Wernicke-Korsakoff Syndrome is a brain disorder caused by thiamine deficiency. Discover its symptoms, causes, and treatment at Meri Sehat.

Wernicke-Korsakoff Syndrome

A. JABBAR DENTAL CLINIC

Sadia Khan

Overview

Wernicke-Korsakoff syndrome is a neurological condition caused by a deficiency of vitamin B1 (thiamine). The syndrome refers to two different illnesses that can occur at the same time - Wernicke&#39;s disease and Korsakoff syndrome. Wernicke&#39;s encephalopathy is another name for Wernicke&rsquo;s disease. 
&nbsp;

In 1881, Carl Wernicke described the postmortem findings of three patients that had suffered from acute encephalopathy with mental confusion, ophthalmoplegia, and gait ataxia. His investigation revealed punctate hemorrhages surrounding the third and fourth ventricles and the aqueduct. A few years later, Russian psychiatrist Sergei Korsakoff reported a chronic amnestic illness with significant memory impairment compared to other cognitive areas. While these discoveries were made in the setting of chronic alcoholism, neither Wernicke nor Korsakoff immediately recognized the connection between the illnesses. It wasn&rsquo;t until later that researchers appreciated the findings<a href="#accrodion">[1]</a>.

Wernicke-Korsakoff syndrome (WKS) is the most well-known neurologic consequence of thiamine (vitamin B1) deficiency. The lack of thiamine causes damage to the brain and results in a life-threatening condition called Wernicke encephalopathy (WE). Confusion, ataxia, and visual problems are the three main symptoms of WE. If left untreated, these symptoms can become long-term and lead to permanent brain and nerve cell damage. Thus, resulting in Korsakoff syndrome, which is characterized by severe memory loss. Korsakoff syndrome affects around half of the people who acquire Wernicke encephalopathy<a href="#accrodion">[5]</a>. Those with an alcohol use disorder have a higher chance (80%) of acquiring WKS<a href="#accrodion">[5]</a>. 
&nbsp;

The worldwide prevalence of Wernicke-Korsakoff syndrome ranges between 1-2% in the general population and 12-14% in alcoholics<a href="#accrodion">[6]</a>. The disorder affects males more than females and is evenly distributed between the ages of 30-70 years<a href="#accrodion">[5]</a> <a href="#accrodion">[9]</a>.&nbsp;Certain groups, such as the homeless, elderly people living in isolation or alone, and psychiatric inpatients, have a higher prevalence of the disease<a href="#accrodion">[2]</a>. Surprisingly, prevalence is unrelated to per capita alcohol intake. For instance, the incidence is roughly 0.4% in France, a nation widely renowned for its wine drinking, whereas it is approximately 3% in Australia<a href="#accrodion">[2]</a>. 
&nbsp;

Note: Wernicke-Korsakoff syndrome requires early medical attention in order to alleviate symptoms and avoid complications. 
&nbsp;

Wernicke-Korsakoff syndrome has no specific subtypes. However, this condition encompasses two different stages of the syndromes: 
&nbsp;

<ol>
	<li>Wernicke Encephalopathy (Acute): A sudden and severe neurological disorder that is reversible. It is characterized by three main symptoms - gait abnormalities, confusion, and vision problems.</li>
	<li>Korsakoff Syndrome (Chronic): This is an irreversible memory disorder. It usually develops from untreated Wernicke encephalopathy. 
	&nbsp;</li>
</ol>

Signs and Symptoms

Most Wernicke-Korsakoff Syndrome patients initially experience Wernicke&#39;s encephalopathy and then Korsakoff syndrome. The signs and symptoms of each illness are listed below: 
&nbsp;

<h5>Wernicke Encephalopathy</h5>

Wernicke encephalopathy (WE) is brought on by a thiamine deficiency. Thiamine is an essential vitamin responsible for converting sugar into energy. It is required by every cell in the body. However, since humans cannot produce it, dietary sources are important in providing the body with thiamine. The liver, kidney, heart, and brain require especially high levels of the vitamin.

