Personality traits and personality problems in Korsakoff syndrome patients.

Personality research is of relevance because it provides insights into the psychological strengths and vulnerabilities of a person. Korsakoff's syndrome (KS) is a severe neuropsychiatric disorder following acute thiamine deficiency, usually as a consequence of alcohol-use disorders. Research on personality traits of KS patients is currently limited. The aim of the current exploratory study was therefore to examine the personality traits and problems of KS patients. We assessed self-reported and informant-reported personality traits and problems in institutionalized KS patients (n = 30). Results indicate that the psychotic personality organization was more frequently present in KS patients compared to a psychiatric reference population, leading to increased vulnerability for the development of severe psychiatric issues. Informants observed more premorbid and current cluster B ("emotional") and C ("anxious") personality behavior in comparison to the general population. Also, rigid- and socially avoidant behavior in KS patients was observed to increase over the years. They also observed personality problems such as emotional-, unpredictable-, dramatic-, anxious and fearful behavior in the present and in the past. KS patients reported themselves as healthy individuals, indicating a lack of self-awareness. We recommend training programs for the medical team focussed on psycho-education and supportive interventions for patients with complex personality problems concomitant to KS.

When Do Korsakoff Patients Justify Immoral Behaviors? The Influence of Premorbid Delinquency and Self-Other Perspectives in Moral Decision-Making and Moral Reasoning.

Korsakoff's syndrome (KS) is a chronic neuropsychiatric disorder caused by a vitamin B1 deficiency. KS is characterized by profound amnesia and often accompanied by poor executive functioning, decreased social-cognitive abilities, and difficulties in behavioral regulation. As moral behaviors and attitudes may provide insight in socio-behavioral interactions, the current study aimed to evaluate everyday moral maturity by administering self- versus other-oriented moral dilemmas in a group of KS patients (n = 20) and healthy controls (n = 20). Responses were scored according to the Kohlberg stages of moral reasoning. Furthermore, we assessed premorbid delinquency and current neurocognitive functioning as possible relevant factors. Our results show that KS patients were prone to lower levels of moral maturity when confronted with moral dilemmas relating to themselves, compared to dilemmas relating to (un)personal others in KS patients, while healthy subjects showed an opposite pattern. Moral immaturity could find its origin already before the onset of the KS diagnosis, as suggested by the elevated premorbid levels of delinquent behavior and correlation between premorbid delinquency and moral maturity in KS. Lower moral maturity could therefore be a possible predisposing factor to both delinquency and later development of Korsakoff's syndrome.

Wernicke's Encephalopathy in Acute and Chronic Kidney Disease: A Systematic Review.

Thiamine (vitamin B1) deficiency is relatively common in patients with kidney disease. Wernicke's encephalopathy (WE) is caused by vitamin B1 deficiency. Our aim was to systematically review the signs and symptoms of WE in patients with kidney disease. We conducted a systematic literature review on WE in kidney disease and recorded clinical and radiographic characteristics, treatment and outcome. In total 323 manuscripts were reviewed, which yielded 46 cases diagnosed with acute and chronic kidney disease and WE published in 37 reports. Prodromal characteristics of WE were loss of appetite, vomiting, weight loss, abdominal pain, and diarrhea. Parenteral thiamine 500 mg 3 times per day often led to full recovery, while Korsakoff's syndrome was found in those receiving low doses. To prevent WE in kidney failure, we suggest administering high doses of parenteral thiamine in patients with kidney disease who present with severe malnutrition and (prodromal) signs of thiamine deficiency.

Self-Reported Pain and Pain Observations in People with Korsakoff's Syndrome: A Pilot Study.

