Thiamine Deficiency Research Articles

Relationship between lactate and thiamine-responsive disorders in hospitalised infants and children in Lao PDR: secondary analysis of a prospective cohort study

ABSTRACT Background Lactate is a by-product of thiamine-deficient cellular metabolism, and hyperlactataemia can indicate severe illness. However, little is known about the clinical significance of hyperlactataemia in thiamine deficiency disorders. Aim To describe the relationship between whole-blood lactate level and thiamine-responsive disorders (TRDs) in children with signs/symptoms of thiamine deficiency in a high-risk region. Methods This is a secondary analysis of data from the Lao Thiamine study, a prospective cohort study which enrolled hospitalised infants and children (aged 21 days to <18 months) who had at least one sign or symptom suggestive of thiamine deficiency in Lao PDR. Therapeutic thiamine was administered, and clinical evaluations were completed at several time-points over the next 72 h. Three paediatricians reviewed individual case reports to evaluate clinical response to thiamine and assigned TRD status. Data from 402 children were analysed by logistic regression and predictive modelling to examine the relationship between hyperlactataemia and TRDs. Results Baseline hyperlactataemia (lactate >4.0 mmol/L) was associated with an increased odds of clinical improvement after thiamine administration [OR (95% CI) 2.32 (1.28–4.45), p = 0.007]. Baseline hyperlactataemia was a significant predictor of thiamine deficiency (thiamine diphosphate <40 nmol/L) [area under the receiver operating curve (95% CI) 0.76 (0.67–0.84), p < 0.001], and increased odds of mortality [OR (95% CI) 3.51 (1.38–8.94), p = 0.009]. Conclusions In children with signs/symptoms of thiamine deficiency, hyperlactataemia is associated with a favourable clinical response to thiamine, biochemical thiamine deficiency, and increased odds of mortality. Lactate may be useful in identifying children who might benefit from therapeutic thiamine.

NEUROBICS: A VITAL ADJUNCT TO PHYSICAL THERAPY IN IMPROVING COGNITIVE AND PHYSICAL FUNCTIONS IN A PREGNANT WOMAN WITH WERNICKE'S ENCEPHALOPATHY - A CASE REPORT.

Background: Wernicke’s Encephalopathy (WE) is a neurological disorder caused by thiamine deficiency, leading to cognitive and physical impairments like confusion, ataxia, and ophthalmoplegia. Pregnant women with WE face additional challenges due to pregnancy demands. This case report examines the use of neurobics—cognitive exercises to stimulate brain function - as an adjunct to physical therapy in enhancing cognitive and physical recovery in a pregnant woman with WE. Objective: This report aims to demonstrate the benefits of combining neurobics with physical therapy to improve cognitive and physical outcomes in a pregnant woman with WE. Case Presentation: A 28-year-old pregnant woman in her second trimester, diagnosed with WE, presented with confusion, ataxia, and visual disturbances. After stabilization with intravenous thiamine, a rehabilitation program combining neurobics and physical therapy was initiated. Physical therapy targeted balance, coordination, and strength, while neurobics engaged the brain with unusual sensory stimuli and non-routine tasks to enhance cognitive function. Results: After four weeks, the patient’s cognitive function improved, with her MoCA score increasing from 4 to 17. Significant gains were seen in executive function, attention, language, visuospatial skills, and orientation, though memory remained unchanged. Ataxia symptoms also improved, with her SARA score reducing from 22 to 14. Conclusion: The integration of neurobics with physical therapy offered a comprehensive approach to rehabilitation in this pregnant woman with WE. Neurobics enhanced cognitive recovery alongside physical rehabilitation, suggesting its potential as a valuable adjunct in managing WE.

Wernicke's Encephalopathy

Wernicke encephalopathy is a thiamine deficiency condition that has a wide range of somatic causes in addition to alcohol abuse. Most patients do not have the classical clinical triad — oculomotor dysfunction, ataxia and cognitive impairment at the onset of the disease, which makes timely diagnosis difficult. The disease may manifest as dizziness, unsteadiness, double vision, or cognitive impairment. Key clinical manifestations include symmetrical gaze-evoked nystagmus, truncal ataxia, bilateral abducens paresis, internuclear ophthalmoplegia, bilateral vestibular-ocular reflex reduction, and anterograde amnesia. To make a diagnosis, the presence of a condition leading to thiamine deficiency is required. The diagnosis is confirmed by MRI, but even with clinical suspicion of Wernicke encephalopathy, it is necessary to initiate parenteral therapy with thiamine in an adequate dose. With timely treatment, the disease has a good prognosis.

