Progressive Susac syndrome with bilateral visual loss and disability

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...

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Bibliographic Details
Published inIndian journal of ophthalmology Vol. 64; no. 9; pp. 678 - 680
Main Authors Entezari, Morteza, Karimi, Saeed, Feizi, Mohammadali
Format Journal Article
LanguageEnglish
Published India Wolters Kluwer India Pvt. Ltd 01.09.2016
Medknow Publications and Media Pvt. Ltd
Medknow Publications & Media Pvt. Ltd
Medknow Publications & Media Pvt Ltd
Wolters Kluwer Medknow Publications
Subjects
Administration, Oral
Adult
Analysis
Blindness
Branch retinal artery occlusion
Brief Communication
Care and treatment
Causes of
deafness
Diagnosis
Disability Evaluation
Disease Progression
Drug Combinations
Fluorescein Angiography
Glucocorticoids - therapeutic use
Health screening
Humans
Injections, Intravenous
Magnetic Resonance Imaging
Male
Methylprednisolone - therapeutic use
Prednisolone - therapeutic use
Retinal Artery Occlusion - diagnosis
Retinal Artery Occlusion - drug therapy
Susac syndrome
Susac Syndrome - diagnosis
Susac Syndrome - drug therapy
Vision, Low - diagnosis
Vision, Low - drug therapy
Visually Impaired Persons
India
magnetic resonance imaging
deafness
Susac syndrome
Branch retinal artery occlusion
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Summary: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.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
ObjectType-Feature-4
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ObjectType-Report-1
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ISSN:0301-4738
1998-3689
DOI:10.4103/0301-4738.194334