Xue Wang,1,* Jiaqi Li,1,* Qichang Fu,2,* Honglin Zheng,1,3 Suying Duan,1 Yuan Gao,1,4 Haiyang Luo,1,3 Yuming Xu1,3,4 

1Department of Neurology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Zhengzhou, Henan, People’s Republic of China; 2Department of Magnetic Resonance, The First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Zhengzhou, Henan, People’s Republic of China; 3NHC Key Laboratory of Prevention and Treatment of Cerebrovascular Disease, Zhengzhou, Henan, People’s Republic of China; 4Henan Key Laboratory of Cerebrovascular Diseases, The First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Zhengzhou, Henan, People’s Republic of China

*These authors contributed equally to this work

Correspondence: Haiyang Luo, Department of Neurology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Jian-She East Road, Zhengzhou, Henan, 450000, People’s Republic of China, Email fccluohy@zzu.edu.cn Yuming Xu, Department of Neurology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Jian-She East Road, Zhengzhou, Henan, 450000, People’s Republic of China, Email xuyuming@zzu.edu.cn

Objective: The present study aims to analyze the clinical manifestations, laboratory results, neuroimaging features, treatment interventions, and outcomes in a cohort of patients with cerebral amyloid angiopathy-related inflammation (CAA-ri), providing a deeper understanding of this rare subtype of CAA and enhancing diagnostic precision in clinical practice.
Methods: We conducted a systematic retrospective review of clinical records from 13 consecutive patients who met the diagnostic criteria for probable CAA-ri and were evaluated at the First Affiliated Hospital of Zhengzhou University between January 2021 and August 2024.
Results: The study cohort comprised 13 patients (7 males, 6 females; mean age 65.2 years, range 42– 81), predominantly presenting with subacute onset (53.8%, n=7). Cognitive impairment (61.5%, n=8) emerged as the most frequent clinical manifestation, followed by headache (46.2%, n=6), epileptic seizures (30.8%, n=4), and focal neurological deficits (23.1%, n=3). Neuroimaging findings across all patients demonstrated asymmetric white matter hyperintensities in conjunction with cortical-subcortical cerebral microbleeds. A subset of patients exhibited cortical superficial siderosis lobar hemorrhage, and/or punctate acute infarction. Among the nine patients who underwent lumbar puncture, five showed elevated cerebrospinal fluid (CSF) pressure and protein levels. All four patients assessed for CSF Alzheimer’s disease biomarkers showed reduced Aβ 42 and Aβ 40 levels, alongside elevated total tau and phosphorylated tau levels. Furthermore, over 70% of the patients who treated with immunosuppressive therapy achieved favorable clinical outcomes.
Conclusion: Clinical manifestations and neuroimaging abnormalities serve as pivotal non-invasive criteria for guiding clinicians in the diagnosis of CAA-ri. Timely initiation of immunosuppressive therapy in CAA-ri patients can lead to favorable outcomes.

Keywords: CAA-ri, clinical manifestations, neuroimaging, immunosuppressive treatment