Search Results (33)

Background: Apathy is a neuropsychiatric symptom that is frequently present in nursing-home residents, including residents with Korsakoff’s syndrome (KS). Although apathy is common in KS, treatment guidelines are lacking. The Shared Action for Breaking through Apathy (SABA) program, developed for people with dementia, was previously shown to be feasible in that group. The applicability of this program for the KS population seems promising, yet it was expected that the program would need to be adapted. This study aims to 1) explore what is important in identifying and managing apathy in individuals with KS, and 2) investigate the appropriate adjustments to the SABA program. Methods: This qualitative study consisted of semi-structured interviews with people with KS (n = 3), family caregivers (one spouse and one sibling) and professional caregivers (two nurses), and a multidisciplinary focus group meeting with professional caregivers (n = 12) experienced in care for people with KS. The focus group meeting was performed to deepen the understanding of the interview findings and further explore recommendations for adjustments to the SABA program. Thematic analysis was used to process the data. Results: Addressing aim 1, two themes were identified: (1) the challenge to appraise signals of apathy, and (2) the challenge to assess the needs of people with KS. Based on these themes, specific adjustments were formulated to respond to aim 2. Conclusions: The themes that were identified in this study gave direction to a KS tailored SABA program, the feasibility of which needs to be studied next. Full article

Korsakoff syndrome (KS) is a chronic neuropsychiatric disorder caused by severe thiamine deficiency. The syndrome is characterized by deficits in explicit memory and executive functions. These impairments severely limit patients with KS in their daily activities like visiting new and familiar places. The aim of the present study was to examine whether patients with KS are still able to learn a route, despite their cognitive impairments. We compared three route learning methods across three groups of patients with KS: passively following the experimenter (PL), trial-and-error learning (TEL), and errorless learning (EL). In the EL group, the participants had to walk towards a landmark that was shown on a tablet by the experimenter, and upon arrival, they had to find and walk towards the next landmark shown by the experimenter. Over 12 training sessions all participants showed improved route retracing performance. There was no difference between the three groups. Moreover, the results indicated positive effects of the walking training on quality of life, physical fitness, and attentional functioning. These promising findings imply that walking training may be beneficial for various aspects of amnestic patients’ daily functioning. It is therefore recommended to integrate route training into standard care for patients with KS. Full article

Background: Wernicke Encephalopathy (WE), a neurological disorder often linked to alcohol use, can also occur under non-alcoholic conditions, including in pediatric populations. Methods: This systematic review examines 88 pediatric WE cases reported over the past 30 years, encompassing diverse etiologies such as cancer (25 cases), gastrointestinal diseases (19), malnutrition (17), psychiatric disorders (13), obesity surgery (5), renal disease (4), COVID-19 (2), PICU complications (1), hyperemesis gravidarum (1), and a genetic mutation (1). Results: Prodromal symptoms included nausea (60%) and vomiting (55%). In total, 37% of the patients received parenteral nutrition without thiamine before WE diagnosis, often progressing to Wernicke–Korsakoff syndrome (WKS). Key findings revealed the classic triad of WKS, eye movement disorders (80%), mental status changes (75%), and ataxia (63%), with MRI demonstrating high diagnostic sensitivity (85%). Treatment varied widely; higher parenteral thiamine doses correlated with faster recovery and better outcomes, while insufficient dosages led to adverse effects. Full remission was achieved in 61% of cases, with improved outcomes in more recent reports due to refined dosing protocols. Conclusions: These findings underscore the importance of early recognition of nausea and vomiting as predictors of pediatric WE and the critical need to incorporate thiamine in parenteral nutrition for children. Optimal dosing remains vital for recovery, particularly in severe cases. Full article

Vitamin B₁ (thiamine) plays an important role in human metabolism. It is essential for the proper growth and development of the body and has a positive effect on the functioning of the digestive, cardiovascular, and nervous systems. Additionally, it stimulates the brain and improves the psycho-emotional state. In vivo, vitamin B₁ occurs in free form as thiamine or as its ester with phosphate residue(s), i.e., as mono-, di-, or triphosphate. It has been proven that supportive therapy with vitamin B₁ can not only provide neuroprotection but also has a positive effect on advanced neurodegenerative diseases, such as Parkinson’s disease, Alzheimer’s disease, Wernicke–Korsakoff syndrome, or Huntington’s disease. This paper presents studies on the effect of free thiamine (T) and thiamine pyrophosphate (TPP) on the activity of acetylcholinesterase (AChE), which is an enzyme considered to play an important role in the therapies for neurodegenerative diseases, especially Alzheimer’s disease. The mechanisms of action of these compounds as potential inhibitors of AChE were evaluated using both experimental (enzymatic activity) as well as computational (molecular docking, molecular dynamics simulations, and MM-GBSA calculations) methods. The results of the current study indicate a non-competitive type of enzyme inhibition, in contrast to the previously published works suggesting a competitive one. Full article

