类固醇激素反应性慢性淋巴细胞性炎性反应伴脑桥血管周围强化症1例并文献复习  

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids:a case report and review of the literature

作　　者：张维雄[1] 黄丽娜[1] 高秀菊[1] 孙聚保[1] 

机构地区：[1]河南科技大学第一附属医院神经内科,471000

出　　处：《中国神经免疫学和神经病学杂志》2015年第5期347-351,共5页Chinese Journal of Neuroimmunology and Neurology

摘　　要：目的探讨类固醇激素反应性慢性淋巴细胞性炎性反应伴脑桥血管周围强化症(chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids,CLIPPERS)临床表现、影像学特点以及发病的可能机制。方法回顾分析1例CLIPPERS患者的临床表现、影像学特点,并结合文献进行复习。结果该例CLIPPERS患者主要临床表现为头晕、头痛、吞咽困难、口周及左侧手指麻木、视物不清、复视、四肢乏力。头颅MRI检查显示脑桥、双侧基底节区斑片状长T1长T2信号,FLARIR像呈高信号,呈小点片状强化,DWI示双侧基底节区异常信号。经糖皮质激素治疗后患者症状明显改善。复查MRI显示脑桥及双侧基底节区增强信号消失,但双侧基底节区病变仍呈长T1长T2信号。结论 CLIPPERS除典型临床和MR表现外,还可能出现双侧基底节区脑梗死,诊断时需排除中枢神经系统血管炎、淋巴瘤样肉芽肿、结缔组织病等具有类似影像学表现的疾病。该病发病机制可能由免疫和炎性反应介导所致。Objective To investigate the pathogenesis, clinical manifestations and imaging features of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids （CLIPPERS）. Methods The clinical manifestations, imaging features and possible pathogenesis of one patient with CLIPPERS were collected, and the related literatures were reviewed. Results A 50-year-old female presented with dizziness, headache, dysphagia, perioral and left fingers numbness, blurred vision, diplopia, and limb weakness. Magnetic resonance imaging （MRI） showed pons, bilateral basal ganglia with T1WI, T2WI, FLARIR hyperintensity lesions and a characteristic pattern of punctate enhancement extending from the brainstem to the bilateral posterior limbs of the internal capsule. Patchy lesions were observed in the bilateral posterior limb of the internal capsule on diffusion-weighted images （DWI）. After treatment with methylprednisolone, the patient＇ s symptoms improved and a follow up MRI revealed no remaining punctate Gd-enhancing lesions in the brainstem or the bilateral posterior limbs of the internal capsule, but the bilateral basal ganglia still had T1WI, T2WI hyperintensity lesions. Conclusions Except for typical clinical manifestations and the previously described MR findings, CLIPPERS also demonstrated bilateral basal ganglia lacunar infarcts. The diagnosis must rule out primary central nervous system vasculitis, lymphomatoid granulomatosis, connective tissue disease with central nervous system involvement and other diseases with similar imaging characteristics. The disease may be caused by immune and inflammatory mediators.

关 键 词：脑桥 血管周围强化 糖皮质激素类 脑梗死 

分 类 号：R742.89[医药卫生—神经病学与精神病学]