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原发性中枢神经系统血管炎(PCNSV):一例强调诊断准确性和治疗方法的病例报告

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

作者信息

Tokue Hiroyuki, Tokue Azusa, Takahashi Issei, Tagami Noriyuki, Tsushima Yoshito

机构信息

Department of Diagnostic Radiology and Nuclear Medicine, Graduate School of Medicine, Gunma University, Maebashi, JPN.

出版信息

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.

摘要

本报告介绍了一例原发性中枢神经系统血管炎(PCNSV)病例,强调了精确诊断和个体化治疗的必要性。PCNSV是一种罕见的仅限于中枢神经系统(CNS)的炎症性疾病,可累及中小血管,若不治疗可导致严重的神经损伤。一名既往无病史的73岁女性出现快速进展的右侧偏瘫和认知障碍。磁共振成像(MRI)结果显示,弥散加权成像(DWI)上出现新的高信号病变、脑膜强化和血管壁增厚,这引发了对血管炎的怀疑。脑血管造影显示左侧大脑中动脉(MCA)狭窄,脑活检证实血管周围淋巴细胞浸润,支持PCNSV的诊断。给予大剂量皮质类固醇和硫唑嘌呤以稳定症状。该病例突出了MRI结果在PCNSV中的诊断价值,以及组织病理学确诊和免疫抑制治疗在管理这种疾病中的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/51f3/11663028/b49b2d2f3321/cureus-0016-00000074132-i01.jpg

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