Wernicke's Encephalopathy Research Articles

Stem Cell Therapy and Thiamine Deficiency-Induced Brain Damage.

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 PO1-20-10: Wernicke Encephalopathy Syndrome in Metastatic Breast Cancer – A Diagnostic Dilemma or An Underdiagnosed Entity

Abstract Wernicke encephalopathy (WE) is an acute medical syndrome that manifests as a clinical triad of encephalopathy, gait ataxia, and oculomotor dysfunction, requiring urgent treatment. Early recognition and treatment can prevent severe neurological morbidity and the chronic neurological consequence of WE, known as Korsakoff syndrome. Thiamine (vitamin B1) deficiency is the root cause of WE, which can occur due to several underlying conditions, including chronic alcoholism, hyperemesis of pregnancy, malnutrition, prolonged fasting, and malignancy. In cases of malignancy, WE is often undiagnosed or misdiagnosed because the presenting symptoms can be confused with several other complications of malignancy. We present the case of a 47-year-old woman with no significant medical history who was diagnosed with triple negative metastatic breast cancer and admitted to the hospital with confusion, memory loss, and progressive lower extremity weakness. During her breast cancer treatments, she experienced significant nausea and vomiting, leading to a prolonged state of nutritional imbalance. Patient underwent extensive infectious and neurological workup for encephalopathy including lumbar puncture which was negative for leptomeningeal disease or infection. MRI brain did not show any new metastatic lesions however revealed enhancement of mammillary bodies bilaterally. Mammillary body enhancement can be seen in WE but not always present on imaging. She was treated for WE with high dose of thiamine infusion for five days, resulting in an improvement of her neurological symptoms. Diagnosing WE in cancer patients is challenging, as there are various factors to consider, such as cerebral metastasis, infection, chemotherapy treatment, and electrolyte imbalances, that can cause neurological symptoms. Cancer patients are at high risk for development of WE due to chemotherapy or whole brain radiation induced nausea and vomiting that can lead to chronic malnutrition. The typical triad of symptoms is not present in many cases and presenting symptoms can be nonspecific which makes the diagnosis challenging. As a result, WE is often underdiagnosed in such patients, making it essential to educate the physician community about this acute neurological manifestation that can be easily treated. A high index of suspicion is necessary for early recognition, and prompt treatment can prevent Korsakoff syndrome, thereby enhancing the overall quality of life of patients with malignancy. Citation Format: Archit Patel, Arya Mariam Roy, Ronald Alberico, Shipra Gandhi. Wernicke Encephalopathy Syndrome in Metastatic Breast Cancer – A Diagnostic Dilemma or An Underdiagnosed Entity [abstract]. In: Proceedings of the 2023 San Antonio Breast Cancer Symposium; 2023 Dec 5-9; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2024;84(9 Suppl):Abstract nr PO1-20-10.

Neurologic complications of thiamine (B1) deficiency following bariatric surgery in adolescents

BackgroundThe prevalence of obesity among children and adolescents is rising and poses a major health concern. Bariatric surgery is well established in adults and has become an option for adolescents. Thiamine (B1) deficiency is common following bariatric surgery in adults. It may present as Beri-Beri, Wernicke encephalopathy, or Korsakoff psychosis. ObjectiveOur aim was to describe the clinical features, diagnosis, and treatment of adolescents who presented with B1 deficiency after bariatric surgery at one center, and to summarize the data from the literature. PatientsThree adolescents with morbid obesity (two boys and one girl, aged 15.5 to- 17-years-old), presented at Schneider Children's Medical Center of Israel with progressive lower limb pain and weakness 2–3 month following a bariatric procedure (sleeve gastrectomy or narrowing of a bariatric band). The girl also had upper limb involvement and cerebellar signs. All three were non-compliant with micronutrient supplementation. After admission, they received intravenous B1 and oral multivitamin supplementation, and their symptoms improved considerably. ConclusionsMicronutrient supplementation following bariatric surgery is crucial to prevent deficiencies. In adolescents, compliance with micronutrient supplementation should be assessed before and after such surgery. Thiamine deficiency may cause polyneuropathy, among other symptoms. Treatment reduces the severity of neurological complications.