Korsakoff's syndrome (KS) is a chronic neuropsychiatric disorder. The large majority of people with KS experience multiple comorbid health problems, including cardiovascular disease, malignancy, and diabetes mellitus. To our knowledge pain has not been investigated in this population. The aim of this study was to investigate self-reported pain as well as pain behavior observations reported by nursing staff. In total, 38 people diagnosed with KS residing in a long-term care facility for KS participated in this research. The Visual Analogue Scale (VAS), Pain Assessment in Impaired Cognition (PAIC-15), Rotterdam Elderly Pain Observation Scale (REPOS), and the McGill Pain Questionnaire-Dutch Language Version (MPQ-DLV) were used to index self-rated and observational pain in KS. People with KS reported significantly lower pain levels than their healthcare professionals reported for them. The highest pain scores were found on the PAIC-15, specifically on the emotional expression scale. Of importance, the patient pain reports did not correlate with the healthcare pain reports. Moreover, there was a high correlation between neuropsychiatric symptoms and observational pain reports. Specifically, agitation and observational pain reports strongly correlated. In conclusion, people with KS report less pain than their healthcare professionals indicate for them. Moreover, there is a close relationship between neuropsychiatric symptoms and observation-reported pain in people with KS. Our results suggest that pain is possibly underreported by people with KS and should be taken into consideration in treating neuropsychiatric symptoms of KS as a possible underlying cause.

Eye Movements as Proxy for Visual Working Memory Usage: Increased Reliance on the External World in Korsakoff Syndrome.

In the assessment of visual working memory, estimating the maximum capacity is currently the gold standard. However, traditional tasks disregard that information generally remains available in the external world. Only when to-be-used information is not readily accessible, memory is taxed. Otherwise, people sample information from the environment as a form of cognitive offloading. To investigate how memory deficits impact the trade-off between sampling externally or storing internally, we compared gaze behaviour of individuals with Korsakoff amnesia (n = 24, age range 47-74 years) and healthy controls (n = 27, age range 40-81 years) on a copy task that provoked different strategies by having information freely accessible (facilitating sampling) or introducing a gaze-contingent waiting time (provoking storing). Indeed, patients sampled more often and longer, compared to controls. When sampling became time-consuming, controls reduced sampling and memorised more. Patients also showed reduced and longer sampling in this condition, suggesting an attempt at memorisation. Importantly, however, patients sampled disproportionately more often than controls, whilst accuracy dropped. This finding suggests that amnesia patients sample frequently and do not fully compensate for increased sampling costs by memorising more at once. In other words, Korsakoff amnesia resulted in a heavy reliance on the world as 'external memory'.

Young Nonalcoholic Wernicke Encephalopathy Patient Achieves Remission Following Prolonged Thiamine Treatment and Cognitive Rehabilitation.

Wernicke encephalopathy (WE), a neurological emergency commonly associated with alcohol use disorder, results from a severe deficiency of vitamin B1. If left untreated, patients either succumb to the illness or develop chronic Korsakoff's syndrome (KS). Recently, an increasing number of nonalcoholic WE case studies have been published, highlighting a lack of understanding of malnutrition-related disorders among high-functioning patients. We present the case of a 26 year old female who developed life-threatening WE after COVID-19-complicated obesity surgery. She experienced the full triad of WE symptoms, including eye-movement disorders, delirium, and ataxia, and suffered for over 70 days before receiving her initial WE diagnosis. Late treatment resulted in progression of WE symptoms. Despite the severity, the patient achieved remission of some of the symptoms in the post-acute phase due to prolonged parenteral thiamine injections and intensive specialized rehabilitation designed for young traumatic brain injury (TBI) patients. The rehabilitation resulted in gradual remission of amnesia symptomatology, mainly increasing her autonomy. The late recognition of this case highlights the importance of early diagnosis and prompt, targeted intervention in the management of nonalcoholic WE, as well as underscores the potential for positive outcomes after delayed treatment through intensive cognitive rehabilitation in specialized treatment centers.

Korsakoff syndrome and altered pain perception: a search of underlying neural mechanisms.

