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40
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Text
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<a href="http://doi.org/10.1080/01913123.2019.1692117" target="_blank" rel="noreferrer noopener">http://doi.org/10.1080/01913123.2019.1692117</a>
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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
1-8
ISSN
1521-0758
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Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Brain microvascular pathology in Susac syndrome: an electron microscopic study of five cases
Publisher
An entity responsible for making the resource available
Ultrastructural Pathology
Date
A point or period of time associated with an event in the lifecycle of the resource
2019
2019-11-16
Subject
The topic of the resource
hearing loss; Susac syndrome; electron microscopy; encephalopathy; branch retinal artery occlusion; cerebral vasculitis; corpus callosal lesions
Creator
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Agamanolis Dimitri P; Prayson Richard A; Asdaghi Negar; Gultekin Sakir H; Bigley Kim; Rennebohm Robert M
Description
An account of the resource
Susac syndrome is a rare, immune-mediated disease characterized by encephalopathy, branch retinal artery occlusion, and hearing loss. Herein, we describe the electron microscopic findings of three brain biopsies and two brain autopsies performed on five patients whose working clinical diagnosis was Susac syndrome. In all five cases, the key findings were basement membrane thickening and collagen deposition in the perivascular space involving small vessels and leading to thickening of vessel walls, narrowing, and vascular occlusion. These findings indicate that Susac syndrome is a microvascular disease. Mononuclear cells were present in the perivascular space, underlining the inflammatory nature of the pathology. Though nonspecific, the changes can be distinguished from genetic and acquired small vessel diseases. The encephalopathy of Susac syndrome overlaps clinically with degenerative and infectious conditions, and brain biopsy may be used for its diagnosis. Its vascular etiology may not be obvious on light microscopy, and electron microscopy is important for its confirmation.
Identifier
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<a href="http://doi.org/10.1080/01913123.2019.1692117" target="_blank" rel="noreferrer noopener">10.1080/01913123.2019.1692117</a>
PMID: 31736417
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Journal Article
2019
Agamanolis Dimitri P
Akron Children's Hospital
Asdaghi Negar
Bigley Kim
branch retinal artery occlusion
Cerebral vasculitis
corpus callosal lesions
Department of Pathology
electron microscopy
Encephalopathy
Gultekin Sakir H
Hearing Loss
Journal Article
NEOMED College of Medicine
November 2019 Update
Prayson Richard A
Rennebohm Robert M
Susac syndrome
Ultrastructural pathology
-
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
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
A point or period of time associated with an event in the lifecycle of the resource
2019
2019-06
Subject
The topic of the resource
Cerebral vasculitis; Deafness; Encephalopathy; Susac syndrome; Visual loss
Creator
An entity primarily responsible for making the resource
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
An unambiguous reference to the resource within a given context
<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
-
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/j.pediatrneurol.2014.07.032" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.pediatrneurol.2014.07.032</a>
Pages
717–720
Issue
5
Volume
51
Dublin Core
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Title
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Use of next-generation sequencing as a diagnostic tool for congenital myasthenic syndrome.
Publisher
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Pediatric neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2014
2014-11
Subject
The topic of the resource
congenital myasthenic syndrome; Congenital/*genetics/therapy; Continuous Positive Airway Pressure/methods; Humans; Infant; Male; Muscle Proteins/*genetics; Mutation/*genetics; Myasthenic Syndromes; next-generation sequencing; pyridostigmine; rapsyn
Creator
An entity primarily responsible for making the resource
Das Alvin S; Agamanolis Dimitri P; Cohen Bruce H
Description
An account of the resource
BACKGROUND: The clinical presentation of congenital myasthenic syndromes is similar to many other neuromuscular disorders of infancy, and with 12 known discrete genetic forms of congenital myasthenic syndromes, both the diagnosis and treatment decisions present clinical challenges. PATIENT DESCRIPTION: We report a
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.pediatrneurol.2014.07.032" target="_blank" rel="noreferrer noopener">10.1016/j.pediatrneurol.2014.07.032</a>
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Article information provided for research and reference use only. All rights are retained by the journal listed under publisher and/or the creator(s).
2014
Agamanolis Dimitri P
Cohen Bruce H
congenital myasthenic syndrome
Congenital/*genetics/therapy
Continuous Positive Airway Pressure/methods
Das Alvin S
Humans
Infant
Male
Muscle Proteins/*genetics
Mutation/*genetics
Myasthenic Syndromes
next-generation sequencing
Pediatric neurology
pyridostigmine
rapsyn