Abstract 125: Amyloid Beta‐Related Angiitis: A Case Presentation

Abstract

Introduction We detail the presentation and diagnostic workup of a woman who was ultimately diagnosed with Amyloid Beta‐Related Angiitis (ABRA), which can be a challenging condition to diagnose. Methods This is a case report which describes the presentation and workup that led to the diagnosis of ABRA. Results 64‐year‐old woman with history of hypertension, hypothyroidism, hyperlipidemia and diabetes mellitus presented to outside hospital with progressive encephalopathy and worsening expressive aphasia for three months who was transferred to our center for a left frontal lobe mass with vasogenic edema. MRI brain with and without gadolinium showed multiple small enhancing cerebral regions, diffuse swelling, and leptomeningeal enhancement. Lumbar puncture was within normal limits. Cytology and CA 19‐9, CEA, and alpha‐fetoprotein were all normal. CT thorax, abdomen, and pelvis with contrast did not reveal findings suspect for malignancy. Given diagnostic uncertainty, brain biopsy of the left frontal lesion was completed. Findings were consistent with amyloid beta deposition in the walls and concerning for an inflammatory angiitis, without features to suggest infectious or malignancy related process. Vasculitic labs were notable for positive ANA (1:160), speckled pattern, but were otherwise negative. She completed a five day course of methylprednisolone 1g IV with some improvement in encephalopathy and aphasia. She was started on a prednisone taper and cyclophosphamide and discharged to a skilled nursing facility with follow up with vascular neurology and neuroimmunology planned. Conclusion ABRA is a complication seen in cerebral amyloid angiopathy and is a rare cause of CNS vasculitis. This condition should be suspected in the appropriate clinical scenario, particularly given the improvement often noted with immunosuppression (1).