Abstract
Susac syndrome was named after J.O. Susac who first described the syndrome in 1979.
It is characterized by the clinical triad of encephalopathy, branch retinal artery
occlusion, and sensorineural hearing loss. It mainly occurs in young women. This underdiagnosed
disease needs to be considered in the differential diagnosis of a broad variety of
disorders. In Susac syndrome, autoimmune processes leading to damage and inflammation-related
occlusion of the microvessels in brain, retina, and inner ear are thought to play
a causal role. The diagnosis is based primarily on the clinical presentation, the
documentation of branch retinal artery occlusion by fluorescence angiography, and
characteristic findings on cerebral MRI, that help in distinguishing Susac syndrome
from other inflammatory entities, like multiple sclerosis. Antiendothelial cell antibodies
could be detected in some patients. Patients are successfully treated with immunosuppression,
however, the best regimen still needs to be defined. As a result of the rarity of
the disease, controlled therapeutic trials are missing so far. In this review, we
want to demonstrate the clinical features, natural history, treatment, and clinical
course of Susac syndrome, illustrated by a typical case history.
Keywords
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Article info
Publication history
Published online: May 28, 2012
Accepted:
May 5,
2012
Received in revised form:
April 29,
2012
Received:
January 31,
2012
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
