Central nervous system vasculitis occurs in a variety of clinical sett
ings. Some exhibit a distinct age preference; others a tissue tropism.
Most frequently encountered are giant cell arteritis (temporal arteri
tis) and vasculitis secondary to infections. The central nervous syste
m may be involved in the antineutrophil cytoplasmic antibody-associate
d systemic vasculitides and occasionally neurologic abnormalities appe
ar as a presenting manifestation of disease. Isolated angiitis of the
central nervous system, a rare form of vasculitis that is restricted t
o the central nervous system, must be distinguished from other causes
of central nervous system inflammation and from noninflammatory vascul
ar disease. We are learning a great deal about the cellular mechanisms
of vascular inflammation in general. Some manifestations of the clini
cal disease result from histologic features of the infiltrate and the
size of affected vessel. However, the local consequences of inflammati
on, such as increased coagulation and altered vasomotor tone, as well
as the systemic consequences, such as activation of the central noradr
energic systems, trigeminovascular system, and hypothalamic pituitary
adrenal axis, contribute both to pathogenesis of disease and to recove
ry. Curr Opin Neurol 11:241-246. (C) 1998 Lippincott-Raven Publishers.