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  • Review Article
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Characteristics of Susac syndrome: a review of all reported cases

Nature Reviews Neurology volume 9, pages 307–316 (2013)Cite this article

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Abstract

In Susac syndrome, occlusions of microvessels—presumed to be mediated by an autoimmune response to an as yet unknown antigen—lead to a characteristic clinical triad of CNS dysfunction, branch retinal artery occlusions, and sensorineural hearing impairment. Susac syndrome is considered a rare but important differential diagnosis in numerous neurological, psychiatric, ophthalmological, and ear, nose and throat disorders. Improved understanding of this disorder is crucial, therefore, to ensure that patients receive appropriate treatment and care. Current knowledge on Susac syndrome is largely based on reports of single patients, small case series, and nonsystematic reviews. The aim of this Review is to extend these previous, primarily anecdotal findings by compiling data from all 304 cases of Susac syndrome that have been published worldwide, which were identified following a literature search with predefined search, inclusion and exclusion criteria. From this data, we present an overview of demographic, clinical and diagnostic data on Susac syndrome, providing a reliable basis for our current understanding of this rare disease. Where possible, we make recommendations for clinical diagnosis, differential diagnosis, and management of patients with suspected Susac syndrome.

Key Points

  • Susac syndrome is a rare but important differential diagnosis in numerous neurological, psychiatric, ophthalmological, and ear, nose and throat disorders

  • On the basis of data obtained from 304 published cases, the mean age of onset of Susac syndrome is estimated at 31.6 years, with a male-to-female ratio of 1:3.5

  • Presentation with the full clinical triad of symptoms (CNS, eye and ear symptoms) at disease onset is rare and, therefore, diagnosis should not rely solely on presence of the triad

  • Presence of oligoclonal bands and/or intrathecal IgG does not exclude a diagnosis of Susac syndrome, but lack of both signs remains an important criterion for differentiation from multiple sclerosis

  • Invasive diagnostic procedures such as brain biopsy or cerebral angiography do not aid establishment of a diagnosis of Susac syndrome, so should be reserved for exceptional cases

  • The role of antibodies targeting endothelial cells in the pathophysiology and diagnosis of Susac syndrome remains to be determined

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Figure 1: Fluorescein angiography and MRI findings in Susac syndrome.
Figure 2: Location of brain lesions in patients with Susac syndrome.

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Acknowledgements

J. Dörr and F. Paul are supported by the German Research Foundation (DFG Exc 257).

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Authors and Affiliations

  1. NeuroCure Clinical Research Centre, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany

    Jan Dörr, Sarah Krautwald & Friedemann Paul

  2. Division of Molecular Neuroimmunology, Department of Neurology, University Hospital Heidelberg, Im Neuenheimer Feld 400, 69120, Heidelberg, Germany

    Brigitte Wildemann & Sven Jarius

  3. Department of Neurology, Heinrich-Heine-University, Medical Faculty, Moorenstrasse 5, 40225, Düsseldorf, Germany

    Marius Ringelstein & Orhan Aktas

  4. Department of Neurology, University of Münster, Albert-Schweitzer-Strasse 33, 48129, Münster, Germany

    Thomas Duning, Erich Bernd Ringelstein & Ilka Kleffner

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Contributions

J. Dörr, S. Krautwald, B. Wildemann, S. Jarius, M. Ringelstein, T. Duning, O. Aktas, E. B. Ringelstein and F. Paul researched data for the article. J. Dörr, B. Wildemann, S. Jarius, M. Ringelstein, T. Duning, O. Aktas, E. B. Ringelstein, P. Friedemann and I. Kleffner provided substantial contributions to discussion of content. J. Dörr wrote the article. All authors provided substantial contribution to review and/or editing of the manuscript before submission.

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Correspondence to Jan Dörr.

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J. Dörr receives a grant to support research on Susac syndrome from Novartis Pharma. The other authors declare no competing interests.

Supplementary information

Supplementary Box 1

Retrieval and processing of data (DOC 37 kb)

Supplementary Table 1

Summary of findings from 164 published case studies or case series of Susac syndrome (DOC 676 kb)

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Dörr, J., Krautwald, S., Wildemann, B. et al. Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol 9, 307–316 (2013). https://doi.org/10.1038/nrneurol.2013.82

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