Due to low thiamine levels, the body is unable to carry out fundamental and essential cell processes. Thus, leading to symptoms of WE. Moreover, brain cells are especially sensitive to low thiamine levels, which explains why many symptoms of WE are neurological in nature.

An individual with Wernicke encephalopathy may present with:

<ul>
	<li>Confusion</li>
	<li>Altered mental status</li>
	<li>Jerky or involuntary eye movement</li>
	<li>Droopy upper eyelids (ptosis)</li>
	<li>Double vision</li>
	<li>Involuntary eye movements (nystagmus)</li>
	<li>Poor balance and difficulty walking (ataxia) 
	&nbsp;</li>
</ul>

WE patients may seem malnourished and underweight. They may also have low blood pressure, cardiac problems, or a low body temperature. 
&nbsp;

<h5>Korsakoff Syndrome</h5>

People with Korsakoff Syndrome (KS) frequently struggle to acquire new information. The person may also mistakenly invent facts to fill holes in their memory. Confabulation is the medical term for this condition. Short-term memory issues might make it difficult to form new memories and recall recent experiences. Moreover, they may experience personality changes, such as apathy and a lack of care, as well as talkative and repetitive conduct. Although the signs and symptoms of KS may gradually get better over time, about 25% of patients have a permanent form of the illness<a href="#accordion">[6]</a>.

WE and KS are related in the following ways:

<ul>
	<li>Wernicke encephalopathy frequently precedes Korsakoff syndrome</li>
	<li>WE symptoms tend to decrease as KS symptoms grow</li>
	<li>If a person is effectively treated for WE, KS may not develop 
	&nbsp;</li>
</ul>

Causes

Wernicke-Korsakoff syndrome results from a deficiency of thiamine, or vitamin B1. The vitamin is required for glucose metabolism, which is used by the brain for energy. Thus, a lack of thiamine means the various brain regions are unable to function. The activity of the hypothalamus, which regulates emotions, temperature, appetite, and growth, is especially affected. Moreover, the cells of the cardiovascular and nervous systems are also affected.

Alcohol use disorder is the number one cause of Wernicke-Korsakoff syndrome. Long-term, heavy alcohol use reduces the absorption and utilization of thiamine in the body. Moreover, alcohol makes it difficult for the body to store thiamine in the liver. As a result, the liver stores of thiamine are also depleted.

Malnourished individuals are also at risk for this condition. Furthermore, individuals suffering from deprivation, starvation, schizophrenia, anorexia nervosa, prisoners of war, and terminal cancer patients can develop the disease. Women who have hyperemesis gravidarum can develop the disorder during the first trimester of pregnancy. Previously, Wernicke-Korsakoff syndrome had also been linked to thiamine-deficient baby formula. Other common causes of Wernicke Korsakoff syndrome are mentioned below:

<ul>
	<li>Bariatric surgery, such as a gastric bypass&nbsp;</li>
	<li>Eating disorders, such as<a href="https://merisehat.pk/disease/anorexia-nervosa"> anorexia nervosa</a></li>
	<li>Terminal cancer</li>
	<li>Chemotherapy</li>
	<li>Starvation</li>
	<li>Strict diet</li>
	<li>Inflammatory bowel syndrome or gastric malignancy</li>
	<li>Bowel obstruction</li>
	<li><a href="https://merisehat.pk/disease/aids">Acquired immune deficiency syndrome (AIDS)</a></li>
	<li>Lactation without supplements</li>
	<li>Systemic disorders, such as <a href="https://merisehat.pk/disease/tuberculosis">tuberculosis</a>, uremia, stem cell transplantation, etc 
	&nbsp;</li>
</ul>

Additionally, infants breastfed by thiamine-deficient mothers are also affected. 
&nbsp;

Diagnosis

Identification of Wernicke-Korsakoff syndrome is critical for effective therapy. A physical exam is performed by healthcare practitioners to diagnose Wernicke-Korsakoff syndrome. They will also inquire about the symptoms and the patient&rsquo;s family history. When patients are experiencing mental confusion or memory loss, it might be difficult to diagnose Wernicke-Korsakoff syndrome. The doctor will also want to rule out other problems that could cause your symptoms. 
&nbsp;

<h5>Signs Of Alcohol Abuse</h5>

Checking for signs of alcoholism is often the first step. The doctor may check blood alcohol levels. At times, a liver function test may be needed to screen for liver damage - a common sign of alcoholism. Moreover, elevated liver enzymes may also point to liver damage.

