VandeVrede Lawren, Gibbs Daniel M, Koestler Mary, La Joie Renaud, Ljubenkov Peter A, Provost Karine, Soleimani-Meigooni David, Strom Amelia, Tsoy Elena, Rabinovici Gil D, Boxer Adam L
Memory and Aging Center Department of Neurology University of California San Francisco San Francisco California USA.
Department of Radiology and Biomedical Imaging University of California San Francisco San Francisco California USA.
Alzheimers Dement (Amst). 2020 Oct 9;12(1):e12101. doi: 10.1002/dad2.12101. eCollection 2020.
Amyloid-related imaging abnormalities (ARIA) are a common, dose-dependent effect of amyloid-targeting antibodies, strongly associated with the apolipoprotein E (APOE) ε4 allele.
We describe the clinical course and management of a 66-year-old white male (APOE ε4/ε4) enrolled in an observational study that included amyloid and tau positron emission tomography (PET), who received aducanumab through the ENGAGE clinical trial.
Acute symptoms included headache and encephalopathy, and magnetic resonance imaging revealed ARIA-E and ARIA-H. Malignant hypertension and epileptiform activity were treated with nicardipine and levetiracetam. Subsequent clinical/imaging worsening prompted a course of methylprednisolone. Symptoms and ARIA-E resolved over 6 months, while ARIA-H persisted. Quantitative analysis of interval amyloid PET showed reduced signal in pre-existing areas but increased signal posteriorly; while tau PET showed increased signal overall.
In an APOE ε4/ε4 patient, ARIA symptoms were accompanied by malignant hypertension and epileptiform activity, and pulsed steroids reversed edema. Studies from larger cohorts may clarify the optimal treatment and pathophysiology of ARIA.
淀粉样蛋白相关成像异常（ARIA）是靶向淀粉样蛋白抗体常见的剂量依赖性效应，与载脂蛋白E（APOE）ε4等位基因密切相关。
我们描述了一名66岁白人男性（APOE ε4/ε4）的临床病程及治疗情况，该患者参与了一项包含淀粉样蛋白和tau正电子发射断层扫描（PET）的观察性研究，并通过ENGAGE临床试验接受了阿杜卡单抗治疗。
急性症状包括头痛和脑病，磁共振成像显示有ARIA-E和ARIA-H。恶性高血压和癫痫样活动分别用尼卡地平和左乙拉西坦治疗。随后的临床/影像学恶化促使采用甲泼尼龙治疗。症状和ARIA-E在6个月内消退，而ARIA-H持续存在。对期间淀粉样蛋白PET的定量分析显示，先前存在的区域信号降低，但后部信号增加；而tau PET显示总体信号增加。
在一名APOE ε4/ε4患者中，ARIA症状伴有恶性高血压和癫痫样活动，脉冲式类固醇治疗可逆转水肿。来自更大队列的研究可能会阐明ARIA的最佳治疗方法和病理生理学。
