Primary Central Nervous System Vasculitis (PCNSV): A Case Report Emphasizing Diagnostic Precision and Therapeutic Approaches

Cureus. 2024 Nov 21;16(11):e74132. doi: 10.7759/cureus.74132. eCollection 2024 Nov.

Abstract

This report presents a case of primary central nervous system vasculitis (PCNSV), emphasizing the need for precise diagnosis and individualized treatment. PCNSV is a rare inflammatory condition confined to the central nervous system (CNS) that affects small- to medium-sized vessels and can cause severe neurological damage if left untreated. A 73-year-old woman with no previous medical history presented with rapidly progressive right-sided hemiparesis and cognitive impairment. Magnetic resonance imaging (MRI) findings revealed new hyperintense lesions on diffusion-weighted imaging (DWI), meningeal enhancement, and vascular wall thickening, raising suspicion of vasculitis. Cerebral angiography showed left middle cerebral artery (MCA) stenosis, and a brain biopsy confirmed perivascular lymphocytic infiltration, supporting the diagnosis of PCNSV. High-dose corticosteroids and azathioprine were administered to stabilize the symptoms. This case highlights the diagnostic value of MRI findings in PCNSV and the importance of histopathological confirmation and immunosuppressive therapy in managing this condition.

Keywords: central nervous system inflammation; magnetic resonance imaging; meningeal enhancement; primary central nervous system vasculitis; vessel wall imaging.

Publication types

  • Case Reports