Abstract
Susac's syndrome is the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss (Susac 1994) [1]. It occurs predominantly in young females and is believed to be an immune-mediated endotheliopathy of small vessels of the brain, retina and cochlea (Neumayer et al. 2009) [2]. Early, aggressive, and sustained immunosuppressive therapy has been recommended for Susac's syndrome and anecdotal evidence has suggested a therapeutic role for monoclonal antibodies (Rennebohm et al. 2008, Lee and Amezcua 2009) [3,4]. We report a case of Susac's syndrome in which the patient improved immediately after tumour necrosis factor (TNF) inhibition with the monoclonal antibody, infliximab.
Copyright © 2010 Elsevier B.V. All rights reserved.
MeSH terms
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Anti-Inflammatory Agents / therapeutic use
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Antibodies, Monoclonal / therapeutic use*
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Azathioprine / therapeutic use
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Brain / pathology
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Cognition Disorders / etiology
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Cognition Disorders / psychology
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Cyclophosphamide / therapeutic use
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Epilepsy, Generalized / etiology
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Female
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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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Infliximab
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Magnetic Resonance Imaging
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Neuropsychological Tests
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Prednisone / therapeutic use
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Reverse Transcriptase Polymerase Chain Reaction
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Seizures / etiology
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Susac Syndrome / drug therapy*
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Susac Syndrome / psychology
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Tumor Necrosis Factor-alpha / antagonists & inhibitors*
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Young Adult
Substances
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Anti-Inflammatory Agents
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Antibodies, Monoclonal
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Immunoglobulins, Intravenous
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Immunosuppressive Agents
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Tumor Necrosis Factor-alpha
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Cyclophosphamide
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Infliximab
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Azathioprine
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Prednisone