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Thiamin deficiency and brain disorders

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https://www.readbyqxmd.com/read/28765488/wernicke%C3%A3-%C3%A2-%C3%A2-s-encephalopathy-associated-with-liver-abscess
#1
Rajesh Verma, Vipul Garg
Wernicke's encephalopathy is a rare neurological disorder caused by thiamine deficiency, characterised by ocular motor dysfunction, ataxia and impairment in consciousness. It predominantly affects brain regions with a high metabolic rate such as mammillary bodies, medial thalamic nuclei, the tectal region and the cerebellum. Although chronic alcoholism is the most common cause of Wernicke's encephalopathy, various other conditions not related to alcohol consumption such as bariatric surgery, acute pancreatitis, hyperemesis gravidarum, prolonged fasting and gastrointestinal surgery have been implicated in its aetiology...
July 31, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28694620/nonalcoholic-wernicke-s-encephalopathy-a-retrospective-study-from-a-tertiary-care-center-in-northern-india
#2
Irfan Ahmad Shah, Ravouf Parvaiz Asimi, Yuman Kawoos, Maqbool Wani, Tahir Saleem, Waqas Nabi Baba
OBJECTIVE: The objective of this study was to describe the demographic features, clinical presentation, and management and outcome of fifty cases of nonalcoholic Wernicke's encephalopathy from a tertiary care hospital of a region with reported incidence of thiamine deficiency disorders. MATERIALS AND METHODS: In a retrospective study, fifty adult cases of Wernicke's encephalopathy were analyzed. The diagnosis of Wernicke's encephalopathy was made according to the European federation of neurological societies guidelines 2010...
July 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28665968/high-dose-thiamine-prevents-brain-lesions-and-prolongs-survival-of-slc19a3-deficient-mice
#3
Kaoru Suzuki, Kenichiro Yamada, Yayoi Fukuhara, Ai Tsuji, Katsumi Shibata, Nobuaki Wakamatsu
SLC19A3 deficiency, also called thiamine metabolism dysfunction syndrome-2 (THMD2; OMIM 607483), is an autosomal recessive neurodegenerative disorder caused by mutations in SLC19A3, the gene encoding thiamine transporter 2. To investigate the molecular mechanisms of neurodegeneration in SLC19A3 deficiency and whether administration of high-dose thiamine prevents neurodegeneration, we generated homozygous Slc19a3 E314Q knock-in (KI) mice harboring the mutation corresponding to the human SLC19A3 E320Q, which is associated with the severe form of THMD2...
2017: PloS One
https://www.readbyqxmd.com/read/28574307/korsakoff-syndrome-from-retrochiasmatic-suprasellar-lesions-rapid-reversal-after-relief-of-cerebral-compression-in-4-cases
#4
Luis E Savastano, Todd C Hollon, Ariel L Barkan, Stephen E Sullivan
Korsakoff syndrome is a chronic memory disorder caused by a severe deficiency of thiamine that is most commonly observed in alcoholics. However, some have proposed that focal structural lesions disrupting memory circuits-in particular, the mammillary bodies, the mammillothalamic tract, and the anterior thalamus-can give rise to this amnestic syndrome. Here, the authors present 4 patients with reversible Korsakoff syndromes caused by suprasellar retrochiasmatic lesions compressing the mammillary bodies and adjacent caudal hypothalamic structures...
June 2, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28506637/thiamine-and-benfotiamine-prevent-stress-induced-suppression-of-hippocampal-neurogenesis-in-mice-exposed-to-predation-without-affecting-brain-thiamine-diphosphate-levels
#5
Julie Vignisse, Margaux Sambon, Anna Gorlova, Dmitrii Pavlov, Nicolas Caron, Brigitte Malgrange, Elena Shevtsova, Andrey Svistunov, Daniel C Anthony, Natalyia Markova, Natalyia Bazhenova, Bernard Coumans, Bernard Lakaye, Pierre Wins, Tatyana Strekalova, Lucien Bettendorff
Thiamine is essential for normal brain function and its deficiency causes metabolic impairment, specific lesions, oxidative damage and reduced adult hippocampal neurogenesis (AHN). Thiamine precursors with increased bioavailability, especially benfotiamine, exert neuroprotective effects not only for thiamine deficiency (TD), but also in mouse models of neurodegeneration. As it is known that AHN is impaired by stress in rodents, we exposed C57BL6/J mice to predator stress for 5 consecutive nights and studied the proliferation (number of Ki67-positive cells) and survival (number of BrdU-positive cells) of newborn immature neurons in the subgranular zone of the dentate gyrus...
May 12, 2017: Molecular and Cellular Neurosciences
https://www.readbyqxmd.com/read/28417285/alcohol-and-thiamine-deficiency-trigger-differential-mitochondrial-transition-pore-opening-mediating-cellular-death
#6
REVIEW
Abdoulaye Bâ
Accumulating evidence has shown that binge-type alcohol intake in mothers interferes with thiamine deficiency (TD) to promote the fetal alcohol syndrome (FAS). Developmental alcohol or TD exposures act either synergistically or separately to reproduce FAS features e.g. intrauterine growth retardation and related microcephaly characterized by extensive cellular death induced by one another neurotoxicant. However molecular and cellular mechanisms underlying apoptosis in both alcohol and TD toxicities are unknown...
June 2017: Apoptosis: An International Journal on Programmed Cell Death
https://www.readbyqxmd.com/read/28284395/treatable-inborn-errors-of-metabolism-due-to-membrane-vitamin-transporters-deficiency
#7
Juan Darío Ortigoza Escobar, Belén Pérez Dueñas
B vitamins act as cofactors for strategic metabolic processes. The SLC19 gene family of solute carriers has a significant structural similarity, transporting substrates with different structure and ionic charge. Three proteins of this family are expressed ubiquitously and mediate the transport of 2 important water-soluble vitamins, folate, and thiamine. SLC19A1 transports folate and SLC19A2 and SLC19A3 transport thiamine. PCFT and FOLR1 ensure intestinal absorption and transport of folate through the blood-brain barrier and SLC19A25 transports thiamine into the mitochondria...
November 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28284389/biochemical-analyses-of-cerebrospinal-fluid-for-the-diagnosis-of-neurometabolic-conditions-what-can-we-expect
#8
Marta Batllori, Marta Molero-Luis, Mercedes Casado, Cristina Sierra, Rafael Artuch, Aida Ormazabal
In this article, we review the state-of-the-art analysis of different biomarkers in the cerebrospinal fluid for the diagnosis of genetically conditioned, rare, neurometabolic diseases, including glucose transport defects, neurotransmitter (dopamine, serotonin, and gamma-aminobutyric acid) and pterin deficiencies, and vitamin defects (folate, vitamin B6, and thiamine) that affect the brain. The analysis of several key metabolites are detailed, which thus highlights the preanalytical and analytical factors that should be cautiously controlled to avoid misdiagnosis; moreover, these factors may facilitate an adequate interpretation of the biochemical profiles in the context of severe neuropediatric disorders...
November 2016: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28225048/thiamine-deficiency-oxidative-metabolic-pathways-and-ethanol-induced-neurotoxicity-how-poor-nutrition-contributes-to-the-alcoholic-syndrome-as-marchiafava-bignami-disease
#9
REVIEW
L M P Fernandes, F R Bezerra, M C Monteiro, M L Silva, F R de Oliveira, R R Lima, E A Fontes-Júnior, C S F Maia
Ethanol is an important risk factor for the occurrence of several brain disorders that depend on the amount, period and frequency of its consumption. Chronic use of ethanol often leads to the development of neurodegenerative syndromes, which cause morphological and functional impairments such as foetal alcohol syndrome in newborns exposed to ethanol during pregnancy, Wernicke-Korsakoff Syndrome and, more rarely, Marchiafava-Bignami disease (MBD). MBD is characterized by primary degeneration of the corpus callosum, without inflammation and is associated with oxidative stress and hypovitaminosis, as well as altered mental status, to mention dementia, seizures, depression and so on...
May 2017: European Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28193519/thiamine-deficiency-induces-endoplasmic-reticulum-stress-and-oxidative-stress-in-human-neurons-derived-from-induced-pluripotent-stem-cells
#10
Xin Wang, Mei Xu, Jacqueline A Frank, Zun-Ji Ke, Jia Luo
Thiamine (vitamin B1) deficiency (TD) plays a major role in the etiology of Wernicke's encephalopathy (WE) which is a severe neurological disorder. TD induces selective neuronal cell death, neuroinflammation, endoplasmic reticulum (ER) stress and oxidative stress in the brain which are commonly observed in many aging-related neurodegenerative diseases, such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD) and progressive supranuclear palsy (PSP). However, the underlying cellular and molecular mechanisms remain unclear...
April 1, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/27984051/brain-endothelial-dysfunction-following-pyrithiamine-induced-thiamine-deficiency-in-the-rat
#11
Sumit Sarkar, Serguei Liachenko, Merle G Paule, John Bowyer, Joseph P Hanig
Prolonged vitamin B1 (thiamine) deficiency can lead to neurological disorders such as Wernicke's encephalopathy and Wernicke-Korsakoff Syndrome (WKS) in humans. These thiamine deficiency disorders have been attributed to vascular leakage, blood-brain barrier breakdown and neuronal loss in the diencephalon and brain stem. However, endothelial dysfunction following thiamine deficiency and its relationship to the phenomenon of neurodegeneration has not been clearly elucidated. The present study sought to begin to address this issue by evaluating vascular morphology and integrity in a pyrithiamine (PT)-induced rat model of thiamine deficiency...
October 28, 2016: Neurotoxicology
https://www.readbyqxmd.com/read/27129864/concomitants-of-alcoholism-differential-effects-of-thiamine-deficiency-liver-damage-and-food-deprivation-on-the-rat-brain-in-vivo
#12
Natalie M Zahr, Edith V Sullivan, Torsten Rohlfing, Dirk Mayer, Amy M Collins, Richard Luong, Adolf Pfefferbaum
RATIONALE: Serious neurological concomitants of alcoholism include Wernicke's encephalopathy (WE), Korsakoff's syndrome (KS), and hepatic encephalopathy (HE). OBJECTIVES: This study was conducted in animal models to determine neuroradiological signatures associated with liver damage caused by carbon tetrachloride (CCl4), thiamine deficiency caused by pyrithiamine treatment, and nonspecific nutritional deficiency caused by food deprivation. METHODS: Magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) were used to evaluate brains of wild-type Wistar rats at baseline and following treatment...
July 2016: Psychopharmacology
https://www.readbyqxmd.com/read/26836322/exercise-leads-to-the-re-emergence-of-the-cholinergic-nestin-neuronal-phenotype-within-the-medial-septum-diagonal-band-and-subsequent-rescue-of-both-hippocampal-ach-efflux-and-spatial-behavior
#13
Joseph M Hall, Lisa M Savage
Exercise has been shown to improve cognitive functioning in a range of species, presumably through an increase in neurotrophins throughout the brain, but in particular the hippocampus. The current study assessed the ability of exercise to restore septohippocampal cholinergic functioning in the pyrithiamine-induced thiamine deficiency (PTD) rat model of the amnestic disorder Korsakoff Syndrome. After voluntary wheel running or sedentary control conditions (stationary wheel attached to the home cage), PTD and control rats were behaviorally tested with concurrent in vivo microdialysis, at one of two time points: 24-h or 2-weeks post-exercise...
April 2016: Experimental Neurology
https://www.readbyqxmd.com/read/26620994/wernicke-s-encephalopathy-following-roux-en-y-gastric-bypass-surgery
#14
Azra Zafar
Wernicke's encephalopathy (WE) is a serious neurological disorder characterized by a classical triad of acute mental confusion, ataxia, and opthalmoplegia due to thiamine deficiency. It was initially described in chronic alcoholics; however, any condition resulting in poor nutritional status places the patient at risk of WE. Bariatric surgery is now considered as an emergent cause of WE. The number of bariatric surgery is increasing for morbid obesity. We present a case of a 40-year-old male who presented with confusion and difficulty in maintaining the balance while walking 3 months after Roux en Y gastric bypass surgery...
December 2015: Saudi Medical Journal
https://www.readbyqxmd.com/read/26557183/dry-beriberi-preceded-wernicke-s-encephalopathy-thiamine-deficiency-after-laparoscopic-sleeve-gastrectomy
#15
Debopam Samanta
In recent times, pediatric obesity has become widely prevalent. If first-line treatment with lifestyle modification fails, bariatric surgery may be indicated for severely obese patients. Many patients now travel abroad to get these surgeries done. Some of these patients receive inadequate postoperative care. We described a morbidly obese 17-year-old girl who had a laparoscopic sleeve gastrectomy procedure for weight loss. Due to severe nausea, she stopped her multivitamin supplementation. Within a few weeks, she developed symptoms of dry beriberi was soon followed by classic symptoms of Wernicke's encephalopathy...
