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Background: Wernicke's encephalopathy can occur in oncology patients independent of alcohol use, likely resulting from poor dietary thiamine intake. High metabolic demands, such as those in acute illnesses seen in the emergency department (ED), can exacerbate thiamine deficiency. In this study, our objective was to assess the incidence of thiamine deficiency in ED oncology patients, which could lead to Wernicke's encephalopathy or other thiamine deficiency disorders if left untreated. Methods: This was a single-center prospective cohort study. We included patients with acute illness and a history of active cancer management in the ED of a large, urban hospital. We also included age and sex-matched control patients with no history of cancer who sought ED care. We excluded patients with a history of alcohol use or parenteral thiamine administration before enrollment. We recorded whole blood thiamine levels to measure total body thiamine stores and collected data on clinical variables, thiamine treatment, and adverse events. Results: In total, 87 oncology and 71 control patients were included in the study. The mean age was 62.1 ± 13.7 and 58.9 ± 12.6 years, respectively, and 48% of oncology vs. 55% of control participants were female. The most common cancers represented were colon (23%), lung (25%), prostate (10%), and breast (9%). Thiamine deficiency was significantly higher in ED oncology patients (25, 28.7%) compared to controls (6, 8.5%), odds ratio 4.4 (95% CI 1.7–11.4). None of the oncology patients with deficiency received thiamine treatment in the ED. Conclusions: Our findings suggest that thiamine deficiency is prevalent in acutely ill oncology patients, yet rarely treated in the ED. [ABSTRACT FROM AUTHOR]

Wernicke's Encephalopathy (WE) is a rare but severe condition primarily caused by thiamine deficiency, often seen in pregnant women who experience severe vomiting, such as in hyperemesis gravidarum. This case report details a 38-year-old woman at 27 weeks of gestation who developed altered consciousness, cerebellar ataxia, and hyperlactatemia following persistent vomiting. Brain MRI demonstrated characteristic bilateral abnormalities consistent with WE. Early recognition and prompt intravenous thiamine administration led to significant clinical improvement. This case emphasizes the importance of maintaining a high level of suspicion and initiating immediate treatment to prevent irreversible neurological damage in pregnant women presenting with severe vomiting and neurological symptoms. A unique feature of this case is the development of Wernicke's encephalopathy secondary to hyperemesis gravidarum, further complicated by severe pre-eclampsia.

The article in the Journal of Neurology discusses two cases of fixational upbeat nystagmus, a rare condition where nystagmus appears or increases while fixating on a target straight ahead. The cases presented involve a 54-year-old man with unsteadiness and a 43-year-old woman with recurrent vertigo. The article also includes a literature review of 37 patients with fixational upbeat nystagmus, highlighting various causes such as acute stroke, Wernicke's encephalopathy, and multiple sclerosis, with lesions mainly affecting the dorsal pons or caudal medulla. [Extracted from the article]

Background: Wernicke's encephalopathy (WE) is an acute neurological syndrome resulting from thiamine (vitamin B1) deficiency. It has been recognized increasingly in non-alcoholic patients, such as in the condition of malnutrition. Recent literature has shed light on uncommon symptoms and neuroimaging findings. Case report: We reported a case of a 44-year-old male who initially presented with bilateral hearing loss, and exhibited abnormality in the splenium of the corpus callosum on magnetic resonance imaging (MRI) diffusion-weighted imaging sequence. On the following day the patient developed new symptoms, including unstable walking, double vision and hallucination. The subsequent brain MRI demonstrated lesions involving periaqueductal grey matter and bilateral medial thalamus, indicating the diagnosis of WE. Empirical treatment with intravenous thiamine resulted in complete clinical and radiological resolution. Conclusion: To the best of our knowledge, the current case is the first report of WE in literature with uncommon but reversible manifestations. This case warns us to maintain a heightened level of suspicion for WE in malnourished patients with neurological deficits, despite the possibility of atypical presentations encompassing bilateral hearing disturbances and unusual neuroradiological results. Early diagnosis and timely administration of thiamine in WE are likely to lead to a favorable outcome and full recovery. [ABSTRACT FROM AUTHOR]

Abstract This case report presents the clinical findings of a female patient diagnosed with Wernicke’s encephalopathy, characterized by pinpoint pupils. While pupillary changes can occur in Wernicke’s encephalopathy, the presence of pinpoint pupils is exceedingly rare. In this report, we aim to document and discuss this unusual presentation, as well as speculate on the potential mechanisms underlying this atypical manifestation of the disease.

