TY - JOUR
T1 - Nonsystemic vasculitic neuropathy
AU - Dyck, Peter James
AU - Benstead, Timothy J.
AU - Conn, Doyt L.
AU - Stevens, J. Clarke
AU - Windebank, Anthony J.
AU - Low, Phillip A.
N1 - Funding Information:
The expert help of Karen Oviatt in the preparation of the manuscript is gratefully acknowledged. This investigation was supported in part by a Peripheral Neuropathy Clinical Center Grant from the National Institutes of Health (NS-14304), by a Center Grant from the Muscular Dystrophy Association, and by Mayo, Borchard, Upton, and Whirlpool Funds.
PY - 1987/8
Y1 - 1987/8
N2 - 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).
AB - 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).
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U2 - 10.1093/brain/110.4.843
DO - 10.1093/brain/110.4.843
M3 - Article
C2 - 3651797
AN - SCOPUS:0023181387
SN - 0006-8950
VL - 110
SP - 843
EP - 853
JO - Brain
JF - Brain
IS - 4
ER -