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Text
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URL Address
<a href="http://doi.org/10.1093/jnen/nlz031" target="_blank" rel="noreferrer noopener">http://doi.org/10.1093/jnen/nlz031</a>
Rights
Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
Pages
515-519
Issue
6
Volume
78
Dublin Core
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Title
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Neuropathological Findings in Susac Syndrome: An Autopsy Report
Publisher
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Journal of Neuropathology and Experimental Neurology
Date
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2019
2019-06
Subject
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Cerebral vasculitis; Deafness; Encephalopathy; Susac syndrome; Visual loss
Creator
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Agamanolis Dimitri P; Klonk Collin; Bigley Kim; Rennebohm Robert M
Description
An account of the resource
A 24-year-old woman developed encephalopathy, branch retinal artery occlusion, hearing loss, and had "snowball" lesions in the corpus callosum, classic findings of Susac syndrome (SuS). Despite intensive immunosuppressive therapy, she lapsed into a coma, and died 7 months after the onset of her illness. Neuropathological examination, revealed perivascular inflammation and vasculitis involving small vessels, associated with vascular narrowing and occlusion, and numerous microinfarcts diffusely throughout the brain. The findings establish SuS as a neuroinflammatory condition that can include vasculitis. This represents the most comprehensive report of the neuropathological findings in SuS.
Identifier
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<a href="http://doi.org/10.1093/jnen/nlz031" target="_blank" rel="noreferrer noopener">10.1093/jnen/nlz031</a>
2019
Agamanolis Dimitri P
Bigley Kim
Cerebral vasculitis
Deafness
Department of Pathology
Encephalopathy
Journal of Neuropathology and Experimental Neurology
June 2019 Update
Klonk Collin
NEOMED College of Medicine
Rennebohm Robert M
Susac syndrome
Visual loss