Background: Susac syndrome is a rare disease attributed to microangiopathy involving the arterioles of the brain, retina, and cochlea. Understanding the pathogenesis is incomplete, but an immune-mediated process remains the leading hypothesis.
Methods: Report of a single case of a previously healthy 22-year-old female patient showing the complete clinical triad.
Results: Diagnosis of Susac syndrome in this patient was first questioned due to the atypical initial ophthalmologic presentation with central retinal artery occlusion. Multiple relapses occurred in the fellow eye during follow-up, showing the typical branch retinal artery occlusions, allowing definite diagnosis.
Conclusion: Susac syndrome should be considered in the differential diagnosis when facing (young) patients with central retinal artery occlusion, especially in the presence of unexplained encephalopathy and/or sensorineural hearing loss.