| Susac's syndrome, a rare, potentially severe or lethal neurological disease. | |
| | |
MedLine Citation:
|
PMID: 20723912 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Susac's syndrome (SS) is a rare, immune-mediated endotheliopathy affecting the microvasculature of the brain, the inner ear and the retina. Clinical presentation is characterised by a triad: encephalopathy, hearing loss and branch retinal artery occlusion (BRAO). Given the rarity of this disease, its natural history still remains partially unknown, but lethal cases appear to be extremely rare since there has never been, to our knowledge, a report of SS leading to death. We report 2 cases of SS illustrating the multiplicity of neurological symptomatology and its unpredictable course. One case is particularly unusual due to its severe neurological evolution, leading to death despite treatments. This report presents clinical and paraclinical findings contributory to SS diagnosis and offers an innovative perspective on disease management. These cases represent the potential severity of this disease. Early, aggressive treatment strategies may be warranted for SS in order to avoid neurological deterioration and lethal evolution. |
| | |
Authors:
|
A Saux; G Niango; M Charif; R Morales; F Mura; A Bonafe; I Mourand |
Publication Detail:
|
Type: Case Reports; Journal Article Date: 2010-08-17 |
Journal Detail:
|
Title: Journal of the neurological sciences Volume: 297 ISSN: 1878-5883 ISO Abbreviation: J. Neurol. Sci. Publication Date: 2010 Oct |
Date Detail:
|
Created Date: 2010-09-13 Completed Date: 2010-12-28 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 0375403 Medline TA: J Neurol Sci Country: Netherlands |
Other Details:
|
Languages: eng Pagination: 71-3 Citation Subset: IM |
Copyright Information:
|
Copyright 2010 Elsevier B.V. All rights reserved. |
Affiliation:
|
Department of Neurology, Gui de Chauliac Hospital, Avenue Auguste Fliche, 34295, Montpellier, France. a-saux@chu-montpellier.fr |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Brain
/
pathology* Female Humans Magnetic Resonance Imaging / methods Middle Aged Susac Syndrome / diagnosis* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Comorbidities in pediatric patients with postural orthostatic tachycardia syndrome.
Next Document: Periodontitis affects neurological deficit in acute stroke.