title: Cauda equina involvement in Susac's 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.
authors.affiliation: Department of Radiology, Brigham and Women's Hospital, Boston, MA, United States.
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Allmendinger
authors.affiliation: Departments of Neurology and Ophthalmology, University of Pennsylvania, Philadelphia, PA, United States. Electronic address: robert.mallery@uphs.upenn.edu.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Mallery
authors.affiliation: Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, New York, NY, United States.
authors.forename: Cynthia M
authors.initials: CM
authors.lastname: Magro
authors.affiliation: Department of Neurology, Brigham and Women's Hospital, Boston, MA, United States.
authors.forename: Nancy
authors.initials: N
authors.lastname: Wang
authors.affiliation: Oregon Neurology, Tualatin, OR, United States.
authors.forename: Robert A
authors.initials: RA
authors.lastname: Egan
authors.affiliation: Department of Neurology, Brigham and Women's Hospital, Boston, MA, United States.
authors.forename: Martin A
authors.initials: MA
authors.lastname: Samuels
authors.affiliation: Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, MA, United States.
authors.forename: Alison
authors.initials: A
authors.lastname: Callahan
authors.affiliation: Department of Radiology, Brigham and Women's Hospital, Boston, MA, United States.
authors.forename: Narayan
authors.initials: N
authors.lastname: Viswanadhan
authors.affiliation: Department of Radiology, Brigham and Women's Hospital, Boston, MA, United States.
authors.forename: Roman A
authors.initials: RA
authors.lastname: Klufas
authors.affiliation: Department of Radiology, Brigham and Women's Hospital, Boston, MA, United States.
authors.forename: Liangge
authors.initials: L
authors.lastname: Hsu
authors.affiliation: Department of Neurology, Brigham and Women's Hospital, Boston, MA, United States.
authors.forename: Sashank
authors.initials: S
authors.lastname: Prasad
mesh_terms: D002420:Cauda Equina / Q000473:pathology*; D003337:Corpus Callosum / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000209:etiology*; D013116:Spinal Cord; D055955:Susac Syndrome / Q000150:complications* / Q000175:diagnosis*
keywords: Antiendothelial cell antibodies; Cauda equina syndrome; Corpus callosum; Endotheliopathy; MRI; Microangiopathy; Retinal artery occlusion; Susac's syndrome