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Brain, Vol. 110, No. 4, 843-853, 1987
© 1987 Guarantors of Brain

research-article

NONSYSTEMIC VASCULITIC NEUROPATHY

PETER JAMES DYCK, TIMOTHY J. BENSTEAD, DOYT L. CONN, J. CLARKE STEVENS, ANTHONY J. WINDEBANK and PHILLIP A. LOW

The Peripheral Neuropathy Research Laboratory, the Division of Rheumatology and Internal Medicine, the Department of Neurology, and the Peripheral Nerve Center Research Laboratory, Mayo Clinic and Mayo Foundation Rochester, Minnesota, USA

Correspondence to: Correspondence to: Dr P. J. Dyck, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA.

Among 65 patients with necrotizing vasculitis, 45 had systemic and 20 had nonsystemic vascuhtic neuropathy. In nonsystemic vasculitic neuropathy, clinically only nerves are affected; there are no, or few, constitutional symptoms or serological abnormalities. The clinical and pathological features are those of an ischaemic neuropathy caused by a necrotizing vasculitis of small arterioles. These 20 patients had neuropathic symptoms for a median time of 11.5 yrs (range 1–35 yrs). The clinical pattern of neuropathy was that of multiple mononeuropathy in 13, asymmetric neuropathy in 4, distal polyneuropathy in 3, and sensory polyneuropathy in 1. As compared with their initial evaluation, 8 are now worse, 5 are better, 4 are approximately the same, and 3 are dead from unrelated causes. Prednisone was thought to prevent the development of new lesions in some cases. By contrast, of the 41 patients with systemic necrotizing vasculitis whose outcome is known, 12 are dead (median time, 1.5 yrs, range 3 months-8yrs) and 29 are alive (median time, 6 yrs, range 6 months-22 yrs).

Received July 8, 1986. Revised September 11, 1986. Accepted September 25, 1986.


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