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Progressive multifocal leukoencephalopathy — SCE Neurology MCQ

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HardCNS InfectionsProgressive multifocal leukoencephalopathySCE Neurology

A 38-year-old has subacute encephalopathy and low-frequency sensorineural hearing loss. MRI shows central corpus-callosum “snowballs”, but she denies visual symptoms and fundoscopy is normal. Which investigation most directly seeks the clinically silent third component of the suspected syndrome?

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Correct answer: BWide-field retinal fluorescein angiography for branch occlusion or arterial-wall hyperfluorescence

Explanation lettering: E = shown as A · A = shown as B · D = shown as C · C = shown as D · B = shown as E

A is correct: Susac syndrome is a brain–retina–cochlea microangiopathy, and fluorescein angiography can demonstrate branch retinal artery occlusions or arterial-wall hyperfluorescence despite absent visual symptoms and normal routine fundoscopy. B and C investigate optic-nerve or visual-pathway disorders rather than retinal microangiopathy. D tests generalized photoreceptor function. E usually misses this exclusively small-vessel retinal process. The central rather than callosal-undersurface lesion distribution also argues against typical multiple sclerosis.

Reference: Clinical Characterization and Ancillary Tests in Susac Syndrome: https://www.neurology.org/doi/10.1212/NXI.0000000000200209