Clinical manifestations, imaging features and treatment strategies of cerebral amyloid angiopathy–related inflammation with chronic onset in the Chinese population

Abstract Objective: Cerebral amyloid angiopathy–related inflammation (CAA-ri) is an increasingly recognized subtype of cerebral amyloid angiopathy, and previously reported CAA-ri patients mainly performed with acute and subacute onset. The study aims to describe the clinical manifestations, imaging features and treatment strategies of CAA-ri with chronic onset in the Chinese population. Methods: We systematically reviewed the records of patients with cerebral amyloid angiopathy–related inflammation who presented from January 1, 2016, to January 1, 2023 at the First Affiliated Hospital of Zhengzhou University for additional detailed clinical, laboratory, imaging, treatment, and outcomes data. Results: The 40 individuals in the study included 23 men and 17 women with a median age of 66 years; 25 patients (62.5%) presented with chronic onset, the most common form of onset. Cognitive impairment (n = 17), headache (n = 6), behavioral alterations (n = 5), focal neurological signs (n = 5), were the relatively common clinical manifestations of CAA-ri in the chronic patients. T2 weighted, and fluid attenuation inversion recovery MRI sequences in all patients showed asymmetric, confluent and patchy hyperintensities in both cerebral hemispheres; Eleven patients were treated with glucocorticoid shock therapy (1 of them combined with immunoglobulin). Eight patients of eleven were discharged with significant remission of clinical symptoms. MRI was re-examined after treatment, and abnormal signals on the T2WI phase partially disappeared in 6 cases, increased in 1 case and MRI was not re-examined in 4 cases. Conclusion : Chronic onset pattern may be the most common form in the CAA-ri Chinese population. Cognitive impairment, behavioral alterations, focal neurological dysfunction and headache are the main clinical manifestations of CAA-ri with chronic onset. Immunosuppressive treatment may improve clinical symptoms and imaging features in patients of CAA-ri with chronic onset.