Wernicke-Korsakoff Syndrome

Wernicke-Korsakoff Syndrome


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 duodenum 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. The brain is particularly vulnerable to impaired
glucose metabolism since it utilizes so much energy. Early on in thiamine deficiency, the cerebellum
gets affected and that can affect movement and balance. In addition, the brainstem can be affected,
and that’s the region that gives rise to the cranial nerves that provide motor and
sensory innervation to the face and eyes. If the medulla region of the brainstem is
affected, it can impair the heart rate and breathing. Later findings in thiamine deficiency are
hemorrhage and necrosis of the mammillary bodies. The mammillary bodies are two small round
structures located under the brain and are part of limbic system which is responsible
for memory, emotion, and behavior. Now Wernicke Korsakoff syndrome can be thought
of as a spectrum of disorders: Wernicke’s encephalopathy symptoms occur first, and can
eventually develop into the more severe symptoms found in Korsakoff syndrome. Wernicke’s encephalopathy is characterized
by ophthalmoplegia, meaning weakness or paralysis of the eye muscles, ataxia or unsteady gait,
and changes in mental state like confusion, apathy, and difficulty concentrating. And untreated Wernicke’s encephalopathy can
lead to coma and death if not treated quickly. Korsakoff syndrome, on the other hand, mainly
targets the limbic system, causing severe memory impairment. This includes anterograde amnesia, which is
the inability to create new memories, as well as retrograde amnesia, which is the inability
to recall previous memories. One of the characteristic findings of Korsakoff
syndrome is confabulation in which the person creates stories to fill in the gaps in their
memory which they believe to be true. Not too different from what young children
sometimes do in retelling a story. Diagnosis of Wernicke Korsakoff syndrome is
typically made by clinical impression of the presenting symptoms, as well as basic blood
and liver function tests to measure thiamine levels. Sometimes diagnosis may be confirmed by an
MRI which would show degeneration of the mammillary bodies. Wernicke’s encephalopathy is considered a
medical emergency and must be treated immediately to prevent the development of Korsakoff’s
syndrome. The therapy is an infusion of thiamine over
a few days to get rid of the deficiency. It’s usually given alongside glucose, but
it’s important to normalize the thiamine levels first, because without thiamine pyrophosphate,
most of the glucose will become lactic acid and that can lead to metabolic acidosis. All right, as a quick recap…Wernicke Korsakoff
syndrome is caused by thiamine deficiency and is commonly seen in chronic alcoholism. It is a combination of Wernicke’s encephalopathy
which consists of the triad of ophthalmoplegia, ataxia, and confusion and if left untreated,
may result in Korsakoff syndrome which manifests as aphasia and confabulation. Treatment involves the prompt administration
of thiamine, followed by glucose.

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21 Replies to “Wernicke-Korsakoff Syndrome”

  1. I am addicted to all of your videos, i have a suggestion could Osmosis make video about Wallenberg syndrome or lateral medullary infarction please?

  2. Will it give false positive for diabetes.oh what antipsy hotics csusecan also be seen in stomach problems etc.

  3. Unbelievable that such a demonic drug is the legal one… I challenge you to find an illegal drug that damages you more than this in its pure pharmaceutical form.

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