AbstractWernicke’s encephalopathy (WE) is a dangerous and potentially fatal neurological condition associated with thiamin deficiency. The standard treatment for WE is intravenous (IV) thiamin, but limited research describes optimal dosing. We present a case of a 40-year-old male with severe alcohol use disorder (AUD) and chronic malnourishment who developed WE. Upon administration of 100 mg IV thiamin, symptoms of WE persisted, but when the dose was increased to 500 mg, altered mental status and ophthalmoplegia resolved rapidly. IV thiamin is a reliable and low-risk treatment for WE, even when administered at high doses. High-dose IV thiamin (i.e., >/100 mg) can treat neurological symptoms and cognitive dysfunction in WE and should be considered for first-line treatment. Further study of WE diagnostic and treatment guidelines is warranted to maximize recovery potential. [ABSTRACT FROM AUTHOR]

Background: Thiamine deficiency disease may occur in infants from thiamine-deficient mothers in developing countries, as well as in infants fed solely with soy-based formula. Thiamine deficiency in infants may present with acute neurological manifestations of infantile encephalitic beriberi. Objective: To review the role of noncontrast CT brain findings in infantile encephalitic beriberi in early diagnosis. Materials and methods: A retrospective review of noncontrast CT scans of the brain in 21 infants with acute-onset infantile encephalitic beriberi was carried out. Results: On noncontrast-enhanced CT brain, hypodense lesions were seen symmetrically in the putamen in all the babies; symmetric hypodensities were seen in the caudate nuclei in 14/21 (67%), in dorsomedial thalami/hypothalamic/subthalamic area in 4/21 (19%), and in the globi pallidi in 2/21 (9.5%) of the infants. Conclusion: Recognition of symmetrical hypodense lesions in the basal ganglia and medial thalami/hypothalamic/subthalamic area on noncontrast CT scan of the brain are important early features to recognize in encephalitic beriberi in at-risk infants. Advances in knowledge: IEBB is a cause of hypodense bilateral basal ganglia and may be identified by this finding in the appropriate clinical settings. [ABSTRACT FROM AUTHOR]

Wernicke's encephalopathy (WE) is a major central nervous system disorder resulting from thiamine deficiency (TD) in which a number of brain regions can develop serious damage including the thalamus and inferior colliculus. Despite decades of research into the pathophysiology of TD and potential therapeutic interventions, little progress has been made regarding effective treatment following the development of brain lesions and its associated cognitive issues. Recent developments in our understanding of stem cells suggest they are capable of repairing damage and improving function in different maladys. This article puts forward the case for the potential use of stem cell treatment as a therapeutic strategy in WE by first examining the effects of TD on brain functional integrity and its consequences. The second half of the paper will address the future benefits of treating TD with these cells by focusing on their nature and their potential to effectively treat neurodegenerative diseases that share some overlapping pathophysiological features with TD. At the same time, some of the obstacles these cells will have to overcome in order to become a viable therapeutic strategy for treating this potentially life-threatening illness in humans will be highlighted. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction: Vitamin B1 deficiency poses a significant risk of impaired consciousness, with manifestations ranging from anorexia and fatigue to severe neurological and cardiovascular disturbances. Wernicke's encephalopathy, a neurological disorder stemming from vitamin B1 deficiency, presents as the triad of ophthalmoplegia, altered mental state, and cerebellar ataxia. However, these symptoms are not consistently present, complicating the diagnosis. In addition, subclinical vitamin B1 deficiency can progress unnoticed until severe complications arise. Studies indicate a high rate of undiagnosed cases, emphasizing the need for early detection and intervention. Case presentation: We present the case of a 65-year-old man in whom hyperlactatemia was incidentally detected, leading to the diagnosis of vitamin B1 deficiency. The patient, presenting with vertigo and vomiting, had been eating boxed lunches bought from convenience stores following the death of his wife 3 years earlier. Vertigo gradually improved with rest, but the persistence of hyperlactatemia prompted further investigation, revealing low vitamin B1 levels and high pyruvate levels. Treatment with dietary adjustments and supplements significantly improved his symptoms. Discussion: In this case, hyperlactatemia was found in a vertigo patient, revealing asymptomatic vitamin B1 deficiency. Elevated lactate is often linked with conditions like sepsis but can also stem from overlooked factors such as low vitamin B1 levels due to poor diet habits like consuming fried foods. Conclusion: This case highlights the importance of considering vitamin B1 deficiency in patients with unexplained hyperlactatemia, even in high-income countries. Early detection can prevent progression to the severe complications associated with Wernicke's encephalopathy. Proactive measurement of lactate levels in at-risk populations may facilitate early diagnosis and intervention, ultimately improving patient outcomes. [ABSTRACT FROM AUTHOR]

This case report presents the clinical findings of a female patient diagnosed with Wernicke's encephalopathy, characterized by pinpoint pupils. While pupillary changes can occur in Wernicke's encephalopathy, the presence of pinpoint pupils is exceedingly rare. In this report, we aim to document and discuss this unusual presentation, as well as speculate on the potential mechanisms underlying this atypical manifestation of the disease. [ABSTRACT FROM AUTHOR]

