TY - JOUR
T1 - Clinical Characterization and Ancillary Tests in Susac Syndrome A Systematic Review
AU - Wilf-Yarkoni, Adi
AU - Zmira, Ofir
AU - Tolkovsky, Assaf
AU - Pflantzer, Barak
AU - Gofrit, Shany G.
AU - Kleffner, Ilka
AU - Paul, Friedemann
AU - Dörr, Jan
N1 - Publisher Copyright:
© 2024 The Author(s).
PY - 2024/2/16
Y1 - 2024/2/16
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85185391272&partnerID=8YFLogxK
U2 - 10.1212/NXI.0000000000200209
DO - 10.1212/NXI.0000000000200209
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C2 - 38364193
AN - SCOPUS:85185391272
SN - 2332-7812
VL - 11
JO - Neurology: Neuroimmunology and NeuroInflammation
JF - Neurology: Neuroimmunology and NeuroInflammation
IS - 3
M1 - e200209
ER -