#4-27E. The Diagnostic Dilemma: Navigating Primary CNS Vasculitis (JNeurol, 2026)

When faced with unexplained, diverse neurological symptoms, how do you confidently distinguish and diagnose primary central nervous system vasculitis (PCNSV) without resorting to risky empirical treatments? This episode explores the 2026 update on PCNSV, which comprehensively reviews the latest advances in recognizing, diagnosing, and managing this rare but highly treatable condition. The most critical takeaway is that cerebral angiography lacks sufficient accuracy—with sensitivity and specificity notably low—reaffirming that histopathological confirmation via brain biopsy remains the undisputed gold standard. Securing a definitive tissue diagnosis is vital to prevent the misidentification of severe mimics, such as infections or lymphomas, and to avoid initiating potentially hazardous immunosuppression unnecessarily. However, when critically appraising this field, clinicians must recognize a significant limitation: there is a complete absence of randomized controlled trials specifically for PCNSV, meaning current treatments are heavily extrapolated from systemic vasculitis data and expert consensus. For frontline physicians, this paper is highly relevant as it challenges the overreliance on neuroimaging, advocating for early biopsy to ensure accurate diagnosis and the safe, tailored management of patients with complex neuroinflammatory presentations.

Citation: Rice C, Scolding N. Primary central nervous system vasculitis: an update. Journal of Neurology. 2026. DOI: 10.1007/s00415-026-13727-y

Disclaimer: This audio summary is based on personal interpretation and does not guarantee the exact content of the original paper. Please refer to the original article for details.