脑淀粉样血管病变相关炎症  

Cerebral amyloid angiopathy associated with inflammation

作　　者：刘丽媛 郑兰 谷文萍[2] 高小平[1] 杨丽萍[1] 高菊华[1] LIU Li-Yuan;ZHENG Lan;GU Wen-Ping;GAO Xiao-Ping;YANG Li-Ping;GAO Ju-Hua(The First Affiliated Hospital of Hunan Normal University,Changsha,Hunan 410005,China;Department of Neurology,Xiangya Hospital,Central South University,Changsha,Hunan 410008,China)

机构地区：[1]湖南师范大学附属第一医院,湖南长沙410005 [2]中南大学湘雅医院神经内科,湖南长沙410008

出　　处：《国际神经病学神经外科学杂志》2022年第6期59-64,共6页Journal of International Neurology and Neurosurgery

基　　金：湖南省自然科学基金(2020JJ4875);湖南省重点研发(2020SK2069);湖南省卫生健康委科研项目(B202203073354)。

摘　　要：为了提高对脑淀粉样血管病变(CAA)相关炎症(CAA-I)的认识。现将该院1例CAA-I患者的临床表现、影像特点、诊疗过程进行整理回顾。患者为男性,70岁,以癫痫发作、认知功能减退为临床表现。脑脊液(CSF)检查提示细胞数及蛋白轻度升高。头颅计算机断层扫描(CT)提示凸面型蛛网膜下腔出血、脑白质变性。头颅磁共振成像(MRI)提示紧邻皮质的斑片状T2WI及FLAIR异常高信号影,非对称性并延伸至皮质下白质,伴软脑膜强化。磁敏感加权成像(SWI)提示≥1处皮质―皮质下微出血灶及皮质表面铁沉积。基因检测为载脂蛋白E(APOE)E2/E3。诊断为很可能的脑淀粉样血管病相关炎症。经甲泼尼龙冲击治疗后病灶明显好转,1个月后随访认知功能改善、无癫痫发作。CAA-I是脑淀粉样血管病的罕见亚型,临床医生多对此病认识不足,多序列的磁共振检查,尤其是SWI有利于其诊断,激素治疗效果显著。In order to improve the awareness of cerebral amyloid angiopathy associated with inflammation(CAA-I),this article reviews the clinical manifestations,imaging features,diagnosis,and treatment of a patient with CAA-I in our hospital.A male patient,aged 70 years,had the clinical manifestations of seizures and cognitive impairment.Cerebrospinal fluid examination showed a slight increase in cell number and protein.Cranial CT showed convex subarachnoid hemorrhage and white matter degeneration;cranial magnetic resonance imaging(MRI)showed abnormal patchy hyperintensity on T2WI and FLAIR adjacent to the cortex,which was asymmetric and extended to subcortical white matter,with the enhancement of leptomeninges;susceptibility-weighted imaging(SWI)showed one or more cortical-subcortical microhemorrhage foci and iron deposition on the surface of the cortex.Genetic testing showed apolipoprotein E E2/E3.The patient was diagnosed most likely with CAA-I.The lesion was significantly improved after methylprednisolone shock therapy,and 1-month followup showed improvement in cognitive function without seizure.CAA-I is a rare subtype of cerebral amyloid angiopathy,and there is a lack of understanding of this disease among clinicians.Multi-sequence MRI examination,especially SWI,may help with the diagnosis of this disease,and corticosteroid therapy shows a marked clinical effect in treatment.

关 键 词：脑淀粉样血管病相关炎症 癫痫发作 Β淀粉样蛋白 磁共振成像 

分 类 号：R743[医药卫生—神经病学与精神病学]