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Neurol Sci. 2009 Feb;30(1):59-64. doi: 10.1007/s10072-008-0004-8. Epub 2009 Jan 15.

The spectrum of Susac's syndrome.

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1
Multiple Sclerosis Center, Department of Neurology, Vanderbilt University Medical Center, 2201 Childrens Way Room 1222, Nashville, TN 37211, USA. siddharama.pawate@vanderbilt.edu

Abstract

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.

PMID:
19145401
DOI:
10.1007/s10072-008-0004-8
[Indexed for MEDLINE]
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