SUSAC SYNDROME.
OBJECTIVE
To describe an atypical presentation of Susac syndrome.


METHODS
Observational case report.


RESULTS
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.


CONCLUSIONS
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.
Kevin H
KH
Patel
Sara J
SJ
Haug
Richard K
RK
Imes
Emmett T
ET
Cunningham
H Richard
HR
McDonald
D000328:Adult; D001167:Arteritis / Q000175:diagnosis; D006801:Humans; D008297:Male; D015356:Retinal Artery Occlusion / Q000175:diagnosis; D055955:Susac Syndrome / Q000175:diagnosis*; D014786:Vision Disorders / Q000175:diagnosis*