Cerebral Amyloid Angiopathy‐Related Inflammation With Abnormal Higher‐Order Visual Dysfunctions

70-year-old white male hypertensive with a 2-week history of headaches and visual disturbances with bilateral visual acuity of 20/100, visual object agnosia, prosopagnosia, simultanagnosia, alexia, achromatopsia, and topographic disorientation. No visual field defects. Neurological examination otherwise unremarkable. Metabolic lab work-up was unremarkable. APOE genotype was heterozygous ε4/ε2. Brain MRI GRE revealed multiple hypointense lesions on bilateral occipital-parietal–temporal regions and hyperintensity on FLAIR (Figure 1). Prednisone significantly improved all higher-order visual deficits. Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare disorder caused by the deposition of β-amyloid peptides with an inflammatory response on the walls of intracranial microvessels responsive to immunotherapy. Probable CAA-ri was diagnosed based on a new criteria: 1. age ≥ 40 years, 2. subacute presentation of high-order visual neurological dysfunction, 3. FLAIR MRI demonstrating subcortical areas of white matter disease lesions, mostly asymmetric, 4. GERD MRI demonstrating cortical–subcortical microbleeds, and 5. no better explanation [1]. APOE ε4, especially homozygous ε4ε4, and ε2 increases the risk for CAA-ri [2]. CAA-ri was linked to prosopagnosia [3], and most recently to simultanagnosia [4]. However, our complex high-order visual dysfunctions presentation remains unpublished. Most high-order visual dysfunctions resulted from damage to the occipital-temporal structures, but simultanagnosia resulted from occipital-parietal damage. In summary, CAA-ri should be considered in any acute/subacute presentation of high-order visual dysfunctions. The authors have nothing to report. The authors have nothing to report. The authors have nothing to report. The authors confirm that they have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. Patient signed a consent for publication that is included in the submitted additional information. The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.