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Korsakoff Syndrome - Causes, Symptoms, Diagnosis, and Treatment

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Korsakoff Syndrome - Causes, Symptoms, Diagnosis, and Treatment

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Korsakoff or Wernicke-Korsakoff syndrome is a neurological disorder caused by a vitamin B1 deficiency. Continue reading the article to learn more about this.

Written by

Dr. Shikha

Medically reviewed by

Dr. Abhishek Juneja

Published At August 8, 2022
Reviewed AtJanuary 19, 2024

What Is Korsakoff Syndrome?

Korsakoff syndrome is a memory disorder that predominantly affects the brain's memory system. It is usually caused by a thiamine (vitamin B1) shortage, which can be caused by alcohol misuse, nutritional deficits, continuous vomiting, eating problems, or chemotherapy side effects.

Thiamine aids in the production of energy from sugar by brain cells. Brain cells cannot generate sufficient energy to function correctly when thiamine levels drop too low. Korsakoff syndrome is usually caused by excessive alcohol consumption. However, it can also be caused by acquired immunodeficiency syndrome (AIDS), malignancies that have metastasized throughout the body, persistent infections, poor nutrition, and a variety of other disorders. It is also prevalent among those whose bodies cannot effectively digest food (malabsorption). This might occur as a result of a chronic condition or after bariatric (weight-loss) surgery.

Wernicke encephalopathy, an abrupt brain reaction to severe thiamine deficiency, is frequently, although not always, a precursor of Korsakoff syndrome. Wernicke encephalopathy is a life-threatening condition that causes confusion, stumbling, poor coordination, and irregular involuntary eye movements. Korsakoff syndrome and Wernicke encephalopathy are not the same things. They are just various stages of the very same illness. Both are caused by a deficiency of vitamin B1 in the brain.

Korsakoff syndrome is frequently referred to as Wernicke-Korsakoff syndrome since chronic memory loss often occurs after an incident of Wernicke encephalopathy. Korsakoff syndrome, on the other hand, can arise in people who have never experienced Wernicke encephalopathy.

What Causes Korsakoff Syndrome?

  • Some people may possess a genetic predisposition to develop Korsakoff syndrome due to hereditary causes. The high-affinity thiamine transporter gene has been linked to the disease. The SLC19A2 gene codes for thiamine transporter 1, a protein that transports thiamine. This protein is to be found on the surface of cells, where it aids in the absorption of vitamin B1 (thiamine). More research is needed on this condition to determine the impact of genetics on the disorder's development.

  • Korsakoff syndrome is caused by thiamine insufficiency (vitamin B1). Thiamine is a necessary vitamin for the brain to convert glucose for energy. The activity of the hypothalamus, which governs temperature, hunger, emotions, and growth, is affected by thiamine deficiency in the most metabolically active brain regions. Thiamine insufficiency has a more substantial impact on cells in the neurological and cardiovascular systems than on cells in other organ systems. Alcohol decreases thiamine absorption in the body, depletes thiamine reserves in the liver, and inhibits the action of the enzyme, which transforms thiamine into an active form.

  • Malnutrition might also cause the disease. Starvation, eating disorders such as anorexia, and prolonged or chronic vomiting, as seen in hyperemesis gravidarum, are all causes of malnutrition that can lead to Korsakoff syndrome. Korsakoff syndrome can also be caused by chronic illnesses such as AIDS, cancer, stomach problems (gastropathy), and kidney problems. Due to the small food quantity consumed, gastric bypass surgery makes it challenging to meet nutritional needs and hence can lead to Korsakoff syndrome.

What Are the Symptoms and Signs of Korsakoff Syndrome?

Korsakoff syndrome is characterized by:

  • Difficulties in acquiring new knowledge.

  • Inability to recall recent events.

  • Long-term memory gaps.

  • Memory problems can be severe, although other intellectual and social abilities remain mostly untouched. Individuals may appear to be able to carry on a cogent discussion one moment and then be unable to recall the conversation or who they spoke with the next.

  • Patients may confabulate or make up things they do not recall. The mechanism by which Korsakoff syndrome may generate confabulation is unknown to scientists.

  • Hallucinations or the possibility that the person sees or hears things that are not there.

How to Diagnose Korsakoff Syndrome?

Korsakoff syndrome is a clinical diagnosis that represents a doctor's best guess as to what is causing a patient's symptoms.

According to experts, a medical examination for loss of memory or other cognitive abnormalities should always include questions regarding an individual's alcohol usage. Anyone who is admitted into the hospital for an alcohol-related ailment should have their memory and cognitive function tested by a professional. Supplementary questions to test recent memory should be included in the screening. If the test reveals that the patient has a cognitive impairment, they should be given a thorough examination.

Since symptoms of other disorders are frequent among people who overuse alcohol, such as intoxication or withdrawal, infection, or head injury, the syndrome gets concealed and can be challenging to detect. The doctor may do an initial examination to look for indicators of alcoholism. They may do a blood alcohol test on the patients. They may perform a liver function test to see whether there is any damage to the liver. A typical symptom of alcoholism is liver damage.

The doctor will check for indicators of vitamin B-1 deficiency in the case. This may entail blood tests to assess the thiamine levels as well as the overall nutritional health. The doctor may conduct the following nutritional tests to ensure that a patient is not malnourished:

  • Test for Serum Albumin in the Blood- Albumin, a protein in the blood, is measured in this test. Low albumin levels can indicate nutritional inadequacies as well as renal or liver issues.

