Thiamine-deficient encephalopathy is characterized by morphologic lesions in the brainstem and less extensively in the cerebellum, but the early neurologic signs reverse rapidly and fully with thiamine, indicating a metabolic disorder. The suggested causal mechanisms of the encephalopathy involve two thiamine-dependent enzymes: (a) impairment of pyruvate decarboxylase activity with decreased cerebral energy (ATP) synthesis, and (b) reduction of transketolase activity with possible impairment of the hexose monophosphate shunt and subsequent decrease in NADPH formation. The latter may be important in maintaining glutathione in a reduced form (GSH), which apparently functions by keeping enzymes in a reduced (active) conformation.
David W. McCandless, Steven Schenker
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Title and authors | Publication | Year |
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Exploring the impact of age, and body condition score on erythrocytic B1-Dependent transketolase activity in cats: A comprehensive analysis of thiamine status
Fascetti AJ, Larsen JA, Min A, Nair M, Montano M, Giulivi C |
Heliyon | 2024 |
Synthetic Thioesters of Thiamine: Promising Tools for Slowing Progression of Neurodegenerative Diseases.
Bettendorff L |
International journal of molecular sciences | 2023 |
Supplemental thiamine as a practical, potential way to prevent Alzheimer's disease from commencing
J Fessel |
Alzheimer's & Dementia: Translational Research & Clinical Interventions | 2021 |
Don’t seek, don’t find: The diagnostic challenge of Wernicke’s encephalopathy
S Kohnke, CL Meek |
Annals of Clinical Biochemistry | 2020 |
Wernicke’s encephalopathy — from basic science to clinical practice. Part 1: Understanding the role of thiamine
M Ott, U Werneke |
Therapeutic Advances in Psychopharmacology | 2020 |
Thiamine deficiency impairs common eider (Somateria mollissima) reproduction in the field
T Mörner, T Hansson, L Carlsson, AL Berg, YR Muñoz, H Gustavsson, R Mattsson, L Balk |
Scientific Reports | 2017 |
Widespread episodic thiamine deficiency in Northern Hemisphere wildlife
L Balk, PÅ Hägerroth, H Gustavsson, L Sigg, G Åkerman, YR Muñoz, DC Honeyfield, U Tjärnlund, K Oliveira, K Ström, SD McCormick, S Karlsson, M Ström, M Manen, AL Berg, HP Halldórsson, J Strömquist, TK Collier, H Börjeson, T Mörner, T Hansson |
Scientific Reports | 2016 |
Integrative Therapies for Depression: Redefining Models for Assessment, Treatment and Prevention
J Greenblatt, P Patel |
Integrative Therapies for Depression: Redefining Models for Assessment, Treatment and Prevention | 2015 |
Role of astrocytes in thiamine deficiency
S Afadlal, R Labetoulle, AS Hazell |
Metabolic Brain Disease | 2014 |
Wernicke’s Encephalopathy: Expanding the Diagnostic Toolbox
ME Lough |
Neuropsychology Review | 2012 |
A comprehensive understanding of thioTEPA metabolism in the mouse using UPLC–ESI-QTOFMS-based metabolomics
F Li, AD Patterson, CC Höfer, KW Krausz, FJ Gonzalez, JR Idle |
Biochemical Pharmacology | 2011 |
Modeling neurodegenerative disease pathophysiology in thiamine deficiency: Consequences of impaired oxidative metabolism
SS Jhala, AS Hazell |
Neurochemistry International | 2011 |
Thiamine (Vitamin B 1 )
A Fattal-Valevski |
Journal of Evidence-Based Complementary & Alternative Medicine | 2011 |
Wildlife Ecotoxicology
JE Elliott, CA Bishop, CA Morrissey |
2011 | |
Thiamine status in humans and content of phosphorylated thiamine derivatives in biopsies and cultured cells