Thiamine: An indispensable regulator of paediatric neuro-cardiovascular health and diseases.

Physicians are recommended to have a low threshold for suspecting thiamine deficiency especially in vulnerable populations. Laboratory diagnosis of thiamine deficiency needs to be implemented as a standard of care, especially in endemic regions. Further, public health policies on food fortification, mandatory supplementation, and surveillance are imperative to eliminate thiamine deficiency-induced health hazards. • South Asian countries report a higher incidence of neonatal and under-five mortalities, many of which are attributed to maternal and perinatal micronutrient deficiencies. • Preventable causes of neonatal/ infantile deaths include birth factors (low birth weight, birth asphyxia), infectious diseases (pneumonia, diarrhoea, tetanus, tuberculosis, measles, diphtheria, malaria, acute infections), deficiency diseases and genetic diseases (vitamin & mineral deficiencies, IEMs, congenital heart disease, unexplained PPHN, SIDS etc). • Acute thiamine deficiency presenting as multisystemic syndromes, has unfortunately been a long standing unresolved public health concern. However, accessible surveillance and diagnostic strategies remain elusive in most clinical settings. • Despite decades of reports and emerging guidelines, diagnosis of thiamine deficiency is often missed and policy mandates at national level are yet to be implemented even in endemic countries. • This review provides a comprehensive summary of the biochemical role of thiamine, its key functions and effects on major organ systems, the diagnostic gap, the enigmatic presentation of acute thiamine deficiency, the plausible role of thiamine in other pathologies and the preventive measures at individual and community level.

Antepartum Wernicke’s Encephalopathy: Common, and Yet Rarely Diagnosed. Discussion on the Disease and Its Treatment

This review article presents a comprehensive discussion on antepartum Wernicke’s encephalopathy (WE), a serious neurological disorder. This disorder stems from thiamine (Vitamin B1) deficiency, which is critical for normal metabolism and neuronal integrity. During pregnancy, women are more susceptible to this condition due to increased nutritional requirement and metabolic changes. In WE, inadequate thiamine (Vitamin B1) causes disruption of metabolism in cells and impedes neurotransmitter synthesis in the brain, ultimately causing adverse neural consequences. Key risk factors contributing to antepartum WE include conditions that hinder thiamine absorption or increase metabolic demand, such as hyperemesis gravidarum (intense antepartum vomiting), malnutrition, alcoholism, and post-bariatric surgery states. WE's classic triad of symptoms, confusion, ataxia (loss of balance and coordination), and ophthalmoplegia (occlumotor incoordination), are not commonly seen in antepartum WE. Therefore, diagnosing the disorder in pregnancy is challenging and the condition is often misdiagnosed. Pregnancy symptoms like nausea, vomiting, and fatigue often overlap with WE's presentation, complicating diagnosis and confounding treatment. While magnetic resonance imaging (MRI) can reveal characteristic lesions in brain areas such as the thalamus and mammillary bodies, these may not always appear in early stages, adding further diagnostic difficulty. Effective management of antepartum WE relies on early recognition and timely administration of high-dose thiamine, particularly parenteral administration for affected pregnant women. Prompt treatment is critical to preventing irreversible sequalae, neurological and otherwise in both the woman and the child. Once acute symptoms are managed, transitioning to oral thiamine supplementation is the norm to prevent recurrence and maintain normal thiamine levels. Balanced diet of macro-nutrients and maintaining fluid and electrolyte balance, also plays an essential role in recovery. In severe or treatment-resistant cases, more intensive, prolonged thiamine therapy and regular monitoring of maternal and fetal health are necessary, usually managed by a multidisciplinary medical team. This coordinated care approach aims to prevent recurrence and minimize risks associated with WE. The article emphasizes following clinical guidelines, which recommend regular thiamine monitoring and preventive measures in high-risk pregnancies, to improve maternal and fetal outcomes. By adhering to established protocols, healthcare providers can reduce the likelihood of severe complications associated with WE, ensuring better care and recovery for affected pregnant women. Categories: Pregnancy, Neurology, Nutrition.