In this case series, the simultaneous occurrence of Wernicke’s encephalopathy (WE) and dry beriberi was reported in three patients who underwent vertical sleeve gastrectomy (VSG) between May 2021 and May 2023. All patients were obese women who underwent vertical sleeve gastrectomy (VSG) without immediate postoperative complications, but two weeks later, hyperemesis and subsequent encephalopathy with ocular movement abnormalities and weakness were observed over the following thirty days. Patients were referred to neurology, where due to the high suspicion of WE, thiamine replacement therapy was initiated; meanwhile, diagnostic neuroimaging and blood tests were conducted. Neurological and psychiatric evaluations and neuroconduction studies were performed to assess the clinical evolution and present sequelae. One year after diagnosis, all patients exhibited affective and behavioral sequelae, anterograde memory impairment, and executive functioning deficits. Two patients met the criteria for Korsakoff syndrome. Additionally, peripheral nervous system sequelae were observed, with all patients presenting with sensorimotor polyneuropathy. In conclusion, Wernicke’s encephalopathy requires a high diagnostic suspicion for timely intervention and prevention of irreversible sequelae, which can be devastating. Therefore, raising awareness among medical professionals regarding the significance of this disease is essential. Full article

Wernicke–Korsakoff Syndrome (WKS) is a severe neurological disorder resulting from thiamine deficiency, commonly associated with alcohol consumption but also stemming from dietary imbalances or other clinical conditions. Cognitive deficits, affecting memory and executive functions, pose a serious concern, with partial recovery often not complete. A 28-year-old woman underwent surgery for acute necrotizing hemorrhagic pancreatitis, leading to admission for post-acute intensive treatment due to prolonged bed rest syndrome. Clinical examinations revealed sensory–motor neuropathy, denervation in the active phase, mammillary body hyperintensity, and cognitive impairment. The patient exhibited poor orientation, lacked awareness of her clinical condition, and experienced impaired nonverbal memory, practical constructive issues, and planning difficulties—consistent with WKS. The patient received high-dose thiamine (300 mg TDS), coupled with daily physiokinesitherapy and occupational therapy. A final neuropsychological evaluation three months later showed substantial remission of executive and memory difficulties, improved spatial–temporal orientation, and enhanced awareness. The complex case required timely multidisciplinary intervention for accurate diagnosis and effective rehabilitation. The patient experienced rapid clinical improvement and cognitive recovery with high-dose thiamine and physiotherapy. Full article

The aim of this study was to objectively evaluate the hypothesis that the neuropsychological presentation of Korsakoff’s syndrome, the chronic phase of Wernicke–Korsakoff syndrome (WKS), is invariably a severe, selective amnesia against a background of relatively preserved general intellectual functions in a consecutive clinical sample. An analysis of the neuropsychological profiles of nine cases with a recorded history of WKS was undertaken. All cases were adult males (ages 32 to 70) with a long history of alcohol use disorder. Eight cases were chosen retrospectively on a consecutive basis from patient referrals. One additional case was recruited prospectively. Conventional understanding and some current opinion of Korsakoff’s syndrome predicts anterograde memory to be consistently more impaired than other cognitive abilities, but this was not found in this case series. The Mean Wechsler Delayed Memory Index was not significantly different from the Wechsler Full-Scale IQ (FSIQ), p = 0.130. Regression of Delayed Memory on FSIQ produced a non-significant intercept, p = 0.213. The ‘hallmark’ criterion of anterograde memory score at least 20 points less than intelligence score was observed in four of eight cases with available data, equating to a ‘sensitivity’ of 50%. Three of eight cases with available data had an FSIQ less than the memory score. Contrary to a common view, general intellectual function was not consistently preserved in Korsakoff’s syndrome relative to memory function. This study illustrates one of the specific merits of case series, namely, to critique an established view. Clinicians and researchers should expand their diagnostic criteria for Korsakoff’s syndrome to include more variable cognitive phenotypes, including a potentially reversible dementia-like impairment of variable severity, and focus on potential treatment opportunities. Full article

(1) Background: chronic alcohol use is consistently associated with impaired executive functioning, but its profile across the spectrum from mild to major alcohol-related cognitive impairment is, to date, unclear. This study aims to compare executive performances of patients with alcohol-induced neurocognitive disorder, including Korsakoff’s syndrome (KS), by using a computerized assessment battery allowing a fine-grained and precise neuropsychological assessment; (2) Methods: performances of 22 patients with alcohol-related cognitive impairment (ARCI) and 20 patients with KS were compared to those of 22 matched non-alcoholic controls. All participants were diagnosed in accordance with DSM-5-TR criteria and were at least six weeks abstinent from alcohol prior to assessment. Executive function was evaluated using four subtests of Cambridge Neuropsychological Test Automated Battery (CANTAB^®); (3) Results: significant differences between groups were found on spatial working memory (updating), sustained attention and inhibitory control, set shifting, and planning. Healthy controls performed significantly better than both patient groups (Games-Howell post hoc; p < 0.05), but no differences in performance were found between the ARCI and KS group; (4) Conclusions: ARCI and KS patients showed significant executive impairments, most prominent in updating, set-shifting and general planning abilities. Findings suggest equivalent levels of executive function in ARCI and KS patients. Our results highlight executive function as a significant hallmark of alcohol-induced neurocognitive disorder and stipulate the importance of early assessment and evaluation of skills to guide treatment. Full article

