Cerebral Amyloid Angiopathy-Related Inflammation (CAA-ri): Presentation at an Unusual Age

Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a less common but aggressive manifestation of CAA caused by an autoimmune reaction to the amyloid-beta (Ab) deposits in affected vessels. Here, we report the case of a 96-year-old patient, with a history of Alzheimer's disease, who presented to our hospital due to a sudden onset of high-intensity holocranial headache followed by dysarthria, left hemiplegia, and gaze deviation to the right. MRI of the brain was performed, which revealed a heterogeneous hypointense signal on the right frontal T2 and fluid-attenuated inversion recovery (FLAIR) sequences, with an asymmetric hyperintensity surrounding the lesion compatible with perilesional vasogenic edema. Given the clinical radiographic findings, a diagnosis of CAA-ri was established and immediate treatment with intravenous corticosteroids was started, with a rapid clinical response and remarkable improvement in follow-up neuroimaging.