The emergence of additional causes of thiamine deficiency in the modern world: The impact of gastric surgery

We present a case of shoshin beriberi with non-specific hyperamylasemia but on a different background of thiamine deficiency. Due to the fulminant of the clinical presentation and the lack of rapid diagnostic tests, shoshin beriberi remains under-diagnosed, particularly among non-alcoholic patients. The present report of presumed shoshin beriberi aims to highlight the emergence of additional causes of thiamine deficiency in the modern world, especially those resulting from esophagogastric surgery, which carries a substantial risk of malnutrition in the medium to long term. The timely administration of thiamine is generally safe and can result in rapid improvement, making it both a valuable therapeutic and diagnostic tool.

Thiamine Deficiency Secondary to Pain-Induced Oral Aversion.

Thiamine Deficiency Secondary to Pain-Induced Oral Aversion.

Thiamine deficiency as a differential diagnosis for severe fatigue in terminally ill cancer patients.

Patients with advanced cancer present various symptoms as their disease progresses. Among these, fatigue is a frequent symptom in patients with advanced cancer and is associated with decreased quality of life (QOL). However, there are few reports regarding its association with thiamine deficiency (TD). We report a case in which we found TD in a patient with advanced lung cancer who presented with weight loss, significant fatigue, and appeared to have a worsening general condition, for whom symptoms were dramatically improved within a short period of time by intravenous administration of thiamine. The patient was a 76-year-old woman who had been diagnosed with lung cancer and liver metastases 6 months earlier. Due to interstitial pneumonia, she was not a candidate for chemotherapy and so palliative care was started. At 8 months after initial diagnosis, the patient complained of fatigue during a medical examination, so a blood sample was taken. A week later, she visited the hospital with a cane. She felt extremely fatigued and was unable to stand, but results from the previous blood test revealed that a TD. The fatigue disappeared 15 minutes after intravenous administration of thiamine and she was able to return home without the cane. Fatigue is a frequent symptom in advanced cancer patients, and TD may be the underlying cause. Inclusion of TD in the differential diagnosis may contribute to improving patient QOL.

The Implications of Atlantic Salmon (Salmo salar L.) Fatty Acid Profiles for Their Thiamine Status.

Thiamine deficiency is an ongoing issue across the Northern Hemisphere, causing reproductive failure in multiple salmonid populations. In the Baltic Sea, a large brackish water system in northern Europe, previous research has suggested that this deficiency is associated with lipid-rich diets with a high proportion of docosahexaenoic acid (DHA, 22:6n-3). The mechanism proposed is that a diet abundant in highly unsaturated fatty acids, such as DHA, depletes thiamine as an antioxidant defense in adult salmonids, rather than allocating thiamine to the offspring. In light of this existing hypothesis, we here explore the relationship between diet history and the related fatty acid (FA), profiles, and thiamine status of Atlantic salmon (Salmo salar L.) in three systems: the Baltic Sea, the North Atlantic Ocean, and Lake Vänern. Atlantic salmon inhabiting each system is known to have unique feeding histories and thiamine status. Our results showed that despite extensive sampling effort and distinct FA profiles, indicative of their diverse diets, there were no correlations between any FAs, including DHA, and the thiamine status of these populations. This finding does not support the above-mentioned hypothesis that diets rich in easily oxidized FAs would lead to lower thiamine concentrations in salmon tissues. Additionally, we found that changes in the salmon FA profiles throughout their life cycle are consistent for both low-thiamine populations from the Baltic Sea and medium-thiamine populations from North Atlantic Ocean, suggesting that these changes might not be involved in thiamine deficiency development.

Wernicke encephalopathy due to hyperemesis gravidarum in pregnancy

Wernicke’s encephalopathy is a life-threatening acute or sub-acute neurological emergency characterized by a triad of ataxia, confusion, ophthalmoplegia caused by thiamine deficiency, mostly observed in chronic alcohol users. Wernicke's encephalopathy due to causes other than alcohol abuse is uncommon and often misdiagnosed. In pregnancy, it is an unknown pathology, with poor prognosis, which occurs secondarily to hyperemesis gravidarum. We present a rare case of a 30-year-old female with mild learning disability, at 15 weeks of gestational age, with a history of previous hospital admission for severe vomiting and referred to Maternal Mental Health Clinic for abnormal behaviour. She had classic symptoms of Wernicke’s Encephalopathy on presentation, and was immediately treated with thiamine. Her symptoms improved in a few days.

