Neuropsychological outcome of a case of Susac syndrome: A two-year follow-up study

Appl Neuropsychol Adult. 2019 Jan-Feb;26(1):89-95. doi: 10.1080/23279095.2017.1359178. Epub 2017 Sep 18.

Abstract

Susac syndrome is a rare condition characterised by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Of the few published cases, there is variability with regard to cognitive outcome. We describe the clinical course and neuropsychological performance of a 21-year-old male patient presenting with severe encephalopathy and later developing the full triad fulfilling the diagnosis of Susac syndrome.

Keywords: Encephalopathy; Susac syndrome; neuropsychological assessment; neuropsychological assessment battery (NAB).

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cognition Disorders* / diagnosis
  • Cognition Disorders* / etiology
  • Cognition Disorders* / physiopathology
  • Humans
  • Male
  • Neuropsychological Tests
  • Susac Syndrome* / complications
  • Susac Syndrome* / diagnosis