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Complex Regional Pain Syndromes: Differential Diagnoses & Workup

Author: Manish K Singh, MD, Assistant Professor, Department of Neurology, Teaching Faculty for Pain Management and Neurology Residency Program, Hahnemann University Hospital, Drexel College of Medicine; Medical Director, Neurology and Pain Management, Jersey Institute of Neuroscience
Coauthor(s): Jashvant Patel, MD, Medical Director, Department of Pain Medicine and Comprehensive Rehabilitation, Medical College of Pennsylvania Hahnemann University; John Grothusen, PhD, Director of Quantitative Sensory and Autonomic Nervous System Laboratory, Assistant Professor, Department of Neurology, MCP Hahnemann University; Patrick M Foye, MD, FAAPMR, FAAEM, Associate Professor of Physical Medicine and Rehabilitation, Co-Director of Musculoskeletal Fellowship, Co-Director of Back Pain Clinic, Director of Coccyx Pain Service (Tailbone Pain Service: www.TailboneDoctor.com), University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Contributor Information and Disclosures

Updated: Sep 22, 2009

Differential Diagnoses

[Injury]
Mononeuritis Multiplex
[Lumbar Degenerative Disc Disease]
Neoplastic Brachial Plexopathy
Achilles Tendon Injuries and Tendonitis
Neoplastic Lumbosacral Plexopathy
Adhesive Capsulitis
Posterior Cruciate Ligament Injury
Ankle Sprain
Postpolio Syndrome
Anterior Cruciate Ligament Injury
Radiation-Induced Brachial Plexopathy
Brachial Neuritis
Radiation-Induced Lumbosacral Plexopathy
Chronic Pain Syndrome
Rotator Cuff Disease
Compartment Syndrome
Spasticity
Diabetic Lumbosacral Plexopathy
Spinal Stenosis and Neurogenic Claudication
Diabetic Neuropathy
Thoracic Outlet Syndrome
Iliotibial Band Syndrome
Traumatic Brachial Plexopathy
Ischemic Monomelic Neuropathy
Medial Collateral and Lateral Collateral Ligament Injury
Meniscal Injury

Other Problems to Be Considered

Erythromelalgia is a rare and poorly understood clinical syndrome characterized by hot, red, painful extremities. The condition commonly affects the lower extremities. The application of cold or elevation of the extremity may provide pain relief.

The syndrome is classified either as primary (idiopathic) erythromelalgia or as secondary erythromelalgia, which is commonly associated with myeloproliferative, syndrome-related thrombocythemia. Adults with erythromelalgia may get relief with a daily dose of aspirin.

Workup

Laboratory Studies

  • No diagnostic criteria have been accepted uniformly for RSD, and no single special investigation has been proven sensitive and specific enough for diagnosing RSD. No criterion standard exists for making this clinical diagnosis, although some tests and findings may contribute to the diagnosis or help to diagnose or exclude other possible medical conditions.
  • As required, routine and specific blood tests, as well as other studies, should be performed to identify precipitating causes. The exact tests vary according to the body region involved, as well as according to the findings related to the history and physical examination.

Imaging Studies

  • Radiographic films may show patchy peri-articular demineralization within 3-6 weeks. The extent of osteoporosis is more than expected from disuse alone, and it is a common abnormality revealed on radiographs.
  • Three-phase bone scan:
    • A 3-phase bone scan may be helpful in revealing findings typical for the diagnosis of RSD and in excluding other conditions that could cause the patient's symptoms. A false-negative bone scan is fairly common.
    • The 3-phase bone scan often is considered sensitive and specific, particularly in the early phase (<20 weeks) of the syndrome, but a study by Werner and colleagues reported that the 3-phase bone scan showed a diagnostic sensitivity of only 44%.9
    • According to Kozin and co-authors, scintigraphic abnormalities were reported in up to 60% of RSD patients and may be useful in arriving at the diagnosis of RSD, as well as in predicting which patients are likely to respond to systemic steroid therapy.10
    • Abnormally increased activity must be diffuse, not focal.
    • The most suggestive and sensitive findings on bone scan include diffuse increased activity, with juxta-articular accentuation uptake on the delayed images (phase 3).
    • Phases 1 and 2 are less sensitive and specific for RSD.
  • Imaging studies have shown to not be reliable screening tests in the differentiation between normal posttraumatic changes and those changes seen in CRPS due to a low positive predictive value (17-60%) and a moderate negative predictive value (79-86%).11

