Korsakoff Syndrome

What is Korsakoff Syndrome?

Thiamine (vitamin B1) helps brain cells produce energy from sugar ¹. However, when B1 levels fall too low, brain cells can't generate enough energy to function properly. That can result in a condition known as Korsakoff syndrome.

Korsakoff syndrome is most commonly caused by alcohol misuse. It can also be associated with AIDS, cancers that have spread throughout the body, chronic illnesses and infections, poor nutrition, and other related conditions ².

For example, people who can’t absorb food properly (malabsorption) or patients who have undergone bariatric weight-loss surgery may also develop Korsakoff syndrome.

It is characterized by an inability to learn new information or remember recent events. It can also produce long-term memory gaps, as well.

Memory gaps can be significant, but other thinking and social skills may appear to be unaffected. For example, a person with Korsakoff syndrome may be able to carry on a clear and coherent conversation.

Still, moments later, they may not be able to recall the conversation or even who they spoke with.

People with Korsakoff syndrome may also resort to making up things they can’t remember. Known as “confabulation,” it is not considered lying because sufferers may believe their manufactured explanations. Another side effect is that people may hallucinate, seeing, or hearing things that aren't real.

Chronic memory loss from Korsakoff syndrome often follows an episode of Wernicke encephalopathy (also known as Wernicke’s disease or WD). As a result, it is commonly referred to as Wernicke-Korsakoff syndrome (WKS).

Some medical professionals believe that they are different stages of the same disorder. Wernicke's disease is considered the acute phase with a shorter duration and more severe symptoms.

Korsakoff syndrome is regarded as the chronic phase and is a long-lasting condition.

When combined, these conditions may also be referred to as:

• Alcoholic Encephalopathy 
• Gayet-Wernicke Syndrome
• Korsakoff Amnesic Syndrome
• Korsakoff Psychosis
• Korsakoff Syndrome
• Psychosis Polyneurotica
• Thiamine deficiency - Wernicke
• Wernicke Disease
• Wernicke Encephalopathy
• Wernicke Syndrome

WD is characterized by a confused mental state (including lethargy, indifference, and in some cases, delirium), an inability to coordinate voluntary movement (ataxia), and eye abnormalities.

Affected individuals may not display all three symptoms.

Wernicke’s disease causes damage to the thalamus and hypothalamus located in the lower parts of the brain.

Signs & Symptoms of Korsakoff Syndrome 

An episode of Wernicke's disease often precedes Korsakoff syndrome. WD is an acute brain reaction to a severe lack of thiamine. WD is a medical emergency that requires immediate attention and is characterized by confusion, lack of coordination, staggering, stumbling, and abnormal involuntary eye movements.

About 80-90% of people with Wernicke syndrome develop Korsakoff syndrome ³.

Korsakoff syndrome symptoms often develop as the mental symptoms of Wernicke syndrome begin to lessen. The telltale sign of a switch from one to the other is the loss of short-term memory or the inability to learn new things.

A severe deficiency of thiamine can cause damage to the thalamus and hypothalamus parts of the brain, which are parts of the limbic system.

The limbic system is a crucial part of a person's emotions and memory. Lack of thiamine can also cause brain lesions and overall cerebral atrophy as well.

While WD is considered an acute medical condition, Korsakoff syndrome produces several long-term signs and symptoms that develop over time.

The combined symptoms of Wernicke’s disease and Korsakoff syndrome (WKS) are evident in many ways. They include:

• Double vision
• A drooping upper eyelid (ptosis)
• Involuntary up-and-down and side-to-side eye movements
• Paralysis of some eye muscles (ophthalmoplegia)
• Loss of muscle coordination that can interfere with walking (ataxia). Advanced ataxia may prevent an affected individual from standing or walking without assistance.
• Apathy and indifference
• Peripheral neuropathy can cause weakness of the arms and legs and contribute to difficulty walking. 
• Many nerves affected throughout the body (polyneuropathy)
• Cardiovascular abnormalities including a rapid heartbeat (tachycardia), low blood pressure upon standing (postural hypotension), and loss of consciousness (syncope)
• Drowsiness and fainting due to cardiovascular issues
• Alcohol-related dementia
• Impaired executive functions (cognitive control of behavior that includes planning and fluid intelligence, such as reasoning and problem-solving. However, IQ is not affected.
• A confused mental state. That can lead to bouts of anger, violent behavior, and combativeness.
• Memory loss in several different ways:
  
  
  • amnesia for events that happen after the onset of the disorder (anterograde amnesia)
  • memory loss for events that took place at times long before the onset of WKS (retrograde amnesia)
  • amnesia fixation which involves the immediate loss of memory for events that happened even just a few minutes prior
  • difficulty understanding the meaning of information
  • difficulty putting words into context
  • hallucinations
  • exaggerated storytelling to rationalize gaps in memory that are believed by the sufferer to be true (confabulation)
  • false memories
  • the inability to form new memories
  • lack of insight
  • limited ability to carry on a conversation
  • false recognition of places
  • euphoria
  • deficits in declarative memory, but continued function in verbal, procedural and implicit spacial memory

Acute treatment for WKS involves administering thiamine either intravenously or by mouth as well as maintaining a balanced diet.

Unfortunately, in a small number of cases, treating thiamine deficiency can produce an adverse reaction. Reactions can vary but may include symptoms consistent with alcohol withdrawal.

