Primary angiitis and angiopathy of the central nervous system and their relationship to systemic giant cell arteritis.

Abstract Three cases of primary angiitis of the central nervous system were compared with previously published cases. Most cases occurred in older adults, were diagnosed histologically, and had a male-female ratio of 2:1. Angiographically identified cases, demonstrating angiopathy but not necessarily vasculitis as might be seen in histologically identified cases, were found in younger adults and showed a male-female ratio of 1:2. The autopsy cases had some similarities to systemic giant cell arteritis, including many cases with vasculitis in large branches of the circle of Willis and foci of systemic vasculitis. Vasculitides in arteries of different sizes may result from different responses to a variety of antigens or immune complexes rather than represent different disease processes. If causative immune mechanisms can be identified, primary angiitis of the central nervous system may become classified immunologically rather than histopathologically.