Long-term cognitive outcomes in Susac syndrome: A case series

Yoav Piura, Noa Bregman, Gitit Kavé, Arnon Karni, Hadar Kolb, Ifat Vigiser, Gregory S. Day, Sebastian Lopez-Chiriboga, Tamara Shiner, Keren Regev

Research output: Contribution to journalArticlepeer-review


Susac syndrome (SuS) presents with encephalopathy, visual disturbances, and hearing loss from immune-mediated microvascular occlusion. While acute SuS is well-described, long-term cognitive outcomes with current treatments are underknown. We assessed ten SuS patients treated in accordance with evidence-based guidelines using immunotherapies targeting humoral and cell-mediated pathways. Patients were followed for a median 3.6 years. Initially, cognition inversely correlated with corpus callosum lesions on MRI. All reported cognitive improvement; 5/10 patients had residual deficits in visual attention and executive function. Early, aggressive treatment was associated with good outcomes; extensive early corpus callosum lesions may identify patients at-risk of persistent cognitive deficits.

Original languageEnglish
Article number578396
Pages (from-to)578396
JournalJournal of Neuroimmunology
StatePublished - 15 Aug 2024

Bibliographical note

Copyright © 2024 Elsevier B.V. All rights reserved.


  • Cognition
  • Corpus callosum
  • Immunotherapy
  • Susac syndrome


Dive into the research topics of 'Long-term cognitive outcomes in Susac syndrome: A case series'. Together they form a unique fingerprint.

Cite this