ABSTRACT
Vasculitis refers to the inflammation of blood vessels with or without vessel wall necrosis. Both the central nervous system (CNS) and peripheral nervous system (PNS) may be involved. This type of involvement is considered as primary nervous system vasculitis, when it is restricted to the nervous system. However, the clinical behaviour and neuroimaging, as well as the histopathology, are not uniform in all such cases. This variation is likely to point to a spectrum, depending on the type and extent of the vascular involvement within the CNS, covering a group of disorders, rather than a single disease, which may explain the different prognosis and response to treatments.
