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Cholesterol emboli neuropathy

Departments of Neurology (Drs. Bendixen and Younger), Pathology (Drs. Hair and Gutierrez), and Medicine (Drs. Meyers, Homma, and Jaffe). Columbia-Presbyterian Medical Center, New York, NY.

We report the clinical and pathologic features of a patient with peripheral neuropathy that was the first clinical expression of cholesterol emboli syndrome (CES). Biopsy of skeletal muscle and peripheral nerve revealed cholesterol clefts in lumens of small arteries, necrotizing arteritis, and severe degeneration of peripheral and intramuscular nerves. At autopsy, the peripheral nervous system was extensively affected by similar changes. We conclude that (1) peripheral neuropathy may be the initial manifestation of CES. Presumably, deposition of cholesterol leads to arteritis. (2) The underlying pathology of CES neuropathy is chronic axonal degeneration, possibly due to chronic ischemia of epineurial arteries. (3) Muscle biopsy is important in the antemortem diagnosis of CES. Nerve biopsy may show involvement of epineurial vessels. (4) CES may resemble polyarteritis nodosa clinically and pathologically. (5) CES may be under-recognized and should be included in the differential diagnosis of any neuropathy of uncertain cause, particularly when there is a history of vascular catheterization, or severe aortic atherosclerosis.

Address correspondence and reprint requests to Dr. David S. Younger, Columbia-Presbyterian Medical Center, 710 West 168th Street. New York, NY 10032–3784.

Supported by the Center Grants from the Muscular Dystrophy Association, the National Institutes of Health (NS-11766), and the General Clinical Research Center (RR-00645).

Received May 9, 1991. Accepted for publication in final form July 18, 1991.

This article has been cited by other articles:

				M. Hauben, J. Norwich, E. Shapiro, L. Reich, K. S. Petchel, and D. Goldsmith Multiple Cholesterol Emboli Syndrome--Six Cases Identified Through the Spontaneous Reporting System Angiology, September 1, 1995; 46(9): 779 - 784. [Abstract] [PDF]