[Primary central nervous system vasculitis imitating a brain tumour--a case report].
[Primary central nervous system vasculitis imitating a brain tumour--a case report].
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类似脑肿瘤的原发性中枢神经系统血管炎一例报告[J].
DOI：
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发表时间：
2008
影响因子：
2.9
通讯作者：
M. Harat
中科院分区：
文献类型：
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作者：
M. Wiszniewska;T. Szylberg;M. Harat
原发性中枢神经系统血管炎（PCNSV）是一种罕见的疾病，年发病率为每100万人年2.4例。PCNSV引起以头痛以及意识和精神障碍为主的各种神经系统症状。这种疾病有时在CT上表现为脑瘤，很少表现为中风。PCNSV的诊断是困难的，通常需要进行脑活检，其中通常发现涉及小脑膜或实质血管的跨壁血管炎症。血管造影改变显示血管的不规则运动和特征性的节段性狭窄，但有时血管造影是正常的。作者报告了一名57岁的男性，其脑活检诊断为PCNSV。患者接受皮质类固醇脉冲治疗18个月，效果良好，持续2.5年。停止糖皮质激素治疗一年后，症状再次出现，尽管糖皮质激素与环磷酰胺联合治疗，患者仍死亡。
Primary central nervous system vasculitis (PCNSV) is a rare disease with an annual incidence rate of 2.4 cases per 1,000,000 person-years. PCNSV causes various neurological symptoms dominated by headache as well as consciousness and mental disturbances. The disease sometimes imitates a brain tumour on CT and rarely presents as stroke. The diagnosis of PCNSV is difficult and frequently requires a brain biopsy in which transmural vascular inflammation involving leptomeningeal or parenchymal vessels is typically found. Angiographic changes indicating an irregular course of vessels with characteristic segmental narrowing can be observed but sometimes the angiogram is normal. The authors present a 57-year-old man in whom PCNSV was diagnosed in brain biopsy. The patient was treated with corticosteroid pulses for 18 months with good effect lasting for 2.5 years. After one year of ending glucocorticoid therapy the symptoms had occurred again and in spite of combined therapy of glucocorticoids with cyclophosphamide the patient died.