Predictors of Wernicke Encephalopathy in Adults Admitted for Acute Alcohol Induced Pancreatitis in the United States (P8-4.005)

Predictors of Wernicke Encephalopathy in Adults Admitted for Acute Alcohol Induced Pancreatitis in the United States (P8-4.005)

Malnutrition-induced Wernicke Encephalopathy Presenting as Bilateral Hearing Loss: A Case Report and Literature Review (P5-10.003)

Malnutrition-induced Wernicke Encephalopathy Presenting as Bilateral Hearing Loss: A Case Report and Literature Review (P5-10.003)

Wernicke encephalopathy in combination with acute polyneuropathy under the guise of a demyelinating disease in a teenager with an eating disorder

Wernicke encephalopathy is a neuropsychiatric syndrome characterized by three main symptoms: oculomotor disturbances, cerebellar ataxia, and psychiatric disturbances. The condition is associated with a high mortality and morbidity rate. Wernicke encephalopathy is most commonly seen in adolescent children presenting with a vitamin B1 deficiency. Thiamine deficiency may also cause polyneuritis syndrome, with or without the aforementioned symptoms. The condition is characterized by sensory-motor impairments in a symmetrical pattern, dysarthria, and paresis or even paralysis of the lower limbs. This report focuses on an adolescent case presenting acute oculomotor paresis, nystagmus, leg weakness, impaired gait, decreased deep tendon reflexes, cognitive impairment, and a history of recurrent vomiting, prolonged starvation, and eating behaviour disorders. The magnetic resonance imaging scan reveals symmetrical pathological foci of increased intensity in T2 in the periaqueductal region, at the Magendie’s central aperture. The patient displays a mixed motor-sensory polyneuropathic syndrome affecting both lower limbs, primarily of the axonopathy type, based on electroneuromyography data. Positive outcomes such as restored eyeball movement, enhanced gait, increased muscle strength in the lower legs and feet, and better management of sensory disorders have occurred due to thiamine treatment.

Wernicke Encephalopathy in a Child Treated for Ewing Sarcoma.

Wernicke Encephalopathy in a Child Treated for Ewing Sarcoma.

Use of Thiamine Supplementation in Pregnant Women Diagnosed With Hyperemesis Gravidarum and Wernicke Encephalopathy

Hyperemesis gravidarum is the most common condition requiring hospital care for women during the first 20 weeks of pregnancy and may lead to malnutrition, dehydration, and vitamin deficiencies. Depletion of vitamins such as thiamine may result in the development of Wernicke encephalopathy, a severe neurological disorder that can increase the risk for mortality and morbidity for the mother and fetus. A lack of awareness regarding the relationship of hyperemesis gravidarum and Wernicke encephalopathy may result in delayed treatment and disease management. Glucose administration in the presence of thiamine deficiency may induce Wernicke encephalopathy; protocols are needed to ensure dextrose is used for women with hyperemesis gravidarum in times of prolonged vomiting and poor oral intake only after first administering thiamine. This article includes a discussion of best practices for thiamine supplementation with hyperemesis gravidarum and Wernicke encephalopathy.

Letter to the Editor: Total parenteral nutrition-induced Wernicke's encephalopathy after oncologic gastrointestinal surgery.

Letter to the Editor: Total parenteral nutrition-induced Wernicke's encephalopathy after oncologic gastrointestinal surgery.

Wernicke Syndrome: Case Report and Literature Review of Contributing Factors—Can Malpractice Dynamics Be Identified?

