Wernicke's encephalopathy treated with high dose intravenous thiamine: a case report.

Wernicke'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.

Clinical Reasoning and Challenges Faced With Onset Psychotic Symptoms in a Case of Combined Huntington's Disease and Gayet-Wernicke Encephalopathy.

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

Wernicke Encephalopathy Associated With Semaglutide Use.

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

Wernicke Encephalopathy Caused by Avoidance-Restrictive Food Intake Disorder in a Child: A Case-Based Review.

BACKGROUND: Wernicke encephalopathy (WE) is an acute and potentially fatal neuropsychiatric disorder resulting from thiamine deficiency: its etiology and clinical presentation can be heterogeneous and arduously recognized, especially in children and adolescents. CASE PRESENTATION: An 8-year-old girl arrived to the emergency room with ataxic gait, nystagmus, and mental confusion after a 10-day history of repeated severe vomiting; her recent clinical history was characterized by restricted nutrition due to a choking phobia, which caused substantial weight loss. Brain magnetic resonance imaging revealed a bilaterally increased T2 signal in the medial areas of the thalami and cerebral periaqueductal region. Diagnosis of WE based on clinical and neuroradiological findings was established and confirmed after labwork showing low serum thiamine. Following psychiatric evaluation, the patient was also diagnosed with avoidance-restrictive food intake disorder (ARFID), which required starting cognitive behavioral therapy and introducing aripiprazole. The patient displayed improvement of the radiological findings after one month and complete resolution of her neurological symptoms and signs. CONCLUSIONS: Eating disorders like ARFID might forerun acute signs of WE; this possibility should be considered even in pediatric patients, especially when atypical neurological pictures or feeding issues come out.

Non-alcoholic Wernicke's Encephalopathy Masquerading As CNS Relapse of Acute Myeloid Leukemia.

While Wernicke's encephalopathy (WE) is mostly caused by thiamine deficiency secondary to chronic alcohol use, other conditions that may affect one's nutritional status, such as bariatric surgery, hyperemesis gravidarum, chronic gastrointestinal disease, HIV/AIDS, and certain malignancies, may also lead to this outcome. We are discussing one such case, WE, in a young man with acute myeloid leukemia (AML) who underwent chemotherapy. The patient presented with blurred vision, paresthesia, weakness, and vomiting. Although he denied alcohol abuse, his symptoms, physical exam findings, and MRI results were consistent with WE. Treatment with thiamine resulted in a significant improvement in his visual disturbances and mental status. The authors highlight the importance of recognizing WE in non-alcoholic patients, particularly those undergoing prolonged hospitalization and chemotherapy, as nutritional deficiencies can develop. They recommend thiamine supplementation for patients receiving chemotherapy and those with poor oral intake. The case underscores the need for high clinical suspicion and early intervention in atypical cases of WE.

Vitamin B1 Deficiency Identified from Incidental Detection of Hyperlactatemia: A Case Report.

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.

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.

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

BACKGROUND: The 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. OBJECTIVE: Our 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. PATIENTS: Three 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. CONCLUSIONS: Micronutrient 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.

Typical and atypical MRI abnormalities in Wernicke's encephalopathy: Correlation with blood vitamin B1 levels.