A 65‐year‐old woman developed metronidazole‐induced encephalopathy (MIE) with prolonged severe total aphasia during treatment for a liver abscess. Brain magnetic resonance imaging revealed restricted diffusion in the left cerebral white matter and splenium of the corpus callosum, along with transient cortical lesions but no typical dentate nucleus involvement. Despite discontinuing metronidazole, aphasia persisted for over a month, leading to her death. Although the white matter lesions persisted, the cortical lesions diminished after 2 weeks. This case highlights that MIE can involve the cerebral cortex, similar to Wernicke's encephalopathy. [ABSTRACT FROM AUTHOR]

This document is a reply to a comment made by Paranhos-Baccalà et al. regarding a recent article published in the journal Diagnostics. The authors of the reply acknowledge the sensitivity controversy surrounding the Biofire Filmarray ME panel, specifically in relation to HSV-1. They discuss the limitations of their study and address concerns raised by Paranhos-Baccalà et al. regarding the representativeness of the samples studied. The authors emphasize the importance of clinical correlation in the diagnosis of meningoencephalitis and suggest that larger comparative studies are needed to confirm their findings. [Extracted from the article]

This document provides an overview of the diagnosis and treatment of hyperemesis gravidarum, a severe form of nausea and vomiting during pregnancy. It highlights that the cause of hyperemesis gravidarum is not fully understood, but certain factors may increase the risk. Treatment options aim to reduce symptoms and improve quality of life, with antiemetics being the most effective. The document also discusses the potential adverse effects on both the pregnant person and their baby, as well as the importance of addressing their physical and mental health needs. It provides information on various treatment options, including approved medications and nonpharmacologic therapies. However, it emphasizes that there are still many unanswered questions and a need for further research in this area. [Extracted from the article]

Introduction: Dural arteriovenous fistula (DAVF) is an uncommon malformation involving an abnormal connection between dural arteries, or the pachymeningeal branches of cerebral arteries, and dural veins. Its exact pathogenesis remains elusive. Known potential triggers for DAVF include cerebral venous sinus thrombosis (CVST), trauma, ear infections, and cranial surgeries. Due to its rarity and diverse clinical presentations, diagnosing DAVF can be a challenge. Case description: We present a case of DAVF associated with CVST, manifesting as rapidly advancing parkinsonism accompanied by dementia over a month. Brain magnetic resonance imaging (MRI) revealed bilateral symmetric T2 hyperintensities in the basal ganglia and brain stem. Cerebral angiography further confirmed a fistula between the torcular herophili and the transversesigmoid sinuses. Despite strong recommendations for transvenous embolization of the fistula, the patient declined the procedure. The anticoagulant therapy and symptomatic treatments administered did not yield any improvement in the patient's condition. Additionally, we reviewed 27 DAVF-derived parkinsonism and dementia cases. Conclusion: DAVF must be considered in the differential diagnosis of cases of rapidly progressive parkinsonism with concurrent dementia. Given its potential for treatment and reversibility, timely diagnosis and intervention for DAVF are paramount. [ABSTRACT FROM AUTHOR]

We describe an overweight but otherwise previously healthy 17-year-old female who presented with altered mental status and rapidly progressive weakness. She was ultimately diagnosed with Wernicke encephalopathy and wet beriberi resulting from severe thiamine deficiency. She required admission to the pediatric ICU because of hypoventilation with progressive weakness and worsening encephalopathy and was found to have impaired cardiac function as assessed by echocardiography. Her heart function and encephalopathy improved on initiation of thiamine repletion. She remained in inpatient rehabilitation for 10 months but still remained weak at discharge. Thiamine deficiency is not commonly considered in the United States as a diagnosis other than in patients with severe alcohol use disorder. However, thiamine may be depleted in as little as 2 weeks if nutrition is inadequate. In such a setting, thiamine deficiency is an important etiology to consider early in the pediatric patient with altered mentation especially because it can be readily and safely treated. [ABSTRACT FROM AUTHOR]

Background Alcohol use has an effect on nutritional status, with nutritional deficiencies being a major contributor to morbidity, for example Wernicke's encephalopathy. Currently, there is an absence of best-practice guidelines to support general practitioners (GPs) in the identification and management of malnutrition and nutritional risk factors in patients who drink at risky levels. Objective This article reviews some of the nutritional considerations in patients who drink at risky levels or who have alcohol dependence, with the aim of enhancing GPs' awareness of the nutritional considerations in this patient group. Discussion Nutritional risk factors extend beyond body mass index (BMI), and patients might present with a healthy BMI and be malnourished. Screening for risk of malnutrition and other nutritional deficiencies followed by supplementation and consideration of referral to multidisciplinary services, including a dietitian, is likely to improve patient outcomes. [ABSTRACT FROM AUTHOR]

