Wernicke-Korsakoff Syndrome

Overview

What is Wernicke-Korsakoff syndrome?

Wernicke-Korsakoff syndrome (WKS) is a brain and memory disorder that requires immediate treatment. It happens due to a severe lack of thiamine (vitamin B1), which causes damage to your brain. Thiamine is an essential vitamin that your body uses to convert food into energy.

WKS consists of two stages:

  1. Wernicke encephalopathy, a sudden and severe (acute) brain disorder
  2. Korsakoff syndrome, a long-term (chronic) memory disorder.

This condition is named for German neurologist Carl Wernicke and Russian neuropsychiatrist Sergei Korsakoff.

Untreated, Wernicke encephalopathy develops into Korsakoff syndrome. Commonly, people with Wernicke encephalopathy have three main symptoms:

  • Confusion.
  • Inability to coordinate voluntary movements (also known as ataxia).
  • Visual changes and additional eye problems.

In addition, people may experience some muscle loss.

When these symptoms become long term, they cause lasting brain and nerve cell damage. This causes severe memory loss and the inability to form new memories, leading to Korsakoff syndrome.

Over time, Wernicke-Korsakoff syndrome worsens and can be life-threatening. Emergency treatment with thiamine is essential. It may relieve symptoms and prevent permanent damage to memory and other brain functions.

If you or a loved one has symptoms of WKS, call your healthcare provider or 911 immediately.

Who does Wernicke-Korsakoff syndrome affect?

Wernicke-Korsakoff syndrome affects more men than women, usually between ages 30 and 70 years. Most alcohol-related cases of WKS involve men and those over age 40. Women and people who are younger are more likely to develop the syndrome due to other causes (aside from alcohol). Wernicke-Korsakoff syndrome is rarely reported in children.

Who is at risk for Wernicke-Korsakoff syndrome?

Wernicke encephalopathy develops most often in people with alcohol use disorder. Long-term (chronic) alcohol use irritates your stomach and digestive tract. It also interferes with your body’s ability to absorb vitamins (malabsorption). An estimated 80% of people with alcohol addiction don’t absorb or retain enough thiamine.

WKS also happens in people who can’t properly absorb food due to certain conditions. It may develop after weight loss (bariatric) surgery or as a side effect of chemotherapy.

Other risk factors include:

  • Bowel or stomach problems, including inflammatory bowel disease or stomach disease (gastropathy).
  • Cancer (advanced stages).
  • Extremely high thyroid hormone levels (thyrotoxicosis).
  • Having certain genetic changes (mutations).
  • Heart failure that’s treated with diuretic therapy for a prolonged period.
  • HIV/AIDS, which can lead to conditions that lessen thiamine.
  • Kidney failure, as long-term dialysis reduces your body’s ability to absorb thiamine.
  • Lack of access to medical care or healthy food.
  • Vomiting and extreme nausea in pregnancy (hyperemesis gravidarum).

Are there other conditions like Wernicke-Korsakoff syndrome?

Other disorders caused by a lack of vitamin B1 include:

  • African seasonal ataxia.
  • Beriberi.
  • Central pontine myelinolysis (CPM).
  • Leighs disease.
  • Nutritional optic neuropathy.

How common is Wernicke-Korsakoff syndrome?

Wernicke-Korsakoff syndrome occurs in up to 2% of people worldwide.

About 50% of those who develop Wernicke encephalopathy eventually develop Korsakoff syndrome. The number is higher (80%) among those who have alcohol use disorder.

Symptoms and Causes

What causes Wernicke-Korsakoff syndrome?

Wernicke-Korsakoff syndrome happens due to a lack of thiamine. This essential vitamin changes (converts) sugar into energy. When you have too little thiamine, your brain doesn’t have enough energy to work properly.

The most common cause of thiamine deficiency is chronic alcohol use. Alcohol makes it harder for your body to absorb thiamine and store it in your liver. It can also make it difficult for your body to use vitamin B1 for other essential functions.

Other causes of Wernicke-Korsakoff syndrome include:

What are the symptoms of Wernicke-Korsakoff syndrome?

Wernicke-Korsakoff syndrome can cause a wide range of symptoms. One symptom in people with severe disease is confabulation, where they invent information to cover memory loss.

People with the syndrome may also be confused or disoriented. The confusion makes it difficult for them to realize anything is wrong and seek treatment.

Some common signs of Wernicke encephalopathy may include:

  • Balance problems or loss of coordination.
  • Confabulation.
  • Confusion (delirium).
  • Difficulty walking (unsteady gait).
  • Drowsiness.
  • Extreme loss of body heat (hypothermia).
  • Heart issues, including rapid heartbeat (tachycardia) or low blood pressure (hypotension).
  • Lack of muscle coordination (ataxia).