Combined Administration of Metformin and Amprolium to Rats Affects Metabolism of Free Amino Acids in the Brain, Altering Behavior, and Heart Rate.

The risk of developing diabetes and cardiometabolic disorders is associated with increased levels of alpha-aminoadipic acid and disturbances in the metabolism of branched-chain amino acids. The side effects of the widely used antidiabetic drug metformin include impaired degradation of branched-chain amino acids and inhibition of intracellular thiamin transport. These effects may be interconnected, as thiamine deficiency impairs the functioning of thiamine diphosphate (ThDP)-dependent dehydrogenases of 2-oxo acids involved in amino acids degradation, while diabetes is often associated with perturbed thiamine status. In this work, we investigate the action of metformin in rats with impaired thiamine availability. The reduction in the thiamine influx is induced by simultaneous administration of the thiamine transporters inhibitors metformin and amprolium. After 24 days of combined metformin/amprolium administration, no significant changes in the total brain levels of ThDP or activities of ThDP-dependent enzymes of central metabolism are observed, but the affinities of transketolase and 2-oxoglutarate dehydrogenase to ThDP increase. The treatment also significantly elevates the brain levels of free amino acids and ammonia, reduces the antioxidant defense, and alters the sympathetic/parasympathetic regulation, which is evident from changes in the ECG and behavioral parameters. Strong positive correlations between brain ThDP levels and contents of ammonia, glutathione disulfide, alpha-aminoadipate, glycine, citrulline, and ethanolamine are observed in the metformin/amprolium-treated rats, but not in the control animals. Analysis of the obtained data points to a switch in the metabolic impact of ThDP from the antioxidant and nitrogen-sparing in the control rats to the pro-oxidant and hyperammonemic in the metformin/amprolium-treated rats. As a result, metformin administration along with the amprolium-reduced thiamine supply significantly perturb the metabolism of amino acids in the rat brain, altering behavioral and ECG parameters.

Triazole-Based Thiamine Analogues as Inhibitors of Thiamine Pyrophosphate-Dependent Enzymes: 1,3-Dicarboxylate for Metal Binding.

Thiamine 1 (vitamin B1) is essential for energy metabolism, and interruption of its utilization pathways is linked to various disease states. Thiamine pyrophosphate 2a (TPP, the bioactive form of 1) functions as a coenzyme of a variety of enzymes. To understand the role of vitamin B1 in these diseases, a chemical approach is to use coenzyme analogues to compete with TPP for the enzyme active site, which abolishes the coenzyme function. Exemplified by oxythiamine 3a and triazole hydroxamate 4, chemical probes require the coenzyme analogues to be membrane-permeable and of broad inhibitory activity to the enzyme family (rather than being too selective to particular TPP-dependent enzymes). In this study, using biochemical assays, we show that changing the hydroxamate metal-binding group of 4 to a 1,3-dicarboxylate moiety leads to the potent inhibition of multiple TPP-dependent enzymes. We further demonstrate that this dianionic thiamine analogue when masked in its diester form becomes membrane-permeable and can be unmasked by esterase treatment. Taken together, our inhibitors are potentially useful chemical tools to study the roles of vitamin B1, using a prodrug mechanism, to induce the effects of thiamine deficiency in cell-based assays.

Wernicke Encephalopathy Associated with Hyperemesis Gravidarum: A Case Report

Introduction: Wernicke encephalopathy is a clinical diagnosis that requires a high degree of clinical suspicion to recognize. We report a case of a pregnant patient developing Wernicke encephalopathy in the setting of severe hyperemesis gravidarum. Case Report: The patient was a 22-year-old female 13 weeks pregnant presenting to the emergency department (ED) with neurological deficits after several weeks of hyperemesis gravidarum requiring hospitalization. Exam and workup ultimately revealed the diagnosis of Wernicke encephalopathy. Her symptoms improved after administration of thiamine. Conclusion: Wernicke encephalopathy is a consequence of thiamine deficiency, commonly seen in patients with alcohol use disorder but also with other causes of nutritional deficiency, such as hyperemesis gravidarum. Wernicke encephalopathy is a clinical diagnosis that requires a high degree of suspicion and is, therefore, often missed in the ED setting. Treatment is supplemental thiamine and management of the root cause for nutritional deficiency.

