Clinical Characterization and Ancillary Tests in Susac Syndrome A Systematic Review

Adi Wilf-Yarkoni*, Ofir Zmira, Assaf Tolkovsky, Barak Pflantzer, Shany G. Gofrit, Ilka Kleffner, Friedemann Paul, Jan Dörr

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

6 Scopus citations

Abstract

Susac syndrome (SuS) is an orphan microangiopathic disease characterized by a triad of encephalopathy, visual disturbances due to branch retinal artery occlusions, and sensorineuronal hearing loss. Our previous systematic review on all cases of SuS reported until 2012 allowed for a better understanding of clinical presentation and diagnostic findings. Based on these data, we suggested diagnostic criteria in 2016 to allow early diagnosis and treatment of SuS. In view of the accumulation of new SuS cases reported in the last 10 years and improved diagnostic tools, we here aimed at updating the demographic and clinical features of SuS and to review the updated ancillary tests being used for SuS diagnosis. Therefore, based on the 2016 criteria, we systematically collected and evaluated data on SuS published from January 2013 to March 2022.

Original languageEnglish
Article numbere200209
JournalNeurology: Neuroimmunology and NeuroInflammation
Volume11
Issue number3
DOIs
StatePublished - 16 Feb 2024

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