%0 Journal Article
%T Long-term cognitive outcomes in Susac syndrome: A case series.
%A Piura Y
%A Bregman N
%A Kavé G
%A Karni A
%A Kolb H
%A Vigiser I
%A Day GS
%A Lopez-Chiriboga S
%A Shiner T
%A Regev K
%J J Neuroimmunol
%V 393
%N 0
%D 2024 08 15
%M 38908330
%F 3.221
%R 10.1016/j.jneuroim.2024.578396
%X 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.