Central Nervous System Vasculitis

To date, CNS vasculitis (CNSV) may also be classified as primary (PCNSV) or secondary (SCNSV) to systemic diseases, based on the size of the vessels involved, by MRI findings, and by histopathology based on brain biopsy. The updated literature points to advancements in radiographic modalities (MRI, vessel wall MRI, angiography with digital subtraction) that further aid in the diagnosis of CNSV and the differentiation between CNSV and its mimickers. However, the newer modalities cannot distinguish between primary and secondary CNSV so extra-CNS systemic findings, and lab tests are required. Cerebrospinal fluid analysis and serologic tests have not yielded more specific results than in the past. MRI-guided biopsy is more specific than a blind biopsy for positive results. Treatment options are limited and include steroids and cyclophosphamide. Occasionally, rituximab, IVIG, and others may be beneficial. We discuss the updated medical literature of the different entities and emphasize the utilization of more than one modality to confirm the proper diagnosis.