Wernicke's encephalopathy (WE) is a serious neurological disorder that is underdiagnosed. Despite limited clinical guidelines, the standard use of intravenous (IV) thiamine is underutilized and remains an area of research deserving much attention. We conducted a systematic review using Medline, Embase, and CENTRAL databases to identify and summarize the literature on IV thiamine treatment in WE. Human studies with WE patients who received ≥100 mg of thiamine IV met inclusion criteria. Randomized controlled trials, cross-sectional studies, and case reports were included. A total of 27 studies were included: 20 case reports, five retrospective studies, one prospective study and one randomized control trial. Of the case reports, 11 (55%) cases were female, and the average age of all cases was 45 years (SD = 15). The other seven studies included 688 patients; the average age was 52 years (SD = 9), and 266 (38.7%) were female. Among the case reports, neurological and clinical findings were used to diagnose WE in 16 (80%) cases. MRI was utilized to diagnose 15 (75%) cases. 500 mg IV thiamine TID was reported in 12 case reports (60%). 18 (90%) of case reports had partial or complete resolution of symptoms following IV thiamine. IV thiamine can alleviate neurological symptoms, cognitive dysfunction, and brain imaging lesions associated with WE. We found key limitations in the evidence for IV thiamine and diagnostic standards for WE. Future targeted research should establish clear diagnostic and treatment guidelines for WE to prevent this serious condition from being underdiagnosed or undertreated. [ABSTRACT FROM AUTHOR]

This article provides an overview of altered mental status (AMS) in geriatric patients, focusing on the causes, risk factors, and diagnostic approach. AMS refers to a condition where there is impairment in a patient's level of consciousness, cognition, or behavior. It is estimated that at least 25% of emergency department patients over the age of 65 have some form of AMS. Risk factors for delirium, a common form of AMS, include low education attainment, poor functional status, advanced age, use of certain medications, malnutrition, and comorbidities. The article emphasizes the importance of a thorough evaluation, including history-taking, physical examination, laboratory testing, and imaging studies, to determine the underlying cause of AMS. Treatment should be tailored to the specific etiology. The text discusses various factors that can contribute to acute brain dysfunction in elderly patients. These factors include electrolyte imbalances, renal dysfunction, hepatic dysfunction, thyroid dysfunction, medication-induced effects, and substance misuse. The management of delirium focuses on addressing the underlying cause, while the treatment of stupor and coma requires immediate evaluation and stabilization. The text emphasizes the importance of considering the unique physiological changes and risks associated with aging when assessing and treating elderly patients with acute brain dysfunction. The text discusses the importance of considering acute ingestion and substance use disorders as potential causes of altered mental status (AMS) in older adults. It highlights alcohol as the most commonly ingested drug in this population, with high-risk drinking and binge [Extracted from the article]

Thiamine deficiency (TD) among adolescents following metabolic and bariatric surgery (MBS) has not been assessed. We assessed TD among adolescents following MBS. University Hospital. A retrospective chart review was conducted for all adolescents and young adults (aged 10–25 years) who had MBS and subsequently presented with TD at our institution (n = 30). Diagnosis used clinical, laboratory, brain imaging, and neurophysiology criteria. Of 1575 patients, 7 subsequently had TD. Another 23 adolescents had MBS at private hospitals or overseas and presented at our institution with TD. Based on MBS undertaken at our institution, TD prevalence was.45 cases per 100 MBS. The mean age of patients was 19.5 ± 3.23 years, 53.3% were male, 96.7% had sleeve gastrectomy, and time from MBS to admission averaged 4.97 ± 11.94 months. Mean weight loss from surgery to admission was 33.68 ± 10.90 kg. Associated factors included poor oral intake (90%), nausea and vomiting (80%), and noncompliance with multivitamins (71%). Signs and symptoms included generalized weakness, nystagmus, numbness, and paraparesis (83.3%–80%). Seven patients had Wernicke encephalopathy full triad; 16 displayed a mixed picture of Wernicke encephalopathy and dry beriberi; and there were no cases of wet beriberi. Half the patients achieved complete resolution of symptoms, whereas 47% and 40% had residual weakness or persistent sensory symptoms, respectively. There was no mortality. Most common concurrent nutritional deficiencies were of vitamins K, D, and A. This is the first in-depth study of TD among adolescents after MBS. Although TD is uncommon among adolescents after MBS, it is serious, requiring diligent suspicion and prompt treatment. Bariatric teams should emphasize compliance with multivitamin regimens and follow it up. • This is the first study to comprehensively assess thiamine deficiency among adolescents after bariatric surgery. • Most common risk factors are poor oral intake, vomiting, and non-compliance to multivitamins. • Seven patients had Wernicke's encephalopathy (WE) triad; 16 displayed a mixed picture of WE and dry beriberi; and no cases of wet beriberi. • At 2 months, 50% patients had completely recovered, about 50% had residual symptoms and no mortality were reported. • Low vitamin K, D, A and albumin were the most common concurrent deficiencies. [ABSTRACT FROM AUTHOR]

