Lobar hemorrhage in the course of recurrent inflammatory cerebral amyloid angiopathy

Introduction: Cerebral amyloid angiopathy–related inflammation (CAA-ri) is a disease that can respond to immunosuppressive treatment, but hemorrhagic risk persists because of the underlying vasculopathy. This report aims to present a patient who developed a lobar hematoma after a recurrent attack of CAA-ri.Case: A seventy-two-year-old man presented with right-sided weakness and imbalance following recurrent right-hand jerking episodes, which were evaluated as transient focal neurological episodes (TFNE). His history included recurrent ischemic stroke. Cranial magnetic resonance imaging showed asymmetric FLAIR hyperintensities in the right frontal subcortical white matter and cortical superficial siderosis on SWI, and probable CAA-ri was considered. He received intravenous methylprednisolone 1,000 mg/day for five days, followed by oral prednisolone 64 mg/day, which was tapered and discontinued. Follow-up imaging showed radiological regression. One year later, he presented again with dragging of the right foot and transient right-sided weakness. On examination, right upper extremity muscle strength was 3/5. Diffusion-weighted imaging showed diffusion restriction in the left parietal region, and FLAIR showed an area of edema in the same region. A recurrent CAA-ri attack was considered, and intravenous methylprednisolone 1,000 mg/day was given for five days, with clinical improvement. While he continued to take acetylsalicylic acid, a large lobar hematoma with extension into the ventricular system developed in the left hemisphere one day after completion of treatment. He was monitored in the intensive care unit and died of sepsis about one month later.Discussion/Conclusion: In CAA-ri, corticosteroid treatment can produce clinical and radiological improvement, but the risk of lobar hemorrhage due to the underlying amyloid vasculopathy persists. Individualized assessment of hemorrhagic risk and close follow-up are important, particularly in patients receiving antithrombotic therapy.Keywords: