Headache in Susac's Syndrome

Curr Pain Headache Rep. 2021 Mar 18;25(4):25. doi: 10.1007/s11916-021-00939-2.


Purpose of review: Provide an overview of the current diagnosis, pathophysiology, and treatment of Susac's syndrome (SuS), with special emphasis on summarizing what is currently known about headache as a symptom of disease activity.

Recent findings: The most recent literature in SuS has focused on furthering the understanding of the underlying pathology and efficacy of treatments for SuS. The importance of early recognition to facilitate timely treatment and avoid long-term disability has been highlighted. Headache, the most common symptom experienced by patients with SuS, can occur up to 6 months in advance of other symptoms, and exacerbations of headache can herald increased disease activity. Susac's syndrome (SuS) is a rare disorder classically characterized by triad of encephalopathy, branch retinal artery occlusion (BRAO), and sensory neuronal hearing loss (SNHL). The full triad is uncommon at initial presentation, which can confound efforts to make timely diagnosis and treatment decisions. Headache is the most common symptom in SuS, is often an early feature, and can help separate SuS from other diagnoses in the differential. However, the features and management of the headache associated with SuS have not been systematically defined in the literature.

Keywords: Headache; Susac syndrome.

Publication types

  • Review

MeSH terms

  • Disease Progression
  • Glucocorticoids / therapeutic use
  • Headache / physiopathology*
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Factors / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Susac Syndrome / drug therapy
  • Susac Syndrome / physiopathology*


  • Glucocorticoids
  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Immunosuppressive Agents