Wernicke encephalopathy (WE) is an acute life-threatening neurological condition caused by thiamine (vitamin B1) deficiency. Patients with WE often present with a triad of symptoms consisting of ophthalmoplegia, gait ataxia, and mental confusion. If WE is not treated in a timely manner, it can lead to serious complications such as confusion, coma, or death. Although alcohol abuse is the most commonly reported cause of WE, nonalcoholic causes-although rare-do exist. Herein, we present the case of a nonalcoholic woman with medullary infarctions who presented with intractable vomiting. Her clinical state subsequently progressed to include ophthalmoplegia and gait ataxia. A diagnosis of WE was suspected based on her clinical presentation; this was confirmed by brain magnetic resonance imaging (MRI) and the finding of decreased serum thiamine levels. Brain magnetic resonance imaging demonstrated the complete resolution of abnormal hyperintensities during a follow-up visit, 6 months after treatment.

We present a case of a woman in her 20s who presented to the emergency department with a 1-month history of blurry vision, lower extremity weakness in both legs and progressive numbness involving the feet and anterior chest. On admission, the patient was unable to ambulate. She was 3 months status post laparoscopic vertical sleeve gastrectomy for weight loss and using transdermal vitamin patches for nutritional supplementation. Laboratory values revealed low levels of vitamin B1, vitamin A, vitamin D, folic acid and copper levels. The patient was diagnosed with Wernicke encephalopathy and possible peripheral neuropathy secondary to thiamine deficiency. She was started on intravenous thiamine 500 mg three times a day and folate 1 mg one time a day for 3 days and then transitioned to oral thiamine 500 mg along with a multivitamin tablet. Improvement in ophthalmoplegia, weakness, sensation and cognition was noticed after initiating treatment.

BACKGROUND: Thiamine is the only therapy for prevention and treatment of Wernicke Encephalopathy among patients with Alcohol Use Disorder (AUD). Despite this fact, up to 75 % of inpatients with AUD are not prescribed thiamine during hospitalization. Even fewer patients are prescribed high-dose thiamine which many experts recommend should be standard of care. Previous attempts to improve thiamine prescribing for inpatients have had limited success.
METHODS: We conducted an evaluation of thiamine prescribing in the year before and year after an intervention to increase high-dose thiamine prescribing. Pre-post study analysis occurred on two distinct study cohorts: those with alcohol-related diagnoses and those with elevated alcohol levels. The intervention was new electronic health record-based decision support which encouraged high-dose thiamine when any thiamine order was sought. No educational support was provided. The primary outcome was prescription of high-dose thiamine before versus after intervention. Of those with alcohol-related diagnoses, the monthly percentage of thiamine treatment courses including high-dose thiamine were graphed on a control chart.
RESULTS: We examined 5307 admissions with alcohol-related diagnoses (2285 pre- and 3022 post-intervention) and 698 admissions with elevated alcohol levels (319 pre- and 379 post-intervention). Among admissions with alcohol-related diagnoses, the intervention was associated with a higher proportion of admissions receiving high-dose thiamine prescriptions in the first 24 h (4.7 % vs. 1.1 %, adjusted odds ratio 4.50, CI 2.93 to 6.89, p < 0.001). A similar difference in high-dose thiamine was seen post-intervention among admissions with elevated alcohol levels (14.3 % vs. 2.5 %, adjusted odds ratio 6.43, CI 3.05 to 13.53, p < 0.001). The control chart among those with an alcohol-related diagnosis demonstrated special cause variation: the median percentage of thiamine treatment courses including high-dose thiamine improved from 8.2 % to 13.0 %.
CONCLUSIONS: Electronic decision support without educational interventions increased the use of high-dose thiamine among patients with alcohol-related diagnoses and with elevated alcohol levels during hospitalization. This increase occurred immediately in the month after the intervention and was sustained in the year-long study period after.

Wernicke\'s encephalopathy (WE) is a prominent neurologic manifestation of thiamine (vitamin B1) deficiency. While often linked to alcoholism, it can also arise from various causes, including malabsorption, inadequate dietary intake, increased metabolic requirement, and among dialysis patients. Here, we present a case of altered mental status from acute metabolic encephalopathy attributed to sepsis, acute kidney injury (AKI), and hypoglycemia. WE was overlooked in the early hospitalization course due to the daily administration of thiamine. However, the patient\'s cognitive decline persisted despite the improvement of sepsis and AKI. Subsequent brain MRI revealed thalamic T2 signal intensity changes, suggesting either a past infarction or WE. Implementing an empirical regimen of high-dose thiamine resulted in the patient\'s rapid cognitive recovery. This therapeutic strategy was integrated into the management of her sepsis and AKI, leading to her full recovery and subsequent hospital discharge without complications.

