Cerebral inflammatory amyloid angiopathy: response to treatment.

Abstract

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.