Introduction: Wernicke's encephalopathy (WE) is a potentially reversible, yet serious neurological emergency due to vitamin B1 (thiamine) deficiency. It is a rare but known complication of hyperemesis gravidarum. Prolonged vomiting in pregnancy results in thiamine depletion. Most frequently Wernicke's encephalopathy is found among persons suffering from chronic alcoholism. It can also occur in any medical condition producing malnourishment and causing thiamine deficiency including gastric bypass, a variety of gastrointestinal disorders, malignant diseases, anorexia nervosa, hyperthyroidism and prolonged intravenous feeding. Magnetic resonance imaging (MRI) is sensitive and specific for diagnosis and follow up evaluation. Most patients present with the triad of ocular signs, ataxia, and confusion. It can be associated with life-threatening complication like central pontine myelinolysis. Although early recognition and treatment with thiamine can reverse the symptoms, the mortality rate remains 10-20% due to underdiagnosis. It is frequently not identified until autopsy. Here we stress upon the importance of early diagnosis and prompt treatment of WE. The aim of this report is to present case of Wernicke's encephalopathy induced by hyperemesis gravidarum. The course of the disease, clinical signs, diagnostic tools, treatment and its results are presented.

Adequate and timely assessment of learning abilities in individuals with alcohol-related cognitive disorders is highly relevant to optimize addiction care. Learning curves of episodic verbal memory tests have been used to assess learning ability in various neurocognitive disorders, but studies in alcohol-related cognitive disorders are lacking. Therefore, this study investigated California Verbal Learning Test (CVLT) learning curves in individuals with alcohol-use disorder (AUD) with and without cognitive impairments and examined associations between learning curves and changes in everyday functioning following a multicomponent care program. We fitted learning curves over the five immediate recall trials of the Dutch version of the CVLT of patients with Korsakoff's syndrome (KS; N = 117), alcohol-related cognitive impairment no KS (ARCI; N = 147), and uncomplicated AUD (N = 43) using a generalized non-linear mixed regression. This model was based on three different parameters: initial memory performance (attention), maximum number of correctly recalled words (maximum learning), and the increase of correctly recalled words over the trials (learning rate). Next, we related these learning curves with ratings of everyday activities using the Patient Competency Rating Scale (PCRS) before and after a care program following admission. Modelled learning curves differed across groups, with significant differences in Attention (KS0.05). Although modelled learning curves may be used to differentiate diagnostic groups in alcohol-related cognitive disorders, future studies are needed to establish the criterion validity of learning curves in this population.

This letter to the editor discusses the case of a 58-year-old man with Acute Wernicke Encephalopathy who underwent PET/CT imaging. The imaging revealed increased uptake in the midbrain, medial thalamus, and periaqueductal gray matter, which contrasts with previously reported decreased uptake associated with Wernicke encephalopathy. Treatment with vitamin B1 injection resulted in clinical improvement, and follow-up imaging showed decreased FDG uptake in the cerebral lesion areas. The study suggests that PET/CT can reflect glucose metabolism changes in vital organs affected by thiamine deficiency in Acute Wernicke's encephalopathy. [Extracted from the article]

