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Background/Objective Amyloid Beta-Related Angiitis (ABRA) is a rare complication of Cerebral Amyloid Angiopathy (CAA) which presents with subacute cognitive decline, headaches and seizures. Although histopathology is required for a confirmed diagnosis, there are suggestive Magnetic Resonance Imaging (MRI) findings such as subcortical vasogenic oedema with microhaemorrhages. Treatment usually involves immunosuppression with corticosteroids.Method Case Report.Results We describe a case of a 66-year-old patient who presented with headaches and initially admitted under rheumatology of a tertiary metropolitan hospital for concerns of giant cell arthritis (GCA). The neurology department was consulted for worsening confusion and agitation during her inpatient admission. Her MRI showed leptomeningeal enhancement and multifocal cortical Diffusion-Weighted Imaging (DWI) restrictions in the right occipital lobe and left frontal lobe. The Susceptibility-Weighted Imaging (SWI) sequence showed superficial siderosis underlying the leptomeningeal enhancement. There was elevated white cells and protein in her cerebrospinal fluid (CSF) but no malignant cells. Her digital subtraction angiography (DSA) was normal. A brain biopsy revealed histological features concerning for a vasculitic process with immunohistochemistry confirming vascular beta-amyloid deposition. The patient was empirically treated with high-dose corticosteroids once infection was excluded pre-biopsy. Further progression was noted on interval imaging, requiring escalation of immunosuppressive therapy with cyclophosphamide and rituximab. The patient has since remained stable in the community with close outpatient monitoring.Conclusion This case demonstrates the diagnostic complexity of ABRA, with a definite diagnosis only being obtained by brain biopsy. It also highlights the need for close monitoring with interval imaging to assess response to immunosuppressive therapy.