Wernicke’s encephalopathy (WE) is an acute neurological condition caused by thiamine deficiency. The typical presentation is characterized by a triad of oculomotor abnormalities, gait ataxia, and altered mental status, though patients rarely present with all three symptoms. WE is a serious medical condition that is associated with high rates of morbidity and mortality if left untreated. It is most commonly seen in patients with severe alcohol use disorder; however, it has also been found in patients with thiamine deficiency due to other causes of malnutrition such as prolonged starvation, hyperemesis, dialysis, cancer, and geriatric surgery. Despite growing research demonstrating WE in non-alcoholic populations, it is frequently misdiagnosed in patients without an extensive alcohol-use history, particularly when they do not present with the typical clinical triad of symptoms. Thus, more knowledge about non-alcoholic WE is needed to improve diagnostic accuracy.

We present a case of a 26-year-old male with an unremarkable alcohol use history, who was diagnosed with WE following a 6-week period of excessive nausea and vomiting of unclear etiology. He presented to the ED three times prior to his diagnosis, and was treated with intravenous hydration, Zofran, and Pepcid. He presented to the ED for the fourth time with altered mental status and gait ataxia and was diagnosed with WE based on MRI findings. He was admitted and treated with high doses of IV thiamine and folate. His clinical course was tracked over time via outpatient neurology examinations, and his cognitive functioning was assessed with an outpatient neuropsychological evaluation approximately six months post-discharge. Record review, including clinical notes, lab tests, and imaging results supplement his outpatient neuropsychological evaluation performance.

Data from a comprehensive outpatient neuropsychological evaluation approximately six months after WE diagnosis is presented. His cognitive profile was characterized by impaired performance on measures of verbal fluency and memory, including encoding and retention of verbal and visual information (with minimal benefit from cueing). Given these impairments and continued functional declines related to cognitive deficits, he met criteria for a Major Neurocognitive Disorder. These results demonstrate persistent cognitive deficits beyond the acute WE period.

WE is a serious neurological condition that can have lasting cognitive effects if left untreated. This case demonstrates persistent cognitive impairments six months after WE diagnosis in a young patient with an unremarkable alcohol history. These findings highlight the necessity of increased diagnostic efficiency of WE in non-alcoholic patients, as immediate thiamine treatment is essential to the recovery process. Neuropsychological functioning at a longer interval will be useful in further elucidating cognitive prognosis as well as providing quality of life recommendations.

Assessment of learning potential in patients with cognitive disorders in individuals with alcohol-related cognitive disorders (including Korsakoff’s syndrome; KS) is highly relevant, as this may help to tailor interventions, guide treatment planning and help to optimize care. However, studies on assessing learning potential or learning ability using neuropsychological assessment in relation to changes in everyday activities during the course of treatment are scarce. In this study we examined whether verbal and visuospatial learning curves could be used as an index of learning ability in relation to everyday activities before and after a treatment program.

We examined the episodic learning ability of patients with KS (N=137), other alcohol-related cognitive impairments (ARCI; N=164), and uncomplicated alcohol use disorder (AUD; N=49). For this, we calculated the learning curves for the California Verbal Learning Test (CVLT) and the Location Learning Test - Revised (LLT-R) and examined their association with ratings of everyday activities by the patient and his/her professional caregiver using the Patient Competency Rating Scale (PCRS) before and after a 10-12 week treatment program following admission to the Korsakoff Centre.

For both verbal and visuospatial memory, the AUD group had a steeper learning curve than the ARCI patients, who in turn had a steeper learning curve than the KS group (p<.01). While the VLGT total score was related to the PCRS in all patient groups (Pearson r>.38, p<.01), this was only the case for the KS group for the LLT-R total score (r>-.29, p<.01). However, the learning curve estimates of both tests were neither related to the PCRS absolute scores (for patients and caregivers, before and after treatment) nor to the APCRS scores during the course of the treatment program.

Episodic learning ability, as measured with the learning curves of the CVLT and LLT-R, were unrelated to the patients everyday activity level as measures by the patients themselves or their professional caregiver. The results will be discussed in relation to other tools for assessing the learning potential of cognitively impaired patients, such as dynamic testing.

