Course of cerebral amyloid angiopathy - related inflammation
Course of cerebral amyloid angiopathy - related inflammation
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DOI：
10.1212/01.wnl.0000260066.98681.2e
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发表时间：
2007-04-24
期刊：
影响因子：
9.9
通讯作者：
Greenberg, S. M.
中科院分区：
文献类型：
--
作者：
Kinnecom, C.;Lev, M. H.;Greenberg, S. M.
背景资料：一部分脑淀粉样血管病（CAA）患者表现为认知症状、癫痫发作、头痛、T2高信号MRI病变和CAA相关血管炎症的神经病理学证据。目的：分析本病的危险因素、诊断特点及长期病程。研究方法：我们评估了14例病理诊断为CAA相关炎症的连续患者，其中12例有可用的神经影像学和随访数据。在46.8 +/- 29.1个月的随访期间评估了患者的MRI表现、APOE基因型和临床病程。结果如下：基线MRI扫描的特征是不对称的T2高信号病变延伸到皮质下白色物质，偶尔覆盖灰质，信号特性提示血管源性水肿。根据对免疫抑制剂治疗的反应，受试者可分为三组：单相改善（7/12）、初始改善后症状复发（3/12）和对治疗无明显反应（2/12）。MRI高信号的体积与临床症状的严重程度相关。1例患者在复发性MRI高信号区域内发生症状性脑出血。APOE β 4/β 4基因型与CAA相关炎症密切相关，76.9%（10/13）的受试者与5.1%（2/39）的有症状但非炎症性CAA受试者存在APOE β 4/β 4基因型（p < 0.0001）。结论：脑淀粉样血管病相关炎症代表了临床、病理学、放射学和遗传学上不同的疾病亚型，对阿尔茨海默病的临床实践和正在进行的免疫治疗方法具有意义。
Background: A subset of patients with cerebral amyloid angiopathy (CAA) present with cognitive symptoms, seizures, headaches, T2-hyperintense MRI lesions, and neuropathologic evidence of CAA-associated vascular inflammation. Objective: To analyze the risk factors, diagnostic characteristics, and long-term course of this disorder. Methods: We assessed 14 consecutive patients with pathologically diagnosed CAA-related inflammation, 12 with available neuroimaging and follow-up data. Patients were evaluated for MRI appearance, APOE genotype, and clinical course over a 46.8 +/- 29.1- month follow-up. Results: Baseline MRI scans were characterized by asymmetric T2-hyperintense lesions extending to the subcortical white matter and occasionally the overlying gray matter, with signal properties suggesting vasogenic edema. Subjects could be divided into three groups based on response to immunosuppressive treatment: monophasic improvement (7/12), initial improvement followed by symptomatic relapse (3/12), and no evident response to treatment (2/12). The volume of MRI hyperintensities correlated with the severity of clinical symptoms. One patient experienced symptomatic intracerebral hemorrhage within a region of recurrent MRI hyperintensity. The APOE epsilon 4/epsilon 4 genotype was strongly associated with CAA-related inflammation, present in 76.9% (10/13) of subjects vs 5.1% (2/39) with symptomatic but noninflammatory CAA ( p < 0.0001). Conclusion: Cerebral amyloid angiopathy - related inflammation represents a clinically, pathologically, radiographically, and genetically distinct disease subtype with implications for clinical practice and ongoing immunotherapeutic approaches to Alzheimer disease.