Liquid Chromatography Mass Spectrometric Method and a Fluorometric 96 Well Plate Assay for Determination of Thiamine in Salmonid Eggs.

Thiamine deficiency is a large contributor to reduced reproduction success among salmonids throughout the northern hemisphere. In Scandinavia, this reproduction disorder is known as M74; while in North America, it is known as early mortality syndrome (EMS). The disorder fluctuates in magnitude from year to year. During years with high prevalence of the disorder, salmonid hatcheries that stock various aquatic systems to maintain the population size experience difficulties filling their quotas without thiamine treatment of alevins. The disorder is monitored both by observing the survival rate and by measuring the thiamine content of prefertilized eggs in the hatcheries. Here, a simple extraction procedure is presented, which allows for quantitative determination of the various phosphorylated forms of thiamine using liquid chromatography mass spectrometry but also allows for extraction in 96 deep-well plates and measurement of the total thiamine content using fluorescence monitoring with a plate reader, following oxidation of thiamine to thiochrome. The latter procedure could also be integrated into a highly portal system where the thiochrome is determined using the DeNovix QFX analyzer. The newly developed extraction procedure and cleanup method for fluorescence measurement represent the most versatile and simple methods to date for monitoring of thiamine in salmonid eggs. The methods produced accurate and precise data with quantification limits below the limit where the deficiency causes 100% lethality.

Suspected Case of Persistent Thiamin Deficiency in a Hand-Reared Caribou Calf.

Hand-reared animals are invaluable and irreplaceable in studies of wildlife nutrition. Hand-rearing protocols provide insights into dietary and training programs, but less information is available on disease management. In young ruminants, thiamin (Vitamin B1) deficiency is a particularly important disease that is treatable early in the disease process, but otherwise can be fatal. In this husbandry report, we describe a case of suspected thiamin deficiency in a hand-reared calf (Rangifer tarandus granti) that resulted in clinical signs of polioencephalomalacia and persisted for > 3 months. We attempted treatment with thiamin injections; injections resolved clinical signs of disease, but clinical signs of disease returned once injections stopped. After > 2 months of thiamin injections, the caribou calf received a rumen transfaunation from a fistulated moose (Alces alces) housed at the same facility. Following rumen transfaunation, we did not observe signs of thiamin deficiency. The calf outgrew other females in the cohort initially and shows no long-term effects of thiamin deficiency or polioencephalomalacia. We recommend rumen transfaunation when thiamin deficiency is suspected and does not resolve with thiamin injections alone. We also recommend heterospecific donors if conspecific donors are not available.

Thiamine Deficiency and its Implications on Microvascular Complications of Diabetes Mellitus

Thiamine is the first vitamin discovered and belongs to Vit B family. The main effect seen with thiamine deficiency is Beri-Beri, Wernicke’s encephalopathy, Wernicke-Korsak off syndrome and are considered as a serious condition but often can be reversed. The deficient status of thiamine can also cause varied affects and can overlap with other conditions to exacerbate its potent effects. It is seen that thiamine is necessary for the metabolism of glucose in the form of cofactors, deficiency of which leads to accumulation of toxic glucose metabolites leading to formation of free radicals and oxidative stress. Glucose is not only important for the formation of energy but its improper metabolism proves to have deleterious effects in the body. In this review, an attempt is made to correlate microvascular complications of diabetes with thiamine deficiency and can be discerned that oxidative stress is one of the important factors for the progress of microvascular complications, as well as diabetic ketoacidosis, atherosclerosis and cardiovascular damage in patients with diabetes mellitus and these can be prevented or maintained by optimizing thiamine levels in the body.

Evolutionary perspectives on thiamine supplementation of managed Pacific salmonid populations

Thiamine deficiency complex (TDC) has been identified in an ever-expanding list of species and populations. In many documented occurrences of TDC in fishes, juvenile mortality can be high — up to 90% at the population level. Such sweeping demographic losses and concomitant decreases in genetic diversity due to TDC can be prevented by treating pre-spawn females or fertilized eggs with supplemental thiamine. However, some fisheries managers are hesitant to widely apply thiamine treatments due to the potential for unforeseen evolutionary consequences. With these concerns in mind, we first review the existing data regarding genetic adaptation to low-thiamine conditions and provide perspectives on evolution-informed treatment strategies with specific population examples. We also provide practical treatment information, consider the potential logistical constraints of thiamine supplementation, and explore the consequences of deciding against supplementation. Until new evidence bolsters or refutes the genetic adaptation hypothesis, we suggest that TDC mitigation strategies should be designed to support maximum population genetic diversity through thiamine supplementation.

