原发性中枢神经系统血管炎1例报告并文献复习

Primary angiitis of the central nervous system (PACNS):report of 1 case and review of the literature

目的提高对原发性中枢神经系统血管炎(PACNS)的认识。方法临床诊治、随访1例有病理证实的PACNS病例,并复习有关文献。结果PACNS临床表现复杂多样,辅助检查缺乏特异性,没有统一的诊断标准,激素和环磷酰胺治疗有效。结论当患者出现中枢神经系统症状不能为其他疾病解释时,要通过脑脊液、影像学检查及脑活检综合判断有无PACNS的可能。

Objective To improve the understanding of PACNS by describing a case. Methods A case of PACNS confirmed by pathology was treated and followed. The relevant literature was reviewed. Results The clinical manifestations and laboratory parameters of PACNS were highly variable. There was no consensus on diagnostic criteria. Treatment with glucocorticoid and cyclophosphamide was effective. Conclusions PACNS should be considered in all patients with CNS symptoms which cannot be explained by other diseases. The diagnosis should be made by cerebrnspinal fluid examination, imaging techniques and brain biopsy.