The case

A 41-year-old woman was diagnosed with Susac’s syndrome in 2012, having presented with confusion, headache, hearing loss and retinal arteriolar occlusions. Her MR scan of the brain showed several T2 hyperintensities in the corpus callosum and she was treated with cyclophosphamide.

She was admitted 3 years later with a 4-week history of cognitive deterioration and visual loss, but with no new MR brain scan changes. Her Montreal Cognitive Assessment (MoCA) had fallen from a baseline of 22/30 to 16/30, while her Snellen central visual acuity had fallen from 6/6 bilaterally to 6/24 in the right eye and 6/36 in the left eye. Retinal photography and fundus fluorescein angiography showed bilateral, superotemporal branch retinal artery occlusions with macular ischaemia (figures 1 and 2).