July 2015: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/26473940/endoplasmic-reticulum-stress-and-ethanol-neurotoxicity
#16
REVIEW
Fanmuyi Yang, Jia Luo
Ethanol abuse affects virtually all organ systems and the central nervous system (CNS) is particularly vulnerable to excessive ethanol exposure. Ethanol exposure causes profound damages to both the adult and developing brain. Prenatal ethanol exposure induces fetal alcohol spectrum disorders (FASD) which is associated with mental retardation and other behavioral deficits. A number of potential mechanisms have been proposed for ethanol-induced brain damage; these include the promotion of neuroinflammation, interference with signaling by neurotrophic factors, induction of oxidative stress, modulation of retinoid acid signaling, and thiamine deficiency...
October 14, 2015: Biomolecules
https://www.readbyqxmd.com/read/26410803/wernicke-encephalopathy-as-rare-complication-of-cytoreductive-surgery-and-hyperthermic-intraperitoneal-chemotherapy
#17
Antonio Macrì, Francesco Fleres, Antonio Ieni, Maurizio Rossitto, Tommaso Mandolfino, Salvatore Micalizzi, Francesco Iaropoli, Carmelo Mazzeo, Massimo Trovato, Eugenio Cucinotta, Edoardo Saladino
BACKGROUND: Peritoneal carcinomatosis of gastric origin is a frequent event with poor survival. A new promising approach is the association of the Cytoreductive Surgery (CRS) with the Hyperthermic Intraperitoneal Chemotherapy (HIPEC), which yet is characterized by high morbidity and mortality. We report, to our knowledge, the first case of Wernicke Encephalopathy (WE) complicating CRS plus HIPEC. WE, caused by a deficiency of thiamine, is characterized by ataxia, ocular motor cranial neuropathies and changes in consciousness...
2015: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/26287559/neuromyelitis-optica-spectrum-disorders-may-be-misdiagnosed-as-wernicke-s-encephalopathy
#18
Fulan Shan, Rong Zhong, Linzhan Wu, Yongxiang Fan, Youming Long, Cong Gao
PURPOSE: To raise doctors' attention to the differential diagnosis of neuromyelitis optica spectrum disorders (NMOSD) and Wernicke's encephalopathy (WE). PATIENTS AND METHODS: We extensively reviewed the medical records of 136 patients who had visited our hospital since 2008 and were suspected of having central nervous system demyelinating diseases. Four of those patients had somnolence, electrolyte imbalance and brain lesions around the third ventricle and were included in the study...
October 2016: International Journal of Neuroscience
https://www.readbyqxmd.com/read/26008863/pyruvate-dehydrogenase-deficiency-presenting-as-isolated-paroxysmal-exercise-induced-dystonia-successfully-reversed-with-thiamine-supplementation-case-report-and-mini-review
#19
REVIEW
Claudia Castiglioni, Daniela Verrigni, Cecilia Okuma, Alejandra Diaz, Karin Alvarez, Teresa Rizza, Rosalba Carrozzo, Enrico Bertini, Marcelo Miranda
BACKGROUND: Pyruvate dehydrogenase (PDH) deficiency is a disorder of energy metabolism with variable clinical presentations, ranging from severe infantile lactic acidosis to milder chronic neurological disorders. The spectrum of clinical manifestations is continuously expanding. METHODS AND RESULTS: We report on a 19-year-old intelligent female with PDH deficiency caused by a Leu216Ser mutation in PDHA1. She presented with recurrent hemidystonic attacks, triggered by prolonged walking or running, as the unique clinical manifestation that manifested since childhood...
September 2015: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/25312751/wernicke-s-encephalopathy-in-a-malnourished-surgical-patient-a-difficult-diagnosis
#20
Stefano Busani, Cinzia Bonvecchio, Arianna Gaspari, Marcella Malagoli, Alessandra Todeschini, Nicola Cautero, Massimo Girardis
BACKGROUND: Wernicke's encephalopathy is an acute neurological disorder resulting from thiamine deficiency mainly related to alcohol abuse. Severe thiamine deficiency is an emerging problem in non-alcoholic patients and it may develop in postoperative surgical patients with risk factors. CASE PRESENTATION: We reported a case of a 46 years old woman who underwent, one year before, to cephalic duodenopancreatectomy complicated with prolonged recurrent vomiting. She underwent to a second surgical operation for intestinal sub-occlusion and postoperatively she developed septic shock and hemorrhagic Wernicke's disease...
October 14, 2014: BMC Research Notes
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