[Two Different Courses of a Rare, Potentially Reversible Encephalopathy]

Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare form of cerebral amyloid angiopathy (CAA). This leads to amyloid deposits and inflammation in the vessel walls with cerebral microbleeds, white matter hyperintensities and edema. Major clinical manifestations vary and are non-specific. They include rapidly progressive dementia, headaches and epileptic seizures. MRI and histology are crucial for further diagnosis, although the definitive diagnosis can only be made in autopsy. Timely therapy, mainly with corticosteroids, is essential for successful healing.