M Gangolf, J Czerniecki, M Radermecker, O Detry, M Nisolle, C Jouan, D Martin, F Chantraine, B Lakaye, P Wins, T Grisar, L Bettendorff |
PloS one | 2010 |
Astrocytes are a major target in thiamine deficiency and Wernicke's encephalopathy
AS Hazell |
Neurochemistry International | 2009 |
Update of Cell Damage Mechanisms in Thiamine Deficiency: Focus on Oxidative Stress, Excitotoxicity and Inflammation
AS Hazell, RF Butterworth |
Alcohol and Alcoholism | 2009 |
Wild birds of declining European species are dying from a thiamine deficiency syndrome
L Balk, PA Hägerroth, G Akerman, M Hanson, U Tjärnlund, T Hansson, GT Hallgrimsson, Y Zebühr, D Broman, T Mörner, H Sundberg |
Proceedings of the National Academy of Sciences | 2009 |
Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management
GP Sechi, A Serra |
The Lancet Neurology | 2007 |
REVIEW: Vitamin transport and homeostasis in mammalian brain: focus on Vitamins B and E
R Spector, CE Johanson |
Journal of Neurochemistry | 2007 |
Tissue thiamine deficiency as potential cause of delayed graft function after kidney transplantation: Thiamine supplementation of kidney donors may improve transplantation outcome
A Klooster, HG Leuvenink, RO Gans, SJ Bakker |
Medical Hypotheses | 2007 |
Three Common Intronic Variants in the Maternal and Fetal Thiamine Pyrophosphokinase Gene (TPK1) are Associated with Birth Weight
D Fradin, P Bougneres |
Annals of Human Genetics | 2007 |
Glucose loading precipitates focal lactic acidosis in the vulnerable medial thalamus of thiamine-deficient rats
D Navarro, C Zwingmann, N Chatauret, RF Butterworth |
Metabolic Brain Disease | 2007 |
Unsteady, unfocused, and unable to hear
J Buscaglia, J Faris |
The American Journal of Medicine | 2005 |
Brain lactate synthesis in thiamine deficiency: A re-evaluation using1H-13C nuclear magnetic resonance spectroscopy
D Navarro, C Zwingmann, AS Hazell, RF Butterworth |
Journal of Neuroscience Research | 2004 |
Encyclopedia of the Neurological Sciences
S Mookherjee |
Encyclopedia of the Neurological Sciences | 2003 |
Beri-Beri
RR Trifiletti |
Encyclopedia of the Neurological Sciences | 2003 |
Diffusion weighted MR imaging of acute Wernicke's encephalopathy
TI Chung, JS Kim, SK Park, BS Kim, KJ Ahn, DW Yang |
European Journal of Radiology | 2003 |
2,4-pentanedione
B Ballantyne |
Journal of Applied Toxicology | 2001 |
In Vivo Microdialysis in an Animal Model of Neurological Disease: Thiamine Deficiency (Wernicke) Encephalopathy
KG Todd, RF Butterworth |
Methods | 2001 |
Thiamine deficiency results in metabolic acidosis and energy failure in cerebellar granule cells: An in vitro model for the study of cell death mechanisms in Wernicke's encephalopathy
P Pannunzio, AS Hazell, M Pannunzio, KV Rao, RF Butterworth |
Journal of Neuroscience Research | 2000 |
Metabolic Impairment Elicits Brain Cell Type-Selective Changes in Oxidative Stress and Cell Death in Culture
LC Park, NY Calingasan, K Uchida, H Zhang, GE Gibson |
Journal of Neurochemistry | 2000 |
Thiamine supplementation to prevent induction of low birth weight by conventional therapy for gestational diabetes mellitus
SJ Bakker, JC Maaten, RO Gans |
Medical Hypotheses | 2000 |
Alcohol-thiamine interactions: an update on the pathogenesis of Wernicke encephalopathy
KG Todd, AS Hazell, RF Butterworth |
Addiction Biology | 1999 |
Nutrition
&NA; |
Current Opinion in Lipidology | 1999 |
