摘要
目的探讨脑淀粉样血管病相关炎症患者临床表现、影像特点及预后。方法回顾性分析我医院收治的7例脑淀粉样血管病相关炎症患者的临床表现、头部MRI检查、血和/或脑脊液检验、药物治疗及预后等临床资料。结果临床表现:急性起病5例,亚急性起病2例;4例患者存在局灶性神经功能缺损,5例患者存在急性认知功能减退,3例患者有头痛表现,1例患者有痫性发作;所有患者均无意识障碍。头部MRI检查:7例患者磁敏感加权成像均可见皮层及皮层下大量弥漫分布的脑微出血,5例患者磁敏感加权成像发现皮质表面铁沉积,2例患者存在凸面蛛网膜下腔出血;7例患者T2WI和Flair均可见皮层及皮质下片状非对称性白质高信号,3例患者头部增强扫描见软脑膜强化,5例患者治疗后头部Flair显示皮质下白质异常信号范围缩小。血或脑脊液检验:6例患者行血载脂蛋白E基因筛查,其中ε4/ε4基因型3例,ε2/ε4基因型2例,ε3/ε4基因型1例;4例患者行脑脊液检验,其中3例患者脑脊液蛋白轻度增高,2例患者脑脊液红细胞稍高,2例患者脑脊液Aβ40和Aβ42水平下降。药物治疗:4例患者接受了糖皮质激素治疗,3例仅予以对症支持治疗。预后:4例经糖皮质激素治疗的患者其临床症状及影像均明显好转,1例患者仅予以对症支持治疗临床症状及影像学亦有改善。结论脑淀粉样血管病相关炎症患者主要临床特点为局灶性神经功能缺损、认知功能障碍及头痛;磁共振主要特点是皮层及皮质下片状非对称性白质高信号及脑微出血,增强扫描部分患者有软脑膜不同程度强化;糖皮质激素治疗可使多数患者获益,但部分轻症患者亦可出现自发的临床症状和影像学改善。
Objective To investigate the clinical manifestations,imaging findings,and prognosis of patients with cerebral amyloid angiopathy-related inflammation.Methods The clinical data of 7 patients with cerebral amyloid angiopathy-related inflammation admitted to our hospital were retrospectively analyzed,including general data,clinical manifestations,head MRI examination,blood and cerebrospinal fluid(CSF)examination,drug treatment and prognosis.Results Clinical manifestations:5 cases with acute onset,2 cases with subacute onset.4 patients had focal neurological deficits,5 patients had acute cognitive impairment,3 patients had headache,1 patient was diagnosed with epilepsy based on clinical manifestations and EEG,and all patients had no disorder of consciousness.MRI examination of the head:the SWI of all patients showed a large number of scattered and diffuse cerebral microhemorrhages in the cortex and subcortex.The SWI of 5 patients showed iron deposition on the surface of the cortex,and 2 patients had convex subarachnoid hemorrhage.All patients had T2WI and flair showed asymmetric high white matter signals in the cortex and subcortex.Enhanced scanning of the pia mater showed enhancement of the pia mater in 3 patients.After treatment,Flair showed that the abnormal white subcortex substance signal area was reduced in 5 patients.Blood and CSF tests:6 cases of apolipoprotein E gene screening,including 3 cases ofε4/ε4 genotype,2 cases ofε2/ε4 genotype and 1 case ofε3/ε4 genotype;4 patients completed the lumbar puncture test of CSF,3 patients had slightly elevated CSF protein,2 patients had slightly elevated red blood cells in CSF and 2 patients had decreased Aβ40 and Aβ42 in CSF.Drug treatment:4 patients received glucocorticoid therapy,and 3 patients received only symptomatic and supportive treatment.Prognosis:The clinical symptoms and imaging of 4 patients treated with glucocorticoid improved significantly,and the clinical symptoms and imaging of 1 patient were improved by symptomatic and supportive treatment only.
作者
李维
周颖
陈婵娟
谷文萍
侯德仁
薛群
谭红
LI Wei;ZHOU Ying;CHEN Chanjuan;GU Wenping;HOU Deren;XUE Qun;TAN Hong(Department of Nerve Medical Center,The First Hospital of Changsha,Changsha 410005,China;Department of Neurology,Xiangya Hospital,Central South University,Changsha 410008,China;Department of Neurology,The Third Xiangya Hospital,Central South University,Changsha 410013,China;Department of Neurology,First Affiliated Hospital of Soochow University,Suzhou 215006,China)
出处
《分子影像学杂志》
2023年第5期805-810,共6页
Journal of Molecular Imaging
基金
长沙市自然科学基金(Kq2202008)
湖南省自然科学基金(2023JJ6039)
湖南省自然科学基金(2020JJ8101)
长沙市科技局(Kq2001004)
湖南省重点研发计划(2023sk2019)。
关键词
脑淀粉样血管病相关炎症
载脂蛋白E基因
脑微出血
磁敏感加权成像
免疫抑制治疗
cerebral amyloid angiopathy-related inflam mation
apolipoprotein E
cerebral microhemorrhage
magnetic susceptibility imaging
immunosuppressive therapy
