The Bottom Line: Primary central nervous system vasculitis (PCNSV) is an uncommon condition in which lesions are limited to vessels of the brain and spinal cord. Because the clinical manifestations are not specific, the diagnosis is often difficult, and permanent disability and death are frequent outcomes.
References: Salvarani C, Brown RD Jr, Christianson, T. An update of the Mayo Clinic cohort of patients with adult primary central nervous system vasculitis: description of 163 patients. Medicine. 2015 May; 94(21):e738. doi:10.1097/MD.0000000000000738.
Salvarani C, Brown RD Jr, Calamia KT, et al. Rapidly progressive primary central nervous system vasculitis. Rhematology (Oxford). 2011 Feb; 50(2):349-358. doi:10.1093/rhematology/keq303.
Summary: PCNSV appears to consist of several subsets defined by the size of the vessels involved, the clinical characteristics at presentation, MRI findings, and histopathological patterns on biopsy. Rapidly progressive PCNSV appears to form a subset of PCNSV at the worst end of the clinical spectrum of this vasculitis, characterized by bilateral, multiple, large cerebral vessel lesions and multiple CNS infarctions. Early recognition and treatment may reduce poor outcomes.