Properties and functions of the thiamin diphosphate dependent enzyme transketolase
G Schenk, RG Duggleby, PF Nixon |
The International Journal of Biochemistry & Cell Biology | 1998 |
Critical role of arg433 in rat transketolase activity as probed by site-directed mutagenesis
Y Soh, BJ Song, J Jeng, AT Kallarakal |
Biochemical Journal | 1998 |
Mechanisms of neuronal cell death in Wernicke's encephalopathy
A S Hazell, K G Todd, R F Butterworth |
Metabolic Brain Disease | 1998 |
In Vivo study of the kinetics of thiamine and its phosphoesters in the deafferented rat cerebellum
A Nauti, C Patrini, C Reggiani, A Merighi, R Bellazzi, G Rindi |
Metabolic Brain Disease | 1997 |
Wernicke’s encephalopathy: an excitotoxicity hypothesis
WJ McEntee |
Metabolic Brain Disease | 1997 |
Increased cerebral free radical production during thiamine deficiency
PJ Langlais, G Anderson, SX Guo, SC Bondy |
Metabolic Brain Disease | 1997 |
Neuronal cell death in Wernicke's encephalopathy: pathophysiologic mechanisms and implications for PET imaging
DK Leong, RF Butterworth |
Metabolic Brain Disease | 1996 |
Pathophysiology of alcoholic brain damage: synergistic effects of ethanol, thiamine deficiency and alcoholic liver disease
RF Butterworth |
Metabolic Brain Disease | 1995 |
Pathogenesis of diencephalic lesions in an experimental model of Wernicke's encephalopathy
PJ Langlais |
Metabolic Brain Disease | 1995 |
Hypothesis on cellular ATP depletion and adenosine release as causes of heart failure and vasodilatation in cardiovascular beriberi
SJ Bakker, KM Leunissen |
Medical Hypotheses | 1995 |
Central neurotoxicity induced by subchronic exposure to 2,4-pentanedione vapour
RH Garman, DE Dodd, B Ballantyne |
Human & Experimental Toxicology | 1995 |
Thiamine in excitable tissues: Reflections on a non-cofactor role
L Bettendorff |
Metabolic Brain Disease | 1994 |
Influence of Thiamine on the Behavioral Sensitivity to Ethanol
DV Gauvin, RJ Briscoe, KL Goulden, FH Wojnicki, R Russin, PR Martin, FA Holloway |
Alcoholism, clinical and experimental research | 1994 |
Further studies on erythrocyte thiamin transport and phosphorylation in seven patients with thiamin-responsive megaloblastic anaemia
G Rindi, C Patrini, U Laforenza, H Mandel, M Berant, MB Viana, V Poggi, AN Zarra |
Journal of Inherited Metabolic Disease | 1994 |
Extracellular Glutamate Is Increased in Thalamus During Thiamine Deficiency-Induced Lesions and Is Blocked by MK-801
PJ Langlais, SX Zhang |
Journal of Neurochemistry | 1993 |
Pathophysiology of Cerebellar Dysfunction in the Wernicke-Korsakoff Syndrome
FB Roger |
CAN J NEUROL SCI | 1993 |
Reappraisal of regional thiamine content in the central nervous system of the normal and thiamine-deficient mice
N Harata, Y Iwasaki, Y Ohara |
Metabolic Brain Disease | 1993 |
Pathophysiologic mechanisms responsible for the reversible (thiamine-responsive) and irreversible (thiamine non-responsive) neurological symptoms of Wernicke's encephalopathy
RF Butterworth |
Drug and Alcohol Review | 1993 |
Thiamine transport by erythrocytes and ghosts in thiamine-responsive megaloblastic anaemia
G Rindi, D Casirola, V Poggi, B Vizia, C Patrini, U Laforenza |
Journal of Inherited Metabolic Disease | 1992 |
Thiamine transport by erythrocytes and ghosts in thiamine-responsive megaloblastic anaemia
G Rindi, D Casirola, V Poggi, BD Vizia, C Patrini, U Laforenza |
Journal of Inherited Metabolic Disease | 1992 |
Effect of thiamin deficiency on energy metabolites in the turkey
