We describe a rare combination of multiple multiple branch retinal arteriolar occlusions (BRAO) and encephalopathy, which developed in an adult male 10 days after smallpox vaccination. A 53-year-old physician presented with sudden temporal visual field loss in the left eye. Ten days earlier he had received a smallpox vaccination. Hyperbaric oxygen was administered because of suspected BRAO. Examination on day 3 revealed a decrease in visual acuity and loss of visual field. Fundoscopy of the left eye disclosed a retinal infarct in the inferior macula and multiple cotton wool spots over the entire posterior pole. Fluorescein angiography showed delayed arterial filling and multiple non-perfused areas. Magnetic resonance imaging of the brain disclosed white matter focal lesions. Neurological examination was normal. Audiological examination revealed mild hearing loss caused by an old phonic trauma. A combination of intravenous methylprednisolone and immunoglobulins, recommended for the treatment of Susac syndrome, was administered. At the follow-up visit 3 months later, visual acuity in the left eye improved, fundoscopy showed resolution of the cotton wool spots and temporal disc pallor, and the visual field was substantially restored.

Landa, Marcovich, Leiba, Springer, Bukelman, Pollack, , , (2006). Multiple branch retinal arteriolar occlusions associated with smallpox vaccination. The Journal of infection, 2006 Jan;52(1):e7-9. https://www.ncbi.nlm.nih.gov/pubmed/15936086