Objectives

We want to report the clinical and radiological features of our cohort of patients diagnosed with cerebral amyloid angiopathy-related inflammation (CAA-RI) according to the Boston Criteria and additionally to disclose some atypical clinical characteristics observed in some of them to provide more knowledge about this novel entity.

Methods

We describe 5 patients with probable CAA-RI according to a validation study of proposed criteria for the diagnosis of CAA-RI at University Hospital Josep Trueta of Girona. We consider some clinical characteristics which include the response to immunotherapy, CSF findings, and MRI features. The patient’s neurologic outcomes were assessed using the modified Rankin Scale (mRS).

Results

We collected 5 patients admitted for probable CAA-RI. Most were women and the median age was 72 years. The median mRS score at the onset of disease was 1. Parietal lobes were most affected clinically as well as radiologically. Two patients had intracranial hemorrhage. Decreased levels of CSF amyloid beta 42 and 40 protein were observed. Corticosteroids were used in four patients and a remarkable improvement was observed in all of them.

Conclusions

CAA-RI is a condition that predominantly affects parietal lobes according to our case series and this involvement seems to be directly related to a greater burden of microbleeds, cortical siderosis, WMH, and lobar hemorrhages on these lobes. Decreased levels of CSF amyloid beta protein plus increased total tau protein should be considered as part of the diagnostic criteria of CAA-RI. We recommend corticosteroids using, as they have been demonstrated to be very effective in managing CAA-RI.

中文翻译：

---

脑淀粉样血管病相关炎症的临床和影像学特征

目标

我们希望报告根据波士顿标准诊断为脑淀粉样血管病相关炎症 (CAA-RI) 的患者队列的临床和放射学特征，此外还披露在其中一些患者中观察到的一些非典型临床特征，以提供更多关于这个新颖的实体。

方法

我们根据赫罗纳大学医院 Josep Trueta 提出的 CAA-RI 诊断标准的验证研究描述了 5 名可能患有 CAA-RI 的患者。我们考虑了一些临床特征，包括对免疫治疗的反应、脑脊液检查结果和 MRI 特征。使用改良的 Rankin 量表 (mRS) 评估患者的神经系统结果。

结果

我们收集了 5 名因可能的 CAA-RI 而入院的患者。大多数是女性，中位年龄为 72 岁。疾病发作时的中位 mRS 评分为 1。顶叶在临床和放射学上受到的影响最大。两名患者有颅内出血。观察到脑脊液淀粉样蛋白 42 和 40 蛋白水平降低。4 名患者使用了皮质类固醇，并且在所有患者中都观察到了显着的改善。

结论

根据我们的病例系列，CAA-RI 是一种主要影响顶叶的疾病，这种受累似乎与这些叶上微出血、皮质铁质沉着症、WMH 和大叶出血的更大负担直接相关。CSF β 淀粉样蛋白水平降低加上总 tau 蛋白水平升高应被视为 CAA-RI 诊断标准的一部分。我们推荐使用皮质类固醇，因为它们已被证明对控制 CAA-RI 非常有效。