title: Progressive Susac syndrome with bilateral visual loss and disability.
abstract: Susac syndrome (SS) is a rare retinal-cochlear-cerebral disease with an unclear etiology. A 35-year-old man presented with sudden painless vision loss in the right eye and 2 months later in the left eye with hemiparesis, behavioral changes, and hearing loss. Ophthalmic examinations revealed multiple branch retinal artery occlusions (BRAOs) in both eyes. Brain magnetic resonance imaging showed inflammatory changes with multiple "punched-out" lesions in the corpus callosum which confirmed the diagnosis of SS. Despite intravenous and oral corticosteroid therapy, the disease progressed with the development of new BRAOs, low vision in both eyes, and disability. Prompt diagnosis and early treatment may save the vision and even patient's life.
authors.affiliation: Department of Ophthalmology, Ophthalmic Research Center, Torfeh Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Morteza
authors.initials: M
authors.lastname: Entezari
authors.affiliation: Department of Ophthalmology, Ophthalmic Research Center, Torfeh Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Saeed
authors.initials: S
authors.lastname: Karimi
authors.affiliation: Department of Radiology, Imam Hossein Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Mohammadali
authors.initials: M
authors.lastname: Feizi
mesh_terms: D000284:Administration, Oral; D000328:Adult; D004185:Disability Evaluation; D018450:Disease Progression; D004338:Drug Combinations; D005451:Fluorescein Angiography; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007275:Injections, Intravenous; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D015356:Retinal Artery Occlusion / Q000175:diagnosis / Q000188:drug therapy; D055955:Susac Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D015354:Vision, Low / Q000175:diagnosis* / Q000188:drug therapy; D019987:Persons with Visual Disabilities
chemical_list: D004338:Drug Combinations; D005938:Glucocorticoids; D011239:Prednisolone; D008775:Methylprednisolone