The Storm Behind the Silence: Detecting Cerebral Amyloid Angiopathy-Related Inflammation in a Patient with Dementia

Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare, treatable inflammatory variant of cerebral amyloid angiopathy that often presents with acute or subacute cognitive or behavioral decline. We describe a 76-year-old man with frontotemporal dementia who was admitted for an episode of unresponsiveness following several weeks of worsening agitation. MRI demonstrated bifrontal vasogenic edema, cortical superficial siderosis, and microhemorrhages, consistent with CAA-RI. The patient was treated with high-dose intravenous corticosteroids, followed by a prolonged taper, with clinical improvement to his baseline mental status prior to discharge. This case underscores the importance of considering CAA-RI in older adults with preexisting cognitive impairment who develop acute mental status changes.