ABSTRACT
BACKGROUND: Cerebral amyloid angiopathy - related inflammation (CAA-ri) is an uncommon manifestation of CAA. METHODS: Single-center, retrospective review of all charts with ICD-code I68.0 (CAA) from 2/2/2016-1/1/2020. RESULTS: Of 152 CAA cases, 13 (8.6%) were consistent with CAA-ri. Corticosteroid-treatment led to short-term reduction in modified Rankin Scale scores (2.6 ± 1.4 vs. 1.6 ± 1.5; p = 0.01) and T2/FLAIR lesion volume (78.1 ± 52.2 cm3 vs. 30 ± 30.9 cm3, p < 0.01) as well as short-term improvement in post-treatment Clinical Global Impression - Global Change scores compared to pre-treatment scores (clinical: 6 ± 1 vs. 2.6 ± 1.3, p = 0.03; radiological: 4.6 ± 1.9 vs. 1.2 ± 0.4, p = 0.03). INTERPRETATION: Corticosteroid-treatment leads to clinical and radiological short-term improvement (class IV evidence).