Guidelines for treatment of Susac syndrome – An update

Robert M. Rennebohm, Negar Asdaghi, Sunil Srivastava, Elie Gertner

Research output: Contribution to journalReview articlepeer-review

31 Scopus citations


Susac syndrome is an immune-mediated, pauci-inflammatory, ischemia-producing, occlusive microvascular endotheliopathy/basement membranopathy that affects the brain, retina, and inner ear. Treatment of Susac syndrome is particularly challenging. The organs involved can easily become irreversibly damaged, and the window of opportunity to protect them is often short. Optimal outcome requires rapid and complete disease suppression. Adding to the challenge is the absence of objective biomarkers of disease activity and the great variability in presentation, timing and extent of peak severity, duration of peak severity, and natural disease course. There have been no randomized controlled trials or prospective treatment studies. We offer treatment guidelines based on cumulative clinical experience and a large cohort of patients followed longitudinally in a comprehensive database project. These guidelines state our preferences but do allow flexibility and discuss other options. The guidelines also serve as an initial step in the planning of prospective treatment studies, future consensus-based recommendations, and future randomized controlled trials.

Original languageEnglish (US)
Pages (from-to)484-494
Number of pages11
JournalInternational Journal of Stroke
Issue number5
StatePublished - Jul 1 2020


  • Susac syndrome
  • branch retinal artery occlusion
  • hearing loss
  • immunosuppression
  • ischemic stroke

ASJC Scopus subject areas

  • Neurology


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