JC Remus, JD Firman |
The Journal of Nutritional Biochemistry | 1990 |
Effects of thiamine deficiency on thiamine-dependent enzymes in regions of the brain of pregnant rats and their offspring
H Fournier, RF Butterworth |
Metabolic Brain Disease | 1990 |
Changes in Brain Lipid Composition in Thiamine Deficient Rats
M OKAZAKI, H SAKAMOTO, A OHTSUKI, K OGUCHI |
The Japanese Journal of Pharmacology | 1990 |
Brain glutamate and γ-aminobutyrate (GABA) metabolism in thiamin-deficient rats
MG Page, V Ankoma-Sey, WF Coulson, DA Benders |
British Journal of Nutrition | 1989 |
Regionally selective alterations in enzymatic activities and metabolic fluxes during thiamin deficiency
G Gibson, P Nielsen, V Mykytyn, K Carlson, J Blass |
Neurochemical Research | 1989 |
Studies on thiamine metabolism in thiamine-responsive megaloblastic anaemia
V Poggi, G Rindi, C Patrini, BD Vizia, G Longo, G Andria |
European Journal of Pediatrics | 1989 |
Pyruvate Dehydrogenase Activity in Osmotically Shocked Rat Brain Mitochondria: Stimulation by Oxaloacetate
RH Haas, G Thompson, B Morris, K Conright, T Andrews |
Journal of Neurochemistry | 1988 |
Transketolase abnormality in cultured fibroblasts from familial chronic alcoholic men and their male offspring
AB Mukherjee, S Svoronos, A Ghazanfari, PR Martin, A Fisher, B Roecklein, D Rodbard, R Staton, D Behar, CJ Berg |
Journal of Clinical Investigation | 1987 |
Muricidal suppression by chlorpheniramine and changes in brain levels following dietary-induced thiamine deficiency in rats
K Onodera |
Physiology & Behavior | 1987 |
Activities of thiamine-dependent enzymes in two experimental models of thiamine deficiency encephalopathy: 3. Transketolase
JF Gigure, RF Butterworth |
Neurochemical Research | 1987 |
Cerebral thiamine-dependent enzyme changes in experimental Wernicke's encephalopathy
RF Butterworth |
Metabolic Brain Disease | 1986 |
Thiamine-deficient encephalopathy in rats: Effects of deficiencies of thiamine and magnesium
I Goto, H Nagara, J Tateishi, Y Kuroiwa |
Brain Research | 1986 |
Cerebral Acidosis in Focal Ischemia: I. A Method for the Simultaneous Measurement of Local Cerebral pH with Cerebral Glucose Utilization or Cerebral Blood Flow in the Rat
AM Hakim, M Arrieta |
Journal of Cerebral Blood Flow & Metabolism | 1986 |
Human Growth
F Falkner, JM Tanner |
1986 | |
Activities of thiamine-dependent enzymes in two experimental models of thiamine-deficiency encephalopathy 2. ?-ketoglutarate dehydrogenase
RF Butterworth, JF Gigure, AM Besnard |
Neurochemical Research | 1986 |
Thiamine deficiency depletes cortical norepinephrine and impairs learning processes in the rat
RG Mair, CD Anderson, PJ Langlais, WJ McEntee |
Brain Research | 1985 |
Wernicke's Encephalopathy
JB Reuler, DE Girard, TG Cooney |
New England Journal of Medicine | 1985 |
Selected Topics from Neurochemistry
RF Butterworth |
Selected Topics from Neurochemistry | 1985 |
Activities of thiamine-dependent enzymes in two experimental models of thiamine-deficiency encephalopathy:: 1. The pyruvate dehydrogenase complex
RF Butterworth, JF Giguere, AM Besnard |
Neurochemical Research | 1985 |
Pathological Neurochemistry
A Lajtha |
1985 | |
Alcohol and the Brain
RE Tarter, DH van Thiel |
1985 | |
Correlation of enzymatic, metabolic, and behavioral deficits in thiamin deficiency and its reversal
GE Gibson, H Ksiezak-Reding, KF Sheu, V Mykytyn, JP Blass |
Neurochemical Research | 1984 |
Alcohol and Brain Damage