Wernicke encephalopathy in a patient with medullary infarctions: a case report.

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.

Thiamine deficiency underlies persistent gut dysbiosis and inflammation in people living with HIV on antiretroviral therapy

BackgroundAntiretroviral therapy (ART) has significantly improved the prognosis for people living with human immunodeficiency virus (PLWH). However, alterations in the gut microbiota of PLWH affect their metabolic environment and may contribute to chronic inflammation and premature aging, despite effective ART. Given that inflammation in the body have been shown to be risk factors for age-related diseases, elucidating changes in the gut microbiota and metabolic levels over time in individuals would be for understanding the dynamics in the body that lead to disease development.MethodsThe study assessed changes in blood markers, gut microbiota, and metabolic environment of PLWH on ART for four years, with participants divided into two groups based on median CD4 cell count (580/µL).ResultsThe high-CD4 group was more obese than the low-CD4 group, but there was no significant difference in comorbidities, such as diabetes, hypertension, and dyslipidemia. An investigation of the alterations in gut microbiota over a 4-year period revealed a reduction in specific groups of bacteria that produce short-chain fatty acids (SCFAs) and an increase in the occurrence of Enterobacteriaceae. In addition, a decline in bacterial thiamine synthesis was predicted, and a decreased abundance of the Ruminococcus gauvreauii group, which relies on supplementation with thiamine from symbiotic bacteria, was observed. The abundance of Collinsella and Catenibacterium at baseline was positively correlated with the predicted number of thiamine-synthesizing pathway. The presence of Catenibacterium at follow-up was inversely correlated with the rate of increase in plasma levels of lipopolysaccharide-binding protein, an indicator of a leaky gut. In terms of assessing the diversity of gut microbiota, a trend towards an increase in opportunistic bacteria with different genetic distances was observed in the high-CD4 group at follow-up.ConclusionThese observations suggested that, despite effective ART, the thiamine-deficient environment in the gut continues to reduce thiamine-requiring bacteria, such as certain SCFA-producing bacteria. As a result, secondary effects, such as proliferation of opportunistic bacteria and chronic inflammation associated with increased intestinal permeability, proceed in PLWH. Thus, certain essential nutrient deficiencies for gut metabolic environment may disrupt the symbiotic relationship between gut bacteria and increase the risk of developing inflammatory diseases.

Low expression of thiamine pyrophosphokinase-1 contributes to brain susceptibility to thiamine deficiency.

Thiamine deficiency is a well-known risk factor for the development of severe encephalopathy, such as Wernicke encephalopathy and Korsakoff syndrome, but the underlying mechanism is still mysterious. This study aims to investigate the expression levels of thiamine metabolism genes in different tissues and their impact on brain susceptibility to thiamine deficiency. The mRNA and protein levels of four genes known to be associated with thiamine metabolism: thiamine pyrophosphokinase-1 (Tpk), Solute carrier family 19 member 2 (Slc19a2), Slc19a3, and Slc25a19, in the brain, kidney, and liver of mice were examined. Thiamine diphosphate (TDP) levels were measured in these tissues. Mice were subjected to dietary thiamine deprivation plus pyrithiamine (PTD), a specific TPK inhibitor, or pyrithiamine alone to observe the reduction in TDP and associated pathological changes. TPK mRNA and protein expression levels were lowest in the brain compared to the kidney and liver. Correspondingly, TDP levels were also lowest in the brain. Mice treated with PTD or pyrithiamine alone showed an initial reduction in brain TDP levels, followed by reductions in the liver and kidney. PTD treatment caused significant neuron loss, neuroinflammation, and blood-brain barrier disruption, whereas dietary thiamine deprivation alone did not. TPK expression level is the best indicator of thiamine metabolism status. Low TPK expression in the brain appears likely to contribute to brain susceptibility to thiamine deficiency, underscoring a critical role of TPK in maintaining cerebral thiamine metabolism and preventing thiamine deficiency-related brain lesions.