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NEUROLOGY 1998;51:20-25
© 1998 American Academy of Neurology

Complex regional pain syndrome type I (RSD)

Pathology of skeletal muscle and peripheral nerve

L. van der Laan, MD, H. J. ter Laak, PhD, A. Gabreëls-Festen, PhD, F. Gabreëls, PhD and R.J.A. Goris, PhD

From the Departments of Surgery (Drs. van der Laan and Goris) and Neurology (Drs. ter Laak, Gabreëls-Festen, and Gabreëls), University Hospital Nijmegen, Nijmegen, The Netherlands.

Address correspondence and reprint requests to Dr. L. van der Laan, Department of Surgery, University Hospital Nijmegen, PO Box 9101, 6500 HB Nijmegen, The Netherlands.

Background: Reflex sympathetic dystrophy (RSD) (recently reclassified as complex regional pain syndrome type I) is a syndrome occurring in extremities and, when chronic, results in severe disability and untractable pain. RSD may be accompanied by neurologic symptoms even when there is no previous neurologic lesion. There is no consensus as to the pathogenic mechanism involved in RSD. To gain insight into the pathophysiology of RSD, we studied histopathology of skeletal muscle and peripheral nerve from patients with chronic RSD in a lower extremity.

Methods: In eight patients with chronic RSD, an above-the-knee amputation was performed because of a nonfunctional limb. Specimens of sural nerves, tibial nerves, common peroneal nerves, gastrocnemius muscles, and soleus muscles were obtained from the amputated legs and analyzed by light and electron microscopy.

Results: In all patients, the affected leg showed similar neurologic symptoms such as spontaneous pain, hyperpathy, allodynia, paresis, and anesthesia dolorosa. The nerves showed no consistent abnormalities of myelinated fibers. In four patients, the C-fibers showed electron microscopic pathology. In all patients, the gastrocnemius and soleus muscle specimens showed a decrease of type I fibers, an increase of lipofuscin pigment, atrophic fibers, and severely thickened basal membrane layers of the capillaries.

Conclusion: In chronic RSD, efferent nerve fibers were histologically unaffected; from afferent fibers, only C-fibers showed histopathologic abnormalities. Skeletal muscle showed a variety of histopathologic findings, which are similar to the histologic abnormalities found in muscles of patients with diabetes.


Received December 11, 1997. Accepted in final form March 28, 1998.




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