Synonyms:
Retinocochleocerebral vasculopathy
Diagnosis:
Clinical:
- Encephalopathy (including psychosis), monocular vision loss, hearing loss
- Headache
- Branch retinal artery occlusion
MRI:
- T2 hyperintensity in basal ganglia, thalamus, white matter (almost always involving corpus callosum). May involve brainstem & cerebellum
- T1 +contrast: leptomeningeal enhancement & parenchymal enhancement
Ophthalmic fluorescein angiography:
- Retinal vasculitis: narrowing, occlusion, vessel wall hyperfluorescence, contrast outflow
Audiogram:
- Sensorineural hearing loss
Angiogram/angiography:
- Normal as the affect vessels are very small <100 micrometer
Pathology, brain biopsy:
Microangiopathy in cortex & white matter:
- Infarcts, Capillary hyaline thrombi, Perivascular inflammatory cells