Wernicke Encephalopathy (WE) is a neurological acute syndrome related to vitamin B1 deficiency and is relatively common in patients with chronic alcoholism. In the case of Hyperemesis Gravidarum, thiamine body stores become unable to meet the increased demand, resulting in acute deficiency. WE is associated with typical clinical and radiological findings. Treatment pathways rely on thiamine replacement. The case herein reported is centered around a 33-year-old diabetic patient at 12 weeks of gestation, with WE due to hyperemesis gravidarum. The disease manifested itself with weakness, mental confusion, headache, and impaired vision. The diagnosis was established after the detection of typical findings by MRI. Thirty days after therapy was started, most of the patient’s neurological disorders were resolved. The patient was discharged 40 days later with instructions to continue daily thiamine supplementation. The pregnancy outcome was good. Unfortunately, mild ataxia persisted in 2-year follow-up as a long-term consequence. When diagnosed and treated, WE has a favorable prognosis. However, roughly 80% of patients experience memory loss, which may continue for a long time, while gait disorders reportedly affect about 35% of patients. Mild ataxia and dysmetria may persist, too. We reviewed the scientific literature on WE in women with HG until February 2023. Hardly any authors report data on long-term sequelae. Our report emphasizes how important it is to take into consideration this complication in clinical practice, referring to published guidelines and recommendations. Neurological maternal sequelae can demonstrably persist despite early diagnosis and appropriate management. For this reason, a long-term follow-up is recommended. Wernicke syndrome management cannot yet rely on well-established conclusive guidelines; hence, a cautionary approach ought to be prioritized in order to ensure medicolegal soundness.

Traversing Antepartum Wernicke’s Encephalopathy: In-Depth Insights and Strategies

Antepartum Wernicke’s encephalopathy (WE) is a severe neurological disorder caused by a deficiency of thiamine (Vitamin B1), essential for glucose metabolism, and neuronal function. This review aims to provide an in-depth analysis of WE during pregnancy, highlighting its pathophysiology, risk factors, clinical presentation, diagnostic challenges, and management strategies. Thiamine is critical in the Krebs cycle and neurotransmitter production, and its deficiency leads to substantial biochemical disruptions and neuronal damage. Pregnant women are particularly susceptible due to their increased nutritional demands to support fetal growth and maternal metabolic changes, making them prone to thiamine deficiency and its severe neurological consequences. The clinical manifestations of WE include ophthalmoplegia, ataxia, confusion or altered mental state, peripheral neuropathy, and cardiovascular issues. Diagnosing WE during pregnancy is challenging due to its atypical presentation, requiring a high degree of clinical suspicion and awareness of risk factors. Diagnostic limitations include variable clinical symptoms and challenges in interpreting serum thiamine levels and neuroimaging findings. While neuroimaging can show characteristic brain lesions, its utility is limited by variability. Managing WE during pregnancy requires prompt recognition, immediate thiamine supplementation, continued supportive measures, and careful monitoring. Severe or refractory cases may require advanced management strategies. Pharmacological treatments include standardized thiamine administration protocols, guided by clinical guidelines and recommendations. Early detection and management of WE in pregnant women are vital to prevent irreversible neurological damage and improve maternal and fetal outcomes. Adherence to clinical guidelines is crucial to mitigate the impact of this condition.

Wernicke Encephalopathy in a Patient of Acute Gastroenteritis: A Case Report

An acute neurological disease known as Wernicke Encephalopathy (WE) is characterised by the clinical triad of symptoms: ophthalmoparesis with nystagmus, ataxia, and disorientation. Thiamine deficiency is the underlying cause of this potentially fatal condition, which mainly affects the central and peripheral nervous systems. Hereby, the authors present a case of a 47-year-old male who presented with multiple episodes of loose stools and altered consciousness to the emergency ward. He had hyperchloremic metabolic acidosis suspected to be due to acute gastroenteritis, which may have caused his altered consciousness. Despite correction of his metabolic acidosis, his sensorium did not improve. Later, he was diagnosed with WE using Magnetic Resonance Imaging (MRI) brain findings, which showed symmetrical subtle hyperintensities in the medial part of the thalami and periaqueductal grey matter, suggestive of WE; his consciousness improved after thiamine supplementation. There should be a high suspicion of WE for timely diagnosis and treatment. Prompt treatment would be rewarding as WE is reversible, if treated in time.

