Document Detail

Cauda equina involvement in Susac's syndrome.
MedLine Citation:
PMID:  24290499     Owner:  NLM     Status:  Publisher    
Susac's syndrome is a rare autoimmune microangiopathy characterized by the clinical triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss. In many cases, the clinical triad is not fully present at the onset of symptoms. MRI studies often show characteristic punched out lesions of the central fibers of the corpus callosum, and leptomeningeal enhancement and deep gray matter lesions may also be seen. Here we present a case of Susac's syndrome in a middle aged man with the unique clinical finding of cauda equina syndrome and spinal MRI showing diffuse lumbosacral nerve root enhancement. Biopsy specimens of the brain, leptomeninges, and skin showed evidence of a pauci-immune endotheliopathy, consistent with pathology described in previous cases of Susac's syndrome. This case is important not only because it expands the clinical features of Susac's syndrome but also because it clarifies the mechanism of a disorder of the endothelium, an important target for many disorders of the nervous system.
Andrew M Allmendinger; Robert M Mallery; Cynthia M Magro; Nancy Wang; Robert A Egan; Martin A Samuels; Alison Callahan; Narayan Viswanadhan; Roman A Klufas; Liangge Hsu; Sashank Prasad
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-11-22
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  -     ISSN:  1878-5883     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  2013 Nov 
Date Detail:
Created Date:  2013-12-2     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2013 Elsevier B.V. All rights reserved.
Department of Radiology, Brigham and Women's Hospital, Boston, MA, United States.
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