RR Tuck, BJ Brew, AM Britton, J Loewy |
Addiction | 1984 |
The induction and reversibility of cerebral acidosis in thiamine deficiency
AM Hakim |
Annals of Neurology | 1984 |
Nervous tissue thiamine metabolism in vivo. II. Thiamine and its phosphoesters dynamics in different brain regions and sciatic nerve of the rat
G Rindi, V Comincioli, C Reggiani, C Patrini |
Brain Research | 1984 |
Sequence of metabolic, clinical, and histological events in experimental thiamine deficiency
AM Hakim, HM Pappius |
Annals of Neurology | 1983 |
Metabolic and Histological Reversibility of Thiamine Deficiency
AM Hakim, S Carpenter, HM Pappius |
Journal of Cerebral Blood Flow & Metabolism | 1983 |
Medical and Social Aspects of Alcohol Abuse
B Tabakoff, PB Sutker, CL Randall |
1983 | |
Effect of tea consumption on the levels ofα-ketoglutarate and pyruvate dehydrogenase in rat brain
P Ruenwongsa, S Pattanavibag |
Experientia | 1982 |
THIAMIN HOMEOSTASIS IN THE CENTRAL NERVOUS SYSTEM
R Spector |
Annals of the New York Academy of Sciences | 1982 |
Kinetics of thiamine transport across the blood-brain barrier in the rat
J Greenwood, ER Love, OE Pratt |
The Journal of Physiology | 1982 |
Kinetic Studies of Mouse Brain Transketolase
JP Blass, S Piacentini, E Boldizsar, A Baker |
Journal of Neurochemistry | 1982 |
Effects of Septal Lesions on Enzymes of Acetyl-CoA Metabolism in the Cholinergic System of the Rat Hippocampus
A Szutowicz, PA Srere, CN Allen, IL Crawford |
Journal of Neurochemistry | 1982 |
ENERGY METABOLISM IN THE LATERAL VESTIBULAR NUCLEUS IN PYRITHIAMIN-INDUCED THIAMIN DEFICIENCY
DW McCandless |
Annals of the New York Academy of Sciences | 1982 |
Thiamine Deficiency Limits Glucose Utilization and Glial Proliferation in Brain Lesions of Symptomatic Rats
FR Sharp, E Bolger, K Evans |
Journal of Cerebral Blood Flow & Metabolism | 1982 |
Ruminant thiamine requirement in perspective
EE Edwin, R Jackman |
Veterinary Research Communications | 1981 |
Determination of pyruvate dehydrogenase and acetyl-CoA synthetase activities using citrate synthase
A Szutowicz, M Stȩpien, G Piec |
Analytical Biochemistry | 1981 |
The role of citrate derived from glucose in the acetylcholine synthesis in rat brain synaptosomes
A Szutowicz, H Bielarczyk, W L̵ysiak |
International Journal of Biochemistry | 1981 |
Nature of the Autofluorescent Material in Cerebrocortical Necrosis
EE Edwin, R Jackman |
Journal of Neurochemistry | 1981 |
Characteristics of an acid active thiamine diphosphatase from beef brain
JV Murphy, FE Frerman, A Hodach |
Neurochemical Research | 1980 |
Regional and Subcellular Distribution of ATP-Citrate Lyase and Other Enzymes of Acetyl-CoA Metabolism in Rat Brain
A Szutowicz, WZ Lysiak |
Journal of Neurochemistry | 1980 |
Effect of thiamine deficiency on levels of putative amino acid transmitters in affected regions of the rat brain
E Hamel, RF Butterworth, A Barbeau |
Journal of Neurochemistry | 1979 |
Genetic Factors in Wernicke-Korsakoff Syndrome
JP Blass, GE Gibson |
Alcoholism, clinical and experimental research | 1979 |
Measurement of Ataxia and Related Neurological Signs in the Laboratory Rat
FB Jolicoeur, DB Rondeau, E Hamel, RF Butterworth, A Barbeau |
CAN J NEUROL SCI | 1979 |
Effects of thiamine deficiency on cerebral and visceral protein synthesis
GI Henderson, AM Hoyumpa, S Schenker |
Biochemical Pharmacology | 1978 |
Abnormality of a Thiamine-Requiring Enzyme in Patients with Wernicke-Korsakoff Syndrome