The physical examination will assess the:

<ul>
	<li>Heart rate</li>
	<li>Eye movement</li>
	<li>Reflexes</li>
	<li>Blood pressure</li>
	<li>Body temperature 
	&nbsp;</li>
</ul>

<h5>Signs Of Malnutrition</h5>

To assess for signs of a deficiency, the doctor may order blood tests that measure thiamine (vitamin B1) &nbsp;levels and the patient&rsquo;s overall nutritional health. The following nutritional tests can help check for malnutrition:

<ul>
	<li>Serum Albumin Test - This test evaluates blood albumin levels. Albumin is a protein whose low levels can signify nutritional deficiencies, as well as liver or kidney problems.</li>
	<li>Serum Vitamin B1 Test - This test measures the blood&rsquo;s vitamin B-1 levels. The activity of enzymes in red blood cells (RBCs) can be measured. Low enzyme activity in RBCs indicates a vitamin B-1 deficiency. 
	&nbsp;</li>
</ul>

<h5>Other Tests</h5>

The doctors may also order imaging tests to help detect any WKS-related damage. WKS diagnostic imaging tests include the following:

<ul>
	<li>Electrocardiogram (ECG) - This is conducted before and after taking vitamin B-1. It helps the doctor detect any irregularities.</li>
	<li>CT scan - Any WKS-related brain lesions can be detected.</li>
	<li>MRI scan - This helps look for brain changes related to WKS.</li>
</ul>

Moreover, doctors may determine the severity of any mental problems with the help of a neuropsychological exam. 
&nbsp;

<h5>Differential Diagnosis</h5>

Following are the differential diagnosis of Wernicke- Korsakoff syndrome:

<ul>
	<li>Anoxic encephalopathy</li>
	<li><a href="https://merisehat.pk/disease/alzheimers-disease">Alzheimer&#39;s disease</a></li>
	<li>Temporal lobe <a href="https://merisehat.pk/disease/epilepsy">epilepsy</a></li>
	<li>Concussive head injury</li>
	<li><a href="https://merisehat.pk/disease/dementia">Dementia </a>with Lewy bodies</li>
	<li>Herpes simplex virus</li>
	<li>Temporal lobe infarction</li>
	<li>Transient global amnesia</li>
	<li>Third ventricle tumor 
	&nbsp;</li>
</ul>

Treatment

Even with extensive therapy, it is unusual for a person with Wernicke-Korsakoff syndrome to recover completely. It generally takes 5 to 12 days, after thiamine therapy, for the symptoms of encephalopathy to improve. The patient is given oral thiamine and referred to a rehabilitation center for treatment of other concomitant disorders. Moreover, the majority of Wernicke-Korsakoff syndrome patients require long-term care in a chronic care facility. Approximately 25% of patients require long-term institutionalization<a href="#accordion">[2]</a>.

The doctor will closely monitor the patient&rsquo;s health, particularly alcohol usage. Recovery from alcohol addiction can take months or years.

The treatment for WKS may include:

<ul>
	<li>Oral or intravenous vitamin B-1</li>
	<li>Balanced diet to keep vitamin B-1 levels up</li>
	<li>Treatment for alcoholism</li>
	<li>IV fluid rehydration&nbsp;</li>
	<li>Physical therapy</li>
	<li>Electrolyte supplementation 
	&nbsp;</li>
</ul>

In alcoholics, vitamin B-1 deficiency treatment may produce negative reactions. These can range from mild to severe and might include alcohol withdrawal symptoms, such as sleeplessness, sweating, or mood changes. Hallucinations, confusion, or anxiety may also occur. 
&nbsp;

Korsakoff syndrome is usually irreversible. In severe situations, it can cause brain damage and persistent issues with memory and gait. Death can also occur from irreversible brain damage, a lung infection, or blood poisoning. 
&nbsp;

Malnutrition and a history of alcoholism are the main risk factors for WKS. Other risk factors include:

