Wernicke-Korsakoff syndrome
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Wernicke-Korsakoff syndrome
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Wernicke-Korsakoff syndrome
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Wernicke-Korsakoff syndrome
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Ataxia
Wernicke-Korsakoff syndrome p. 64, 528, 595
Mammillary bodies p. 521, 528
Wernicke-Korsakoff syndrome p. 595
Memory loss
Wernicke-Korsakoff syndrome p. 64, 528, 595
Ophthalmoplegia p. 64
Wernicke-Korsakoff syndrome p. 64, 595
Vitamin B1 deficiency
Wernicke-Korsakoff syndrome p. 595
Wernicke-Korsakoff syndrome p. 528, 595
vitamin B1 deficiency p. 64
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Transcript
Contributors
Royce Rajan, MD, MBA
Ursula Florjanczyk, MScBMC
Evan Debevec-McKenney
Tanner Marshall, MS
Wernicke Korsakoff syndrome is named after Carl Wernicke and Sergei Korsakoff, the physicians who discovered the condition in the late 1800s. Wernicke Korsakoff syndrome is caused by Vitamin B1 or thiamine deficiency and it refers to a spectrum of disease. Wernicke's encephalopathy is the acute, reversible stage of the syndrome, and if left untreated it can later lead to Korsakoff syndrome, which is chronic and irreversible.
Thiamine is typically stored in the liver and absorbed in the jejunum and ileum, and then moves throughout the body, where it’s involved in numerous cellular processes that require thiamine. The enzyme thiamine pyrophosphate synthetase transfers a pyrophosphate group from ATP to thiamine, turning it into the coenzyme thiamine pyrophosphate - which is the metabolically active form of thiamine. Now, as a coenzyme, thiamine pyrophosphate functions to assist other enzymes such as pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase, and transketolase carry out reactions, particularly regarding glucose metabolism. Furthermore, within the brain, thiamine pyrophosphate helps metabolize lipids and carbohydrates as well as maintain normal amino acid and neurotransmitter levels. In some neurons, thiamine even helps with propagation of a neural impulses down the axon.
Given it’s multifaceted role, a deficiency of thiamine can have serious consequences. Specifically, thiamine deficiency impairs glucose metabolism and this leads to a decrease in cellular energy. One of the major causes of thiamine deficiency, and therefore Wernicke Korsakoff syndrome, is alcohol abuse. Alcohol leads to decreased thiamine levels in various ways. First, alcohol interferes with the conversion of thiamine to its active form, thiamine pyrophosphate by blocking the phosphorylation of thiamine. Second, thiamine is normally absorbed through the first portion of the small intestine called the duodenum. However, ethanol prevents this absorption process, and it is believed that alcohol does this by reducing the gene expression for thiamine transporter-1 within the intestinal brush border. Third, chronic alcohol abuse can lead to fatty liver or cirrhosis which interferes with the storage of thiamine within the liver. Other causes of thiamine deficiency are inadequate intake like in malnutrition and anorexia or due to malabsorption like in stomach cancer and inflammatory bowel disease.
Summary
Wernicke-Korsakoff syndrome (WKS) is a neurological disorder caused by a deficiency of thiamine (vitamin B1), often due to chronic alcohol abuse. It is a combination of Wernicke encephalopathy and Korsakoff syndrome, presenting with confusion, ophthalmoplegia, and ataxia. Wernicke encephalopathy can progress to Korsakoff syndrome, which causes severe memory loss, disorientation, and confabulation. Treatment includes thiamine supplements, rehabilitation, and cognitive therapy to manage symptoms.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "THE NATURAL HISTORY AND PATHOPHYSIOLOGY OF WERNICKE'S ENCEPHALOPATHY AND KORSAKOFF'S PSYCHOSIS" Alcohol and Alcoholism (2005)
- "Health problems and care needs in patients with Korsakoff’s syndrome: A systematic review" Journal of Psychiatric and Mental Health Nursing (2020)
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