When time is brain: a systematic review about Wernicke encephalopathy as a dramatic consequence of thiamin deficiency in hyperemesis gravidarum.

Nausea and vomiting affect up to 80% of all pregnancies, sometimes so severely that the condition of hyperemesis gravidarum (HG) is established. HG may in addition be a predisposing factor for Wernicke encephalopathy (WE), a severe and life-threatening condition due to vitamin B1 (thiamin) deficiency. If untreated, WE may progress to Korsakoff's syndrome, an irreversible cognitive disorder. We reported a case that recently occurred at our clinic and performed a systematic review of the literature to investigate the clinical presentation, maternal and perinatal outcomes and treatment of WE in women with HG. METHODS: We performed a systematic review of case series and case reports searching the Medline database on Pubmed from inception until December 2021. We used as search terms (Wernicke encephalopathy) OR (Wernicke-Korsakoff syndrome) AND (hyperemesis gravidarum) AND (pregnancy) AND (thiamin deficiency). Articles were considered eligible for inclusion in our review if they described at least one case of WE due to thiamin deficiency in relation to HG. An overall of 82 cases of WE due to HG in pregnancy from 66 manuscripts, including our own, were selected. RESULTS: The maternal mean age was 26.38 ± 5.23 years, while mean gestational week at hospitalization was 14.57 ± 4.12 after a mean of 6.6 ± 3.14 weeks of vomiting duration. WE manifestation occurred at a mean gestational age of 16.54 ± 3.06 weeks. Regarding clinical presentation, ocular signs and symptoms were reported by 77/82 (93.9%) women, 61/82 (74.4%) presented with ataxia and 63/82 (76.8%) with confusion. Dysarthria affected 15/82 women (18,3%), while muscular weakness was present in 36/82 (43.9%) and impaired reflexes in 42/82 (51.2%). Memory impairment involved 25/82 (30.5%) of the study population. Almost all cases reported a thiamin administration treatment, however data regarding the clinical course of the neurological condition and the perinatal outcomes were often missing and showed a great heterogeneity when reported. CONCLUSION: WE is a challenging diagnosis, as its clinical presentation is nonspecific. A high clinical suspicion and the awareness of its possible predisposing conditions such as HG may help clinicians to get a prompt diagnosis and starting treatment, which are vital to prevent possible life-impairing neurological sequelae.

Fetal demise and Wernicke-Korsakoff syndrome in a patient with hyperemesis gravidarum: a case report.

BACKGROUND: Wernicke-Korsakoff syndrome is a neuropsychiatric disorder caused by thiamine deficiency composed of two related disorders accounting for an acute presentation and chronic progression. Hyperemesis gravidarum presents a significant risk factor for Wernicke-Korsakoff syndrome as symptoms may rapidly progress in the setting of pregnancy. We present the first-reported case of hyperemesis-gravidarum-associated Wernicke encephalopathy in a patient in the first half of pregnancy in which a missed diagnosis led to septic shock, fetal demise, and eventual profound Korsakoff syndrome. CASE PRESENTATION: We present the case of a 33-year-old primigravid African American woman at 15 weeks gestational age who initially presented at a community emergency department with nausea and vomiting that ultimately progressed to severe hyperemesis-gravidarum-associated Wernicke-Korsakoff syndrome, fetal demise, and septic shock. The patient received a total of 6 weeks of high-dose parenteral thiamine. Magnetic resonance imaging of the head and formal neuropsychological assessment following treatment plateau confirmed the diagnosis of Wernicke-Korsakoff syndrome. CONCLUSIONS: The multisystem complications seen in severe thiamine deficiency can delay timely administration of high-dose thiamine, particularly in pregnancy, in which the classic triad of Wernicke-Korsakoff syndrome may not raise clinical suspicion due to rapid progression of neurological sequelae in this population. We advise a low threshold for parenteral thiamine repletion in pregnant women with persistent vomiting as hyperemesis gravidarum-induced severe thiamine deficiency can result in Wernicke-Korsakoff syndrome, sepsis, and fetal demise.

Wernicke-Korsakoff syndrome in a patient with tuberculous peritonitis.

