eMedicine Specialties > Neurology > Headache and Pain

Reflex Sympathetic Dystrophy: Differential Diagnoses & Workup

Author: Eugenia-Daniela Hord, MD, Instructor, Departments of Anesthesia and Neurology, Massachusetts General Hospital Pain Center, Harvard Medical School
Coauthor(s): Sajjad Mueed, MD, Consulting Staff, Department of Medicine, Division of Neurology, Carle Clinic Association
Contributor Information and Disclosures

Updated: Apr 18, 2006

Differential Diagnoses

Alcohol (Ethanol) Related Neuropathy
Thoracic Outlet Syndrome
Diabetic Neuropathy
Toxic Neuropathy
Lyme Disease
Traumatic Peripheral Nerve Lesions
Median Neuropathy
Ulnar Neuropathy
Neurosarcoidosis
Nutritional Neuropathy
Peroneal Mononeuropathy

Other Problems to Be Considered

Causalgia
Chronic arterial insufficiency
Hysterical conversion
Irritative lesions of peripheral nerve
Malingering
Neurotic compulsive postures
Peripheral nerve entrapment
Peripheral nerve neuroma
Phlebothrombosis
Plexopathies
Polyneuropathies
Raynaud disease
Rheumatologic disease
Soft-tissue infection

Workup

Laboratory Studies

  • No specific blood workup is indicated for RSD/CRPS type 1. The following tests are used for routine screening to rule out infections or rheumatologic disease:
    • CBC
    • Complement fixation
    • Erythrocyte sedimentation rate
    • Antinuclear antibody
    • Rheumatoid factor

Imaging Studies

  • Plain radiographs show patchy demineralization of the epiphyses of long bones and also of the bones of the hands and feet. These findings cannot be appreciated until the syndrome is advanced. The sensitivity and specificity have been reported to be 69% and 71%, respectively.
  • Three-phase bone scanning enables evaluation of total extremity perfusion, which typically is increased in stage 1, normalized in stage 2, and decreased in stage 3. The sensitivity and specificity are reported to be 60% and 86%, respectively.

Other Tests

  • Thermography has a debatable value in the evaluation of RSD/CRPS type 1.
  • EMG/NCS can help differentiate early phases of CRPS type 1 and type 2.
  • Quantitative sudomotor axon reflex test demonstrates abnormal function of sudomotor reflex loops in RSD. Abnormal findings on this test are highly predictive of a positive response to sympathetic blocks.
  • Laser Doppler flowmetry may demonstrate changes in the skin blood flow.

Histologic Findings

Skeletal muscles demonstrate (1) decrease in type 1 fibers, (2) increase in lipofuscin pigment, (3) atrophic fibers with a slight angular appearance, and (4) severely thickened basal membrane layers of the capillaries.

Peripheral nerve: Efferent nerve fibers are unaffected histologically; from afferent fibers, only C-fibers have demonstrated histopathologic abnormalities consisting of denervation of Schwann cell stacks, miniature axon sprouts, and an obvious increase of collagen pockets. Loss of C-fibers can be noted as well. Sometimes, multiple basal membranes surround the small endoneural vessels.

More on Reflex Sympathetic Dystrophy

Overview: Reflex Sympathetic Dystrophy
Differential Diagnoses & Workup: Reflex Sympathetic Dystrophy
Treatment & Medication: Reflex Sympathetic Dystrophy
Follow-up: Reflex Sympathetic Dystrophy
References
[ CLOSE WINDOW ]

References

  1. Allen G, Galer BS, Schwartz L. Epidemiology of complex regional pain syndrome: a retrospective chart review of 134 patients. Pain. Apr 1999;80(3):539-44. [Medline].

  2. Baron R, Levine JD, Fields HL. Causalgia and reflex sympathetic dystrophy: does the sympathetic nervous system contribute to the generation of pain?. Muscle Nerve. Jun 1999;22(6):678-95. [Medline].

  3. Bruehl S, Harden RN, Galer BS. External validation of IASP diagnostic criteria for Complex Regional Pain Syndrome and proposed research diagnostic criteria. International Association for the Study of Pain. Pain. May 1999;81(1-2):147-54. [Medline].

  4. Evans JA. Reflex sympathetic dystrophy. Surg Gynecol Obstet. 1946;82:36-44.

  5. Galer BS, Jensen M. Neglect-like symptoms in complex regional pain syndrome: results of a self-administered survey. J Pain Symptom Manage. Sep 1999;18(3):213-7. [Medline].

  6. Geertzen JH, Dijkstra PU, van Sonderen EL. Relationship between impairments, disability and handicap in reflex sympathetic dystrophy patients: a long term followup study. Clin Rehabil. 1998;12:402-412.

