A review and update on the ophthalmic implications of Susac syndrome

Surv Ophthalmol. 2019 Jul-Aug;64(4):477-485. doi: 10.1016/j.survophthal.2019.01.007. Epub 2019 Jan 29.


Susac syndrome is a rare condition presumed to be immune-mediated occlusion of small arterial vasculature principally of the brain, inner ear, and retina. Clinically, the syndrome manifests as a pathognomonic triad of encephalopathy, hearing loss, and branch retinal artery occlusion. Early recognition and diagnosis is important as delayed treatment may be profound and result in deafness, blindness, dementia, and other neurological deficits. The plethora of imaging technology, including magnetic resonance imaging, retinal fluorescein angiography, optical coherence tomography, and optical coherence tomography angiography, allows deeper and more discrete anatomical-physiological correlation of underlying pathology, early diagnosis, and imaging biomarkers for early detection of relapse during follow-up. We highlight the current clinical classification of Susac syndrome, available investigations, treatment, and care pathways.

Keywords: OCT angiography; Susac's syndrome; branch retinal artery occlusion; fluorescein angiography; treatment.

Publication types

  • Review

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use
  • Diagnosis, Differential
  • Diagnostic Techniques, Ophthalmological
  • Fluorescein Angiography
  • Humans
  • Immunologic Factors / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Imaging
  • Retinal Artery Occlusion / diagnosis
  • Retinal Artery Occlusion / drug therapy
  • Retinal Artery Occlusion / etiology*
  • Susac Syndrome / complications*
  • Susac Syndrome / drug therapy
  • Tomography, Optical Coherence
  • Vision Disorders / diagnosis
  • Vision Disorders / etiology*


  • Anti-Inflammatory Agents
  • Immunologic Factors
  • Immunosuppressive Agents