Amyloid Beta Related Angiitis (ABRA) is a rare subtype of cerebral amyloid angiopathy (CAA), typically presenting with rapid neurological decline due to microinfarcts, microhemorrhages, and cortical superficial siderosis. Histologically, it features amyloid-beta deposition with angio-destructive inflammation. We present a case highlighting the importance of early recognition of ABRA following a 5.5-year history of chronic intermittent headaches.

A 75-year-old female with chronic intermittent headaches and word-finding difficulty, alongside a history of intraparenchymal hemorrhage (IPH), presented to the ED with a sudden, severe headache. CT revealed a large right temporal lobe IPH, multiple hemorrhagic foci in the bilateral thalami and posterior temporal lobe, and subarachnoid hemorrhages in the right frontal and parietal lobes. MRI showed diffuse chronic siderosis and leptomeningeal enhancement, prompting CSF studies. CSF analysis indicated pleocytosis with elevated protein, suggesting an inflammatory process. The patient experienced recurrent neurological symptoms, including new microhemorrhages and ischemia in the right centrum semiovale. A brain biopsy revealed focal transmural inflammation with epithelioid histiocytes, confirming ABRA. Despite steroid treatment, new hemorrhages occurred, leading to Cyclophosphamide therapy, followed by long-term azathioprine, which improved her aphasia and alertness without further decline.

The prolonged headaches and vascular lesions preceding her decline indicate a delayed diagnosis of ABRA. Earlier imaging and intervention may have mitigated her rapid neurological deterioration. This case underscores the importance of recognizing early ABRA manifestations to facilitate prompt diagnosis and treatment, potentially preventing severe cerebrovascular complications.