JP Blass, GE Gibson |
New England Journal of Medicine | 1977 |
Alcohol and Aldehyde Metabolizing Systems
U Rydberg, AL Myrsten, A Neri |
Alcohol and Aldehyde Metabolizing Systems | 1977 |
Untersuchungen zur biologischen Wirksamkeit sogenannter Thiaminantagonisten bei Ratten mit suboptimaler Thiaminversorgung1
K Schaller, C Brammertz-Buchen, KH Brammertz, H Höller |
Zeitschrift für Tierphysiologie Tierernährung und Futtermittelkunde | 1977 |
Peripheral nerve changes in thiamine deficiency and starvation: An electron microscopic study
F Pawlik, A Bischoff, I Bitsch |
Acta Neuropathologica | 1977 |
Thiamine transport in thiamine-deficient rats role of the unstirred water layer
AM Hoyumpa, S Nichols, S Schenker, FA Wilson |
Biochimica et Biophysica Acta (BBA) - Biomembranes | 1976 |
Glycolytic metabolism in cultured cells of the nervous system IV. The effects of thiamine deficiency on thiamine levels, metabolites and thiamine-dependent enzymes of the C-6 glioma and C-1300 neuroblastoma cell lines
JP Schwartz, DW McCandless |
Molecular and Cellular Biochemistry | 1976 |
Effect of diet-induced thiamine deficiency on visceral DNA synthesis and tissue composition
GI Henderson, M Dillon, S Schenker |
Biochemical Pharmacology | 1976 |
Glycolytic metabolism in cultured cells of the nervous system IV. The effects of thiamine deficiency on thiamine levels, metabolites and thiamine-dependent enzymes of the C-6 glioma and C-1300 neuroblastoma cell lines
JP Schwartz, DW McCandless |
Molecular and Cellular Biochemistry | 1976 |
Wernicke's encephalopathy
NK Blank, NA Vick, S Schulman |
Acta Neuropathologica | 1975 |
Increased muricide and decreased avoidance and discrimination learning in thiamine deficient rats
CV Vorhees, RJ Barrett, S Schenker |
Life Sciences | 1975 |
Alcohol and Abnormal Protein Biosynthesis
M Oratz, MA Rothschild |
Alcohol and Abnormal Protein Biosynthesis | 1975 |
Metabolism of Sulfur Compounds
IG Leder |
Metabolism of Sulfur Compounds | 1975 |
Biology of Brain Dysfunction
GE Gaull |
1975 | |
Wernicke's encephalopathy: An experimental study in the rhesus monkey
NK Blank, NA Vick, S Schulman |
Acta Neuropathologica | 1975 |
Thiamine deficiency encephalopathy in the developing rat
SE Geel, PM Dreyfus |
Brain Research | 1974 |
The Biology of Alcoholism
B Kissin, H Begleiter |
1974 | |
THOUGHTS ON THE PATHOPHYSIOLOGY OF WERNICKE'S DISEASE
PM Dreyfus |
Annals of the New York Academy of Sciences | 1973 |
Abnormalities in Pyruvate Dehydrogenase and Neurologic Function
J Blass |
International Journal of Neuroscience | 1972 |
Relationship of nervous tissue transketolase to the neuropathy in chronic uremia
RB Sterzel, M Semar, ET Lonergan, G Treser, K Lange |
Journal of Clinical Investigation | 1971 |
Effect of thiamine deficiency on the competence of the blood-brain barrier to albumin labeled with fluorescent dyes
DM Robertson, HJ Manz |
The American Journal of Pathology | 1971 |
Hepatic metabolism and transport in thiamine deficiency
S Schenker, D Chen, V Speeg, CO Walker, DW McCandless |
The American Journal of Digestive Diseases | 1971 |
Erythrocyte Transketolase Activity in Dialyzed Patients: A Reversible Metabolic Lesion of Uremia
ET Lonergan, M Semar, RB Sterzel, G Treser, MA Needle, L Voyles, K Lange |
New England Journal of Medicine | 1971 |
A defect in pyruvate decarboxylase in a child with an intermittent movement disorder
JP Blass, J Avigan, BW Uhlendorf |
Journal of Clinical Investigation | 1970 |