Tuberculous peritonitis is an uncommon type of extrapulmonary tuberculosis and can be present in different and rare conditions. Wernicke-Korsakoff syndrome is a neuropsychiatric disorder due to thiamine deficiency which is caused by poor nutrition for any reason. The relationship between tuberculous peritonitis and the Wernicke-Korsakoff has not been declared yet. Therefore, we aim to report a case of tuberculous peritonitis which presented with Wernicke-Korsakoff syndrome.

Identifying, Assessing, and Treating Korsakoff Syndrome Patients: Updated Perspectives.

ABSTRACT: Objectives consist of updating published reports on the recognition, assessment, and care of patients with Wernicke-Korsakoff syndrome (WKS). Methods included defining relevant terms, describing core clinical phenomena, conducting meaningful reviews for latter-day WKS publications, and selecting instructive case examples. Findings covered epidemiology, precipitants, neuroimaging studies, alternate learning strategies in WKS, adjunctive treatments, and promising research. In conclusion, patients, their family members, clinicians, and public health experts should benefit from this updated knowledge. Countries with substantial alcohol consumption should consider emulating Holland in designating WKS research centers, founding regional clinical facilities, and funding multidisciplinary expert teams.

Low rates of thiamine prescribing in adult patients with alcohol-related diagnoses in the emergency department.

Background: Alcohol use disorder (AUD) is associated with thiamine deficiency and Wernicke-Korsakoff Syndrome (WKS). Thiamine supplementation for the prevention of WKS in patients with suspected AUD in the Emergency Department (ED) is generally recommended. As alcohol-related diagnoses are frequent reasons for visits to EDs, ED thiamine prescribing practices are relevant to the overall management and prevention of WKS in patients with AUD.Objective: To determine the prescription rates of thiamine to patients with alcohol-related diagnoses in the ED.Methods: This was a retrospective chart review conducted at two New York City urban teaching hospitals from January 1 to December 31, 2017. All patients 18 years or older who were given an alcohol-related diagnosis (all F10 ICD-10-CM codes) upon disposition were included. Collected data included details of thiamine prescribing practices, patient demographics and patient disposition.Results: A total of 7,529 patient visits with an alcohol-related diagnosis were identified. The overall median age of included patients was 44; 5747 (76.3%) patient visits were by men; 310 (4.1%) patient visits resulted in admission. Out of all patient visits, thiamine was ordered during 167 (2.2%) visits, with thiamine administered parenterally during 129 (77.2%) visits and orally in 38 (22.8%) visits. Out of patient visits specifically associated with an F10.2 (Alcohol Dependence) diagnosis, thiamine was ordered during 105 (17.8%) visits.Conclusion: We found a very low rate of thiamine prescribing during patient visits associated with alcohol-related diagnoses. This may be a missed opportunity to reduce morbidity and mortality among AUD ED patients.

Early Identification of Alcohol Use Disorder Patients at Risk of Developing Korsakoff's Syndrome.

BACKGROUND: The aim of the present study was to determine whether the Brief Evaluation of Alcohol-Related Neuropsychological Impairments (BEARNI), a screening tool developed to identify neuropsychological deficits in alcohol use disorder (AUD) patients, can also be used for the early identification of AUD patients at risk of developing Korsakoff's syndrome (KS). METHODS: Eighteen KS patients, 47 AUD patients and 27 healthy controls underwent BEARNI testing (including 5 subtests targeting episodic memory, working memory, executive function, visuospatial abilities, and ataxia) and a comprehensive neuropsychological examination. RESULTS: Performance of AUD and KS patients on BEARNI subtests was consistent with the results on the standardized neuropsychological assessment. On BEARNI, ataxia and working memory deficits observed in AUD were as severe as those exhibited by KS patients, whereas for visuospatial abilities, a graded effect of performance was found. In contrast, the subtests involving long-term memory abilities (episodic memory and fluency) were impaired in KS patients only. AUD patients with a score lower than 1.5 points (out of 6) on the episodic memory subtest of BEARNI exhibited the lowest episodic memory performance on the neuropsychological battery and could be considered at risk of developing KS. CONCLUSIONS: These findings suggest that BEARNI is a useful tool for detecting severe memory impairments, suggesting that it could be used for the early identification of AUD patients at high risk of developing KS.

