类固醇激素反应性慢性淋巴细胞炎症伴脑桥血管周围强化症的临床进展  

Recent advance in chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids

作　　者：李攀星 高远[1] 宋波[1] 许予明[1] 李玉生[1] 

机构地区：[1]郑州大学第一附属医院神经内科,郑州450052

出　　处：《中华神经医学杂志》2016年第12期1294-1296,共3页Chinese Journal of Neuromedicine

摘　　要：类固醇激素反应性慢性淋巴细胞炎症伴脑桥血管周围强化症（CLIPPERS）是新定义的罕见的中枢神经系统炎症性疾病。共济失调、构音障碍和复视是其最常见的临床症状，脑干和小脑为其常见受累部位，组织病理学常表现为小血管周围T淋巴细胞为主的炎性细胞浸润，“胡椒粉样”增强病灶是其特征性影像学表现．大剂量的激素冲击治疗可获得快速的临床及影像学的缓解，而过早停用激素或减量过快易导致临床症状及影像学的复发。Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a newly defined inflammatory central nervous system disorder. Ataxia, dysphasia, and diplopia are the most common clinical symptoms. The radiological distribution is focused on the brain stem and cerebellum; the perivascular and T cell predominant inflammatory cell infiltrates in the affected lesions. Rapid clinical and radiological improvements of symptoms were observed with the use of glucocorticosteroid based immunosuppression, while any attempts to withdrawal or taper GCS below a particular lower dose limit may provoke the recurrence of clinical or radiological symptoms.

关 键 词：脑干 脑炎 磁共振成像 类固醇激素 淋巴细胞 

分 类 号：R743[医药卫生—神经病学与精神病学]