| Summary: | Purpose: To report a fatal case of Susac syndrome in a 24-year-old female. Observations: A 24-year-old female presented with progressive encephalopathy of unknown etiology. Her previous evaluation consisted of laboratory testing, imaging, and a brain biopsy to investigate for infectious and rheumatologic diseases. Several months after onset of symptoms, she underwent ophthalmic examination, which demonstrated bilateral branch retinal artery occlusions. Further review of her medical record revealed a recent history of hearing loss. Based on the retinal and systemic findings, the patient was diagnosed with Susac syndrome. The patient was started on intensive immunosuppression; however, she became more obtunded and succumbed several months after her diagnosis. Conclusions and importance: The timely and accurate diagnosis of Susac syndrome, which classically manifests as the triad of encephalopathy, vestibulocochlear abnormalities, and retinal arteriolar occlusions, may help to reduce the morbidity of invasive testing and to prevent fatality Keywords: Susac syndrome, Retinal artery occlusion, Encephalopathy
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