Wernicke-Korsakoff Syndrome

Wernicke-Korsakoff syndrome (WKS) is a type of brain disorder caused by lack of vitamin B1. The syndrome is actually two separate conditions that can occur at the same time. Usually you’ll experience symptoms of Wernicke’s encephalopathy first. Also called Wernicke’s disease, people with Wernicke’s encephalopathy have bleeding in the lower sections of the brain, including the thalamus and hypothalamus. These areas of the brain control the nervous and endocrine systems. The bleeding causes brain damage that presents itself through symptoms involving vision, coordination, and balance.
 
Signs of Korsakoff psychosis tend to follow as the Wernicke’s symptoms wane. If Wernicke’s disease is treated quickly and effectively, Korsakoff syndrome may not develop. Korsakoff psychosis is the result of chronic brain damage, and affects the areas of your brain that control memory.

Risk Factors

Risk factors for WKS are related to a patient’s diet and lifestyle. The major risk factors for developing WKS are malnourishment and chronic alcoholism. Other risk factors for WKS include:
 
- being unable to afford medical care and proper food
- undergoing kidney dialysis, which reduces vitamin B1 absorption
- acquired immunodeficiency syndrome (AIDS), which makes you more likely to develop conditions that lead to vitamin B1 shortage

Causes

The number one cause of WKS is alcoholism. Less common causes are conditions that limit nutritional absorption. Conditions that restrict eating and nutrient absorption include:
 
- gastric bypass surgery - It is difficult for nutritional needs to be met on small food portions.
- colon cancer - The pain related to this cancer may cause an individual to put off eating.
- eating disorders (anorexia, bulimia, etc.)
 
Alcoholism is the number one cause of WKS because alcoholics generally have a poor diet. Alcohol also prevents vitamin B1 absorption and storage.

Symptoms

Lesions on the brain cause Wernicke’s disease (WD). These lesions are the result of a vitamin B1 deficiency. Prominent symptoms of WD are double vision, a drooping upper eyelid, up and down or side-to-side eye movements, loss of muscle coordination and confused mental state.
 
WD can later develop into Korsakoff syndrome. Patients who have WKS have a variety of issues relating to memory. You may suffer from memory loss or be unable to form new memories. Wernicke’s patients may also exhibit the following symptoms: amnesia for events that happen after the onset of the disorder, difficulty understanding the meaning of information, difficulty putting words into context, hallucinations and exaggerated storytelling (confabulation).

Diagnosing

Diagnosing WKS is not always easy. An individual with WKS is often mentally confused. This can make patient and doctor communication difficult, and doctors may overlook the possibility of a physical disorder when dealing with a confused patient.
The physician may first check for signs of alcoholism, check an individual’s blood alcohol levels. Sometimes, a they will take a liver function test to check for liver damage, which is a common sign of alcoholism.
Axial MRI FLAIR image showing hyperintense signal in the mesial dorsal thalami, a common finding in Wernicke encephalopathy. This patient was nearly in coma when IV thiamine was started, he responded moderately well but was left with some Korsakoff type deficits.
Axial MRI FLAIR image showing hyperintense signal in the
 mesial dorsal thalami, a common finding in Wernicke
encephalopathy. This patient was nearly in coma when IV
vitamin B1 was started, he responded moderately well but
was left with some Korsakoff type deficits. (Image source)
The individual may also appear to be malnourished. Therefore, the doctor may also order tests to check nutritional levels. Nutritional tests may include:

- serum albumin test: a blood test that measures the levels of albumin (a protein) in the blood. Low levels may signal nutritional deficiencies as well as kidney or liver problems.
- serum vitamin B1 test: a blood test to check vitamin B1 levels in the blood
- Transketolase (enzyme) activity in the red blood cells: Low enzyme activity signals a vitamin B1 deficiency.
 
Sometimes, the doctor will perform imaging tests. These tests can find damage that is characteristic of WKS. Diagnostic imaging tests for WKS include:
 
- electrocardiograph (ECG), which looks for abnormalities before and after giving vitamin B1
- computed tomography (CT) scan to check for brain lesions related to WD
- magnetic resonance imaging (MRI) scan to look for brain changes related to WD
 
Physicians may also use neuropsychological tests to judge the severity of mental deficiencies.

Treatment 

WKS treatment should begin immediately. Prompt treatment may delay or stop disease progression, and are also able to reverse non-permanent brain abnormalities. Treatment may first involve hospitalization where the patient will be monitored to ensure that his or her digestive system is absorbing food properly. The doctor may also administer vitamin B1 to the patient through an IV. Treatment for WKS may include:
 
- intravenous vitamin B1 (vitamin B1 given through an IV in the arm or hand)
- oral vitamin B1 (for continued maintenance after initial treatment)
- balanced diet (to keep vitamin B1 levels up)
- treatment for alcoholism (help with withdrawal, support in quitting, etc.)
 
In a small number of cases, treatment of vitamin B1 deficiency produces a negative reaction. This is more common in alcoholics. Negative reactions to receiving vitamin B1 vary, but may include alcohol withdrawal symptoms like insomnia, sweating, or mood swings. The patient may also experience hallucinations, confusion, and/or agitation.

Outlook

The outlook for WKS is based on how far the disease has advanced. Receiving early treatment before irreversible damage has occurred dramatically improves patient outlook. Mortality rates are high if WKS is left untreated. Most deaths are the result of a lung infection, blood poisoning (septicemia), or irreversible brain damage. Those who receive fast treatment can see progress in:
 
- eye problems (begin to improve in hours to days)
- muscle coordination (improves in days to weeks, but some damage may remain)
- confusion (improves in days to weeks)
 
To continue recovery of memory and mental function, patients must abstain from alcohol. They also need to eat a balanced diet to prevent future vitamin B1 deficiencies. Vitamin B1-rich foods include lean pork, rice, peas, whole wheat bread, spinach, oranges, and milk.
 
For further information on Wernicke-Korsakoff syndrome, please contact National Organization for Rare Disorders or National Institute on Alcohol and Alcohol Abuse.