Особенности диагностики энцефалопатии Вернике: клинический случай

Wernicke's encephalopathy (WE) is a rare neurological disorder caused by vitamin B1 (thiamine) deficiency and is associated with a significant risk of developing persistent neurological deficiency, chronification, disability and ultimately death if the aetiopathogenetic therapy with high doses of vitamin B1 is initiated late. Objective: to raise awareness among physicians about the characteristics of clinics, diagnostics and treatment of Wernicke's encephalopathy. Materials and methods. Retrospective analysis of the medical records of a patient with alcoholic Wernicke's encephalopathy. Results. This paper presents the own clinical observation of a patient with newly diagnosed alcoholic Wernicke's encephalopathy, clinical manifestations and MRI picture, describes the main features of aetiology and pathogenesis of this pathological process. Conclusion. An important role in the diagnosis of WE is played by the correct collection of anamnestic data and magnetic resonance imaging (MRI) of the brain, which has a sufficiently high specificity and is the only diagnostic method that allows this disease to be identified with high reliability. Thus, it is the thorough collection of anamnesis and the timely MRI examination that allow the doctor to point in the right direction of the diagnostic search in a timely manner.

Analysis of factors leading to brain MRI lesions in Wernicke's encephalopathy

[Objective] To investigate association between Wernicke encephalopathy (WE) and brain MRI. [Subjects] 26 patients (7 females, mean age 63.9 ± 12.7 years) with WE admitted to our department between May 2008 and September 2022. [Methods] Wernicke's encephalopathy in patients with MRI lesions was defined as "MRI-positive group" (MPG), and those without MRI lesions as "MRI-negative group" (MNG). The following parameters were assessed between the two groups: age, sex, alcoholism, neurological symptoms, vitamin B1, lymphocyte, total cholesterol, albumin, and outcome at discharge. [Results] There were 17 patients in MPG. Compared to MNG, MPG had lower rates of alcohol abuse (10.0% vs 77.8%, P = 0.025), lower vitamin B1 (median 10.0 ‍ng/ml vs 29.0 ‍ng/ml, P < 0.001), and more vitamin B1 treatment dose (median 1900 ‍mg vs 600 ‍mg, P = 0.016). [Conclusion] Alcoholic WE may be overlooked if the focus is solely on brain MRI findings.

ЭНЦЕФАЛОПАТИЯ ВЕРНИКЕ ПОСЛЕ БАРИАТРИЧЕСКОЙ ОПЕРАЦИИ: СЛУЧАЙ ИЗ ПРАКТИКИ

Objective: To review the signs and symptoms of Wernicke encephalopathy (WE) after bariatric surgery based on 2 case reports and a literature data analysis. Methods: We present two case reports of WE. In these patients, thiamine deficiency developed due to bariatric gastric surgery, which was performed to treat class III obesity, formerly known as morbid obesity. The patients underwent comprehensive physical and neurological examinations; clinical, laboratory, and instrumental data were collected from medical records and analyzed. The researchers also performed a computerized search using the keywords "Wernicke encephalopathy after bariatric surgery" to find relevant publications in the PubMed/Medline, Scopus, and Web of Science databases. Results: The presented acute neurological condition related to vitamin B1 deficiency in two patients after undergoing a weight loss surgery, specifically a mini-gastric bypass. The clinical picture in one patient was dominated by memory impairment, ataxic, and polyneuritic syndrome, while in the other, it was only polyneuritic syndrome. Conclusion: The initial symptoms of WE are often overlooked, leading to the progression of the disease with the development of severe neurological deficits. To prevent the progression of encephalopathy, it is necessary to begin treatment with high doses of parenteral thiamine replacement therapy as early as possible. Keywords: Obesity, bariatric surgery, Wernicke encephalopathy, thiamine, case report

Wernickes Encephalopathy in a Non-Alcoholic Patient with Pancreatic Pseudocyst: A Case Report

Wernickes Encephalopathy in a Non-Alcoholic Patient with Pancreatic Pseudocyst: A Case Report

Hemosiderin Detection inside the Mammillary Bodies Using Quantitative Susceptibility Mapping on Patients with Wernicke-Korsakoff Syndrome.

