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A 54-year-old man had a 12 month history of weakness of both lower limbs with urge urinary incontinence, 6 month history of visual loss and hearing impairment and a 1 month history of cognitive dysfunction. He had no constitutional symptoms. On examination, his visual acuity was reduced to perception of light only in both eyes; funduscopy identified branch retinal artery occlusion (figure 1). He had spastic weakness and hyperreflexia in the lower limbs. Pure-tone audiometry suggested severe sensorineural hearing loss. MR scan of the brain showed characteristic features of Susac’s syndrome (figure 2). Following high-dose methylprednisolone pulses, intravenous immunoglobulin and rituximab, …
Contributors AE conceived the report and was involved in diagnosis and management of the case and in writing the manuscript. AK was involved in diagnosis and in writing the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally reviewed by Alasdair Coles, Cambridge, UK.