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Open Access Highly Accessed Case Report

Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model

Robert M Rennebohm1*, Martin Lubow2, Jerome Rusin3, Lisa Martin3, Deborah M Grzybowski2 and John O Susac4

Author Affiliations

1 Department of Pediatrics, Division of Pediatric Rheumatology, Ohio State University College of Medicine, Columbus, Ohio, USA

2 Department of Ophthalmology, Ohio State University College of Medicine, Columbus, Ohio, USA

3 Department of Radiology, Nationwide Children's Hospital, Columbus, Ohio, USA

4 Neurology and Neurosurgery, PA, Winter Haven, FL, USA

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Pediatric Rheumatology 2008, 6:3  doi:10.1186/1546-0096-6-3

Published: 29 January 2008

Abstract

We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear. Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM. The patient's outcome provides evidence for the importance of prompt, aggressive, and sustained immunosuppressive treatment of encephalopathic SS.