Síndrome de Wernicke-Korsakoff em paciente gestante de 18 anos / Wernicke-Korsakoff syndrome in an 18-year-old pregnant patient

A síndrome de Wernicke-Korsakoff é uma rara encefalopatia desencadeada pela deficiência de tiamina, uma vitamina do complexo B, que atua como importante cofator de enzimas responsáveis pela manutenção da homeostase da energia cerebral. Apresentamos o caso de uma paciente de 18 anos, gestante, com diagnóstico prévio de pancreatite aguda biliar, que evoluiu à hiperêmese gravídica e à Wernicke-Korsakoff. Objetivamos, com este trabalho, chamar a atenção para a importância do diagnóstico imediato dessa síndrome diante de seu potencial em causar danos cerebrais irreversíveis, caso não tratada precocemente.

Wernicke-Korsakoff syndrome is a rare encephalopathy triggered by deficiency of thiamine, a B-complex vitamin, which acts as an important cofactor of enzymes responsible for maintaining brain energy homeostasis. We present the case of an 18-year-old pregnant woman with previous diagnosis of acute biliary pancreatitis, who developed hyperemesis gravidarum and Wernicke-Korsakoff. With this work, we aim to draw attention to the importance of the immediate diagnosis of this syndrome in view of its potential to cause irreversible brain damage if not treated early.

[The knowledge of resident doctors on diagnostics, etiology and treatment of Wernicke encephalopathy]. / De kennis van a(n)iossen over diagnostiek, etiologie en behandeling van wernicke-encefalopathie.

BACKGROUND: Wernicke encephalopathy (we) is a severe, acute neuropsychiatric disorder caused by a deficiency in thiamine. There have been indications that we is undertreated, which can lead to the Korsakoff syndrome, delirium or death. Treatment according to protocol is simple and effective. The knowledge of physicians about we has not been researched before.
AIM: To test the knowledge of resident doctors on diagnosis, etiology and treatment of we.
METHOD: The knowledge of 70 resident doctors in different medical specialties was examined through two clinical cases: the first with we due to hyperemesis gravidarum and the second due to alcohol abuse. Both open and multiple-choice questions were asked. Cues of the classical triad of we (cognitive disorder, eye movement disorder and gait disorder) were given accumulatively.
RESULTS: The classical triad of we was not recognized by 73% of the resident doctors in the case of hyperemesis gravidarum and they missed we in the case of alcohol abuse. Many of the resident doctors were not able to name the thiamin deficiency, the triad of we, more than three causes of we or the correct treatment with thiamine sufficiently. 67% of resident doctors indicated that their knowledge of we was insufficient and 76% expressed a need for more information about we.
CONCLUSION: The knowledge of resident doctors about the diagnostics, etiology and management of we is insufficient. Moreover, the resident doctors evaluate their knowledge about we to be insufficient. Medical school and postgraduate specialization have to focus more on this common and severe syndrome, which can appear in different medical areas.

Hyperemesis-induced Wernicke-Korsakoff Syndrome due to Hypergastrinemia during Long-term Treatment with Proton Pump Inhibitors.

We herein report a patient with Wernicke-Korsakoff syndrome (WKS) who had neither a history of alcoholism or of history of gastric surgery. A 56-year-old woman was transferred to our hospital because of the loss of consciousness and she was diagnosed to have Wernicke encephalopathy. She showed proton pump inhibitor-induced refractory hypergastrinemia with the subsequent development of hyperemesis and a vitamin B1 deficiency.

Wernicke-Korsakoff Syndrome: A Case Series in Liaison Psychiatry.

Wernicke-Korsakoff syndrome (WKS) is a life-threatening and underdiagnosed neuropsychiatric condition caused by thiamine deficiency that comprises Wernicke encephalopathy and Korsakoff syndrome. Although mainly associated with chronic alcoholism, WKS can arise from other circumstances. This report describes a series of cases of WKS that were clinically evaluated by liaison psychiatrists on a nonpsychiatric inpatient unit. The cases illustrate a deficit in the recognition and adequate treatment of WKS, demonstrating its clinical complexity and the need to improve physicians' knowledge.

[The 473rd case: renal failure, abdominal pain, and mental abnormality].