  7. Harden RN, Bruehl S, Galer BS. Complex regional pain syndrome: are the IASP diagnostic criteria valid and sufficiently comprehensive?. Pain. Nov 1999;83(2):211-9. [Medline].

  8. Kemler MA, van de Vusse AC, van den Berg-Loonen EM. HLA-DQ1 associated with reflex sympathetic dystrophy. Neurology. Oct 12 1999;53(6):1350-1. [Medline].

  9. Kingery WS. A critical review of controlled clinical trials for peripheral neuropathic pain and complex regional pain syndromes. Pain. Nov 1997;73(2):123-39. [Medline].

  10. Kurvers HA. Reflex sympathetic dystrophy: facts and hypotheses. Vasc Med. 1998;3(3):207-14. [Medline].

  11. Merskey H, Bogduk N. Classification of Chronic Pain: Descriptions of Chronic Pain Syndromes and Definitions of Pain Terms. Seattle, Wash: IASP Press. 1994.

  12. Ochoa JL. Truths, errors, and lies around "reflex sympathetic dystrophy" and "complex regional pain syndrome". J Neurol. Oct 1999;246(10):875-9. [Medline].

  13. Oerlemans HM, Oostendorp RA, de Boo T. Pain and reduced mobility in complex regional pain syndrome I: outcome of a prospective randomised controlled clinical trial of adjuvant physical therapy versus occupational therapy. Pain. Oct 1999;83(1):77-83. [Medline].

  14. Oerlemans HM, Oostendorp RA, de Boo T. Signs and symptoms in complex regional pain syndrome type I/reflex sympathetic dystrophy: judgment of the physician versus objective measurement. Clin J Pain. Sep 1999;15(3):224-32. [Medline].

  15. Pappagallo M. Complex regional pain syndromes. Neurology Reviews. 2000;25-29.

  16. Portenoy RK. Neuropathic Pain. Pain Management: Theory and Practice. Philadelphia: FA Davis Company;1996:83-125.

  17. Rommel O, Gehling M, Dertwinkel R. Hemisensory impairment in patients with complex regional pain syndrome. Pain. Mar 1999;80(1-2):95-101. [Medline].

  18. Schurmann M, Gradl G, Andress HJ. Assessment of peripheral sympathetic nervous function for diagnosing early post-traumatic complex regional pain syndrome type I. Pain. Mar 1999;80(1-2):149-59. [Medline].

  19. Thimineur MA, Saberski L. Complex regional pain syndrome type I (RSD) or peripheral mononeuropathy? A discussion of three cases. Clin J Pain. Jun 1996;12(2):145-50. [Medline].

  20. Veldman PH, Reynen HM, Arntz IE. Signs and symptoms of reflex sympathetic dystrophy: prospective study of 829 patients. Lancet. Oct 23 1993;342(8878):1012-6. [Medline].

  21. Zyluk A. The natural history of post-traumatic reflex sympathetic dystrophy. J Hand Surg [Br]. Feb 1998;23(1):20-3. [Medline].

  22. van der Laan L, ter Laak HJ, Gabreels-Festen A. Complex regional pain syndrome type I (RSD): pathology of skeletal muscle and peripheral nerve. Neurology. Jul 1998;51(1):20-5. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

acute peripheral trophoneurosis, algodystrophy, causalgia, chronic traumatic edema, mimocausalgia, neurovascular posttraumatic painful syndrome, neurovascular reflex dystrophy, neurovascular reflex sympathetic dystrophy, posttraumatic chronic edema, posttraumatic osteoporosis, posttraumatic pain syndrome, posttraumatic sympathetic dystrophy, RSD, shoulder-hand syndrome, spreading neuralgia, Sudeck atrophy, sympathalgia, thermalgia, traumatic angiospasm, traumatic vasospasm, complex regional pain syndrome type 1

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Eugenia-Daniela Hord, MD, Instructor, Departments of Anesthesia and Neurology, Massachusetts General Hospital Pain Center, Harvard Medical School
Eugenia-Daniela Hord, MD is a member of the following medical societies: American Academy of Neurology and American Pain Society
Disclosure: Nothing to disclose.

Coauthor(s)

Sajjad Mueed, MD, Consulting Staff, Department of Medicine, Division of Neurology, Carle Clinic Association
Sajjad Mueed, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Epilepsy Society, and American Society of Neuroimaging
Disclosure: Nothing to disclose.

Medical Editor

Jorge E Mendizabal, MD, Consulting Staff, Corpus Christi Neurology
Jorge E Mendizabal, MD is a member of the following medical societies: American Academy of Neurology, American Headache Society, National Stroke Association, and Stroke Council of the American Heart Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, Barnes Jewish Hospital
Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.