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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 25:382-388, March 2004
© 2004 American Society of Neuroradiology

Case Report
BRAIN

Susac Syndrome: Report of Four Cases and Review of the Literature

Tai Hung Do^a, Christiane Fisch^a and François Evoy^a

^a From the Department of Radiology, CHUS—Hôpital Fleurimont, Sherbrooke, Quebec, Canada

Address correspondence to Dr. Tai Hung Do, 8550 rue Daoust, Lasalle, Quebec, Canada, H8N 2C2

Summary: Susac syndrome is a rare disease of unknown pathogenesis. It is caused by a microangiopathy affecting the arterioles of the brain, retina, and cochlea, giving the classic clinical triad of subacute encephalopathy, visual loss secondary to retinal branch occlusions, and sensorineural hearing loss. The features of four cases of this syndrome are presented. MR imaging, retinal fluorescein angiography, and audiography findings enable diagnosis. Early therapy may reduce sequelae and improve recovery.

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