A 43-year-old male presented with elevated serum creatinine for 4 years and developed abdominal pain for 3 days. He started peritoneal dialysis 2 months ago. Dialysis-related peritonitis was ruled out and acute gastroenteritis was diagnosed. The patient was administrated with ertapenem 500 mg/d. An acute mental abnormality developed 3 days later. After excluded organic encephalopathy, ertapenem was discontinued for the suspicion of antibiotic-related encephalopathy. The frequency of peritoneal dialysis was increased to accelerate the clearance of antibiotics. However, the metal abnormality became even more severe. Then a diagnosis of Wernick-Korsakoff syndrome was considered. After the administration of high dose vitamin B(1), the mental disorder dramatically relieved. Vitamin B(1) 30 mg/d is maintained during peritoneal dialysis and the mental disorder does not relapse.

Prevalence and Improvement of Caine-Positive Wernicke-Korsakoff Syndrome in Psychiatric Inpatient Admissions.

BACKGROUND: Wernicke-Korsakoff Syndrome (WKS) resulting from thiamine deficiency is classically defined as including encephalopathy, ataxia, and ophthalmoplegia. Only 16% of autopsy-confirmed patients with WKS exhibit all three signs. Caine-positive WKS criteria include two or more of the following: nutritional deficiency, delirium or mild memory impairment, cerebellar dysfunction/ataxia, and oculomotor abnormalities. OBJECTIVE: We describe Caine-positive WKS prevalence among psychiatric inpatients and compare pretreatment-versus-posttreatment neurocognitive improvement to an unaffected group. METHODS: This 6-month quality-improvement evaluation included two-stage screening for Caine-positive WKS, administering high-dose intravenous thiamine (day 1: 1200 mg; days 2-4: 200 mg) with reexamination on day 5. We used descriptive statistics and fitted random effects models to examine rate-of-change differences in pre-/posttreatment Montreal Cognitive Assessment (MoCA), delayed 5-item recall, and gait/coordination scores between treated Caine-positive patients with WKS and untreated Caine-negative patients. RESULTS: Of 262 patients, 32 (12%) had Caine-positive WKS; 17 (53%) used alcohol currently. Treated Caine-positive WKS (n = 26) versus Caine-negative comparison (n = 34) before and after treatment observed a mean change (standard deviation) in the MoCA score of 3.6 (2.5) versus 1.8 (2.5) (P < 0.01); 5-item recall: 1.8 (1.4) versus 0.5 (1.4) (P < 0.001); gait/coordination scores: -0.6 (1.2) versus -0.1 (0.6) (P < 0.001). Oculomotor abnormalities were infrequent (n = 4 in Caine-positive WKS, n = 2 in Caine-negative comparison groups). CONCLUSIONS: Caine-positive WKS prevalence among psychiatric inpatients was 12%; only half used alcohol. Patients treated with high-dose thiamine demonstrated clinically significant neurocognitive improvement.

Future Thinking in Korsakoff Syndrome.

AIMS: Prior research has been mainly concerned with the ability of patients with Korsakoff syndrome (KS) to project themselves into the past. Little is known about the patients' ability to project themselves into the future. We therefore compared past and future thinking in patients with KS. METHODS: We invited patients with KS and control participants to retrieve past events and reconstruct future events. Participants were also invited to rate subjective characteristics (i.e. time travel, emotional feeling, and visual imagery) of the past and future events. RESULTS: Patients with KS demonstrated low specificity, time travel, and emotional experience during past and future thinking. However, while lower emotional experience was observed in patients with KS than in the control participants during future thinking, no significant differences were observed between the two populations during past thinking. Regarding within-group comparisons, patients with KS demonstrated no significant differences between past and future thinking in terms of specificity, time travel, and visual imagery; however, they demonstrated higher emotional experience during past than during future thinking. Regarding control participants, they demonstrated no significant differences between past and future thinking in terms of specificity, time travel, emotional experience, and visual imagery. CONCLUSION: Our findings demonstrate a diminished ability to construct specific future scenarios as well as a diminished subjective experience during future thinking in KS.

Wernicke-Korsakoff syndrome: Focus on low-threshold diagnosis and prompt treatment in the primary care setting.

