Korsakoff syndrome — a neurocognitive disorder stemming from thiamine (vitamin B1) deficiency resulting often from untreated Wernicke encephalopathy — proves challenging to diagnose and frequently leads to adverse outcomes, according to a retrospective study.
Among 1,320 patients diagnosed with Korsakoff syndrome, 30.2% died over a median follow-up of 3.1 years, reported Imelda Coffi, MD, from Assistance Publique-Hôpitaux de Paris in France, alongside her co-authors.
Factors such as malnutrition (HR 1.54), alcoholic liver disease (HR 1.45), male gender (HR 1.45), and age between 80 and 89 years (HR 1.89) were associated with mortality, as highlighted in a publication in JAMA Neurologyopens in a new tab or window.
In a well-documented subgroup of 114 patients, it was found that 61% experienced anterograde amnesia, with 19% having a history of Wernicke encephalopathy. MRI abnormalities affecting the Papez circuit and vascular leukoencephalopathy were observed in 44.4% of this subgroup.
This study illustrates the significant clinical challenges posed by Korsakoff syndrome and emphasizes the urgent need for standardized diagnostic protocols to allow for earlier recognition and effective management of this disorder in both clinical practice and research, according to Coffi and her colleagues.
Korsakoff syndrome mainly impacts episodic memory relative to other cognitive functions in patients who may otherwise appear alert. It often arises following undiagnosed or inadequately managed Wernicke encephalopathy, an acute condition resulting from insufficient thiamine supply to the brain. High doses of intravenous thiamine are typically effective in treating Wernicke encephalopathy.
Diagnosis of Korsakoff syndrome remains complex due to a lack of agreed-upon diagnostic criteria, as noted by the researchers. The onset of symptoms can be gradual, with diagnostic delays being reported from months to years.
Extensive studies have been conducted regarding Korsakoff syndrome in patients with alcohol use disorders, given their elevated risk of thiamine deficiency. However, thiamine deficiency can also be present in individuals with poor nutrition or eating disorders.
A recent case report revealed a 62-year-old breast cancer patient diagnosed with Wernicke-Korsakoff syndrome unrelated to alcohol consumption, likely resulting from malnutrition. Rare instances of Wernicke encephalopathy have also been identified in patients taking semaglutide (Wegovy, Ozempic), with some developing Korsakoff syndrome or succumbing to the condition.
To gain a clearer understanding of Korsakoff syndrome, Coffi and colleagues assessed electronic health records from the Greater Paris University Hospitals for individuals diagnosed with Korsakoff from 2017 to 2022. The average age among these patients was 62.9 years, with 72.9% being men.
According to ICD-10 codes, 43.7% of those with Korsakoff syndrome had alcohol use disorder while 17.9% were smokers. Common comorbidities included high blood pressure (22.1%) and alcoholic liver disease (15.7%). Notably, 43.6% were admitted through the emergency department.
A significant amount of missing data existed for patients diagnosed with Korsakoff syndrome, so researchers crafted a representative subgroup of 114 well-documented patients for better insights into specific features.
In this subgroup, 95.6% of patients had Korsakoff syndrome connected to alcohol use disorder. Other less frequent causes included vascular dementia (17.5%), eating disorders (1.75%), brain tumors (1.75%), and gastrointestinal surgery complications leading to malabsorption (0.8%).
Reasons for admission in the well-documented subgroup included cognitive impairment (36%), altered health status (30.7%), and confusion (29%).
Anterograde amnesia was the most common memory deficit reported within the subgroup, highlighting the seriousness of memory impairment in Korsakoff syndrome and the difficulties this poses for timely diagnosis and management, according to Coffi and co-authors.
Only 22 individuals (19%) in the well-documented subgroup had a previous diagnosis of Wernicke encephalopathy, further supporting the view that Wernicke encephalopathy frequently goes unrecognized prior to the onset of Korsakoff syndrome.
Given that both Wernicke encephalopathy and Korsakoff syndrome represent different clinical stages of thiamine deficiency-related brain injury, the results raise concerns that some cases of thiamine deficiency may have gone underdiagnosed or inadequately treated.
The study acknowledged several limitations as noted by Coffi and colleagues. A substantial proportion of data was missing from medical records, and detailed evaluations including neuropsychological assessments, brain MRIs, and thiamine levels were only available for a minority of patients. Some diagnoses may have also been misclassified.
The high mortality rate observed in this study aligns with previous research but must be contextualized within the comorbidities and social vulnerabilities that can impact these patients.
Disclaimer: This content is for informational purposes only and is not intended as medical advice. Always consult a healthcare professional for medical concerns.
Fuente: https://www.medpagetoday.com/neurology/dementia/123047
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