| Update on Susac's syndrome. | |
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MedLine Citation:
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PMID: 15891418 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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PURPOSE OF VIEW: We review recent developments in the clinical course and imaging modalities for Susac's syndrome, a clinical triad consisting of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. RECENT FINDINGS: Susac's syndrome has variable clinical presentations; recently described presentations include epileptic seizures and transient inverted vision. Advances in neuroradiology suggest that magnetic resonance imaging demonstrates distinctive patterns in the white and grey matter and in the leptomeninges. Reports have verified that Susac's syndrome is under-diagnosed because of its multisystem involvement and confusion with other imitating disorders (such as multiple sclerosis), and because of the fact that neuroradiologists are not acquainted with this syndrome. SUMMARY: The precise aetiology of Susac's syndrome is still unknown and many areas have not yet been explored. Magnetic resonance imaging is the neuroimaging study of choice. Findings include multiple small hyperintense foci on T2-weighted images and contrast enhancement in white and grey matter of both supratentorial and infratentorial structures, corpus callosum and, occasionally, leptomeninges. Callosal lesions typically involve the central fibres and are probably pathognomonic for Susac's syndrome. When assessing patients with unexplained encephalopathy involving white and grey matter, leptomeninges and corpus callosum, the findings of sensorinueral hearing loss or visual disturbances may yield important clues regarding the possibility of Susac's syndrome. |
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Authors:
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Menachem Gross; Ron Eliashar |
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Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: Current opinion in neurology Volume: 18 ISSN: 1350-7540 ISO Abbreviation: Curr. Opin. Neurol. Publication Date: 2005 Jun |
Date Detail:
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Created Date: 2005-05-13 Completed Date: 2005-08-09 Revised Date: 2011-01-11 |
Medline Journal Info:
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Nlm Unique ID: 9319162 Medline TA: Curr Opin Neurol Country: England |
Other Details:
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Languages: eng Pagination: 311-4 Citation Subset: IM |
Affiliation:
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Department of Otolaryngology/Head and Neck Surgery, The Hebrew University School of Medicine, Hadassah Medical Center, Jerusalem, Israel. drgrossm@hotmail.com |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Autoimmune Diseases of the Nervous System
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complications,
pathology* Brain Diseases / complications, diagnosis* Corpus Callosum / pathology Hearing Loss, Sensorineural / complications, pathology* Humans Hypertensive Encephalopathy / complications, pathology* Magnetic Resonance Imaging / methods Retinal Artery Occlusion / complications, pathology* Syndrome |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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