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Susac Syndrome — SCE Neurology MCQ

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HardCNS InfectionsSusac SyndromeSCE Neurology

A 47-year-old develops encephalopathy and new migraine-like headaches. MRI shows central corpus-callosum “snowball” lesions. Fluorescein angiography identifies branch retinal artery occlusions and audiometry shows bilateral low-frequency sensorineural hearing loss. Which diagnosis best unifies the findings?

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Correct answer: E — Susac syndrome

The encephalopathy–retinopathy–hearing-loss triad with characteristic central corpus-callosum lesions is Susac syndrome, a retinal, cochlear and cerebral microangiopathy. MS can produce white-matter lesions but does not explain branch retinal artery occlusions and the characteristic low-frequency hearing loss. CADASIL, CNS vasculitis and neurosarcoidosis have different imaging and systemic patterns.

Reference: European Susac Consortium diagnostic criteria: https://pubmed.ncbi.nlm.nih.gov/28103199/