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

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.

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Publication Details

Journal
Cureus
Published
2024-11-21
DOI
https://doi.org/10.7759/cureus.74132
Primary Topic
Vasculitis and related conditions
Type
article
Field-Weighted Citation Impact
0.00
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article

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

Azusa Tokue, Yoshito Tsushima, I. Takahashi, Hiroyuki Tokue et al.
Cureus
Vasculitis and related conditions
article

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

Azusa Tokue, Yoshito Tsushima, I. Takahashi, Hiroyuki Tokue, Noriyuki Tagami
article en

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.

CureusVol. 16(11)
Gunma University (JP)
Good health and well-being
Openalex Percentile: Top 60%
Vasculitis and related conditions
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