Clinical Characteristics, Neuroimaging Findings, and Mortality in Korsakoff Syndrome

Importance Korsakoff syndrome is a severe neurocognitive disorder resulting from thiamine deficiency, most often associated with alcohol use disorder. Contemporary data describing its clinical presentation, neuroimaging findings, and long-term outcomes remain limited. Objective To characterize the clinical features and outcomes of patients diagnosed with Korsakoff syndrome in routine clinical practice and to identify factors associated with mortality. Design, Setting, and Participants This cohort study included electronic health records of adults with a diagnosis of Korsakoff syndrome between August 1, 2017, and December 31, 2022, from the Clinical Data Warehouse of the Greater Paris University Hospitals (AP-HP), which includes 39 hospitals. Data were analyzed March 2023. Exposure Korsakoff syndrome diagnosis. Main Outcomes and Measures The primary outcome was all-cause mortality. Secondary outcomes included clinical manifestations, comorbidities, and neuroimaging findings in patients diagnosed with Korsakoff syndrome. Results Among 1320 patients with Korsakoff syndrome (mean [SD] age, 62.9 [11.1] years; 962 men [72.9%]), a well-documented subgroup of 114 patients most frequently presented with anterograde amnesia (61.4%). Among this subgroup, 22 patients (19%) had a reported history of Wernicke encephalopathy. Among patients who underwent brain magnetic resonance imaging, abnormalities involving the Papez circuit and vascular leukoencephalopathy were each observed in 40 patients (44.4%) each. During a median (IQR) follow-up of 3.1 (1.2-5.0) years, 398 patients (30.2%) died. In multivariable analyses, malnutrition (hazard ratio [HR], 1.54; 95% CI, 1.15-2.05), alcoholic liver disease (HR, 1.45; 95% CI, 1.10-1.91), male sex (HR, 1.45; 95% CI, 1.13-1.86), and age 80 to 89 years (HR, 1.89; 95% CI, 1.27-2.81) were associated with mortality. Conclusions and Relevance In this large clinical cohort, patients diagnosed with Korsakoff syndrome had a high burden of vascular comorbidities, frequent under-recognition of Wernicke encephalopathy, and substantial mortality. These findings highlight the need for earlier recognition and more standardized diagnostic evaluation of Korsakoff syndrome.