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. 2020 Sep 2;20(1):332.
doi: 10.1186/s12883-020-01892-0.

Increased incidence of Susac syndrome: a case series study

A Wilf-Yarkoni  1 , O Elkayam  2   3 , O Aizenstein  4 , Y Oron  5 , V Furer  2   3 , D Zur  3   6 , M Goldstein  3   6 , D Barequet  3   6 , H Hallevi  7 , A Karni  3   7   8 , Z Habot-Wilner  3   6 , K Regev  7
Affiliations

Increased incidence of Susac syndrome: a case series study

A Wilf-Yarkoni et al. BMC Neurol. .

Abstract

Background: Susac syndrome (SuS) is a rare condition characterized by a clinical triad of sensorineural hearing loss, branch artery occlusion and encephalopathy. This study reports an increased incidence of SuS in Israel. We describe the clinical characteristics of these patients, diagnostic procedures and the use and subsequent outcomes of newly published treatment guidelines.

Methods: This is a single center retrospective study. Patients who were diagnosed with SuS between July 2017 and August 2018 were enrolled in this study.

Results: Seven patients were diagnosed with SuS according to the diagnostic criteria in a time period of 13 months. The annual incidence was recently evaluated in Austria to be 0.024/100000, therefore, our case series represent at least a 5.4- fold increase in the annual incidence of SuS expected in Israel and a 7-fold increase in the annual incidence expected in our medical center. Mean time from the onset of the symptoms to diagnosis was three weeks and follow-up time was twenty four months. Recent exposure to cytomegalovirus was serologically evident in three patients and one patient had high titer of anti-streptolysin antibody. All patients underwent brain MRI, fluorescein angiography and audiometry. All patients were treated according to the newly recommended guidelines. All patients achieved clinical and radiological stability.

Conclusions: We report of an increased incidence of SuS in Israel. Infectious serological findings may imply a post infectious mechanism. The use of the recommended diagnostic procedures reduced the time to diagnosis. Newly published treatment guidelines led to favorable clinical outcomes.

Keywords: Branch retinal artery occlusion; Susac syndrome; Treatment; cmv post infectious.

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Conflict of interest statement

The authors declare that they have no competing interest. None of the authors reports conflicts of interest related to this work. Dr. Karni Arnon received research support from Stem Cell Medicine Ltd., Medison Pharma Ltd. and from Novartis Pharmaceutical Ltd.

Figures

Fig. 1
Fig. 1
Typical diagnostic procedures findings. a Brain MRI. Sagittal and axial T2 FLAIR sequence, showing hyperintense lesions in the corpus callosum (“snow balls”). A1. Periventricular and subcortical areas. A2-3. Sagittal T1 sequence corpus callosum (“black holes”). A4. Axial DWI sequence which show restricted diffusion A5. Leptomeningeal enhancement with gadolinium A6. b Wide field Fluorescein angiogram demonstrating right eye peripheral (superior, temporal and inferior) branch artery occlusions and focal arterial leakage in an inferior branch artery. c. A bilateral low-tone and middle-tone sensorineural hearing loss is seen, with abnormal low scores in speech audiometry
See this image and copyright information in PMC

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