Case ReportACUTE MACULAR NEURORETINOPATHY IN SUSAC SYNDROME: A NEW ASSOCIATIONYang, Yong-Sheng MD; Zhang, Lily BS; Asdaghi, Negar MD; Henry, Christopher R. MD; Davis, Janet L. MDAuthor Information Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida. Reprint requests: Janet L. Davis, MD, Bascom Palmer Eye Institute, 900 NW 17th Street, Miami, FL 33136; e-mail: [email protected] Supported by Research to Prevent Blindness Unrestricted Grant. None of the authors has any financial/conflicting interests to disclose. Retinal Cases & Brief Reports: Fall 2020 - Volume 14 - Issue 4 - p 310-314 doi: 10.1097/ICB.0000000000000738 Buy Metrics AbstractIn Brief Purpose: To report a case of acute macular neuroretinopathy in a patient with Susac syndrome. Methods: Case report. Results: A 39-year-old male patient presented with severe headache, photopsias, and a sudden onset of hearing loss in the right ear. Fluorescein angiography of the right eye revealed multiple branch retinal artery occlusions. Clinical presentation of encephalopathy, hearing loss, and branch retinal artery occlusions, along with characteristic magnetic resonance imaging findings, led to a diagnosis of Susac syndrome. Despite aggressive immunosuppression for four months, the patient later presented with acute macular neuroretinopathy in the left eye. Conclusion: Acute macular neuroretinopathy and Susac is a new association of two well-defined disorders. The concurrence of both disorders supports retinal ischemia as the proximate cause of acute macular neuroretinopathy and inflammation as a potential etiology. Acute macular neuroretinopathy is conceptualized as ischemia in the watershed zone of the retinal deep capillary plexus. Acute macular neuroretinopathy in Susac syndrome, an autoimmune small-vessel vasculitis that causes ischemia in the brain and eye, is a new association that supports retinal ischemia as the proximate cause of acute macular neuroretinopathy.