What Goes Around Comes Around? Wernicke Encephalopathy and the Nationwide Shortage of Intravenous Multivitamins Revisited

Perko, Ross; Harreld, Julie H.; Helton, Kathleen J.; Sabin, Noah D.; Haidar, Cyrine E.; Wright, Karen

doi:10.1200/jco.2012.42.7237

Cited by 10 publications

(16 citation statements)

References 10 publications

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“…This triad is pathognomonic for thiamine deficiency and is classically associated with malnourished alcoholics. However, a variety of diseases can lead to WE in pediatric patients with a wide range of underlying diagnoses including malignancies and post‐solid organ transplantation . Rapid diagnosis and treatment is critical as prolonged deficiency can lead to irreversible severe anterograde amnesia known as Korsakoff Syndrome .…”

Section: Discussionmentioning

confidence: 99%

“…Rapid diagnosis and treatment is critical as prolonged deficiency can lead to irreversible severe anterograde amnesia known as Korsakoff Syndrome . The recent nationwide shortage of intravenous multivitamins has intensified the importance of adequate oral vitamin supplementation in patients with malabsorption, medical non‐compliance, or persistent nausea and emesis …”

Section: Discussionmentioning

confidence: 99%

“…However, a variety of diseases can lead to WE in pediatric patients with a wide range of underlying diagnoses including malignancies and postsolid organ transplantation. [6][7][8] Rapid diagnosis and treatment is critical as prolonged deficiency can lead to irreversible severe anterograde amnesia known as Korsakoff Syndrome. [9] The recent nationwide shortage of intravenous multivitamins has intensified the importance of adequate oral vitamin supplementation in Pediatr Blood Cancer DOI 10.1002/pbc Vitamin B1 Deficiency During Stem Cell Transplant patients with malabsorption, medical non-compliance, or persistent nausea and emesis.…”

Section: Discussionmentioning

confidence: 99%

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Stem Cell Transplant-Associated Wernicke Encephalopathy in a Patient with High-Risk Neuroblastoma

Darlington

Pinto

Hecktman

et al. 2015

Pediatr Blood Cancer

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Children undergoing intense cancer treatment frequently require total parenteral nutrition (TPN). Rarely, vitamins are removed due to hypersensitivity to the carrier vehicle in the formulation. We present the case of a 5-year-old patient with stage 4, high-risk neuroblastoma who developed altered mental status, ataxia, and tachycardia during consolidative autologous stem cell transplantation. Skin findings and brain MRI were consistent with thiamine (vitamin B1) deficiency and Wernicke encephalopathy. Vitamin B1 administration rapidly reversed all skin and neurologic symptoms. This case highlights the importance of close monitoring of micronutrients in pediatric patients receiving prolonged courses of chemotherapy and stem cell transplantation.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Stem Cell Transplant-Associated Wernicke Encephalopathy in a Patient with High-Risk Neuroblastoma

Darlington

Pinto

Hecktman

et al. 2015

Pediatr Blood Cancer

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“…The diagnosis of Wernicke's encephalopathy is challenging due to nonspecificity of symptoms. The most sensitive diagnostic test is magnetic resonance imaging (MRI), with typical abnormalities being increased T2‐weighted symmetrical signal intensity in the medial (periventricular) thalami, mammillary bodies, tectal plate, and periaqueductal area of the midbrain . Authors report complete remission of symptoms within hours to days after thiamine supplementation and improvement of brain lesions on MRI in a few weeks.…”

Section: Acquired Conditions Leading To Thiamine Deficiencymentioning

confidence: 99%

“…Wernicke's encephalopathy occurs in children that suffer from malnutrition or malabsorption conditions in the context of gastrointestinal surgical procedures, prolonged total parenteral nutrition with low contents of thiamine, malignancies, organ transplantations, pancreatitis, etc. [39][40][41][42] A minority of children presents with the triad of ataxia, ophthalmoplegia and decreased consciousness. 4 Most commonly, mental status changes are present in isolation at the beginning of symptoms.…”

Section: Wernicke's Encephalopathymentioning

confidence: 99%

Genetic defects of thiamine transport and metabolism: A review of clinical phenotypes, genetics, and functional studies

Marcé‐Grau

Martí-Sánchez

Baide‐Mairena

et al. 2019

J of Inher Metab Disea

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Thiamine is a crucial cofactor involved in the maintenance of carbohydrate metabolism and participates in multiple cellular metabolic processes within the cytosol, mitochondria, and peroxisomes. Currently, four genetic defects have been described causing impairment of thiamine transport and metabolism: SLC19A2 dysfunction leads to diabetes mellitus, megaloblastic anemia and sensory‐neural hearing loss, whereas SLC19A3, SLC25A19, and TPK1‐related disorders result in recurrent encephalopathy, basal ganglia necrosis, generalized dystonia, severe disability, and early death. In order to achieve early diagnosis and treatment, biomarkers play an important role. SLC19A3 patients present a profound decrease of free‐thiamine in cerebrospinal fluid (CSF) and fibroblasts. TPK1 patients show decreased concentrations of thiamine pyrophosphate in blood and muscle. Thiamine supplementation has been shown to improve diabetes and anemia control in Rogers' syndrome patients due to SLC19A2 deficiency. In a significant number of patients with SLC19A3, thiamine improves clinical outcome and survival, and prevents further metabolic crisis. In SLC25A19 and TPK1 defects, thiamine has also led to clinical stabilization in single cases. Moreover, thiamine supplementation leads to normal concentrations of free‐thiamine in the CSF of SLC19A3 patients. Herein, we present a literature review of the current knowledge of the disease including related clinical phenotypes, treatment approaches, update of pathogenic variants, as well as in vitro and in vivo functional models that provide pathogenic evidence and propose mechanisms for thiamine deficiency in humans.

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Thiamine deficiency in childhood with attention to genetic causes: Survival and outcome predictors

Ortigoza‐Escobar

Alfadhel

Molero-Luís

et al. 2017

Annals of Neurology

119

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Primary and secondary conditions leading to thiamine deficiency have overlapping features in children, presenting with acute episodes of encephalopathy, bilateral symmetric brain lesions, and high excretion of organic acids that are specific of thiamine-dependent mitochondrial enzymes, mainly lactate, alpha-ketoglutarate, and branched chain keto-acids. Undiagnosed and untreated thiamine deficiencies are often fatal or lead to severe sequelae. Herein, we describe the clinical and genetic characterization of 79 patients with inherited thiamine defects causing encephalopathy in childhood, identifying outcome predictors in patients with pathogenic SLC19A3 variants, the most common genetic etiology. We propose diagnostic criteria that will aid clinicians to establish a faster and accurate diagnosis so that early vitamin supplementation is considered. Ann Neurol 2017;82:317-330.

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What Goes Around Comes Around? Wernicke Encephalopathy and the Nationwide Shortage of Intravenous Multivitamins Revisited

Cited by 10 publications

References 10 publications

Stem Cell Transplant-Associated Wernicke Encephalopathy in a Patient with High-Risk Neuroblastoma

Stem Cell Transplant-Associated Wernicke Encephalopathy in a Patient with High-Risk Neuroblastoma

Genetic defects of thiamine transport and metabolism: A review of clinical phenotypes, genetics, and functional studies

Thiamine deficiency in childhood with attention to genetic causes: Survival and outcome predictors

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