Primary central nervous system vasculitis: an update

Primary CNS vasculitis (PCNSV) is uncommon, difficult to diagnose, and potentially fatal or at least likely to cause major and persistent neurological deficits, and yet appears highly treatable (although controlled clinical trials remain to be done). Here, we summarize recent advances in the recognition and diagnosis of PCNSV, the crucial role of biopsy and histopathological confirmation of the diagnosis, and currently recommended approaches to treatment. We also sign post areas of potentially fruitful future collaborative study that might advance this difficult disease.