Journal
NEUROLOGICAL SCIENCES
Volume 30, Issue 1, Pages 59-64Publisher
SPRINGER
DOI: 10.1007/s10072-008-0004-8
Keywords
Susac's syndrome; Branch retinal artery occlusion; Sensorineural hearing loss; Encephalopathy
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We report a series of four patients with Susac's syndrome, which is characterized by the triad of visual loss due to branch retinal artery occlusions, sensorineural hearing loss due to cochlear involvement, and encephalopathy due to cerebral microangiopathy. However, as we describe in this series, the clinical triad may not be apparent for years, resulting in delays in diagnosis. We also report the variable cerebrospinal fluid and brain magnetic resonance imaging findings, and treatment using a combination of steroids and intravenous immunoglobulin, followed by mycophenolate mofetil.
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