This paper begins with a short case report of florid, spontaneous confabulation in a 61-year-old man with an alcohol-induced Wernicke-Korsakoff syndrome. His confabulation extended across episodic and personal semantic memory, as well as orientation in time and place, as measured on Dalla Barba’s Confabulation Battery. Five other brief case summaries will then be presented, followed by a summary of the clinical, neurological, and background neuropsychological findings in three earlier series of Korsakoff patients. These observations will be considered in light of Wijnia’s recent and my own, earlier reviews of the Korsakoff syndrome. Taken together, they indicate the need for a multi-faceted approach (clinical, neurological, neuropsychological, and neuroimaging) to the assessment and diagnosis of the disorder. Full article

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. Full article

Alcohol-related cognitive disorders have long been an area of study, yet they continue to pose challenges in the diagnosis, treatment, and understanding of underlying neuropsychiatric mechanisms. The present article offers a comprehensive review of Wernicke’s Encephalopathy and Korsakoff’s Syndrome, two conditions often seen on a continuum of alcohol-related brain damage. Drawing on current medical literature, neuroimaging studies, and clinical case reports, we explore the neuropsychiatric and neuropsychological profiles, symptomatology, and differential diagnoses of these disorders. We delve into the biochemical pathways implicated in the development of WE and KS, notably thiamine deficiency and its impact on neurotransmitter systems and neural networks. The article also addresses the challenges in early diagnosis, often complicated by non-specific symptoms and co-occurring psychiatric conditions. Furthermore, we review the current state of treatment protocols, including pharmacological and non-pharmacological interventions. Finally, the article highlights gaps in current knowledge and suggests directions for future research to improve diagnosis, treatment, and patient outcomes. Understanding the nuanced interplay between the neuropsychiatric and neuropsychological aspects of WE and KS is crucial for both clinicians and researchers alike, in order to provide effective treatment and to advance our understanding of these complex conditions. Full article

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. Full article

In this perspective article, the authors give insight into the beneficial effects and the current developments in music therapy for patients with Korsakoff’s syndrome (KS) in the Netherlands. Music may be the key to distracting patients from negative moods, to help them express emotions and to teach them new skills on physical, psychosocial and cognitive levels. This may lead to improving the quality of life of patients with KS. Emphasis is placed on the personal experience of the authors and on the future directions in the field. Their experience, as well as the experience of music therapy colleagues working in the field with the same target population (joined together in the Music Therapy Korsakoff Expertise Group), is situated in the context of existing literature and showcases current developments in the specific field of music therapy and KS. Since literature on this specific topic is limited, the authors allowed themselves to delve into somewhat older but still leading and representative literature. There is still little knowledge on how music therapy may contribute to reducing the impairments patients with KS suffer and to improving their quality of life in general. Using the Empathic Directive Approach (EDA) as the starting point, the authors elaborate on different potential approaches and interventions. With this article, the authors aim to gain more insight into the potential role of the music therapist by highlighting music–therapeutic micro-interventions and to provide recommendations for future directions on how to integrate music therapy in the treatment of patients with Korsakoff’s syndrome. Full article

Background: Research on the use of psychotropic drugs in people with alcohol-related neurocognitive disorders is virtually nonexistent. We examined the prevalence of antipsychotic drug use and its effect on mortality among patients with Wernicke–Korsakoff syndrome (WKS) or alcohol-related dementia (ARD). Methods: In this nationwide register study, we collected data on the medication use and mortality of all persons aged ≥40 diagnosed with WKS (n = 1149) or ARD (n = 2432) between 1998 and 2015 in Finland. We calculated the prevalence of antipsychotic use within one year of diagnosis and the adjusted cumulative mortality of antipsychotic users versus non-users in relation to the age-, sex-, and calendar year-matched general population. Results: Of the WKS and ARD patients, 35.9% and 38.5%, respectively, purchased one or more antipsychotic drugs in the year following diagnosis. The adjusted cumulative mortality of the antipsychotic users was significantly lower than that of non-users in both the WKS and ARD groups, where the adjusted hazard ratios (95% CI) were 0.85 (0.72–0.99) and 0.73 (0.65–0.81), respectively. WKS and ARD patients using antipsychotics were less likely to die of alcohol-related causes than antipsychotic non-users, but the difference was significant only in the ARD group. Conclusions: This population-based study shows that antipsychotic use is common in patients with WKS or ARD. In contrast to other dementia studies, our results indicate that the mortality of antipsychotic users is significantly lower than that of non-users. The lower mortality could be explained by decreased alcohol use and better healthcare coverage in antipsychotic users. Full article