原发性中枢神经系统脉管炎是一种少见的仅限于中枢神经组织内的血管炎症病变,届于肉芽肿性小血管炎,临床特点为中枢神经功能障碍。1959年 Cravoto等第1次把本病作为一独立的疾病报道。随着脑血管造影术的出现和发展,报道的病例也增多,但各报道中对本病的命名不一,如脑肉芽肿性血管炎,中枢神经系统肉芽肿性血管炎,单纯性中枢神经系统脉管 Primary central nervous system vasculitis is a rare vascular inflammation within the central nervous system lesions, the granulomatous vasculitis, the clinical features of central nervous system dysfunction. For the first time in 1959, Cravoto reported the disease as an independent disease. With the appearance and development of cerebral angiography, the reported cases also increased, but the reports of the disease of different names, such as brain granulomatous vasculitis, central nervous system granulomatous vasculitis, simple central nervous system System vasculature