OBJECTIVES: Patients with Korsakoff syndrome (KS) may have a diminished pain perception. Information on KS and pain is scarce and limited to case descriptions. The present study is the first to investigate the underlying neural mechanisms of altered pain perception in patients with KS more systematically. METHODS: We conducted a literature search on neural correlates of pain perception in other neurocognitive disorders in which extensive research was done. RESULTS: The brain areas that are affected in KS showed considerable overlap with the neural correlates of pain perception in other neurocognitive disorders. We discussed which different aspects of disturbed pain perception could play a role within KS, based on distinct neural damage and brain areas involved in pain perception. CONCLUSIONS: Combining current knowledge, we hypothesize that diminished pain perception in KS may be related to lesioned neural connections between cerebral cortical networks and relays of mainly the thalamus, the periaqueductal gray, and possibly lower brain stem regions projecting to the cerebellum. Based on these neural correlates of altered pain perception, we assume that increased pain thresholds, inhibition of pain signals, and disturbed input to cerebral and cerebellar cortical areas involved in pain processing, all are candidate mechanisms in cases of diminished pain perception in KS. We recommend that clinicians need to be alert for somatic morbidity in patients with KS. Due to altered neural processing of nociceptive input the clinical symptoms of somatic morbidity may present differently (i.e. limited pain responses) and therefore are at risk of being missed.

Eye movement desensitization and reprocessing (EMDR) and mediative behavioral therapy for the treatment of suffocation related post-traumatic stress disorder (PTSD) in amyotrophic lateral sclerosis (ALS): A case report.

OBJECTIVE: Eye movement desensitization and reprocessing (EMDR) is an established treatment for post-traumatic stress disorders (PTSD). Some patients diagnosed with amyotrophic lateral sclerosis (ALS) experience PTSD following choking or suffocation in the course of progressive loss of the ability to breathe. Although a loss of breathing functions in ALS is relatively common, there are currently no studies available on treatment for the fear of choking following advanced ALS. METHODS: In this case study, we describe the positive effects of EMDR, an evidence-based form of trauma therapy, in a 48-year-old female, suffering from advanced ALS. As the consequence of ALS, she was not able to speak or breath independently, but could communicate through a speech-generating device. She experienced panic attacks, flashbacks, nightmares, and severe anxiety after her tracheostomy jammed, and she almost suffocated. RESULTS: Mediative treatment was started by instructing the care staff to respond neutrally with step-by-step instructions following tracheostomy jam, resulting in significantly less panic attacks and flashbacks. EMDR was initiated two weeks later, and resulted in full remittance of the trauma symptomatology. SIGNIFICANCE OF THE RESULTS: The present case study suggests that symptoms of PTSD, namely the strong fear of suffocation, can be successfully treated by means of mediative behavioral therapy combined with EMDR.

The era of our lives: The memory of Korsakoff patients for the first Covid-19 pandemic lockdown in the Netherlands.

Memories for worldwide and emotional events (such as 9/11) are more vividly relived and recalled than memories for everyday events. Previous studies have shown that flashbulb memories of a single event enhanced the memory strength in severe amnesia. It is currently unknown whether macro-events that stretch out over longer periods of time (weeks, months) strengthen memory even further. Our aim was therefore to investigate to what extent patients with severe amnesia, due to Korsakoff's syndrome (KS), were able to relive the first Covid-19 lockdown in the Netherlands, and whether experienced emotions enhanced reliving of the participants. We included 22 KS patients and 24 age-, education-, and gender-matched healthy controls. Covid-19 related memories were assessed by measures of autobiographical memory specificity, phenomenological reliving, emotional intensity and semantic-and episodic knowledge about the first lockdown in March 2020 - May 2020 in the Netherlands. Although amnesia patients remembered significantly fewer autobiographical details regarding the Covid-19 lockdown than healthy controls, one fourth of the KS patients recalled specific events. Amnesia patients reported levels of emotional intensity equivalent to those in the control group. Stronger autobiographical reliving was associated with higher emotional intensity. Both amnesia patients and healthy controls had higher recall of episodic than semantic lockdown related information. In conclusion, results demonstrate that information for macro-events can still be memorized and relived, most specifically when emotional valence is high, even by highly amnestic patients.

The Use of an Errorless Learning Application to Support Re-Learning of (Instrumental) Activities for People Living with Korsakoff Syndrome.

