脑淀粉样血管病相关炎症的临床特征分析  

Clinical characteristics of cerebral amyloid angiopathy-related inflammation

作　　者：苏娅 付佳玉 谭海波 王琼[3] 董强 程忻 Su Ya;Fu Jiayu;Tan Haibo;Wang Qiong;Dong Qiang;Cheng Xin(Department of Neurology,Huashan Hospital,Fudan University,Shanghai 200040,China;PET Centre,Huashan Hospital,Fudan University,Shanghai 200040,China;Department of Neurology,the First Affiliated Hospital of University of Science and Technology of China(Anhui Provincial Hospital),Hefei 230036,China)

机构地区：[1]复旦大学附属华山医院神经内科,上海200040 [2]复旦大学附属华山医院PET中心,上海200040 [3]中国科学技术大学附属第一医院(安徽省立医院)神经内科,合肥230036

出　　处：《中华神经科杂志》2021年第10期1001-1008,共8页Chinese Journal of Neurology

基　　金：国家重点研发计划重大慢性非传染性疾病防控研究(2016YFC1300500-3);国家自然科学基金面上项目(81971123);上海市“脑与类脑智能”市级重大科技专项(2018SHZDZX01)。

摘　　要：目的探讨脑淀粉样血管病(CAA)相关炎症(CAA-ri)患者的临床、脑脊液和影像学特征,并分析与临床预后的相关因素。方法纳入2015年11月至2020年5月在复旦大学附属华山医院以及2018年1月至2020年5月在中国科学技术大学附属第一医院确诊的17例CAA-ri患者和同期确诊的59例CAA脑出血、15例CAA认知障碍患者,比较3组患者的血管性危险因素和脑小血管病影像学特征。收集CAA-ri患者的临床表现、脑脊液检查结果、磁共振成像病灶特征、治疗方案和随访资料;良好预后定义为临床和影像缓解且无疾病复发;通过二元Logistic回归模型分析临床特征与使用免疫抑制治疗、良好预后的相关性。结果CAA-ri患者相比CAA脑出血患者,发病年龄更早[(61.5±11.7)岁比(70.9±8.6)岁,t=9.428,P=0.001]、脑叶微出血灶数目更多[69.0(43.5,134.3)个比10.0(5.0,59.0)个,H=3.363,P=0.002];相比CAA认知障碍患者,男性更多(14/17比6/15,χ^(2)=6.099,P=0.014)、脑白质高信号Fazekas评分较低[4.0(2.0,6.0)比6.0(5.0,6.0),H=2.461,P=0.042]。CAA-ri患者中,使用免疫抑制治疗与脑脊液蛋白>600 mg/L呈正相关(比值比16.50,95%置信区间1.09~250.18,P=0.043);随访(3.0±1.9)年,良好预后与脑脊液蛋白<1000 mg/L且接受免疫抑制治疗呈正相关(比值比20.00,95%置信区间1.39~287.60,P=0.028)。结论CAA-ri是CAA的一种特殊亚型,与CAA脑出血、认知障碍相比,其发病年龄更早、出血性影像学标志物更多见。在脑脊液蛋白正常或轻度升高的CAA-ri患者中应用免疫抑制治疗更易获得良好预后。Objective To investigate the clinical,cerebrospinal fluid(CSF)and neuroimaging characteristics and their associations with prognosis in cerebral amyloid angiopathy(CAA)-related inflammation(CAA-ri).Methods Seventeen patients with CAA-ri,59 patients with CAA-related intracerebral hemorrhage(ICH)and 15 patients with CAA-related cognitive decline were recruited from Huashan Hospital,Fudan University from November 2015 to May 2020 and the First Affiliated Hospital of University of Science and Technology of China from January 2018 to May 2020.Vascular risk factors and imaging features of cerebral small vessel disease were compared among three groups.Clinical manifestations,CSF results,lesion features on magnetic resonance imaging,treatment options and follow-up data were collected in patients with CAA-ri.The good prognosis was defined by clinical and radiographic improvement with no disease recurrence.The associations between clinical characteristics and the immunosuppressive therapy or the good prognosis were analyzed by binary Logistic regression models.Results Patients with CAA-ri showed earlier disease onset[(61.5±11.7)years vs(70.9±8.6)years,t=9.428,P=0.001]and more lobar cerebral microbleeds[69.0(43.5,134.3)vs 10.0(5.0,59.0),H=3.363,P=0.002]compared to patients with CAA-ICH,and higher prevalence of male(14/17 vs 6/15,χ^(2)=6.099,P=0.014)and lower white matter hyperintensity Fazekas score[4.0(2.0,6.0)vs 6.0(5.0,6.0),H=2.461,P=0.042]compared to patients with CAA-related cognitive decline.In patients with CAA-ri,the immunosuppressive therapy was positively correlated with CSF protein>600 mg/L(odds ratio 16.50,95%confidence interval 1.09-250.18,P=0.043),and during a follow-up of(3.0±1.9)years,the good prognosis was positively correlated with CSF protein<1000 mg/L plus immunosuppressive therapy(odds ratio 20.00,95%confidence interval 1.39-287.60,P=0.028).Conclusions CAA-ri is a special subtype of CAA with earlier disease onset and higher prevalence of hemorrhagic imaging makers compared to CAA-ICH and CAA-related

关 键 词：脑淀粉样血管病 炎症 磁共振成像 免疫抑制治疗 预后 

分 类 号：R743[医药卫生—神经病学与精神病学]