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Susac Syndrome

*Corresponding author for this work
Research Output:
Chapter in Book/Report/Conference proceeding
Chapter
Peer-review

Publication Information

Output type

Research Output:
Chapter in Book/Report/Conference proceeding
Chapter
Peer-review

Host publication Subtitle

Systemic and Organ-specific Diseases, Second Edition

Original language

English

Pages from-to (Number of pages)

Pages 167-171 (5 pages)

Publication milestones

  • Published - 01/01/2025

Publication status

Published - 01/01/2025

Publisher

Springer Nature, United States
9783031698941

ISBN (Electronic)

9783031698958

Publication IDs

  • Scopus: 105002537274

Host publication title

Autoimmune Disease Diagnosis

Abstract

The Susac syndrome is a microangiopathy causing small infarcts in the brain, cochlea, and retina. It is characterized by the clinical triad of acute or subacute encephalopathy, sensorineural hearing loss, and retinal branch artery occlusions. This syndrome is probably an immune-mediated endotheliopathy that affects these organs’ microvasculature. The definite diagnosis is made by the presence of the triad of brain, retinal, and vestibulocochlear involvements. If only two domains are affected, a probable diagnosis can be proposed. Treatment of this condition includes immunosuppression with high-dose steroids and intravenous immunoglobulin, and in some cases cytotoxic drugs.