<ul>
	<li>An inability to afford proper food and medical care</li>
	<li>Kidney dialysis - It lowers the absorption of vitamin B-1</li>
	<li>AIDS - It increases the risk of diseases that cause vitamin B-1 deficiency</li>
	<li>Recent bariatric surgery - Patients are required to limit their portion size and this may affect nutrient absorption</li>
	<li>Eating disorders, such as anorexia nervosa and bulimia</li>
	<li>Severe and persistent vomiting 
	&nbsp;</li>
</ul>

By quitting alcohol and adopting a healthy diet high in vitamin B1, one can prevent Wernicke-Korsakoff Syndrome.&nbsp;

The following foods are high in thiamine:

<ul>
	<li>Rice</li>
	<li>Peas</li>
	<li>Whole wheat bread</li>
	<li>Spinach</li>
	<li>Oranges</li>
	<li>Milk 
	&nbsp;</li>
</ul>

Key Points

<ul>
	<li>Wernicke-Korsakoff syndrome is a neurological condition caused by a deficiency of vitamin B1 (thiamine). The syndrome constitutes two illnesses - Wernicke encephalopathy and Korsakoff syndrome - that can occur at the same time. Wernicke encephalopathy (WE) is a life-threatening condition that needs immediate medical attention to avert death and neurologic damage. Korsakoff syndrome (KS) is a persistent neurologic disease that generally develops as a result of WE.</li>
	<li>Korsakoff syndrome affects around half of the people who acquire Wernicke encephalopathy. Those with an alcohol use disorder have a higher chance, about 80%, of acquiring WKS.</li>
	<li>The worldwide prevalence of Wernicke- Korsakoff syndrome ranges between 1-2% in the general population and 12-14% in alcoholics. Homeless people, elderly people who live alone or in isolation, and psychiatric inpatients also have higher occurrence rates.</li>
	<li>Thiamine is a vitamin that is needed by every cell in the body, but since humans cannot make it, all supplies of the vitamin must come from food. The need for thiamine in the liver, kidney, heart, and brain is especially high. The body is unable to carry out fundamental and essential cell processes when thiamine levels are insufficient.</li>
	<li>People with Korsakoff Syndrome (KS) frequently struggle to acquire new information. The person may also mistakenly invent facts to fill holes in their memory. Confabulation is the medical term for this condition.</li>
	<li>Chronic alcohol misuse is the most prevalent social component linked with Wernicke-Korsakoff syndrome, resulting in reduced thiamine absorption and usage. However, nonalcoholic causes of thiamine deficiency can also result in Wernicke-Korsakoff syndrome.</li>
	<li>Wernicke- Korsakoff Syndrome identification is critical for effective therapy. A physical exam is performed by healthcare practitioners to diagnose Wernicke-Korsakoff syndrome. They will also inquire about the patient&rsquo;s symptoms and family history.</li>
	<li>Even with extensive therapy, it is unusual for a person with Wernicke-Korsakoff syndrome to recover completely. The symptoms of encephalopathy will improve in 5 to 12 days after receiving thiamine therapy. The patient should be given oral thiamine and referred to a rehabilitation center for treatment of other concomitant disorders. The majority of Wernicke-Korsakoff syndrome patients require long-term care in a chronic care facility.</li>
	<li>Korsakoff syndrome is usually irreversible. In severe situations, it can cause brain damage and persistent issues with memory and walking. 
	&nbsp;</li>
</ul>

[1] Wernicke encephalopathy - UpToDate, December 6, 2022.

[2] StatPearls. Wernicke-Korsakoff syndrome. StatPearls.

[3] Arts NJ, Walvoort SJ, Kessels RP. Korsakoff's syndrome: a critical review. Neuropsychiatr Dis Treat. 2017;13:2875-2890. Published 2017 Nov 27. doi:10.2147/NDT.S130078.

[4] Harper C, Fornes P, Duyckaerts C, Lecomte D, Hauw JJ. An international perspective on the prevalence of the Wernicke-Korsakoff syndrome. Metab Brain Dis. 1995;10(1):17-24. doi:10.1007/BF01991779.