Cauda equina involvement in Susac's syndrome.

TitleCauda equina involvement in Susac's syndrome.
Publication TypeJournal Article
Year of Publication2014
AuthorsAllmendinger AM, Mallery RM, Magro CM, Wang N, Egan RA, Samuels MA, Callahan A, Viswanadhan N, Klufas RA, Hsu L, Prasad S
JournalJ Neurol Sci
Volume337
Issue1-2
Pagination91-6
Date Published2014 Feb 15
ISSN1878-5883
KeywordsCauda Equina, Corpus Callosum, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Peripheral Nervous System Diseases, Spinal Cord, Susac Syndrome
Abstract

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.

DOI10.1016/j.jns.2013.11.023
Alternate JournalJ Neurol Sci
PubMed ID24290499
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