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- W2894968785 abstract "Susac syndrome is a rare disease involving a triad of subacute encephalopathy, vision loss involving branch retinal artery occlusions (BRAOs), and sensorineural hearing loss. Clinical presentation is variable and generally does not involve the entire triad. The suspected pathophysiology is an immune-mediated endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear. MRI of the brain reveals abnormal white matter lesions similar to demyelinating disease, but with distinct characteristic central callosal lesions, as well as deep gray matter and leptomeningeal involvement. There is no standardized therapy for Susac’s, but suspicion of an immune-mediated endotheliopathy has informed empirical treatment with corticosteroids and various immunosuppressant regimens, which appear to improve the prognosis." @default.
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- W2894968785 date "2018-08-01" @default.
- W2894968785 modified "2023-10-16" @default.
- W2894968785 title "The Snowball Effect" @default.
- W2894968785 doi "https://doi.org/10.1093/med/9780190693190.003.0010" @default.
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