类固醇激素反应性慢性淋巴细胞性炎症伴脑桥血管周围强化综合征的临床特点分析  

Clinical Features of Chronic Lymphocytic in Flammation with Pontine Perivascular Enhancement Responsive to Steroids in 28 Chinese Patients

作　　者：刘海林 LIU Hailin(Department of Neurology,Luoyang Central Hospital Affiliated to Zhengzhou University,Luoyang 471000,China)

机构地区：[1]郑州大学附属洛阳中心医院神经内科,河南洛阳471000

出　　处：《河南医学研究》2021年第14期2501-2504,共4页Henan Medical Research

基　　金：河南省联合共建项目基金课题(LHGJ20191203)。

摘　　要：目的分析类固醇激素反应性慢性淋巴细胞性炎症伴脑桥血管周围强化综合征(CLIPPERS)的临床特点。方法电子检索万方医学网、维普全文数据库、中国知网中的CLIPPERS综合征病例报告,分析并总结其临床特点。结果检索出的25篇文献包括28个病例,男11例,女16例,1例性别不详。患者发病年龄5~73岁。27例首发症状描述清楚(个别病例首发症状非单一),1例未进行描述。首发症状:10例(37%)共济失调,5例(18.5%)头晕,4例(14.8%)复视,4例(14.8%)肢体运动障碍,3例(11.1%)肢体感觉异常,2例(7.4%)头痛、面部感觉异常,吞咽困难、耳鸣、言语不清、周围神经病变各1例(3.7%)。临床表现:24例(85.7%)共济失调,15例(53.6%)头晕,10例(35.7%)复视、饮水呛咳、肢体运动障碍,8例(28.6%)眼震,7例(25.0%)构音障碍、头痛,6例(21.4%)肢体感觉障碍。6例(21.4%)完善病理检查,1例出现CD3~+T细胞和CD20~+B细胞浸润且胶质细胞增生。1例反复发病9 a,左侧额叶侧脑室前角病理结果示,血管周围大量CD3~+T细胞聚集。结论CLIPPERS无特异性生物标志物,其确诊依赖于影像检查和临床症状,对激素治疗敏感。Objective To analyze the clinical characteristics of steroid-responsive chronic lymphocytic inflammation with pontine perivascular enhancement syndrome(CLIPPERS).Methods Case reports of CLIPPERS syndrome in the three Chinese databases(Wanfang,CNKI and VIP)were searched electronically and its clinical characteristics were analyzed and summarized.Results Twenty-five literatures were retrieved,which included 28 cases constituted by 11 males,16 females and 1 case with unknown sex.The onset age of the patients ranged from 5 to 73.The initial symptoms of 27 cases were clearly described(some cases with non-single initial symptoms)and that of 1 case was not described.The initial symptom was ataxia in 10 cases(37%),dizziness in 5 cases(18.5%),diplopia in 4 cases,motor dysfunction in 4 cases(14.8%),limb paresthesia in 3 cases(11.1%),headache and facial paraesthesia in 2 cases(7.4%),tinnitus,drinking water choking cough,dysarthria or peripheral neuropathy in 1 case(3.7%).As to the clinical manifestations,24 cases(85.7%)had ataxia,15 cases(53.6%)had dizziness,10 cases(35.7%)had diplopia,drinking water choking cough and motor dysfunction,8 cases(28.6%)had nystagmus,7 cases(25.0%)had dysarthria and headache and 6 cases(21.4%)had limb paresthesia.Pathological examination was performed in 6 cases.There was 1 case showed infiltration of CD3~+T and CD20~+B cells and glial cells proliferation.One case with a recurrent disease for 9 years showed a large number of CD3~+T lymphocytes clustered around the vessels in the left frontal ventricle anterior horn.Conclusion CLIPPERS sensitive to hormone therapy and it has no specific biomarkers,its diagnosis depends on imaging examination and clinical symptoms.

关 键 词：类固醇激素反应性慢性淋巴细胞性炎症伴脑桥血管周围强化综合征 临床特征 二次文献 

分 类 号：R58[医药卫生—内分泌]