LAUSR

Isolated vasculitis of the central nervous system (CNS) is an extremely rare condition affecting approximately 2.4 patients per one million patient years, as described in a US study.[1] It is described commonly as primary angiitis of the CNS (PACNS) or primary CNS vasculitis . Although PACNS can occur at any age and in both sexes, the incidence is usually higher in males in their 50s.[1,2] Until the introduction of the diagnostic criteria,[3] PACNS has very rarely been described and was probably under-diagnosed and rarely reported. Large case series are very rarely published except from a handful of centers.