Rapidly Progressive Primary Central Nervous System Vasculitis: A Rare Case with Multifocal Small and Medium Vessel Involvement

Abstract

Introduction: Primary central nervous system vasculitis (primary CNS vasculitis) is a rare inflammatory disorder that affects small-to-medium-sized cerebral vessels, often leading to recurrent strokes. Diagnosis is vague due to non-specific neurological symptoms. Imaging findings, cerebrospinal fluid (CSF) analysis and exclusion of systemic vasculitis are essential for diagnosis. Early immunosuppressive therapy is crucial, but the prognosis remains unpredictable.

Case description: A 45-year-old female with diabetes, hypertension and multiple ischaemic strokes presented with altered consciousness due to hypoglycaemia. Initial workup at a different hospital suggested cerebral vasculitis based on the findings of cerebral angiography. She had negative systemic vasculitis markers and was treated with corticosteroids and rituximab and discharged. On admission to our facility, an MRI of the brain revealed multiple infarcts of varying ages, and a CTA showed arterial beading, suggestive of primary CNS vasculitis. CSF analysis demonstrated elevated protein and IgG without pleocytosis. Despite high-dose corticosteroids and rituximab, she developed progressive neurological deterioration with new infarcts in the vertebrobasilar territory, leading to brainstem dysfunction and brain death.

Conclusion: Primary CNS vasculitis remains a diagnostic and therapeutic challenge because of its diverse presentation and similarities with other vascular conditions. This case emphasises the importance of early recognition, comprehensive diagnostic evaluation and aggressive treatment to prevent irreversible neurological decline. Disease progression can be rapid even with treatment, highlighting the need for further research to enhance early detection and optimise management strategies.