ABSTRACT

Among the many fonns of organ-limited vasculitis, that affecting the central nervous system (CNS) remains one of the most challenging from a clinical and pathophysiological perspective. Reasons for this include (1) lack of specificity of signs and symptoms, (2) limited accessibility of the end-organ for pathological examination, (3) lack of efficient noninvasive diagnostic tests, and (4) the relative rarity of different fonns of CNS vasculitis. Despite these limitations, considerable progress has been made over the past decade in our understanding of these conditions.