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.
MeSH terms
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Adult
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Brain / blood supply
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Brain / pathology
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Cerebral Arteries / pathology
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Cerebral Arteries / physiopathology
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Cerebrovascular Disorders / cerebrospinal fluid
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Cerebrovascular Disorders / pathology*
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Cerebrovascular Disorders / physiopathology
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Cochlea / blood supply
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Cochlea / physiopathology
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Disease Progression
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Endothelial Cells / pathology
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Female
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Hearing Loss, Sensorineural / cerebrospinal fluid
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Hearing Loss, Sensorineural / pathology*
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Hearing Loss, Sensorineural / physiopathology
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Humans
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Immunoglobulins, Intravenous / therapeutic use
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Immunosuppressive Agents / therapeutic use
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Magnetic Resonance Imaging
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Male
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Retinal Artery / pathology
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Retinal Artery / physiopathology
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Retinal Artery Occlusion / cerebrospinal fluid
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Retinal Artery Occlusion / pathology*
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Retinal Artery Occlusion / physiopathology
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Steroids / therapeutic use
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Syndrome
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Treatment Outcome
Substances
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Immunoglobulins, Intravenous
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Immunosuppressive Agents
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Steroids