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 T2W 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.
关键词
脑淀粉样血管病/脑淀粉样血管病相关炎症/载脂蛋白E
Key words
cerebral amyloid angiopathy/cerebral amyloid angiopathy associated with inflammation/apolipoprotein E
维普
万方数据