The authors present the case of an inpatient hospitalized at the Veterans Affairs psychiatric unit diagnosed with Wernicke-Korsakoff syndrome to promote awareness of this prevalent yet often underdiagnosed and undertreated condition. Although Veterans present with a unique predisposition for alcohol abuse, it remains problematic in the general population as well. Analysis from 2000 to 2003 reveals alcohol use in the past month in Veterans at 56.6% and 50.8% in comparable non-Veterans. According to the National Survey on Drug Use and Health, it is estimated that of those who are 18 and older, 86.4% have used alcohol, 26.9% have engaged in binge drinking in the past month, 7% engaged in heavy alcohol use in the past month, and 6.2% (15.1 million) carried the diagnosis of alcohol use disorder., The lifetime prevalence of alcohol abuse in the general population is estimated to be between 4.5% and 13.2%. Primary care providers should maintain a high degree of vigilance in evaluating patients for timely diagnosis and prompt treatment of those suspected to have thiamine deficiency. Indeed, Wernicke's encephalopathy carries a significant level of morbidity and mortality associated with the syndrome, even in cases when it does not present with all of the classic signs. This article aims to raise the primary and ambulatory care provider's ability to recognize the condition, emphasize a low threshold to treat, and highlight current treatment recommendations.

Executive Dysfunction in Patients With Korsakoff's Syndrome: A Theory-Driven Approach.

AIMS: In addition to amnesia, executive deficits are prominent in Korsakoff's syndrome (KS), yet poorly studied. This study investigates the degree of executive dysfunction in patients with KS for the three main executive subcomponents shifting, updating and inhibition using novel, theory-driven paradigms. SHORT SUMMARY: Compared to healthy controls, patients with KS show impairments on the executive subcomponents shifting and updating, but not on inhibition. METHODS: Executive functions were measured with six carefully designed tasks in 36 abstinent patients with KS (mean age 62.3; 28% woman) and compared with 30 healthy non-alcoholic controls (mean age 61.8; 40% woman). ANOVAs were conducted to examine group differences and effect sizes were calculated. RESULTS: Compared to healthy controls, patients with KS were impaired on the executive subcomponents shifting and updating. No statistically significant group difference was found on the factor inhibition. CONCLUSIONS: Executive dysfunction in long-abstinent patients with alcoholic KS shows a profile in which shifting and updating ability are affected most. It also highlights that executive dysfunction is an important feature of KS and requires more attention in scientific and clinical practice, as these deficits may also affect daily functioning.

Review of thiamine deficiency disorders: Wernicke encephalopathy and Korsakoff psychosis.

Wernicke encephalopathy (WE) and Korsakoff psychosis (KP), together termed Wernicke-Korsakoff syndrome (WKS), are distinct yet overlapping neuropsychiatric disorders associated with thiamine deficiency. Thiamine pyrophosphate, the biologically active form of thiamine, is essential for multiple biochemical pathways involved in carbohydrate utilization. Both genetic susceptibilities and acquired deficiencies as a result of alcoholic and non-alcoholic factors are associated with thiamine deficiency or its impaired utilization. WKS is underdiagnosed because of the inconsistent clinical presentation and overlapping of symptoms with other neurological conditions. The identification and individualized treatment of WE based on the etiology is vital to prevent the development of the amnestic state associated with KP in genetically predisposed individuals. Through this review, we bring together the existing data from animal and human models to expound the etiopathogenesis, diagnosis, and therapeutic interventions for WE and KP.

Tell about yourself to improve your autobiographical memory: A study of Korsakoff's syndrome.

Korsakoff's syndrome (KS) has been associated with a difficulty to retrieve specific autobiographical memories. We investigated whether this difficulty can be alleviated after the retrieval of statements describing self-images. KS patients and control participants were recruited and asked to retrieve autobiographical memories after providing statements to the question "Who am I?" and after a control condition consisting of verbal fluency. Analysis showed higher autobiographical specificity in the "Who am I?" than in verbal fluency condition in both patients with KS and control participants. At a theoretical level, our findings demonstrate how retrieval of information related to conceptual self may influence autobiographical memory in KS. At a clinical level, our procedures are important as they demonstrate how a simple task (i.e., "Who am I?" statements) may serve as a tool to cue specific autobiographical memories in patients with KS.