Park Jeong-Ho, Jung Myung-Keun, Bang Cha-Ok, Park Hyung-Kook, Sung Ki-Bum, Ahn Moo-Young, Bae Won-Kyeong, Chi Je G
Department of Neurology, College of Medicine, Soonchunhyang University Hospital, Chunan, Korea.
J Korean Med Sci. 2002 Oct;17(5):718-22. doi: 10.3346/jkms.2002.17.5.718.
We report a rare case of neuro-Behcet's disease (NBD) presenting as an inflammatory pseudotumor in the brain. A 52-year-old woman was evaluated for subacute dizziness and headache. Brain magnetic resonance (MR) imaging showed a right cerebellar mass, which disappeared 2 weeks later. After a year, recurrent mucocutaneous manifestations of Beh et's disease were observed. Immunosuppressant and steroid maintenance treatment were started. She experienced two more neurologic attacks and brain MR imaging revealed an enhancing mass in the right temporal lobe. The second attack showed a good response to steroid pulse therapy, but the third attack did not respond to steroid and her neurologic signs suggested an impending transtentorial hernia. The right temporal lobectomy was performed for the purpose of life-saving. The pathologic finding of the mass was a chronic inflammatory vasculitis, compatible with NBD.
我们报告了一例罕见的以脑部炎性假瘤形式出现的神经白塞病(NBD)。一名52岁女性因亚急性头晕和头痛接受评估。脑部磁共振(MR)成像显示右侧小脑有一个肿块,两周后消失。一年后,观察到白塞病复发性黏膜皮肤表现。开始使用免疫抑制剂和类固醇维持治疗。她又经历了两次神经系统发作,脑部MR成像显示右侧颞叶有一个强化肿块。第二次发作对类固醇脉冲疗法反应良好,但第三次发作对类固醇无反应,且她的神经体征提示即将发生小脑幕切迹疝。为挽救生命进行了右侧颞叶切除术。肿块的病理发现为慢性炎症性血管炎,符合神经白塞病。