2例类固醇激素反应性慢性淋巴细胞性炎性反应伴脑桥周围血管异常强化症(CLIPPERS)并文献复习  

Analysis of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids(CLIPPERS)in 2 Cases and literatures review

作　　者：黄琳[1,2] 沙瑞娟[1] 葛剑青[1] 余传勇[1] HUANG Lin;SHA Ruijuan;GE Jianqing(Department of Neurology,Nanjing Brain Hospital,Nanjing 210029,China)

机构地区：[1]南京医科大学附属脑科医院神经内科,江苏南京210029 [2]安徽医科大学第一附属医院,安徽合肥230022

出　　处：《中风与神经疾病杂志》2022年第3期251-255,共5页Journal of Apoplexy and Nervous Diseases

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周围血管异常强化症(Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids,CLIPPERS)临床表现、影像及诊疗经过,以提高对该病的认识。方法回顾性分析2例CLIPPERS综合征的临床表现、影像及诊疗经过。结果1例头颅MRI示脑干、基底节区、小脑等部位多发长T_(1)、稍长T_(2)病灶,DWI为稍高或等信号,1例头颅MRI示脑干、两侧小脑半球片状长T_(1)稍长T_(2)信号,DWI右侧病灶低信号,左侧稍高信号,两例增强扫描提示病灶呈典型的“胡椒粉”样点状及曲线状强化,头颅MRA或头颈部CTA检查均无异常。2例患者初次使用类固醇激素治疗后症状及影像学均明显好转,1例后长期口服激素维持仍然有病情复发,第二次使用激素冲击后,长期口服激素及免疫抑制剂维持治疗,第三次复诊住院,仅口服硫唑嘌呤,目前症状及影像学明显好转,另外1例出院1 m后因肺部感染去世。结论CLIPPERS综合征在MRI上有典型部位和影像学表现,使用激素治疗有效也提示CLIPPERS综合征可能。CLIPPERS综合征可能是一种异质性病因的综合征,而不是一种独立的疾病。Objective To Invetigate chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids(CLIPPERS)clinical diagnosis、the imaging manifestations and the course of treatment to raise the awareness of the disease.Methods To retrospectively analyse clinical diagnosis、the imaging manifestations and the course of treatment of 2 patients with CLIPPERS.Results One patient’s Brain MRI having long T_(1) and slightly longer T_(2) signal,not significantly high signal in the DWI,in the brain stem、basal ganglia and cerebellar hemisphere.Another patient had long T_(1) and slightly longer T_(2) signal,not significantly high signal in the DWI,in the brain stem and cerebellar hemisphere.Two patients’brain MRI with gadolinium foci of enhancement with a curvilinear pattern highly.Theirs’magnetic resonance vascular examination were normal.Theirs’symptoms and imagings had improving significantly after glucocorticosteroid shocking treatment.One patient relapsed with long-term oral prednisone,after the second glucocorticosteroid shocking,with long-term oral prednisone and glucocorticosteroids,he had obviously improvement.So far,the patient only uses oral azathioprine,he is well.Another patient died of pneumonia for a week after leaving hospital.Conclusion CLIPPERS syndrome has obviously imaging findings on MRI,and having to get better after glucocorticosteroid treating,prompting CLIPPERS syndrome may be a syndrome of heterogeneous etiology,not an independent disease.

关 键 词：CLIPPERS 激素 头颅MRI 曲线状强化 

分 类 号：R743[医药卫生—神经病学与精神病学]