Objective: Pregnancy, a nutritionally demanding situation in terms of macro‐ and micronutrient supply owing to heightened maternal, placental, and fetal needs, significantly affects thiamine reserves. Thiamine deficiency during pregnancy and the postpartum period, presenting with varied manifestations and outcomes, is a relatively common condition in our population. The study aimed to understand the various manifestations and outcomes of acute thiamine deficiency in pregnant and postpartum women, emphasizing the significance of early recognition and thiamine therapy to prevent serious complications during pregnancy and after childbirth. Methods: This prospective study conducted in a tertiary care center in North India enrolled consecutive pregnant and postpartum women presenting with clinical features consistent with thiamine deficiency disorders, such as thiamine deficiency‐related neuropathy, high‐output heart failure, heart failure with reduced ejection fraction, Wernicke's encephalopathy, gastric beriberi, and thiamine‐responsive acute pulmonary hypertension. In addition to capturing medical history including drug intake, dietary consumption, and comorbidities, women underwent brief relevant clinical examinations and laboratory assessments, including whole‐blood thiamine levels. Response to intravenous thiamine supplementation was also monitored. Results: Data of 31 women (12 pregnant, 19 postpartum) with a diagnosis of acute thiamine deficiency and a mean age of 28.88 ± 2.69 years were analyzed. The mean thiamine level was 1.28 ± 0.44 μg/dL with mean blood lactate of 3.46 ± 3.33. The most common presentation was gastric beriberi (n = 10), followed by paraparesis (n = 6), high‐output heart failure (n = 6), acute pulmonary hypertension, heart failure with reduced ejection fraction (n = 3 each), and an acute confusional state (n = 2). All patients responded to thiamine challenge. Conclusion: In the context of borderline thiamine status, particularly in our population with endemic thiamine deficiency and heightened demand for thiamine during pregnancy and the peripartum period, the deficiency can have varied and serious manifestations of dry and wet beriberi. Early recognition of the clinical features and thiamine therapy can be life‐saving. There is a need for validated clinical criteria owing to the non‐availability of thiamine testing in resource‐limited settings. Synopsis: Thiamine deficiency in hypermetabolic states like pregnancy can have serious consequences for the mother as well as the infant. Early recognition can be life‐saving. [ABSTRACT FROM AUTHOR]

This document is an update of the British Society for Haematology guideline on the management of myelofibrosis. It provides healthcare professionals with clear guidance on the stratified management of primary myelofibrosis, as well as post-polycythemia myelofibrosis and post-essential thrombocythemia myelofibrosis. The guidelines discuss various treatment options, including medications like ruxolitinib, fedratinib, momelotinib, and pacritinib, as well as other therapies such as hydroxycarbamide, pegylated interferons, and anti-platelet agents. The document also covers the management of MF-associated anemia, the use of erythropoiesis-stimulating agents, danazol, immunomodulating drugs, and iron chelation therapy. It provides recommendations for special situations and emphasizes the importance of individualized treatment approaches and patient support. Additionally, the document provides recommendations for pretransplant workup, donor choice, conditioning choice, and post allo-HSCT monitoring for patients considering allogeneic stem cell transplantation. [Extracted from the article]

Dihydrolipoamide dehydrogenase (DLD) deficiency is a rare disease of genetic origin due to the malfunctioning of a shared subunit of three mitochondrial multi-enzyme complexes. Phenotypes of this disease are a set of clinical manifestations ranging from neonatal disorders to myopathy or recurrent episodes of liver failures, and vomiting for which no adequate or definitive treatment is currently available. This study described a case involving a 16-year-old boy who had experienced recurrent vomiting of unknown cause from age two. Normal value ranges for the basic metabolic panel were reported in previous years. The patient was admitted with Wernicke's encephalopathy after the last vomiting attack, also indicating metabolites of organic acids compatible with DLD deficiency. Whole exome sequencing identified a known pathogenic mutation in the DLD gene, leading to a diagnosis of DLD deficiency. Our patient was treated with a high dose of thiamine supplementation and continued treatment, has not experienced any vomiting attacks or related problems in the last two years and has adequately responded to the treatment prescribed. Normal urine organic acid levels in patients with recurrent vomiting cannot roll out DLD deficiency. However, although thiamine deficiency typically induces Wernicke's encephalopathy, it can also be implicated in pyruvate dehydrogenase complex (PDHc) deficiency, and high-dose thiamine therapy (with doses up to 30 mg/kg) is recommended for deficient patients. [ABSTRACT FROM AUTHOR]

Thus, the preferential saccadic impairments and truncal ataxia observed in our patient may corroborate the cerebellar midline lesion observed in most patients. Indeed, metronidazole-associated peripheral neuropathy is found in one-third of patients with metronidazole CNS toxicity [[1]]. C Fluid-attenuated inversion recovery (FLAIR) images show high-signal-intensity lesions involving the dentate nuclei (yellow arrows), superior colliculi (yellow arrowheads), and splenium of the corpus callosum. Notably, a characteristic reversible dentate nuclei lesion is found in 90% of patients [[1], [4]]. [Extracted from the article]

