- Stage 1: Pain, cold/touch intolerance, swelling
- Stage 2: Muscle wasting, soft tissue edema, brawny skin, ↑ pain
- Stage 3: ↓ range of motion, digit/joint contracture, waxy skin; brittle, ridged nails; ↓ pain
- No longer widely used due to lack of evidence to support definite progression through these stages
- Painful disorder of extremities
IMAGING
General Features
Nuclear Medicine Findings
Radiographic Findings
CT Findings
MR Findings
Electromyography and Nerve Conduction Studies
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Prevention
DIAGNOSTIC CHECKLIST
Consider
Selected References
- Gupta PK et al: Complex regional pain syndrome-A forgotten entity. J Family Med Prim Care. 8(5):1778-80, 2019
- Howard BA et al: Utility of radionuclide bonescintigraphy in complex regional pain syndrome. Curr Pain Headache Rep. 22(1):7, 2018
- Narimatsu H et al: Bone SPECT/CT localizes increased bone metabolism and subsequent bone resorption in reflex sympathetic dystrophy. Clin Nucl Med. 42(10):784-6, 2017
- AlSharif A et al: Is there a correlation between symptoms and bone scintigraphic findings in patients with complex regional pain syndrome? Ann Nucl Med. 26(8):665-9, 2012
- Cappello ZJ et al: Meta-analysis of imaging techniques for the diagnosis of complex regional pain syndrome type I. J Hand Surg Am. 37(2):288-96, 2012
- Hsu W et al: Radionuclide imaging in the diagnosis and management of orthopaedic disease. J Am Acad Orthop Surg. 20(3):151-9, 2012
- Moon JY et al: Analysis of patterns of three-phase bone scintigraphy for patients with complex regional pain syndrome diagnosed using the proposed research criteria (the 'Budapest Criteria'). Br J Anaesth. 108(4):655-61, 2012
- Ringer R et al: Concordance of qualitative bone scintigraphy results with presence of clinical complex regional pain syndrome 1: meta-analysis of test accuracy studies. Eur J Pain. 16(10):1347-56, 2012
- Wüppenhorst N et al: Sensitivity and specificity of 3-phase bone scintigraphy in the diagnosis of complex regional pain syndrome of the upper extremity. Clin J Pain. 26(3):182-9, 2010
- Park SA et al: Patterns of three-phase bone scintigraphy according to the time course of complex regional pain syndrome type I after a stroke or traumatic brain injury. Clin Nucl Med. 34(11):773-6, 2009
- Park SG et al: Quantitative evaluation of very acute stage of complex regional pain syndrome after stroke using three-phase bone scintigraphy. Nucl Med Commun. 28(10):766-70, 2007
- Harden RN et al: Diagnosis of complex regional pain syndrome: signs, symptoms, and new empirically derived diagnostic criteria. Clin J Pain. 22(5):415-9, 2006
- Lee E et al: Role of radionuclide imaging in the orthopedic patient. Orthop Clin North Am. 37(3):485-501, viii, 2006
- Shehab D et al: Impact of three-phase bone scintigraphy on the diagnosis and treatment of complex regional pain syndrome type I or reflex sympathetic dystrophy. Med Princ Pract. 15(1):46-51, 2006
- Wilder RT: Management of pediatric patients with complex regional pain syndrome. Clin J Pain. 22(5):443-8, 2006
- Intenzo CM et al: The role of nuclear medicine in the evaluation of complex regional pain syndrome type I. Clin Nucl Med. 30(6):400-7, 2005