Hemorrhage inside the mammillary bodies (MMBs) is known to be one of the findings of Wernicke encephalopathy. Brain MRI of two patients with Wernicke-Korsakoff syndrome (WKS) demonstrated high susceptibility values representing hemosiderin deposition in MMBs by using quantitative susceptibility mapping (QSM). QSM provided additional information of susceptibility values to susceptibility-weighted imaging in diagnosis of WKS.

Gestational Malnutrition is Still the Skeleton in the Closet: Where Are the Malnutrition Risk Screening Criteria?

In 2020 there were 3,613,647 live births in the US. Fifty to 90% of those pregnancies were affected by nausea and vomiting of pregnancy (NVP) while the most severe NVP, known as hyperemesis gravidarum (HG) affected 1.5- 3.0% of those gravidas. In 2014 58,436 gravidas were hospitalized with HG. While hospital admissions for HG were down 42%, the number seen in Emergency departments rose by 27.7%. Although HG has been viewed as a positive predictor of a favorable pregnancy outcome, patients who also demonstrate weight loss and electrolyte disturbance may be a distinct entity and at greater risk for growth retardation and fetal anomalies. Poorly managed HG can result in compounded maternal injuries, increased rates of therapeutic abortions and suicide ideation, as well as a high rate of fetal loss. This study focuses on one of the most severe catastrophes originating from HG: Wernicke’s encephalopathy (WE) continues to be an under-recognized and often misunderstood disease in all populations. The acknowledged cause of WE is vitamin B1 or thiamin deficiency, a specific form of malnutrition. Unfortunately, this syndrome is most often recognized at autopsy, especially among non-alcoholics. One manifestation of WE is cognitive dysfunction which may explain the increase in suicidal ideation and/or elected terminations. The current mounting legislation of pregnancy termination limits will complicate the decision to abort for critically ill gravidas and their medical providers. However, antecedent events to WE are extremely poor nutritional intake leading to excessive weight loss and delayed nutritional interventions which can mitigate these situations.

Celiac disease in a teenager revealed with Wernicke Encephalopathy

Background: Wernicke encephalopathy is an acute neurological condition defined by a clinical triad of ophthalmoplegia, ataxia, and confusion. This disease is due to thiamine deficiency. Case presentation: After persistent diarrhea and vomiting, a 15-year-old boy presented up-beating nystagmus, with binocular vertical diplopia and unstable gait. An etiological workup revealed a celiac disease. Magnetic resonance imaging showed bilateral periaqueductal region lesions. Treatment and discussion: Because of suspected Wernicke’s encephalopathy, the patient was treated with thiamine replacement and significant improvement of symptoms took place. Wernicke encephalopathy is a serious medical disorder with enormous morbidity and mortality. Evaluation should include patient history with a physical and ophthalmologic examination and laboratory workup with appropriate imaging. Conclusion: Undiagnosed celiac disease can lead to malabsorption of vitamin B1 causing acute symptoms of Wernicke encephalopathy.

Ataxic variant of Guillain Barre syndrome: a case report

A 17-year-old girl was admitted after acute onset of unsteady gait succeeding acute gastroenteritis. Neurological examination reported normal power in all four limbs, impaired finger-nose, heel-shin tests, areflexia and ataxic gait. We eliminated other diseases with cerebellar symptoms; for example, Wernicke encephalopathy, multiple sclerosis, cerebellar vascular disease , encephalitis in the brain stem and cerebellum. Blood serum collected from the patient during the acute phase showed no anti-ganglioside antibodies. As the patient presented with evident cerebellar ataxia without muscle weakness, ophthalmoplegia or proprioceptive sensory disruption a diagnosis of ataxic form of Guillain-Barré syndrome (GBS) after nerve conduction studies. Though ataxic GBS is not a settled impression, we should have to give heed to the potential existence of such a scarce GBS variant.