PURPOSE: Clinical features of Wernicke's encephalopathy (WE) confirmed strictly through the low blood vitamin B1 (VB1) levels are limited. This study aimed to analyse magnetic resonance imaging (MRI) findings, and clinical characteristics, in patients with WE who have confirmed low blood VB1 levels. METHODS: Clinical and laboratory records of 12 consecutive patients with WE admitted to our hospital during the past 11 years were reviewed. The WE diagnosis was confirmed based on low blood VB1 levels and the presence of at least one of the classical triad. RESULTS: Ophthalmoplegia and nystagmus were recorded in 75% and 50% of the patients, respectively. Eleven of 12 patients presented with consciousness disturbance/memory loss. All patients experienced gait disturbances. Eight of the 12 patients exhibited MRI abnormalities at typical sites (the dorsal midbrain [n = 7], medial thalamus [n = 6], mammillary bodies [n = 5], and dorsal pons [n = 5]). Of the 12 patients, six showed abnormalities at atypical sites (the splenium of the corpus callosum [n = 4], fornix [n = 3], cerebral cortex [n = 2], cerebellar vermis [n = 2], and dorsal medulla [n = 1]). Patients with positive MRI abnormalities had significantly lower blood VB1 levels than those without abnormalities (9.5 vs. 16.0 ng/mL). CONCLUSIONS: In cases of confirmed WE with low blood VB1 levels, the corpus callosum, fornix, and cerebral cortex were more frequently involved than in previous studies. MRI abnormalities at both typical and atypical sites were correlated with low blood VB1 levels in WE, suggesting that lower blood VB1 levels are associated with more severe brain damage in patients with WE.

[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.

Wernicke Encephalopathy After Roux-en-Y Gastric Bypass Presenting with Altered Mental Status-A Video Case Report.

Wernicke encephalopathy (WE) is a seldom encountered yet significant neuropsychiatric ailment resulting from a deficiency in thiamine (vitamin B1). While commonly linked with chronic alcoholism or insufficient dietary intake, instances of WE following bariatric and metabolic surgeries, notably laparoscopic Roux-en-Y gastric bypass (RYGB), have been sporadically documented. This case study elucidates the condition of a male patient who, 3 months after undergoing RYGB to address severe obesity, displayed abrupt alterations in mental status, swiftly ameliorated by immediate administration of intravenous high-dose thiamine.

The role of CT brain findings in the early diagnosis of infantile encephalitic beriberi.

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.

Dural arteriovenous fistula and sinus thrombosis presenting as parkinsonism and dementia: a case report with literature review.

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 transverse-sigmoid 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.

Bilateral hearing loss as the initial presentation of reversible Wernicke's encephalopathy with splenial lesion.

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.

Neuromyelitis Optica Spectrum Disorder Resembling Wernicke's Encephalopathy: A Case Report and Review of the Literature.

We describe a case of Neuromyelitis Optica Spectrum Disorder (NMOSD) mimicking Wernicke's Encephalopathy (WE) to highlight an atypical presentation of NMOSD. A 39-year-old female presented with subacute encephalopathy and progressive ophthalmoplegia. Her MRI revealed T2 hyperintensities involving the mammillary bodies, periaqueductal grey matter, medial thalami, third ventricle, and area postrema. Whole blood thiamine levels were elevated and she did not improve with IV thiamine. CSF was notable for lymphocytic pleocytosis and elevated protein. She tested positive for serum Aquaporin-4 (AQP4) antibody. Subsequent imaging revealed multilevel lesions in the cervical and thoracic spinal cord. Her CSF GFAP antibody also came back positive. She steadily and significantly improved after high-dose IV steroids and plasmapheresis. She later started on chronic rituximab therapy. This represents a unique case of NMOSD presenting with the classical clinical and imaging features of WE, as opposed to the typical presenting symptoms of NMOSD. As such, demyelinating disorders should be considered when there is concern for diencephalic and midline pathologies, particularly without classic WE risk factors. Conversely, clinicians should be aware of secondary nutritional complications arising from severe area postrema syndrome.

Wernicke encephalopathy induced by glucose infusion: A case report and literature review.

Introduction: Wernicke encephalopathy (WE) is a potentially fatal condition caused by thiamine (vitamin B1) deficiency. Chronic alcoholism is the most common cause of WE; however, other conditions responsible for thiamine deficiency should also be considered. Case Report: We report the case of a 64-year-old woman with a history of diabetes who presented with confusion and apathy. Magnetic resonance imaging of the brain showed T2 hyperintensities involving dorsolateral medulla oblongata, tegmentum of the pons, vermis of the cerebellum, periaqueductal region, and the bilateral mammillary bodies. She had a history of intravenous glucose administration before her mental symptoms developed. On suspicion of WE, she was treated with a high dose of thiamine empirically. Her clinical condition improved rapidly in 2 weeks. Conclusion: Endogenous thiamine stores can be rapidly depleted in the case of enhanced glucose oxidation. Patients who receive glucose should also be prescribed thiamine to avoid inducing or exacerbating WE.