  • Test for Serum Vitamin B1 in the Blood- This test measures the amount of vitamin B-1 in the blood. The activity of enzymes in red blood cells (RBCs) can be measured. Vitamin B-1 insufficiency is indicated by low enzyme activity in RBCs.

There are no specific laboratory tests or neuroimaging procedures that can be used to confirm whether or not someone has this condition.

How to Treat Korsakoff Syndrome?

According to some medical professionals, heavy and chronic drinkers, as well as others who are at risk of thiamine deficiency, should take oral Thiamine and other vitamin supplements under the supervision of their doctor. Many experts also advise giving injectable Thiamine to anyone with a history of heavy alcohol use, who is experiencing symptoms of Korsakoff syndrome, such as acute confusion, prolonged nausea and vomiting, and hallucinations, until the clinical picture becomes more evident.

Individuals should be carefully investigated once acute symptoms have subsided to see if their medical history, alcohol use, and pattern of memory issues are consistent with korsakoff syndrome. Extensive medication using oral Thiamine, other vitamins, and magnesium may improve the likelihood of symptom improvement in patients who acquire Korsakoff syndrome. If there is no improvement, treatment of medical illnesses and underlying comorbidities and the requirement for long-term assisted residential care or supported housing should be considered.

Conclusion:

A cornerstone of good long-term treatment is abstaining from alcohol and eating a balanced diet. Korsakoff syndrome patients have a lower tolerance for alcohol and are more likely to develop alcohol-related health problems. According to available data, approximately 25 % of persons who develop Korsakoff syndrome eventually recover. About half of the patients improve but do not fully recover, and about one-fourth of them remain unchanged. According to some studies, persons who recover from an incident may have a normal life expectancy if they do not drink.

Frequently Asked Questions

1.

What Does Korsakoff’s Syndrome Mean?

Korsakoff’s syndrome is a memory disorder caused by thiamine (vitamin B1) deficiency. It is also known as Korsakoff’s amnesic syndrome, which may develop in association with alcoholism. Due to the lack of vitamin B1, the brain may not function properly, resulting in symptoms like tremors, disorientation, vision problems, etc.

2.

What Are the Classic Features of Korsakoff Syndrome?

The characteristics features of Korskoff’s syndrome are:
- Trouble in retaining information.
- Difficulty in learning new things.
- Inability to recall recent events.
- Hallucination.

3.

What Causes Korsakoff’s Syndrome?

The exact reason for the occurrence of Korsakoff’s syndrome is unknown. However, the following may cause it:
- Genetic factors.
- Vitamin B1 deficiency.
- Other risk factors include malnutrition, chronic illness, etc.

4.

What Are the Initial Signs of Korsakoff Syndrome?

The signs that develop first with Korsakoff’s syndrome are:
- Mental confusion.
- Vision problems.
- Tremor.
- Disorientation.
- Improper muscle coordination.

5.

At What Age Does Korsakoff’s Syndrome Develop?

Korsakoff’s syndrome usually develops during the age of 30 to 70 years. In addition, it affects both men and women. However, according to reports, males are more commonly affected than females due to the strong association with alcohol consumption. Other factors like chronic illness and malnutrition might increase the chance of Korsakoff’s syndrome development. However, children are rarely affected.

6.

Does Korsakoff’s Syndrome Develop Quickly?

Korsakoff’s syndrome mainly occurs as a result of thiamine deficiency. Its initial symptoms, like confusion and disorientation, may develop over a few weeks. The disease usually progresses gradually and may lead to memory problems. The symptoms may subside with vitamin B supplements and the removal of risk factors, especially alcohol consumption.

7.

What Can Help a Person With Korsakoff’s Syndrome?

The following treatment may help the person affected by Korsakoff’s syndrome:
- Restrain alcohol.
- Follow a nutritious diet.
- Oral thiamine.
- Hydration.

8.

Can Korsakoff’s Syndrome Be Reversed?

No, Korsakoff’s syndrome is not reversible. In addition, vitamin supplements and other supportive care may improve the symptoms but not treat the condition completely. However, staying away from alcohol can help in recovery and enhances life expectancy. The disease may also progress gradually with prolonged thiamine deficiency. Therefore hydration along with nutritional supplementation is crucial in such patients.

9.

Is Korsakoff Syndrome Considered Dementia?

Korsakoff’s syndrome is believed to be a type of alcohol-related dementia. It is because, due to prolonged alcohol intake, the symptoms of Korsakoff’s syndrome may develop. It includes tremors, disorientation, vision problems, lack of muscle coordination, etc. In addition, it may gradually cause problems with memory. However, other factors may also lead to Korsakoff’s syndrome.

10.

Is Wernicke and Korsakoff Syndrome the Same?

Wernicke’s and Korsakoff’s syndrome may occur due to thiamine deficiency. According to a few studies, Wernicke’s encephalopathy is an acute form of the disease and may progress to Korsakoff’s syndrome. In addition, it can be reversed, while Korsakoff’s syndrome is irreversible. The treatment involves discontinuation of alcohol and vitamin B1 supplementation.
Dr. Abhishek Juneja
Dr. Abhishek Juneja

Neurology

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