{Reference Type}: Journal Article
{Title}: Long-term cognitive outcomes in Susac syndrome: A case series.
{Author}: Piura Y;Bregman N;Kavé G;Karni A;Kolb H;Vigiser I;Day GS;Lopez-Chiriboga S;Shiner T;Regev K;
{Journal}: J Neuroimmunol
{Volume}: 393
{Issue}: 0
{Year}: 2024 08 15
{Factor}: 3.221
{DOI}: 10.1016/j.jneuroim.2024.578396
{Abstract}: 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.