Probable Cerebral Amyloid Angiopathy-Related Inflammation With Spontaneous Remission. A Rare Presentation of a Rare Disease.

Abstract

Cerebral Amyloid Angiopathy-related inflammation (CAA-ri) is a rare condition caused by an inflammatory response against perivascular β-amyloid deposits. CAA-ri with spontaneous remission has been described in a few cases. We report a 76-year-old man who presented with headache and confusion. No neurological deficit was evidenced on physical examination. Brain MRI showed asymmetric white matter hyperintensities with leptomeningeal enhancement and multiple cortical microbleeds, compatible with probable CAA-ri. One month later, brain MRI showed complete resolution of the white matter abnormalities without treatment. This case suggests that the course of CAA-ri may be self-limited. Furthermore, we conducted a review of previously reported cases. This allows physicians to know this entity, properly rule out differential diagnoses, and recognize findings in imaging studies.