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Reversible Cerebral Vasoconstriction Syndrome — SCE Neurology MCQ

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HardCerebrovascular DiseaseReversible Cerebral Vasoconstriction SyndromeSCE Neurology

A 46-year-old has progressive cognitive decline, seizures and multifocal white-matter lesions over four months. CSF shows lymphocytes and raised protein. Catheter angiography is normal, but vessel-wall MRI shows patchy enhancement of small cortical vessels. Infection, malignancy and systemic vasculitis have been excluded. What investigation offers the strongest diagnostic evidence?

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Correct answer: A — Targeted brain and leptomeningeal biopsy of an active lesion

The best answer is “Targeted brain and leptomeningeal biopsy of an active lesion”. Small-vessel primary CNS vasculitis can be angiography-negative; when uncertainty remains and treatment is high risk, a targeted biopsy that includes cortex and leptomeninges provides the most specific evidence and can reveal mimics. “Repeat catheter angiography as the definitive small-vessel exclusion test” is less appropriate because angiography lacks sensitivity for disease below its spatial resolution “Empirical cyclophosphamide after non-invasive inflammatory assessment” is less appropriate because toxic long-term immunosuppression should not substitute for diagnostic reassessment when biopsy is feasible “Temporal-artery biopsy for possible systemic granulomatous arteritis” is less appropriate because temporal artery tissue does not sample the affected intracranial small vessels “Serum inflammatory markers as confirmation of CNS-restricted vasculitis” is less appropriate because normal or raised systemic markers neither establish nor exclude CNS-restricted vasculitis

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