Eye issues are also common, including:

  • Abnormal eye movement (nystagmus).
  • Double vision.
  • Drooping eyelid (ptosis).
  • Eye muscle paralysis or weakness (ophthalmoplegia).

Left untreated, Wernicke encephalopathy can lead to Korsakoff syndrome, a chronic memory disorder. Symptoms include:

  • Amnesia or inability to form new memories.
  • Behavioral changes, such as agitation or anger.
  • Confabulation.
  • Delirium and disorientation.
  • Fatigue or lethargy.
  • Hallucinations, especially in those withdrawing from alcohol.
  • Lack of focus or attention.
  • Unsteady gait.

Some symptoms may not improve, even with treatment. In severe cases, people may experience loss of consciousness (coma). If you or a loved one has lost consciousness or has symptoms of Wernicke-Korsakoff syndrome, call your healthcare provider or 911 immediately.

Diagnosis and Tests

How is Wernicke-Korsakoff syndrome diagnosed?

Early detection of WKS is crucial for effective treatment. Healthcare providers diagnose Wernicke-Korsakoff syndrome using a physical exam. They also ask about your symptoms and family history. Diagnosing Wernicke-Korsakoff syndrome can be difficult when people are experiencing mental confusion or memory loss.

It’s important for your healthcare provider to check for symptoms of:

  • Alcohol use disorder.
  • Malnutrition.
  • Thiamine deficiency.

What tests help diagnose Wernicke-Korsakoff syndrome?

Your healthcare provider may recommend blood tests to check blood sugar and thiamine levels. Healthcare providers often check for signs of alcohol use disorder with a blood alcohol test and liver function test. A common sign of this condition is liver damage.

Healthcare providers may perform additional tests to rule out other causes, such as:

  • Arterial blood gas (ABG) test to measure carbon dioxide and oxygen levels in your blood.
  • Complete blood count (CBC).
  • Toxicology (drug) screening.

You may also need imaging tests, which may include:

Management and Treatment

Is Wernicke-Korsakoff syndrome curable?

Wernicke-Korsakoff syndrome isn’t curable. But healthcare providers can help relieve your symptoms with immediate treatment. With treatment, Wernicke encephalopathy might not progress to Wernicke-Korsakoff syndrome.

In many cases, treatment can slow or reverse symptoms such as confusion, eye problems and muscle coordination. Your healthcare provider can discuss treatment options with you to tailor a care plan for your needs.

How is Wernicke-Korsakoff syndrome treated?

Treatment for Wernicke-Korsakoff syndrome depends on your symptoms and usually involves:

  • Vitamin B1 tablets or injection.
  • IV fluids (rehydration).
  • Alcohol use disorder treatment.
  • Nutritional support.
  • Medications.

Prevention

How can I reduce my risk of developing Wernicke-Korsakoff syndrome?

In many cases, stopping or limiting alcohol use is essential to reduce the risk of developing Wernicke-Korsakoff syndrome. Proper nutrition and supplementation (thiamine [vitamin B1] injection or tablets) may help reduce risk, especially for those with alcohol addiction.

Outlook / Prognosis

What’s the prognosis for someone with Wernicke-Korsakoff syndrome?

If you or a loved one has Wernicke-Korsakoff syndrome, the outlook depends on the severity of symptoms and a timely diagnosis. Immediate treatment can help reverse symptoms and avoid long-term complications. Left untreated, Wernicke-Korsakoff syndrome may lead to disability and even death.

Living With

When should I seek care for Wernicke-Korsakoff syndrome?

Seek care immediately if you or a loved one experiences symptoms of Wernicke-Korsakoff syndrome. Call your healthcare provider or 911 for emergency treatment.

How do I take care of myself?

A diagnosis of Wernicke-Korsakoff syndrome can be challenging. You may need to go to the hospital to receive treatment. Stopping or limiting alcohol use (follow the advice of your healthcare provider) and having proper nutrition can support your recovery.

Follow your healthcare provider’s recommendations to manage complications and treat symptoms. They can work with you to develop a personalized care plan to help restore your health.

A note from Cleveland Clinic

Wernicke-Korsakoff syndrome is an unusual memory disorder. It requires immediate treatment to relieve symptoms and avoid complications. Advanced stages of the disease include neurological symptoms such as severe confusion and disorientation. These symptoms may make it difficult for you or a loved one to recognize signs of the condition, so early diagnosis is key. Your healthcare provider can recommend treatment options to address your needs.

Last reviewed by a Cleveland Clinic medical professional on 04/04/2022.

References

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