Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an uncommon but treatable cause of rapidly progressive dementia (RPD). Unlike prion or degenerative dementias, it can show partial reversibility with immunotherapy. A 68-year-old man developed 3 months of fluctuating cognitive decline with disorientation, behavioural disturbances and memory disturbances. MRI revealed periventricular white matter hyperintensities and multiple cortical microbleeds, consistent with probable CAA-ri. He improved after intravenous methyl-prednisolone, with improvement in memory and behavioural domains, though minor memory deficits persisted. CAA-ri should be considered in elderly patients with relapsing RPD. Recognition of its characteristic MRI features enables timely immunotherapy, which may stabilize or partially reverse cognitive decline.
Lakhanpal et al. (Mon,) studied this question.