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MR angiography vasculitis — SCE Neurology MCQ

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HardNeurological InvestigationsMR angiography vasculitisSCE Neurology

A patient with subacute multifocal deficits has inflammatory CSF and multiple small cortical infarcts. CTA is unrevealing. High-resolution vessel-wall MRI shows smooth concentric enhancement of several intracranial arteries. Which competing diagnosis is made less likely by this pattern and the non-thunderclap course?

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Correct answer: E — Primary angiography-negative small-vessel CNS vasculitis

The best answer is “Primary angiography-negative small-vessel CNS vasculitis”. RCVS usually presents with recurrent thunderclap headache, near-normal CSF and segmental vasoconstriction with little or no persistent concentric wall enhancement; the stated inflammatory pattern instead requires vasculitis and mimic evaluation. “Varicella-zoster virus-associated cerebral vasculopathy” is less appropriate because PACNS remains an important consideration with inflammatory CSF and concentric enhancement “Reversible cerebral vasoconstriction syndrome with thunderclap headache” is less appropriate because infectious vasculopathy must be excluded before immunosuppression “Neurosarcoidosis with secondary inflammatory vasculopathy” is less appropriate because granulomatous systemic or meningeal disease can produce an inflammatory vascular pattern “Intravascular lymphoma with secondary small-vessel injury” is less appropriate because malignancy is a recognised mimic and may require tissue diagnosis

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