What is Wernicke-Korsakoff Syndrome?
Wernicke-Korsakoff syndrome, often seen in people who frequently consume alcohol, is a common problem that arises from lack of thiamine, or vitamin B1. This was originally defined by a set of three symptoms: confusion or dementia, unusual eye movements or paralysis, and poor coordination or balance (ataxia). However, patients often exhibit fewer than all three of these symptoms.
The term Wernicke-Korsakoff syndrome is more broadly used to describe two related conditions, Wernicke encephalopathy and Korsakoff syndrome. Wernicke encephalopathy is severe confusion, which usually improves with treatment. On the other hand, Korsakoff syndrome includes symptoms like making up events and experiences (confabulation), losing memories, and issues with walking and this typically cannot be reversed. Korsakoff syndrome may develop if Wernicke encephalopathy is not treated in time.
What Causes Wernicke-Korsakoff Syndrome?
Wernicke-Korsakoff syndrome is caused by a lack of thiamine, also known as vitamin B1 in the body. People who don’t get enough nutrients in their diet, for whatever reason, are more likely to have this condition. The most common factor linked with Wernicke-Korsakoff syndrome is regular abuse of alcohol, which leads to lesser absorption and use of thiamine in the body. However, it’s important to note that this disease can also be caused by non-alcohol related reasons that lead to a lack of thiamine.
Some examples of getting Wernicke-Korsakoff syndrome due to reasons other than alcohol are malnutrition, going without food, severe mental disorders like schizophrenia, anorexia nervosa, being a prisoner of war, or having a terminal cancer. In the past, baby formula that didn’t have enough thiamine also caused this syndrome. Pregnant women in their first trimester who have severe nausea and vomiting, a condition known as hyperemesis gravidarum, can also experience Wernicke-Korsakoff syndrome.
Another common cause is weight-loss surgery and cancers of the digestive system.
Additional causes of Wernicke-Korsakoff syndrome can include:
* Eating disorders like anorexia
* Advanced-stage cancer
* Going without food
* Strict diets
* Inflammatory bowel disease, a condition causing inflammation in the digestive tract
* Obstruction of the bowel
* AIDS
* Breastfeeding without taking additional nutritional supplements
* Whole body diseases like tuberculosis, a bacterial infection that mainly affects the lungs, or uremia, which is a high level of waste products in the blood due to kidney failure.
Risk Factors and Frequency for Wernicke-Korsakoff Syndrome
The occurrence of certain conditions is between 0 and 2% around the world. However, these conditions are more common in specific groups of people. These include homeless individuals, older people who live alone or in seclusion, and individuals receiving care in psychiatric hospitals. While it may seem likely, the rate of these conditions does not relate to the total amount of alcohol consumed in a country. For example, in France, which is famous for its wine consumption, the rate is about 0.4%. On the other hand, in Australia, the rate is relatively higher at around 3%.
Signs and Symptoms of Wernicke-Korsakoff Syndrome
Wernicke-Korsakoff syndrome is a condition that can cause a range of symptoms. Classically, individuals with this syndrome may experience eye problems like double vision, painless loss of sight or crossed eyes, difficulties with walking such as staggering or shuffling steps and needing help to stand or move around. There may also be changes in mental state, which can include lack of interest, reduced speech and lack of response to the environment. However, it’s important to note that not everyone experiences all these symptoms. As the condition progresses additional symptoms like restlessness, anger, hallucinations and false memories might occur.
- Eye problems: Double vision, painless vision loss, crossed eyes
- Walking difficulties: Staggering or shuffling steps, inability to stand or move without help
- Mental status changes: Lack of interest, reduced speech, lack of response to the environment
- Progressive symptoms: Restlessness, anger, hallucinations, false memories
Testing for Wernicke-Korsakoff Syndrome
Wernicke-Korsakoff syndrome, a condition with serious health consequences, is mainly diagnosed by examining your medical history and observing any symptoms you may have. Doctors may also use lab tests and imaging for a more in-depth evaluation. It’s important to identify this condition as early as possible because it can lead to severe health problems and even death if left untreated.
Normally, the treatment process begins with taking thiamine (vitamin B1), which can help improve symptoms, before any diagnosis has been confirmed. This step is taken as a precautionary measure due to the high likelihood of this condition and the fact that thiamine is generally safe to use. However, it’s important to start thiamine before giving glucose (a type of sugar your body uses for energy). This avoids the risk of making Wernicke-Korsakoff syndrome worse.
Treatment Options for Wernicke-Korsakoff Syndrome
Unfortunately, Wernicke-Korsakoff syndrome usually doesn’t completely heal, even with intensive treatment. This condition is linked to a severe deficiency of thiamine, a crucial nutrient for brain health. After receiving treatment that boosts thiamine levels, the severe symptoms linked with brain impacts will typically get better in 5 to 12 days.
Patient should be given thiamine supplements orally, and a discussion about further treatment options for other related health conditions should follow. Most people with Wernicke-Korsakoff syndrome will require consistent care in a long-term healthcare setting. The overall outlook for these patients is generally uncertain.
What else can Wernicke-Korsakoff Syndrome be?
When it comes to diagnosing particular brain-related conditions, doctors often have to rule out several possible illnesses that could be causing similar symptoms. These include:
- Damage due to lack of oxygen in the brain (Anoxic encephalopathy)
- Alzheimer’s disease (a type of dementia affecting memory, thinking and behavior)
- Temporal lobe epilepsy (a type of seizure that happens in one area of your brain)
- Injury from a concussion to the head
- Dementia with Lewy bodies (a progressive brain disorder)
- Herpes simplex virus (infection that can cause encephalitis)
- Temporal lobe infarction (a stroke that happens in the temporal lobe of your brain)
- Transient global amnesia (temporary loss of all memory)
- Tumor located in the third ventricle of your brain
What to expect with Wernicke-Korsakoff Syndrome
About a quarter of people with Wernicke-Korsakoff syndrome—a neurological disorder often linked to alcohol abuse—will require long-term care in a medical facility, especially if they have other physical or mental health conditions, too.
Mental health complications can emerge with this condition. Many people experience some confusion, which slowly subsides following treatment. About one in five people showing signs of memory loss after starting treatment will fully recover, but others might still struggle with memory and learning.
Recovery can take years and usually requires a commitment to avoiding alcohol.
Several physical complications might also occur. Half of the people with Wernicke-Korsakoff syndrome might face difficulties with coordination and balance, even after recovery. In these cases, symptoms might still include a slow, wide-based walk, and difficulty with balance during walking. Half the patients also improve their vestibular function, which is related to balance and spatial orientation.
Eye-related complications are common, but tend to improve quickly when treated with thiamine, a vitamin that these patients lack. If the eye problems persist even after taking thiamine, it might be a sign that the diagnosis of Wernicke-Korsakoff syndrome should be reconsidered. Certain types of involuntary eye movements or other nerve-related eye problems can persist for months or even indefinitely, although they typically recover completely eventually.
People with Wernicke-Korsakoff syndrome can also face life-threatening complications such as infections and liver failure. These, along with the effects of long-term vitamin deficiency, can lead to a mortality rate of 10% to 15% for the most severe cases.
The outcome for a patient largely depends on how advanced the disease is when it is detected and how quickly treatment is initiated.