Alcohol use disorders (AUDs) are prevalent in intensive care units (ICUs). Alcohol abuse and/or dependence, leading to alcohol withdrawal syndrome (AWS), is as high as 10% or more. There seem to be wide variations in management strategies used to manage these patients, prompting an evaluation of the knowledge gap as well as finding the barriers. Noting lack of such literature in the Indian setting, a survey is undertaken to evaluate practice patterns surrounding the identification and management of alcohol dependence/abuse and AWS in the Indian critical care scenario. The main respondents of the survey are independent practitioners with anesthesia as their base specialty and overwhelmingly practice in multidisciplinary ICUs. They estimated AUD prevalence to be under 10%. The reason most expressed for lack of AUD documentation is fear of insurance rejection. Very few used risk assessment tool in evaluation of AUDs and AWS. Awareness of ICD 10/DSM-V components of AWS diagnosis was negligible. Chlordiazepoxide and lorazepam were used either in a fixed- or symptom-based therapy. Compared to available literature, haloperidol use is excessive, while barbiturates rarely. The wide variation is seen with the dose and frequency of thiamine in AWS without neurological complications. The impact on mortality and morbidity is poorly understood. In conclusion, the survey reported a lower prevalence compared to international literature. Insurance rejection is one of the main factors in limiting adequate history taking or documenting AUDs. Alcohol withdrawal syndrome risk assessment, monitoring, and management is variable and suboptimal. Variability in all aspects of AUDs is attributable to the knowledge gap. Further studies are needed to bridge the research gap. [ABSTRACT FROM AUTHOR]

This article discusses a case study of a pregnant woman from The Gambia who developed Wernicke's encephalopathy (WE) in the United States. The article emphasizes the importance of understanding the patient's cultural beliefs and seeking effective communication in healthcare. It also explores the challenges in diagnosing and treating WE, as well as the potential long-term effects of the condition. The article briefly mentions other neurologic conditions that should be considered in the differential diagnosis for altered mental status in pregnancy. Ultimately, the patient had a successful delivery and a healthy baby. [Extracted from the article]

Background and Purpose: Wernicke's encephalopathy (WE) is a severe acute disorder related to thiamine deficiency. This study was aimed at revealing the relationship between clinical and imaging findings and WE recovery. Methods: We retrospectively reviewed 34 cases of WE diagnosed between 2003 and 2020 (median age: 57 years, 14 females) at two academic institutions. WE cases were divided into two groups with symptomatic recovery within 4 weeks (group 1) or later (group 2). The lesion sites were divided into typical and atypical sites (total sites defined as when either typical or atypical sites were involved). Clinical and MRI features were compared between them as appropriate. Results: WE patients were divided into group 1 (19 cases, median age: 57 years, 10 females) and group 2 (15 cases, median age: 57 years, four females). Regarding clinical features, only cerebellar ataxia was more often observed in group 1 than in group 2. Regarding MRI features, signal abnormality on T2‐weighted image (WI)/fluid‐attenuated inversion recovery (FLAIR) was more often observed in atypical sites between groups 1 and 2 (1/19 vs. 7/15; p =.01). There were significant differences between groups 1 and 2 regarding the presence of both vasogenic edema and cytotoxic edema in total sites (4/11 vs. 11/15, p =.005; 1/19 vs. 6/15, p =.03), with a significant difference in the presence of vasogenic edema in typical sites (4/19 vs. 10/15, p =.01). Conclusion: The early recovered group showed a lower incidence of T2WI/FLAIR abnormality in atypical sites and diffusion signal abnormality in total or typical sites with a lower incidence of cerebellar ataxia. [ABSTRACT FROM AUTHOR]

This article presents a case report of Wernicke's encephalopathy misdiagnosed as schizophrenia. The patient was hospitalized and diagnosed with schizophrenia at a local psychiatric hospital over a month ago. Twelve days ago， the patient gradually developed symptoms such as salivation， difficulty in eating， trembling hands， unsteady walking， glossoptosis and so on. The above symptoms were interpreted as drug-induced adverse reactions and managed accordingly， whereas the patient showed a poor response to treatment， and began to experience intermittent consciousness disorder. After referral to our hospital， findings were diagnostic for Wernicke's encephalopathy and peripheral polyneuropathy. Due to the progression of the disease， the family members abandoned further treatment and requested discharge from the hospital， and the patient died outside the hospital. This case report aims to improve the prognosis of Wernicke's encephalopathy in patients with mental disorder by raising the awareness of the disease and enhancing the early prevention.

Hyperemesis gravidarum occurs in up to 2 % of pregnancies. It is customary to regard the condition as purely an obstetric issue. However, there are reasonable grounds for reframing it as a neuropsychiatric state dependent upon genetic factors and involving peptides active within the central nervous system. Neurological abnormalities can also arise due to Wernicke's encephalopathy and the electrolyte abnormalities of refeeding syndrome. Aside from rehydration and antiemetic drugs, the condition can respond well to certain psychotropic medications and to psychological interventions, it may therefore be reasonable to regard hyperemesis of pregnancy as requiring both psychiatric and medical expertise for comprehensive management. [ABSTRACT FROM AUTHOR]

