原发性中枢性神经系统血管炎研究进展

The research progress of primary angiitis of central nervous system

原发性中枢神经系统血管炎(primary angiitis of central nervous system,PACNS)是一种少见的仅累及中枢神经系统的疾病,临床表现多样,主要临床特点表现为头痛、认知功能障碍、癫痫发作及神经功能缺损。血生化、免疫指标、脑脊液及影像学检查均缺乏特异性,目前针对病变部位脑实质及脑膜组织活检是诊断PACNS的"金标准"。在治疗方面,不同类型的PACNS对糖皮质激素及免疫抑制剂的敏感性差别较大。作者对PACNS的临床特点及分类、影像学及病理特点进行综述。

Primary angiitis of central nervous system is a rare disease that only affects central nervous system with diversity of clinical manifestations. The main clinical features are headache,seizures,cognitive dysfunction and neural defect. Blood biochemistry,immune parameters,cerebrospinal fluid and imaging studies are lack of specificity. Parenchymal and meningeal biopsy is the 'gold standard'in the diagnosis of the disease. In terms of treatment,different types of PACNS have different sensitivity to glucocorticoid and immune suppressants. In this paper,clinical features and classification,imaging and pathological features of PACNS are summarized.