Other Tests

  • Skin temperatures - This measurement is simple, but important, to record during examination of the patient with RSD. Skin temperature is measured by (1) tactile perception, (2) surface thermistors, and (3) hand-held infrared thermometers:
    • Vascular changes in RSD
      • Hyperemic phase - Increase in the temperature of the skin early in the course of RSD
      • Cold limb - Decrease in the temperature of the skin later in the course of RSD
    • Thermography - This test demonstrates limb temperature differences quantitatively, but it is nonspecific. Bruehl and colleagues noted that thermography may be useful in situations in which sensitivity and specificity are equally important; an asymmetry cutoff of 0.6 º C appears optimal.12 If specificity is more important, a cutoff of 0.8 º C or 1.0 º C may be considered.
  • Sudomotor function testing - Through the autonomic testing of 396 patients with pain, Chelminsky and colleagues demonstrated that abnormalities in resting sweat output, in resting skin temperature, and in a quantitative sudomotor axon reflex test predicted the diagnosis of CRPS I with 98% specificity13 :
    • Sweat test - The sympathetic skin response (SSR) provides useful information on sudomotor dysfunction in patients with RSD; however, it is not possible yet to determine the final value of SSR for the diagnosis of RSD.
    • Quantitative sudomotor axon reflex test (QSART)
    • Chemical sweat test - This uses agents such as ninhydrin, cobalt blue, or starch iodine.
    • Testing sweat output - In QSART, the stimulated sweat output is greater and is prolonged when sympathetic hyperfunction is present.
  • Electrodiagnostic studies:
    • Results of electromyography (EMG) and nerve conduction studies (NCS) typically are within the reference range in RSD. In fact, if the EMG and nerve studies identify a nerve lesion, the condition is not by definition CRPS I but may instead be CRPS II.
    • Single-fiber EMG examination also shows no definite abnormalities.
    • The electrodiagnostic studies may be normal because C-fiber abnormalities cannot be well detected.
    • Patients with allodynia (demonstrating, for example, extreme pain even when clothing touches the involved limb or when a breeze blows across it) may have a difficult time tolerating EMG and NCS.
  • Quantitative sensory testing:
    • The purpose of quantitative sensory testing (QST) is to quantify perception thresholds objectively.
    • QST uses very precise, reproducible stimuli, allowing comparison of symptomatic areas with asymptomatic areas, comparison with age-matched and sex-matched controls, and changes with time or treatment. This provides the physician with information about the severity and progression of the sensory dysfunction.
    • The standard QST involves determination of vibrotactile detection thresholds (an Ab-fiber – mediated sensation), cool detection thresholds (an Ad-fiber – mediated sensation), and warm thermal thresholds (a C-fiber – mediated sensation) in appropriate areas. Heat and cold pain thresholds also are obtained with the patient's permission and with the patient controlling the amount of stimulus applied.
  • Laser Doppler imaging (see images below and Images 5-7):
    • Laser Doppler imaging, with appropriate stressors, provides a simple, fast, noninvasive, and painless method for the study of segmental autonomic function. This type of imaging study provides excellent spatial information, eliminates many sources of artifact, and can be used to rapidly and repeatedly test skin autonomic reflexes bilaterally.
    • Along with baseline images, mild stressors (inspiratory gasp, cold pressor positional dependency) are used to quantify skin vasoconstrictor reflexes (see Image 5). The first author has found these methods to be especially useful in helping to distinguish between sympathetically mediated and sympathetically independent pain conditions.
Normal laser Doppler study of the upper extremiti...

Normal laser Doppler study of the upper extremities. When the patient performs inspiratory gasp repeatedly during laser Doppler image acquisition, the transient capillary flow decreases are displayed easily and dramatically (as dark bands) in the pseudocolor image.

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Normal laser Doppler study of the upper extremiti...

Normal laser Doppler study of the upper extremities. When the patient performs inspiratory gasp repeatedly during laser Doppler image acquisition, the transient capillary flow decreases are displayed easily and dramatically (as dark bands) in the pseudocolor image.


Laser Doppler study of the upper extremities in a...

Laser Doppler study of the upper extremities in a patient with right hand reflex sympathetic dystrophy.

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Laser Doppler study of the upper extremities in a...

Laser Doppler study of the upper extremities in a patient with right hand reflex sympathetic dystrophy.


Laser Doppler study of the lower extremities in a...

Laser Doppler study of the lower extremities in a 25-year-old woman with reflex sympathetic dystrophy in the right foot.

