title: The spectrum of Susac's syndrome.
abstract: We report a series of four patients with Susac's syndrome, which is characterized by the triad of visual loss due to branch retinal artery occlusions, sensorineural hearing loss due to cochlear involvement, and encephalopathy due to cerebral microangiopathy. However, as we describe in this series, the clinical triad may not be apparent for years, resulting in delays in diagnosis. We also report the variable cerebrospinal fluid and brain magnetic resonance imaging findings, and treatment using a combination of steroids and intravenous immunoglobulin, followed by mycophenolate mofetil.
authors.affiliation: Multiple Sclerosis Center, Department of Neurology, Vanderbilt University Medical Center, 2201 Childrens Way Room 1222, Nashville, TN 37211, USA. siddharama.pawate@vanderbilt.edu
authors.forename: Siddharama
authors.initials: S
authors.lastname: Pawate
authors.forename: Anita
authors.initials: A
authors.lastname: Agarwal
authors.forename: Harold
authors.initials: H
authors.lastname: Moses
authors.forename: Subramaniam
authors.initials: S
authors.lastname: Sriram
mesh_terms: D000328:Adult; D001921:Brain / Q000098:blood supply / Q000473:pathology; D002536:Cerebral Arteries / Q000473:pathology / Q000503:physiopathology; D002561:Cerebrovascular Disorders / Q000134:cerebrospinal fluid / Q000473:pathology* / Q000503:physiopathology; D003051:Cochlea / Q000098:blood supply / Q000503:physiopathology; D018450:Disease Progression; D042783:Endothelial Cells / Q000473:pathology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000134:cerebrospinal fluid / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D012161:Retinal Artery / Q000473:pathology / Q000503:physiopathology; D015356:Retinal Artery Occlusion / Q000134:cerebrospinal fluid / Q000473:pathology* / Q000503:physiopathology; D013256:Steroids / Q000627:therapeutic use; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D013256:Steroids