{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T03:33:03Z","timestamp":1790652783248,"version":"4.1.0"},"reference-count":30,"publisher":"American Medical Association (AMA)","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA Neurol"],"abstract":"\n Importance<\/jats:title>\n 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.<\/jats:p>\n <\/jats:sec>\n \n Objective<\/jats:title>\n To characterize the clinical features and outcomes of patients diagnosed with Korsakoff syndrome in routine clinical practice and to identify factors associated with mortality.<\/jats:p>\n <\/jats:sec>\n \n Design, Setting, and Participants<\/jats:title>\n 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 between April 2023 and September 2024.<\/jats:p>\n <\/jats:sec>\n \n Exposure<\/jats:title>\n Korsakoff syndrome diagnosis.<\/jats:p>\n <\/jats:sec>\n \n Main Outcomes and Measures<\/jats:title>\n The primary outcome was all-cause mortality. Secondary outcomes included clinical manifestations, comorbidities, and neuroimaging findings in patients diagnosed with Korsakoff syndrome.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n 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.<\/jats:p>\n <\/jats:sec>\n \n Conclusions and Relevance<\/jats:title>\n 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.<\/jats:p>\n <\/jats:sec>","DOI":"10.1001\/jamaneurol.2026.3114","type":"journal-article","created":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T15:00:24Z","timestamp":1789398024000},"source":"Crossref","is-referenced-by-count":1,"title":["Clinical Characteristics, Neuroimaging Findings, and Mortality in Korsakoff Syndrome"],"prefix":"10.1001","author":[{"given":"Imelda","family":"Coffi","sequence":"first","affiliation":[{"name":"Service de M\u00e9decine Interne, Centre de R\u00e9f\u00e9rence Maladies Auto-immunes et Auto-inflammatoires Syst\u00e9miques Rares d\u2019Ile de France, de l\u2019Est et de l\u2019Ouest, H\u00f4pital Cochin, Assistance Publique\u2013H\u00f4pitaux de Paris (AP-HP), Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuel","family":"Lec\u0153ur","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de Recherche Clinique-Centre d\u2019Investigation Clinique, CIC1419, H\u00f4pital Cochin-Necker, AP-HP, Universit\u00e9 Paris Cit\u00e9, Paris, France"},{"name":"Unit\u00e9 de recherche UMR1343, \u00c9valuation des th\u00e9rapeutiques et pharmacologie p\u00e9rinatale et p\u00e9diatrique, INSERM, Universit\u00e9 Paris Cit\u00e9, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin","family":"Zarca","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de recherche clinique en \u00e9conomie de la sant\u00e9, URC-ECO d\u2019Ile de France, H\u00f4pital H\u00f4tel-Dieu, AP-HP, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lidia","family":"Kardas-Sloma","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de recherche clinique en \u00e9conomie de la sant\u00e9, URC-ECO d\u2019Ile de France, H\u00f4pital H\u00f4tel-Dieu, AP-HP, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Durand-Zaleski","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de recherche clinique en \u00e9conomie de la sant\u00e9, URC-ECO d\u2019Ile de France, H\u00f4pital H\u00f4tel-Dieu, AP-HP, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Marc","family":"Treluyer","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de recherche UMR1343, \u00c9valuation des th\u00e9rapeutiques et pharmacologie p\u00e9rinatale et p\u00e9diatrique, INSERM, Universit\u00e9 Paris Cit\u00e9, Paris, France"},{"name":"Centre R\u00e9gional de Pharmacovigilance, Assistance Publique-H\u00f4pitaux de Paris, Universit\u00e9 de Paris, H\u00f4pital Cochin, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luc","family":"Mouthon","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, Centre de R\u00e9f\u00e9rence Maladies Auto-immunes et Auto-inflammatoires Syst\u00e9miques Rares d\u2019Ile de France, de l\u2019Est et de l\u2019Ouest, H\u00f4pital Cochin, Assistance Publique\u2013H\u00f4pitaux de Paris (AP-HP), Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin","family":"Chaigne","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, Centre de R\u00e9f\u00e9rence Maladies Auto-immunes et Auto-inflammatoires Syst\u00e9miques Rares d\u2019Ile de France, de l\u2019Est et de l\u2019Ouest, H\u00f4pital Cochin, Assistance Publique\u2013H\u00f4pitaux de Paris (AP-HP), Paris, 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