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Primary CNS vasculitis — SCE Neurology MCQ

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HardStrokePrimary CNS vasculitisSCE Neurology

A 46-year-old has recurrent headaches, cognitive change and multifocal cerebral infarcts. Extensive infectious, embolic and systemic inflammatory evaluation is negative. Catheter angiography shows multifocal narrowing, but reversible vasoconstriction remains a differential. Before committing her to prolonged cyclophosphamide, which investigation can establish definite primary CNS vasculitis?

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Correct answer: D — Targeted brain and leptomeningeal biopsy

Angiographic beading, inflammatory CSF and vessel-wall enhancement can support the diagnosis, but none is sufficiently specific to establish definite primary CNS vasculitis. Mimics include RCVS, atherosclerosis, infection and malignancy. Tissue demonstrating vascular inflammation is required for a definite diagnosis; biopsy should be targeted to an abnormal, safely accessible region and include leptomeninges when feasible. A negative biopsy does not absolutely exclude patchy disease, but it can also reveal important mimics. The potential toxicity of prolonged immunosuppression makes the distinction between possible and tissue-proven disease clinically important.

Reference: The diagnosis of primary central nervous system vasculitis: https://pn.bmj.com/content/20/2/109