Susac’s Syndrome: A Case Presentation and Radiological Approach to this Rare Autoimmune Endotheliopathy

Goda Randakevičienė,Rymantė Gleiznienė,Rūta Nylander

doi:10.3390/medicina49010003

Abstract

Susac's syndrome is an uncommon neurologic disorder, consisting of the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. We report a case of a sudden vision and hearing impairment in a 35-year-old female patient. Magnetic resonance imaging of the brain revealed multiple lesions in the white matter and the corpus callosum, typical of Susac's syndrome.

Highlights

Clinical CASE REportSusac’s Syndrome: A Case Presentation and Radiological Approach to this Rare Autoimmune Endotheliopathy
The clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss was first described by Susac et al in 1979: they presented a case of an unusual microangiopathy of the brain and retina and sensorineural hearing loss in 2 women
The diagnosis is difficult to make due to syndrome polymorphism, especially when the clinical triad is lacking; in most of cases, the 3 elements are not all present at the onset of the disease and may occur days, months, or even years later

Summary

Clinical CASE REport

Susac’s Syndrome: A Case Presentation and Radiological Approach to this Rare Autoimmune Endotheliopathy. Goda Randakevičienė[1], Rymantė Gleiznienė[1], Rūta Nylander2 1Department of Radiology, Medical Academy, Lithuanian University of Health Sciences, Lithuania, 2Department of Radiology, Uppsala University Hospital, Sweden. Susac’s syndrome is an uncommon neurologic disorder, consisting of the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. We report a case of a sudden vision and hearing impairment in a 35-year-old female patient. Magnetic resonance imaging of the brain revealed multiple lesions in the white matter and the corpus callosum, typical of Susac’s syndrome

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