Case of Cerebral Amyloid Angiopathy-Related Inflammation – Is the Absence of Cerebral Microbleeds A Good Prognostic Sign?

Abstract

The aim of this study is to describe a case of pathologically proven cerebral amyloid angiopathy-related inflammation (CAA-I) without cerebral microbleeds (CMBs) and its clinical course. CAA-I is an uncommon variant of cerebral amyloid angiopathy. Keys to diagnosis rely on the physician's awareness of this entity, CMBs on magnetic resonance imaging (MRI), an often favorable response to immunosuppression, and ultimately brain biopsy. CAA-I with no CMBs is rarely reported. A 76-year-old woman presented with 4 weeks of headaches and was found to have visual neglect on the left part of the visual field. MRI of the brain showed sulcal/gyriform hyperintensity with associated leptomeningeal enhancement in the right occipital lobe on fluid-attenuated inversion recovery (FLAIR) imaging. No CMBs or large parenchymal FLAIR lesions were seen on MRI. Biopsy was consistent with CAA-I. The patient's headaches resolved spontaneously and no immunosuppression was initiated. The patient remained asymptomatic for the 18 months of follow-up. To the best of our knowledge, there has been only one previous case of pathology-proven CAA-I without CMBs reported and this was associated with a good prognosis. Lack of CMBs and/or large parenchymal FLAIR lesions may be a prognostic factor in this disease.