Susac syndrome - the current review of knowledge and own experience presentation

Otolaryngol Pol. 2023 Jan 31;77(2):1-5.

Abstract

Susac syndrome is a rare autoimmune vasculopathy involving the small precapillary arterioles of the brain, retina, and inner ear. It is characterized by a triad of symptoms: encephalopathy, visual disturbances due to obstruction of retinal artery branches, and sensorineural hearing loss. The study aimed to review the current medical knowledge on Susac syndrome and present our clinical experience regarding this disease entity. The paper also presents a case of a 25-year-old patient who was diagnosed with Susac's syndrome based on the clinical picture and the results of additional tests. This syndrome should be considered in the differential diagnosis of multiple sclerosis and other multifocal lesions of the central nervous system because early diagnosis of the disease and immunosuppressive treatment significantly alleviates its course and improves the prognosis.

Keywords: Susac syndrome; autoimmune vasculopathy; encephalopathy; hearing loss; retinal occlusions.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Brain / pathology
  • Diagnosis, Differential
  • Hearing Loss, Sensorineural* / diagnosis
  • Hearing Loss, Sensorineural* / etiology
  • Humans
  • Magnetic Resonance Imaging
  • Prognosis
  • Susac Syndrome* / diagnosis
  • Susac Syndrome* / drug therapy
  • Susac Syndrome* / pathology