Due to an increase in the worldwide prevalence of obesity and the efficiency of bariatric surgery, this procedure is more often performed. Besides its benefits, it has also disadvantages and may be the cause of nutritional deficiencies. Thiamin deficiency is particularly important to diagnose and to treat early as it can lead to major sequelae and even to death. Wernicke\'s encephalopathy is the most frequent presentation associating confusion, ataxia, ophtalmoplegia and nystagmus. The full triad is not usually observed, which may lead to sub-diagnosis of this affection. The diagnosis is clinical, biological and radiologic thanks to the brain MRI. Intravenous thiamin supplementation therapy must be administered as fast as possible in order to avoid long-term damages. In the ophthalmological field, the potential sequelae are ophthalmoplegia, nystagmus and optic neuropathy. Therapeutics for nystagmus are pharmacological, surgical and/or optical. We illustrate this condition with a case report of an 18-year-old man developing Wernicke\'s encephalopathy as early as six weeks after a sleeve gastrectomy.
Suite à une augmentation de la prévalence de l’obésité dans le monde et à l’efficacité de la chirurgie bariatrique, cette technique est pratiquée de plus en plus fréquemment. Malgré ses avantages, elle n’est pas sans risque et peut être responsable de déficits nutritionnels multiples. Le déficit en vitamine B1 ou thiamine est particulièrement important à connaître et, à rapidement diagnostiquer en raison des nombreuses séquelles invalidantes, voire le décès du patient, dont il peut être responsable. Le tableau classique est l’encéphalopathie de Gayet-Wernicke associant confusion, ataxie et troubles oculomoteurs. Néanmoins, il n’est pas toujours complet, ce qui participe au sous-diagnostic de cette pathologie. Le diagnostic est clinique, biologique et/ou radiologique grâce à l’IRM cérébrale. La supplémentation vitaminique intraveineuse doit être instaurée le plus rapidement possible afin d’éviter des séquelles à long terme. D’un point de vue ophtalmologique, les séquelles potentielles sont les ophtalmoplégies, les nystagmus et les neuropathies optiques. Les thérapies envisageables du nystagmus, outre la supplémentation en thiamine en aigu, sont pharmacologiques, chirurgicales et/ou optiques. Nous illustrons cette pathologie par un cas clinique d’encéphalopathie de Gayet-Wernicke dès la 6ème semaine post-opératoire d’une chirurgie bariatrique de type «sleeve» chez un patient de 18 ans.

Both neuromyelitis optica spectrum disorder (NMOSD) and Wernicke\'s encephalopathy (WE) involve brain lesions. However, their treatments are quite different. In this report, we describe the case of a 29-year-old woman with NMOSD, who presented with clinical and imaging findings similar to those of WE. She was admitted to our hospital with a headache, vomiting, and loss of appetite for two weeks and diplopia for nine days. Magnetic resonance imaging revealed lesions in the area postrema, periaqueductal gray matter, thalamus, and right frontal lobe. Vitamin B1 supplementation was ineffective. The patient was diagnosed with NMOSD because serum aquaporin-4 antibody was detected after admission. Her symptoms improved with immunotherapy. The possibility of NMOSD should be considered in patients with suspected WE.

Visual analysis of the current status, research hotspots, evolving trends, and future prospects in the field of thiamine-responsive megaloblastic anemia syndrome (TRMA), providing new insights and directions for subsequent research on the pathogenic mechanisms and prevention strategies of TRMA. Taking the core database of Web of Science as the literature source, selecting TRMA-related literature records published from 1997 to 2023 as the research object, and using R software and Citexs database to conduct visual analysis and discussion of the research content. The results showed that a total of 89 publications related to the topic were published from 1997 to 2023, with an average annual publication volume of 3 papers. Classified by country, it was found that the United States, and Israel among other countries and institutions, published a significant number of papers. Through keyword frequency analysis, high frequencies of keywords such as diabetes, deafness, thiamine-responsive megaloblastic anemia, and mutations in the solute carrier family 19 member 2 (SLC19A2) gene were observed, indicating that to date, these keywords have been the main research directions, highlighting a gradually reached consensus on the mechanism exploration of TRMA. In conclusion, TRMA research focuses on the mechanisms of hot topics such as diabetes, deafness, and thiamine-responsive megaloblastic anemia, and the core gene SLC19A2 research may currently become a new breakthrough point for future molecular studies.
对硫胺素响应性巨幼细胞贫血综合征（thiamine-responsive megaloblastic anemia syndrome，TRMA）研究领域的现状、研究热点、演变趋势和未来展望进行可视化分析，为TRMA发病机制的后续研究与防治策略提供新的思路和方向。本研究以Web of Science核心数据库为文献来源，以1997—2023年间发表的TRMA相关文献记录为研究对象，利用R软件及Citexs数据库对研究内容进行可视化分析和讨论。结果显示，1997—2023年共发表相关文献89篇，文献年均发文量3篇。按国家分类，发现美国和以色列等国家和机构发表论文较多。通过关键词频率分析，糖尿病、耳聋、硫胺素响应性巨幼细胞贫血及溶质载体家族19成员2（SLC19A2）基因突变等关键词出现的频率高，表明至今以上述关键词作为主要研究方向，凸显了对TRMA的机制探索逐渐达成共识。综上，当前TRMA以糖尿病、耳聋、硫胺素响应性巨幼细胞贫血等为研究热点，而核心基因SLC19A2的研究可能成为未来分子研究的新突破点。.