Korsakoff syndrome (KS) is a severe neuropsychiatric syndrome derived from acute thiamine deficiency and concomitant alcohol use disorders. KS patients need lifelong assistance because of the severity of their cognitive problems. In clinical practice and research, errorless learning has proven to be an effective cognitive rehabilitation method for patients with KS. Our study focused on optimizing errorless learning by introducing new software technology to support the training process of errorless learning. Although the benefits of errorless learning for patients with Korsakoff's syndrome have been thoroughly investigated, it is currently unclear whether new technology could contribute to better learning and maintenance of everyday tasks. Therefore, an errorless learning application was built. This device is a web application and can be used on a tablet, laptop, or smartphone. The application allows clinicians and researchers to insert pictures, videoclips, timers, and audio fragments in the different steps of an errorless learning training plan. This way, the different steps are visible and easy to follow for patients. Moreover, it ensures as a learning method that the training is executed exactly the same way for each and every training. The aim of this study was twofold: to examine whether the use of the errorless learning application is effective, and whether it leads to better results than a regular errorless learning of everyday activities. In total, 13 patients with KS were trained in instrumental activities of daily living by means of the application, and 10 patients were trained with traditional instructions. Results showed an equal improvement for both training methods. Importantly, the technology group could better remember the training when probed at a later moment than the traditional errorless learning group. These results are promising for further development of novel technology to support errorless learning applications in clinical practice.

Smartwatch reminders are as effective as verbal reminders in patients with Korsakoff's syndrome: three case studies.

Korsakoff's syndrome (KS) is a neurocognitive disorder caused by severe malnutrition. KS patients typically show severe impairments in prospective memory (PM), thus, have difficulties with remembering to perform delayed intentions. The current study investigated the possible benefits of a smartwatch aid for PM tasks in patients with KS and compared its efficacy with verbal in-person reminders. Three patients participated in the present study and were asked to complete everyday PM tasks. The results of each patient were analyzed as a single-case study. The results highlight the great potential of using smartwatches as external memory aids in KS patients in everyday life.

Exploring episodic and semantic contributions to past and future thinking performance in Korsakoff's syndrome.

Korsakoff's syndrome (KS) is a neuropsychiatric disorder characterized by severe declarative memory disruption. While episodic memory deficits and confabulation are well documented, it remains unclear to what extent semantic memory is compromised in this syndrome. Moreover, how such impairments relate to the capacity for future-oriented thinking remains unknown. Here, we sought to determine the extent to which episodic and semantic forms of past and future thinking are impacted in KS and the interrelationship between different classes of memory in this syndrome. Twenty patients with KS and 17 matched healthy controls took part in this study. We included well-established indices of past and future thinking capacity, enabling us to compare episodic (event-based) versus semantic (nonpersonal knowledge) across past and future conditions. We also included a novel event generation task to probe implausible event simulation (i.e., spending a day on the moon). Our findings revealed marked impairments in KS across all forms of past and future thinking, as well as the generation of episodic details on the implausible event simulation task. Correlation analyses revealed significant associations between implausible event construction and episodic and semantic future thinking in KS; however, no significant associations were found between future thinking performance and confabulation. This study is the first, to our knowledge, to reveal striking impairments in the capacity for past and future thinking across episodic and semantic domains in KS. Our findings resonate with current theoretical perspectives in which the lines between episodic and semantic memory systems are viewed as increasingly blurred.

Confabulations in post-acute and chronic alcoholic Korsakoff's syndrome: a cross-sectional study conducted in two centres.

INTRODUCTION: Confabulations refer to the emergence of memories of experiences and events that are incorrect in place and time, or never took place. In alcoholic Korsakoff's syndrome, confabulations have been frequently reported, but seldomly been investigated. Traditional reports on confabulations state that confabulations in KS mainly occur in the post-acute phase of the illness. The aim of the study was to investigate whether confabulations extinguish in KS. METHODS: An observational rating of confabulation behaviour (the NVCL-R) was completed for 172 KS patients with alcoholic KS. Post-acute and chronic KS patients were compared cross-sectionally in two centres. RESULTS: Provoked and spontaneous confabulations were present in post-acute and chronic patients. Patients residing in a long-term care facility more often presented themselves with spontaneous confabulations than patients in a diagnostic centre. CONCLUSIONS: In contrast to the traditional view, confabulations may be present throughout the course of KS, and are possibly more frequently present in patients receiving care in specialised long-term care facilities than in patients who receive less intensive support.Key pointsConfabulations are a central characteristic of Korsakoff's syndromeIn contrast to popular belief, confabulations may be present in acute and chronic Korsakoff's syndromeThe severity of confabulations is related to an unfavourable disease outcome in KSA longitudinal approach would help the confirmation of finding no decline in confabulations over time.

