Clinical Presentation of Inflammatory Amyloid Angiopathy in Seville, Spain

ICAA is an underdiagnosed inflammatory response to β-amyloid (Aβ) deposition in the cortical and leptomeningeal microcirculation, and is clinically heterogeneous. We present a retrospective observational study conducted at a tertiary center from 2011 to 2024, including 11 patients who met the 2016 clinicoradiological criteria. Eleven patients were included in the study. Seven patients (64%) were female. The mean age was 70.6 years. The most frequent symptoms were seizures (n=9, 82%) and headaches (n=8, 73%). All patients presented with cortical microbleeds and white matter hyperintensities in MRI. Cortical and leptomeningeal biopsies were performed in 2 cases due to diagnostic uncertainty. Corticosteroid therapy was used in 9 (82%) patients, but clinical relapse occurred in 55%, and 45% were functionally dependent after 1 year. This case series highlights the heterogeneous clinical and radiologic presentation of ICAA and the challenges in its diagnosis and management, highlighting the need for standardized therapeutic approaches and further research to improve its prognosis.