Thiamine (thiamin, B1) is a vitamin necessary for proper cell function. It exists in a free form as a thiamine, or as a mono-, di- or triphosphate. Thiamine plays a special role in the body as a coenzyme necessary for the metabolism of carbohydrates, fats and proteins. In addition, it participates in the cellular respiration and oxidation of fatty acids: in malnourished people, high doses of glucose result in acute thiamine deficiency. It also participates in energy production in the mitochondria and protein synthesis. In addition, it is also needed to ensure the proper functioning of the central and peripheral nervous system, where it is involved in neurotransmitter synthesis. Its deficiency leads to mitochondrial dysfunction, lactate and pyruvate accumulation, and consequently to focal thalamic degeneration, manifested as Wernicke’s encephalopathy or Wernicke–Korsakoff syndrome. It can also lead to severe or even fatal neurologic and cardiovascular complications, including heart failure, neuropathy leading to ataxia and paralysis, confusion, or delirium. The most common risk factor for thiamine deficiency is alcohol abuse. This paper presents current knowledge of the biological functions of thiamine, its antioxidant properties, and the effects of its deficiency in the body. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: Rare disease. Background: Wernicke encephalopathy is traditionally associated with chronic alcoholism, nutritional imbalance, prolonged intravenous feeding, hyperemesis, anorexia nervosa, and malabsorption syndromes. We report a case of Wernicke's encephalopathy in a 12-year-old girl with avoidant restrictive food intake disorder. Case Report: The patient had lost 45.4 kg of body weight due to self-imposed changes to her diet, before presenting with decreased oral intake for 2-3 weeks, intermittent nausea, crampy epigastric pain, and post-prandial emesis. Her weight on admission was 78.2 kg. She received intravenous fluids of dextrose 5% with normal saline while she initially attempted to eat, but the post-prandial emesis persisted. She developed a fear of vomiting, which led to even more severe food intake restriction. After a week, she began to report double vision and blurred peripheral vision, with physical findings of nystagmus and an ataxic gait. She was empirically started on thiamine after negative neurology workup, with improvement of her gait, blurry vision, and nystagmus. Thiamine deficiency was later confirmed. Conclusions: In patients with large amounts of weight loss presenting with neurological symptoms, Wernicke's encephalopathy must be considered in the differential diagnosis. Avoidant restrictive food intake disorder is rarely reported to cause Wernicke's encephalopathy. To the best of our knowledge, this is the first pediatric case demonstrating that Wernicke encephalopathy can occur in this type of eating disorder and not just in anorexia nervosa. [ABSTRACT FROM AUTHOR]

Achalasia is primarily a smooth muscle motility disorder of the esophagus driven by aberrant peristalsis and failure of sphincter relaxation. Notably, achalasia is a heterogeneous disease with primarily 3 possible pattern subtypes. According to the review of current cases and literature regarding achalasia, patients primarily present with dysphagia, usually to solids and, if progressed, to solids and liquids. Rarely, untreated achalasia may result in thiamine deficiency and present as Wernicke-Korsakoff syndrome (WKS). This acute neurologic condition primarily affects the central and peripheral nervous system and is known by the triad of ataxia, ophthalmoplegia, and confusion. Individuals who present with WKS typically have a notable history of chronic alcohol abuse with decreased thiamine intake and metabolism. Although less common, individuals with WKS may have a pertinent history of starvation, anorexia nervosa, and malnutrition. This case highlights a unique presentation of Wernicke's encephalopathy (WE) in a 30-year-old woman with severe type II achalasia complicated by a 60-pound weight loss in a span of 2 months. According to our literature review, there have only been 2 previously reported cases of severe achalasia leading to the development of WE. Considering the limited number of case reports available, WE must be in the differentials in patients with underlying achalasia, and our case report highlights this unusual presentation with corresponding brain imaging and manometry testing. [ABSTRACT FROM AUTHOR]

Ethanol use is common to most cultures but with varying doses and to varying extents. While research has focused on the effects on the liver, alcohol exerts a range of actions on the function and structure of the nervous system. In the central nervous system (CNS) it can provoke or exacerbate neurological and psychiatric disease; its effects on the peripheral nervous system are not included in this review. Sustained alcohol intake can predispose to acute neurochemical changes which, with continued ingestion and incomplete treatment, can lead to chronic structural changes in the CNS: these include generalised cortical and cerebellar atrophy, amnesic syndromes such as Korsakoff's syndrome, and specific white matter disorders such as central pontine myelinolysis and Marchiafava-Bignami syndrome. Alcohol in pregnancy commonly and significantly affects fetal health, though this receives less medical and political attention than other causes of fetal harm. This review looks at the range of disorders that can follow acute or chronic alcohol use, and how these should be managed, and we provide a practical overview on how neurologists might diagnose and manage alcohol addiction. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Thiamine deficiency can present with rare neurological symptoms such as urinary retention, along with common symptoms like ataxia and decreased limb muscle strength. Early recognition and treatment are crucial to improve symptoms and prevent complications. [ABSTRACT FROM AUTHOR]