OBJECTIVE: Clinical diagnosis of Wernicke encephalopathy (WE) can be challenging due to incomplete presentation of the classical triad. The aim was to provide an update on the relevance of standard MRI and to put typical and atypical imaging findings into context with clinical features.
METHODS: In this two-center retrospective observational study, the local radiology information system was searched for consecutive patients with clinical or imaging suspicion of WE. Two independent raters evaluated T2-weighted imaging (WI), fluid-attenuation inversion recovery (FLAIR), diffusion WI (DWI), T2*WI and/or susceptibility WI (SWI), and contrast-enhanced (CE)-T1WI, and noted the involvement of typical (i.e., mammillary bodies (MB), periaqueductal grey (PAG), thalamus, hypothalamus, tectal plate) and atypical (all others) lesion sites. Unusual signal patterns like hemorrhages were also documented. Reported clinical features together with the diagnostic criteria of the latest guidelines of the European Federation of Neurological Societies (EFNS) were used to test for relationships with MRI biomarkers.
RESULTS: 47 patients with clinically confirmed WE were included (Jan \'99-Apr \'23; mean age, 53 yrs; 70% males). Interrater reliability for imaging findings was substantial (κ = 0.71), with lowest agreements for T2WI (κ = 0.85) compared to all other sequences and for PAG (κ = 0.65) compared to all other typical regions. In consensus, 77% (n = 36/47) of WE cases were rated MRI positive, with FLAIR (n = 36/47, 77%) showing the strongest relation (χ2 = 47.0; P < 0.001) compared to all other sequences. Microbleeds in the MB were detected in four out of ten patients who received SWI, not visible on corresponding T2*WI. Atypical findings were observed in 23% (n = 11/47) of cases, always alongside typical findings, in both alcoholics (n = 9/44, 21%) and non-alcoholics (n = 2/3, 67%). Isolated involvement of structures, explicitly PAG (n = 4/36; 11%) or MB (n = 1/36; 3%), was present but observed less frequently than combined lesions (n = 31/36; 86%). A cut-off width of 2.5 mm for the PAG on 2D axial FLAIR was established between cases and age- and sex-matched controls. An independent association was demonstrated only between short-term memory loss and changes in the MB (OR = 2.2 [95% CI: 1.1-4.5]; P = 0.024). In retrospect, EFNS criteria were positive (≥ 2 out of 4) in every case, but its count (range, 2-4) showed no significant (P = 0.427) relationship with signal changes on standard MRI.
CONCLUSIONS: The proposed sequence protocol (FLAIR, DWI, SWI and T1WI + CE) yielded good detection rates for neuroradiological findings in WE, with SWI showing microbleeds in the MB with superior detectability. However, false negative results in about a quarter of cases underline the importance of neurological alertness for the diagnosis. Awareness of atypical MRI findings should be raised, not only in non-alcoholics. There is limited correlation between clinical signs and standard MRI biomarkers.

A patient presenting to the emergency room with neurological symptoms is more commonly found to have manifestations of stroke, transient ischemic attack, or nervous system injury. Alcoholic Wernicke encephalopathy (WE) is also another common manifestation of neurological dysfunction; however, the prevalence of non-alcoholic WE is relatively uncommon. We discuss a 37-year-old male who presented to the ED with dysphagia, slurred speech, word-finding difficulty, and restricted extraocular movements from non-alcoholic WE in the setting of semaglutide use.

Physicians are occasionally confronted with patients presenting psychotic symptoms of organic origin. Therefore, precision in diagnosing the organic basis is pivotal for targeted treatment, addressing the underlying etiology. This case study delineates the nuanced phases of clinical reasoning employed to ascertain a diagnosis of Huntington\'s disease (HD), notably amidst concurrent alcohol dependence. A comprehensive clinical examination and meticulous review of the patient\'s medical history served as linchpins in guiding subsequent investigations toward identifying the etiological underpinnings of the psychotic symptomatology. Furthermore, this case sheds light on the uncommon overlap of HD and Wernicke\'s encephalopathy, compounding diagnostic complexities, especially given the polymorphic nature of HD. The diagnostic intricacies needed precise analysis of the clinical picture and a deep understanding of potential interactions between neurological pathologies and the deleterious effects of alcoholism on the nervous system.