脑淀粉样血管病相关炎症的临床特点分析

Clinical Analysis of Cerebral Amyloid Angiopathy Associated with Inflammation

目的分析脑淀粉样血管病相关炎症(CAA-I)的临床特点。方法回顾性分析8例CAA-I患者的临床资料,综合相关文献分析其临床特点。结果8例患者中,6例表现为不同程度的认知功能障碍,5例合并头痛,3例合并无力、失语、视力下降等局灶性神经功能缺损症状,2例伴发癫痫。8例患者头颅T_(2)W和Flair相表现为多发的白质高信号,SWI均表现为皮质-皮质下多发微出血。4例基因检测表现型为APOE4。7例患者经激素治疗后症状和影像学改变均明显改善,1例逐渐进展,1例治疗13个月后复发。结论CAA-I为临床罕见的可治性疾病,对免疫抑制治疗敏感,可动态观察影像学改变评估治疗效果。

Objective To analyz the clinical characteristics of cerebral amyloid angiopathy associated with inflammation(CAA-I).Methods A retrospective analysis on the clinical data of 8 CAA-I patients was performed.The relevant literatures were reviewed to summarize the clinical characteristics of the disease.Results Of the 8 patients,6 cases had different levels of cognitive dysfunction,5 had headaches,and 3 were combined focal neurological deficits such as weakness,aphasia,and visual impairment,and 2 patients with epilepsy.All 8 patients showed hyperintensities on T_(2)W and Flair and microbleeds at cortical-subcortical junction on susceptibility-weighted images.There were 4 cases were detected as APOE4 phenotype.There were 7 patients responds well to corticosteroids with significant improvement in symptoms and imaging changes,while 1 case had relentless disease progression despite aggressive and 1 relapsed after thirteen months.Conclusion CAA-I is a rare but potentially treatable disorder,and sensitive to immunosuppression.Dynamic imaging changes can be evaluated to assess the effect of treatment.