High Mortality Associated with Korsakoff Syndrome Due to Thiamine Deficiency

The study, led by Imelda Coffi, MD, from Assistance Publique-Hôpitaux de Paris, identified various factors influencing mortality, including malnutrition, alcoholic liver disease, male sex, and advanced age. Specific hazard ratios for these factors were found to be 1.54 for malnutrition, 1.45 for both alcoholic liver disease and male sex, and 1.89 for patients aged 80 to 89 years, as reported in JAMA Neurology.

In a more detailed subgroup analysis involving 114 well-documented patients, 61% exhibited anterograde amnesia, while 19% had a history of Wernicke encephalopathy. MRI scans revealed abnormalities in the Papez circuit and vascular leukoencephalopathy in 44.4% of these patients. The study emphasized the considerable clinical burden of Korsakoff syndrome and called for more standardized diagnostic criteria to enhance early recognition and management.

Korsakoff syndrome often arises after untreated Wernicke encephalopathy, which is caused by insufficient thiamine supply to the brain and can be treated with high doses of intravenous thiamine. The researchers noted the diagnostic difficulties, attributing them to a lack of consensus on criteria and the insidious onset of the disorder, which can delay diagnosis by months or years.

The team highlighted that Korsakoff syndrome has commonly been studied in patients with alcohol use disorders, where thiamine deficiency is prevalent. However, it can also affect individuals with poor nutrition or eating disorders. A recent case report documented Wernicke-Korsakoff syndrome in a 62-year-old breast cancer patient, likely resulting from malnutrition, further illustrating the disorder's complexity.

The researchers evaluated electronic health records from the Greater Paris University Hospitals for Korsakoff diagnoses from 2017 to 2022. They found that the mean age of patients was 62.9 years, with a predominance of male patients (72.9%). Alcohol use disorder was noted in 43.7% of cases, while other health issues like high blood pressure and alcoholic liver disease were also common.

In the well-documented subgroup, 95.6% had Korsakoff syndrome related to alcohol use, with lesser occurrences linked to vascular dementia, eating disorders, brain tumors, or gastrointestinal surgery impacting absorption. Symptoms leading to hospital admission frequently included cognitive impairment, altered state of health, and confusion.

The subgroup analysis indicated that only 19% had prior diagnoses of Wernicke encephalopathy, supporting the notion that this condition often goes unrecognized before the onset of Korsakoff syndrome. The findings suggest that thiamine deficiency may frequently be underdiagnosed or inadequately treated.

Coffi and colleagues acknowledged limitations in their study, citing significant gaps in data for neuropsychological evaluations, brain MRI, and thiamine levels among many patients. While the findings correspond with existing literature regarding high mortality rates, they emphasized the need to consider the comorbidities and social vulnerabilities of affected patients.