Behavioural effects of light intervention in people with Korsakoff Syndrome: A pilot study.

Korsakoff Syndrome (KS) is commonly associated with behavioural symptoms such as agitation, apathy, and disinhibition. People with KS often reside in long-term care facilities, which reduces their exposure to natural light. Little is known regarding positive effects of light intervention in KS. Our objective was to evaluate the influence of a dawn simulation therapy on behavioural symptoms in KS. 38 patients residing in a 24-hour care facility were exposed for 6 weeks to a dawn simulation system in their bedrooms, which gradually increased from 0 lux to 290 lux. Behavioural symptoms were measured over 9 weeks. Weeks 1-3 consisted of the baseline phase and weeks 3-9 consisted of the light intervention phase. Our study showed that total severity of neuropsychiatric symptoms was less prominent during light intervention. More specifically, a decrease on the apathy, disinhibition, behaviour at night and appetite and eating behaviour subscales was found during the light intervention phase compared to the baseline phase. Additionally, a significant effect was found on decreasing emotional distress for caregivers. Results suggest that light intervention therapy has a positive effect on reducing behavioural symptoms in KS as well as the levels of stress experienced by the patients' caregivers.

Lifelogging as a rehabilitation tool in patients with amnesia: A narrative literature review on the effect of lifelogging on memory loss.

Visual lifelogging is the procedure that enables individuals to visually record daily life activities by means of small wearable cameras, which can be worn around the neck or on the clothing. Lifelogging devices automatically take pictures or videos after pre-set time intervals or after dynamic input changes. Although some studies have shown effectivity of reviewing lifelogging images in brain-damaged individuals with forms of amnesia as a rehabilitation tool, systematic endeavours to overview this literature is yet missing. The aim of this narrative literature review was to review all the available case-studies, experimental studies and group studies in brain-damaged individuals applying lifelogging devices in a clinical context. The included studies showed efficacy for both subjective and objective measures of memory. In mild to severe amnesia, reviewing images recorded by the lifelogging device was beneficial to subjective and objective measures of memory. Lifelogging is demonstrated to have a great potential in helping people who are suffering from memory loss. It can offer an excellent alternative to currently more frequently used memory rehabilitation techniques and can be applied more in clinical settings.

Wernicke-Korsakoff syndrome despite no alcohol abuse: A summary of systematic reports.

BACKGROUND: Wernicke-Korsakoff syndrome (WKS) is a neurological disorder typically found in alcohol use disorder. The fact that it also occurs in nonalcoholic patients is less well known and often ignored. For the first time, this review offers a systematic investigation of the frequency and associated features of nonalcoholic WKS in the published literature. METHOD: We included 11 recent systematic reports, with a total of 586 nonalcoholic WKS cases following hyperemesis gravidarum (n = 177), cancer (n = 129), bariatric surgery (n = 118), hunger strike (n = 41), soft drink diet in children (n = 33), depression (n = 21), Crohn's disease (n = 21), schizophrenia (n = 15), anorexia nervosa (n = 12), ulcerative colitis (n = 10), and incidental thiamine-deficient infant formula (n = 9). FINDINGS: Vomiting and extreme weight loss were strong predictors of nonalcoholic WKS in adults. Blurred vision was a common presenting sign in about one-fourth of the patients. The classic triad of WKS is characterized by confusion, ataxia, and eye-movement disorders. All reviewed studies reported high percentages of patients presenting with an altered mental status, while both motor symptoms were variably present. INTERPRETATION: The foregoing observations led to several important conclusions. First, we can see that nutritional impoverishment leads to profound brain damage in the form of WKS. Second, it seems that physicians are either unaware of or underestimate the risks for nonalcoholic WKS. Physicians must be specifically vigilant in detecting and treating WKS in patients with sudden and severe weight loss and vomiting. Third, lower doses of thiamine frequently lead to chronic Wernicke-Korsakoff syndrome. We noticed that when thiamine treatment for WKS was administered, in many cases doses were too low. In line with proven interventions we therefore recommend a parenteral thiamine treatment of 500 mg 3 times per day in adults.

