Chapter 23 - Central Nervous System Vasculitis

Abstract

Inflammation of arteries supplying the central nervous system is one of the rare causes of ischemic and hemorrhagic stroke. Diagnosis of CNS vasculitis is challenging and often delayed due to slow onset of nonspecific symptoms prior to sudden presentation with stroke, seizures, or altered mental status. Diagnostic testing is imperfect and often requires a sequence of supportive studies, typically including at least one invasive test: lumbar puncture, catheter angiography, and/or brain biopsy. Treatment for CNS vasculitis carries its own challenges in terms of monitoring for disease control and drug toxicities or infections related to immunosuppression. This chapter reviews common presentations, diagnostic strategies, common mimics, and treatment for CNS vasculitis.