School of Medicine, Tohoku Medical and Pharmaceutical University, Japan.
Division of Neurology, Tohoku Medical and Pharmaceutical University, Japan.
Intern Med. 2024 Jul 1;63(13):1939-1943. doi: 10.2169/internalmedicine.2793-23. Epub 2023 Nov 27.
A 79-year-old woman presented with difficulty walking and disturbance of consciousness. Magnetic resonance imaging revealed diffuse white matter lesions and abnormal signals along the surface of the brain and sulci. A brain biopsy revealed granulomatous vasculitis with eosinophil infiltration. There was no peripheral blood eosinophilia or evidence of angiitis in other organs, and primary angiitis of the central nervous system (PACNS) with pathological findings of eosinophilic granulomatosis with polyangiitis (EGPA) was diagnosed. Steroids and other immunosuppressant therapies showed only limited effects. PACNS with pathological findings of EGPA is extremely rare, and a prompt brain biopsy is necessary for a diagnosis.
一位 79 岁女性因行走困难和意识障碍就诊。磁共振成像显示弥漫性脑白质病变和脑表面及脑沟异常信号。脑活检显示肉芽肿性血管炎伴嗜酸性粒细胞浸润。外周血嗜酸性粒细胞增多或其他器官血管炎证据，诊断为中枢神经系统原发性血管炎（PACNS），病理表现为嗜酸性肉芽肿性多血管炎（EGPA）。类固醇和其他免疫抑制剂治疗仅显示有限效果。PACNS 病理表现为 EGPA 极为罕见，及时进行脑活检有助于诊断。
