SUSAC SYNDROME

Retinal Cases & Brief Reports
Kevin H PatelH Richard McDonald

Abstract

To describe an atypical presentation of Susac syndrome. Observational case report. A 44-year-old man with no significant medical history presented with inferonasal visual field loss in his left eye of several months of duration. He was found to have bilateral migratory arteritis with focal areas of arteriolar occlusion in both eyes and peripheral ischemia superotemporally in his left eye. An extensive hematologic workup was negative for autoimmune disease or coagulopathy. Magnetic resonance imaging with contrast of his brain revealed a hyperintense lesion in the splenium of the corpus callosum. Auditory testing was significant for nonspecific high-frequency hearing loss in the right ear. Given the full clinical picture, a diagnosis of Susac syndrome was made. Susac syndrome is a multisystemic, immune-mediated occlusive endotheliopathy characterized by the clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. However, patients may present with varying degrees of this triad; thus, there should be a high index of suspicion in patients presenting with multiple artery occlusions or multifocal arteritis.

References

Mar 1, 1979·Neurology·J O SusacJ B Selhorst
Mar 26, 2003·American Journal of Ophthalmology·Robert A EganJohn O Susac
Dec 25, 2003·Neurology·J O SusacR B Daroff
Feb 28, 2007·Journal of the Neurological Sciences·Robert M Rennebohm, John O Susac
Oct 1, 2010·Journal of the Neurological Sciences·Robert A EganJohn O Susac
Sep 8, 2011·Current Opinion in Ophthalmology·Ratna K Bitra, Eric Eggenberger
May 30, 2012·Journal of the Neurological Sciences·I KleffnerE B Ringelstein
May 1, 2013·Nature Reviews. Neurology·Jan DörrIlka Kleffner

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