Korsakoff’s syndrome (KS) is a neuropsychiatric disorder, caused by malnutrition. Central to KS are severe amnesia and executive dysfunctions. KS patients often fail to remember future intentions (prospective memory),and rely heavily on external support by caregivers. Recently, specialized smartwatches have been developed to support prospective memory verbally and by displaying pictures of future events. We investigated the benefit of a smartwatch and smartphone compared to no aid in supporting time accuracy and the ability to carry out future intentions in one case study. In three subsequent case studies, we investigated the possible benefits of a smartwatch aid for prospective memory (PM) compared to verbal in-person reminders.

In the first case study, one high-functioning KS patient with a WAIS IQ of 127 points, performed a total of 36 novel prospective memory tasks in three conditions (smartwatch, smartphone and no-aid).

In the second case series, three KS patients with average IQ performed 30 everyday PM tasks in two conditions (smartwatch, in-person).

Two dependent variables were indexed in both studies: PM time accuracy (in minutes), this was calculated as minutes difference from the assigned time, and precision of the PM task (correct or incorrect).

In the first study, time accuracy was improved with a smartwatch compared to a smartphone and no-aid condition. Furthermore, the smartwatch and smartphone conditions were more effective than no aid in assisting memory for task content. In the second study, both the smartwatch and in-person instructions were equally effective in supporting prospective memory tasks.

Since prospective memory is compromised in KS, patients require assistance throughout the day in performing everyday and non-everyday tasks. The results of our case studies suggest that a smartwatch that gives specific verbal and visual reminders can be particularly helpful in supporting prospective memory for KS patients. Giving in-person instructions was equally effective as the use of this smartwatch, highlighting the possibility to support KS patients with less intensive everyday coaching. Together, these results are promising in applying smartwatches clinically to support prospective memory.

People with Korsakoff syndrome (KS) experience severe neuropsychological and neuropsychiatric complications following vitamin B1 deficiency predominantly due to alcoholism. KS often presents itself with neuropsychological symptoms such as problems in episodic memory, executive functioning, and social cognition. Common neuropsychiatric symptoms in KS are disorders of affect, confabulations, anosognosia, and apathy. Apathy can be defined by a pathological lack of goal-directed behaviors, goal-directed cognitions, and goal-directed emotions. Patients with KS have an increased risk of cerebrovascular comorbidity. Cerebrovascular accidents are known to increase the risk for developing apathy. Apathy in KS patients can negatively influence the ability to live an autonomous life, often making 24-hour care a necessity. Limited research on apathy in KS patients has been published to this day. Our aim was to assess apathy in Korsakoff patients with and without neurovascular comorbidity.

General apathy and related subconstructs, such as judgment and decision-making skills, emotional blunting, and the intentions to perform pleasurable activities, were studied in fifteen KS patients, fifteen KS patients with additional cerebrovascular comorbidity, and fifteen healthy controls. The first responsible caregiver of each patient filled in the Apathy Evaluation Scale and Scale for Emotional Blunting. An examiner administered the interview-based Judgement scale of the Neuropsychology Assessment Battery with the KS patients and each KS patient filled in the self-report section of the Pleasurable Activities List. Both KS patient groups receive 24-hour care in a specialized facility for Korsakoff Syndrome.

Our study found higher levels of general apathy in both KS patient groups, when rated by their caregiver compared to healthy controls. No difference was found between the KS patient groups and the healthy control group on the self-reported section of the Pleasurable Activities List, which might suggest the presence of intrinsic motivation in KS patients. However, a discrepancy was found between the self-reported activity levels and proxy reported levels of apathy. KS patients with cerebrovascular comorbidity showed increased levels of emotional blunting compared to KS patients without cerebrovascular comorbidity and healthy controls. Decreased judgment and decision-making skills were found in both patient groups compared to healthy controls, with no difference found between KS patients with cerebrovascular comorbidity and KS patients without.

Our findings suggest that people with Korsakoff syndrome experience more general apathy compared to healthy controls. Both patient groups showed decreased judgement and decision-making skills and increased emotional blunting. Intrinsic motivation was found to be intact in KS patients. Experiencing cerebrovascular comorbidity in KS carries a risk for developing emotional blunting. Our findings show that apathy greatly affects people with KS. Future scientific research is warranted to further benefit the care for this complex patient population.