Vitamin deficiencies in children: Lessons from clinical and neuroimaging findings.

BACKGROUND AND AIMS: Water-soluble vitamins play an essential coenzyme role in the nervous system. Acquired vitamin deficiencies are easily treatable, however, without treatment, they can lead to irreversible complications. This study aimed to provide clinical, laboratory parameters and neuroimaging data on vitamin deficiencies in an attempt to facilitate early diagnosis and prompt supplementation. METHODS: From July 1998 to July 2023, patients at Necker-Enfants-Malades Hospital presenting with acute neurological symptoms attributed to acquired vitamin deficiency were included. Clinical data were extracted from Dr Warehouse database. Neuroimaging, biochemical and electrophysiological data were reviewed. RESULTS: Patients with vitamin B1 deficiency exhibited abnormal eye movements (n = 4/4), fluctuations in consciousness (n = 3/4), and ataxia (n = 3/4). Brain MRI showed alterations of fourth ventricle region (n = 4/4), periaqueductal region (n = 4/4), tectum (n = 3/4), and median thalami (n = 3/4). Patients with vitamin B2 deficiency presented with early onset hypotonia (n = 3/4), hyperlactatemia (n = 4/4), and hyperammonemia (n = 4/4). Plasma acylcarnitines revealed a multiple acyl-coA dehydrogenase deficiency-like profile (n = 4/4). In vitamin B12 deficiency, young children presented with developmental delay (n = 7/7) and older children with proprioceptive ataxia (n = 3/3). Brain MRI revealed atrophy (n = 7/7) and spinal MRI hyperintensity in posterior cervical columns (n = 3/3). Metabolic findings showed elevated methylmalonic acid (n = 6/7) and hyperhomocysteinemia (n = 6/7). Patients with vitamin C deficiency exhibited gait disturbances and muscle weakness (n = 2/2). CONCLUSIONS: Acquired vitamin deficiencies may display reversible clinical symptoms mimicking inherited metabolic disorders. Some situations raise suspicion for diagnosis: concordant clinical presentation, suggestive neuroimaging findings, and/or biochemical evidence. Any acute neurological condition should be treated without waiting for definitive biochemical confirmation.

Presentation of Wernicke encephalopathy in patient secondary to one anastomosis gastric bypass. Case report and literature review.

Wernicke encephalopathy, which is caused by a thiamine deficiency, occurs in 0.8-2% of the population. Only 16% present the typical triad of this disease: nystagmus, confusion and ataxia. We present the case of a postoperative patient with a one anastomosis gastric bypass with reoperation undergoing a Roux-en-Y gastric bypass that begins with confusion and nystagmus on her third postoperative day. The diagnosis of Wernicke encephalopathy is made by imaging, and vitamin B1 is administered with total improvement of nystagmus and altered state of consciousness (lethargy, bradypsychia, bradylalia).

La encefalopatía de Wernicke se produce por una deficiencia de tiamina se presenta en un 0.8-2% de la población. Solo el 16% de los casos presentan la tríada típica de esta enfermedad: nistagmo, confusión y ataxia. Presentamos el caso de una paciente operada de bypass gástrico de una anastomosis con reintervención convirtiendo a bypass gástrico en Y de Roux que en su tercer día de posoperatorio comienza con confusión y nistagmo. Se realiza por imagen el diagnóstico de encefalopatía de Wernicke se administra vitamina B1 con mejoría total del nistagmo y alteración del estado de consciencia (letargia, bradipsiquia, bradilalia).