表现为复发性视神经炎的肿块样原发性中枢神经系统血管炎一例报道并文献复习

目的加强对肿块样原发性中枢神经系统血管炎(tl-PACNS)的认识。方法回顾性分析1例经病理证实的tl-PACNS患者的临床、影像学、病理表现以及治疗和预后情况。结果患者临床表现为复发性视神经炎,头MRI表现为肿块样长T1、长T2病灶,术后病理表现为软脑膜和小血管壁上淋巴细胞和浆细胞浸润,对糖皮质激素(激素)单用或联合免疫抑制剂治疗有效。结论 tl-PACNS临床表现复杂多样,影像学上极易误诊为肿瘤,脑活检是其确诊的主要依据。tl-PACNS对激素和免疫抑制剂治疗效果好。

Tumor-like primary angiitis of the central nervous system presented with relapsing optic neuritis: One case report and literature review

Objective To improve the understanding of tumor-like primary angiitis of the central nervous system(tl-PACNS) by reporting a case. Methods The clinical manifestations,neuroimaging and neuropathological features as well as therapeutical and prognostic profiles were analyzed retrospectively. Results The patient presented with relapsing optic neuritis.Brain MRI showed a mass-like lesion,which was hypointense on T1 and hyperintense on T2.Lymphocyte and plasmocyte infiltrating in small arteries in brain and leptomeningeal was the main finding in postoperative pathology.Treatment with steroids alone or steroids combined with immunosuppressive agents was effective. Conclusions tl-PACNS is easily misdiagnosed to neoplasm with highly variable clinical features,diagnosis is confirmed by cerebral biopsy.Steroids and immunosuppressive agents are recommended.