To illustrate the clinical characteristics, radiographic features, and treatment course of patients with cerebral amyloid angiopathy (CAA) that may raise red flags of possible progression to amyloid beta related angiopathy (ABRA) or CAA-related inflmmation (CAA-RI).

We identified 12 patients with the diagnosis of ABRA or CAA-RI seen in our institution between 2011 and 2019. Demographics, clinical presentation, MRI imaging, and follow up were obtained.

Ages ranged between 55-86 years with 7 men and 5 women. Clinical presentations included: rapid decline in memory or confusion in 6, headaches in 5, behavioral changes in 3, symptomatic intracerebral hemorrhages (ICH) in 3, imbalance or fall in 2, seizures in 2, chronic memory decline in 2 and transient ischemic attack, aphasia and incidentally found in 1. Radiographic features included, vasogenic edema, ICH, multifocal punctate infarcts and/or leptomeningeal enhancement. Three patients had a prior MRI; of those 5, 6, and 13 years passed until discovery of inflammatory changes on MRI. Of those 9 patients presenting with inflammation without prior MRI, only one had prior symptomatic ICH. Nine patients received steroids, with significant clinical and/or radiographic improvement in 8; 1 patient also warranted an immunosuppressant for inflammation recurrence. Of the remaining 3, 2 were asymptomatic and not treated and 1 had spontaneous improvement without treatment.