Pimenta João Roberto Ribeiro, Freitas Júlian Letícia, Rocha Maria Sheila Guimarães, Brucki Sonia Maria Dozzi
Hospital Santa Marcelina, Departamento de Neurologia, São Paulo SP, Brazil.
Dement Neuropsychol. 2025 Aug 18;19:e20240263. doi: 10.1590/1980-5764-DN-2024-0263. eCollection 2025.
Cerebral Amyloid Angiopathy (CAA) is a small vessel disease associated with β-amyloid (Aβ) deposition in cortical and leptomeningeal vessels. Traditionally diagnosed through invasive methods, it can now be identified via advanced imaging modalities, enhancing non-invasive diagnostic accuracy. A subset of patients exhibits an inflammatory presentation, termed Inflammatory Cerebral Amyloid Angiopathy (CAA-RI), characterized by cognitive decline, behavioral changes, and neurological deficits. This study highlighted two cases of CAA-RI with subacute onset, detailed clinical progression, and distinct MRI findings consistent with revised diagnostic criteria, enabling early suspicion. Both cases showed significant improvement with immunosuppressive therapy, reinforcing the potentially reversible nature of CAA-RI and the importance of early recognition. This article underscores the relevance of advanced imaging in the differential diagnosis of CAA and the potential for improved patient outcomes with timely treatment.
脑淀粉样血管病（CAA）是一种与β-淀粉样蛋白（Aβ）在皮质和软脑膜血管中沉积相关的小血管疾病。传统上通过侵入性方法诊断，现在可以通过先进的成像方式进行识别，提高了非侵入性诊断的准确性。一部分患者表现出炎症性表现，称为炎症性脑淀粉样血管病（CAA-RI），其特征为认知能力下降、行为改变和神经功能缺损。本研究重点介绍了2例亚急性起病的CAA-RI病例，详细描述了临床进展以及与修订诊断标准一致的独特MRI表现，有助于早期怀疑。两例患者经免疫抑制治疗后均有显著改善，这强化了CAA-RI潜在的可逆性以及早期识别的重要性。本文强调了先进成像在CAA鉴别诊断中的相关性以及及时治疗改善患者预后的潜力。
