Article
Susac Syndrome: a case report
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Published: | September 22, 2004 |
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Outline
Text
Objective
SUSAC-Syndrome is characterized by branch retinal artery occlusion and microangiopathy of the inner ear and the brain in young subjects, mostly women. The pathogenesis is unknown.
Methods
A 22-years-old female patient developed a dramatic loss of hearing that mostly involved low frequencies. This was followed by cochleovestibular symptoms. Funducsopy and fluorescein angiography demonstrated bilateral branch artery occlusion. Brain MRI T2-weighted images revealed small multifocal hyperintensities of white and gray matter.
Results
A first remission occurred some weeks after antithrombotic and immunosuppression by prednisolone, but some new branch occlusions were detected following withdrawal of the medication. Thus, systemic application of prednisolone has been continued for a couple of months (Ophthalmology 1998; 105:1038-44) followed by a stabilization of the clinical findings.
Conclusions
The symptoms are often misinterpretated as Multiple Sclerosis. Only a rare number of cases - less than 80 - have described in the literature. Treatment options are not standardized, but the disease seems to be self-limited after a fluctuating active phase. Its prognosis is good in most cases, the disease tends to be monophasic.