Neuropathological Findings in Susac Syndrome: An Autopsy Report

J Neuropathol Exp Neurol. 2019 Jun 1;78(6):515-519. doi: 10.1093/jnen/nlz031.


A 24-year-old woman developed encephalopathy, branch retinal artery occlusion, hearing loss, and had "snowball" lesions in the corpus callosum, classic findings of Susac syndrome (SuS). Despite intensive immunosuppressive therapy, she lapsed into a coma, and died 7 months after the onset of her illness. Neuropathological examination, revealed perivascular inflammation and vasculitis involving small vessels, associated with vascular narrowing and occlusion, and numerous microinfarcts diffusely throughout the brain. The findings establish SuS as a neuroinflammatory condition that can include vasculitis. This represents the most comprehensive report of the neuropathological findings in SuS.

Keywords: Cerebral vasculitis; Deafness; Encephalopathy; Susac syndrome; Visual loss.

Publication types

  • Case Reports

MeSH terms

  • Autopsy
  • Blood Vessels / pathology
  • Brain / diagnostic imaging
  • Brain / pathology
  • Cerebral Infarction / pathology
  • Coma / etiology
  • Eye / pathology
  • Fatal Outcome
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Magnetic Resonance Imaging
  • Susac Syndrome / drug therapy
  • Susac Syndrome / pathology*
  • Treatment Failure
  • Vasculitis / pathology
  • Young Adult


  • Immunosuppressive Agents