Wernicke's encephalopathy in Crohn's disease and ulcerative colitis.

Crohn's disease (CD) and ulcerative colitis (UC) are chronic and debilitating inflammatory conditions of the gastrointestinal tract. Thiamine can deplete rapidly in CD and UC, which can lead to Wernicke's encephalopathy (WE), is an acute neurologic disorder. Our objective was to systematically review the presentation of WE in CD and UC. We conducted our search from inception using the MeSH terms "Crohn's disease," "ulcerative colitis," and "Wernicke's encephalopathy." Our search yielded 28 case studies reporting on 31 cases. CD was diagnosed in 21 cases, and UC in 10. The first signs of WE were nausea and vomiting (13 cases), double vision (10), blurred vision (10), and hearing loss (4). In 12 cases, partial or complete bowel resection was one of the etiologies of thiamine depletion. In nine cases, thiamine was not supplemented intramuscularly or intravenously while parenteral nutrition or glucose was given to the patient. In 10 cases, detailed descriptions of thiamine treatment were given. Thiamine treatment at suboptimal levels (7 of 10 cases) turned out to lead to residual cognitive deficits in three cases. In three cases with optimal treatment (1500 mg/d intravenously), complete remission of WE symptoms was achieved. Rapid treatment with high doses (500 mg, 3 times/d) of thiamine saves lives, and treats WE in its core symptomatology.

Moral reasoning, moral decision-making, and empathy in Korsakoff's syndrome.

Korsakoff's syndrome (KS) is a neuropsychiatric disorder, caused by a vitamin B1 deficiency. Although it is known that patients with KS display diminished theory of mind functioning and frequently exhibit marked antisocial interactions little attention has so far focused on the integrity of moral decision-making abilities, moral reasoning, and empathy. In an experimental cross-sectional design, 20 patients diagnosed with KS, and twenty age-, education-, and gender-equivalent healthy participants performed tests assessing moral decision-making, moral reasoning maturity, empathy, and executive functioning. Participants were administered the Moral Behaviour Inventory (MBI) for everyday moral dilemmas, and ten cartoons of abstract moral dilemmas. Responses were scored according to the Kohlberg stages of moral reasoning. Empathy and executive functioning were assessed with the Interpersonal Reactivity Index (IRI) and the Frontal Assessment Battery (FAB). In contrast to frontal traumatic brain injury patients, KS patients did not display a utilitarian bias, suggesting preserved moral decision-making abilities. Of interest, KS patients had significantly lower levels of moral reasoning maturity on everyday moral dilemmas, and abstract moral dilemmas. In patients, empathy was moderately related to the level of moral maturity on both tasks, while executive functioning was not. In conclusion, KS patients have preserved moral decision-making abilities, but their moral reasoning abilities are poorer in everyday and abstract situations. Lower moral reasoning abilities and lower levels of empathy together may be responsible for adverse social functioning in KS.

Confabulations in Alcoholic Korsakoff's Syndrome: A Factor Analysis of the Nijmegen-Venray Confabulation List.

Confabulations generally refer to the emergence of memories of experiences and events that, in reality, never took place, and which are unintentionally produced. They are frequently observed in alcoholic Korsakoff's syndrome. The aim of the current study was to validate the Nijmegen-Venray Confabulation List (NVCL), an observation scale for quantifying both spontaneous and provoked confabulations. The NVCL was completed for 252 patients with alcoholic Korsakoff's syndrome. Exploratory and confirmatory factor analyses were conducted to test three- and four-factor models of the NVCL structure. A four-factor model (provoked confabulations, spontaneous confabulations, severity of spontaneous confabulations, and distorted sense of reality) fitted the data better than the initially proposed three-factor model (provoked confabulations, spontaneous confabulations, memory, and orientation). The new instrument is therefore referred to as the NVCL-R. We encourage clinicians to include the assessment of confabulations in the neuropsychological examination, and to do so with validated instruments such as the NVCL-R.