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Laser Doppler study of the lower extremities in a...

Laser Doppler study of the lower extremities in a 25-year-old woman with reflex sympathetic dystrophy in the right foot.

  • Diagnostic sympathetic ganglion block:
    • The IASP consensus group did not recognize response to sympathetic ganglion block as part of the diagnostic criteria for CRPS, since such responses, while often dramatic and impressive, are not universal.
    • Further, response to such blocks is more indicative of sympathetically maintained pain, which includes other etiologies in addition to CRPS.

More on Complex Regional Pain Syndromes

Overview: Complex Regional Pain Syndromes
Differential Diagnoses & Workup: Complex Regional Pain Syndromes
Treatment & Medication: Complex Regional Pain Syndromes
Follow-up: Complex Regional Pain Syndromes
Multimedia: Complex Regional Pain Syndromes
References
Further Reading
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Keywords

complex regional pain syndrome, CRPS, RSD, regional pain syndrome, reflex sympathetic dystrophy, reflex sympathetic dystrophy syndrome, RSD symptoms, RSD CRPS, RSD pain, RSD treatment, causalgia, complex regional pain syndrome I, CRPS I, complex regional pain syndrome II, CRPS II, mimo-causalgia, Sudeck atrophy of bone, shoulder-hand syndrome, algoneurodystrophy, reflex dystrophy, reflex neurovascular dystrophy

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Contributor Information and Disclosures

Author

Manish K Singh, MD, Assistant Professor, Department of Neurology, Teaching Faculty for Pain Management and Neurology Residency Program, Hahnemann University Hospital, Drexel College of Medicine; Medical Director, Neurology and Pain Management, Jersey Institute of Neuroscience
Manish K Singh, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American Association of Physicians of Indian Origin, American Headache Society, American Medical Association, and American Society of Regional Anesthesia and Pain Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Jashvant Patel, MD, Medical Director, Department of Pain Medicine and Comprehensive Rehabilitation, Medical College of Pennsylvania Hahnemann University
Jashvant Patel, MD is a member of the following medical societies: Alberta Medical Association, American Academy of Pain Medicine, American Academy of Physical Medicine and Rehabilitation, American Medical Association, American Society of Regional Anesthesia and Pain Medicine, and Medical Society of the State of New York
Disclosure: Nothing to disclose.

John Grothusen, PhD, Director of Quantitative Sensory and Autonomic Nervous System Laboratory, Assistant Professor, Department of Neurology, MCP Hahnemann University
Disclosure: Nothing to disclose.

Patrick M Foye, MD, FAAPMR, FAAEM, Associate Professor of Physical Medicine and Rehabilitation, Co-Director of Musculoskeletal Fellowship, Co-Director of Back Pain Clinic, Director of Coccyx Pain Service (Tailbone Pain Service: www.TailboneDoctor.com), University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Patrick M Foye, MD, FAAPMR, FAAEM is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, Association of Academic Physiatrists, and International Spine Intervention Society
Disclosure: Nothing to disclose.

Medical Editor

Robert J Kaplan, MD, James E Van Zandt VA Medical Center, Staff Physician, Department of Rehabilitation Medicine
Robert J Kaplan, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, Association of Academic Physiatrists, and Physiatric Association of Spine, Sports and Occupational Rehabilitation
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Patrick M Foye, MD, FAAPMR, FAAEM, Associate Professor of Physical Medicine and Rehabilitation, Co-Director of Musculoskeletal Fellowship, Co-Director of Back Pain Clinic, Director of Coccyx Pain Service (Tailbone Pain Service: www.TailboneDoctor.com), University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Patrick M Foye, MD, FAAPMR, FAAEM is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, Association of Academic Physiatrists, and International Spine Intervention Society
Disclosure: Nothing to disclose.

CME Editor

Kelly L Allen, MD, Regional Medical Director, IMX-Medical Management Services
Disclosure: Nothing to disclose.

Chief Editor

Rene Cailliet, MD, Professor-Chairman Emeritus, Department of Rehabilitation Medicine, University of Southern California School of Medicine; Former Director, Department of Rehabilitation Medicine, Santa Monica Hospital Medical Center
Rene Cailliet, MD is a member of the following medical societies: American Academy of Pain Medicine, American Academy of Physical Medicine and Rehabilitation, American Pain Society, Association of American Medical Colleges, International Association for the Study of Pain, and Pan American Medical Association
Disclosure: Nothing to disclose.

 
 
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