That might result in mood swings, sweating, insomnia, agitation, confusion, or hallucinations.

More advanced cases of WKS can lead to serious complications, including:

• Permanent loss of thinking skills
• Permanent memory loss
• Difficulty with social and personal interactions
• Severe alcohol withdrawal 
• Injuries due to falls caused by lack of coordination
• A shortened lifespan

Causes of Korsakoff Syndrome 

Scientists don't know precisely how Korsakoff syndrome damages a person's brain or why some people develop the condition, and others do not.

Prevalent theories suggest that severe vitamin B1 thiamine deficiency disrupts key biochemicals that carry signals between brain cells and in storing and retrieving memories.

The disruptions can destroy brain cells and cause microscopic bleeding and scar tissues throughout the brain.

Alcoholism and poor nutrition often go hand-in-hand. Alcoholism also produces inflammation in the stomach lining, which can also cause a thiamine deficiency.

There are several genetic variations that may increase the likelihood of developing Korsakoff syndrome. Poor nutrition is also a risk factor.

WKS can also be associated with anorexia, stringent dieting, chronic stomach conditions (gastropathies), fasting, or weight loss surgery.

Uncontrolled vomiting, AIDS, kidney dialysis, chronic infections, and widespread cancers are also linked to WKS. Gastric and colon cancers have also been linked to WKS as well. 

Pregnant women with extreme morning sickness also are at greater risk for WKS as well.

Although it is rare, structural lesions of the central nervous system can also contribute to symptoms of WKS. 

Studies suggest that about 25% of those who develop Korsakoff syndrome eventually recover, about half improve but don’t recover completely, and about 25% remain unchanged ⁴.

Lack of coordination may begin to improve after about a week but may take several months to clear up completely. Mental confusion also takes several months to clear up.

Men get WKS slightly more often than women. Overall, WKS typically happens in people ages 45-65 ⁵. It is also more common in people who are homeless, older adults living alone, and people with serious mental health issues because these groups are more prone to abusing alcohol or not eating correctly.

How Common is Korsakoff Syndrome? 

Because it can be challenging to diagnose at times, it's difficult to say precisely how prevalent Korsakoff syndrome is among the general population.

Best estimates are that it occurs in 1-2% of the general population in the United States ⁶, but this number may not be accurate due to being underdiagnosed.

Among heavy drinkers, it’s estimated that one out of every eight people will be affected by Korsakoff syndrome at some point. WKS affects males slightly more often than females.

Sufferers are evenly distributed between the ages of 30 and 70 ⁷. Other studies indicated that it might more commonly affect men over age 45 with a long history of alcohol abuse.

Anyone who drinks heavily over a long period is at risk, but researchers aren't able to determine why some people develop WKS, and others do not. There are some indications that lifestyle and diet may play a role.

Diagnosis of Korsakoff Syndrome 

Diagnosing Korsakoff syndrome takes place in several different ways. However, because a person with the condition may be mentally confused, communication with a doctor can be difficult, making an accurate diagnosis more challenging.

One of the first things a doctor will do is check for signs of alcoholism, including a check of your blood alcohol levels.

Because liver damage is a common sign of alcoholism, your doctor may also perform a liver function test to see if this organ is damaged.

Your liver enzymes are checked because alcoholism produces elevated levels of these highly specialized and complex proteins that aid in chemical changes throughout your body.

A doctor will also check for a fast heart rate, unusual eye movements, low blood pressure, low body temperature, and reflexes for abnormalities ⁸. You will also be examined for muscle weakness and atrophy, as well as any problems walking or with general coordination.

Additionally, a doctor will also look for clinical signs that indicate a thiamine deficiency. Blood tests will look for signs of malnourishment, and will include:

• Serum albumin test to measure levels of albumin. That is a protein in the blood, and low levels may be an indication of a nutritional deficiency or kidney or liver problems.
• Serum vitamin B1 test to check levels of thiamine in your blood. Low enzyme activity in red blood cells indicates a vitamin B1 deficiency.
• Transketolase activity in red blood cells. It is reduced in people with a thiamine deficiency.

In some cases, a doctor will look for damage that’s characteristic of Korsakoff syndrome through the diagnostic imaging tests. These tests may include:

• An EKG can help your doctor find abnormalities before and after taking B-1
• A CT scan will check for WD-related brain lesions
• An MRI scan will check for WD-related brain changes

These tests may be augmented by a neuropsychological test to determine the severity of any mental deficiencies.

As part of the diagnosis process, your medical history will also be taken into consideration. Several conditions can cause a B1 deficiency, including:

• Cancers that have metastasized throughout the body
• HIV/AIDS
• Extreme nausea and vomiting during pregnancy
• Heart failure that has been treated with long-term diuretic therapy
• Long periods of intravenous therapy without receiving thiamine supplements
• Long-term dialysis
• Very high thyroid hormone levels

Despite all of these procedures, there is no specific lab test or neuroimaging procedures that can confirm a person has Korsakoff syndrome.

Diagnosis is based on a doctor’s best clinical judgment. Part of the diagnosis also includes ruling out other disorders with similar symptoms. This can range from infections to head injuries or even tumors, internal bleeding, and other similar maladies.

Korsakoff Syndrome Treatment & Management 

Treating WKS depends on how severe your symptoms are. The first step in all cases is to get plenty of vitamin B1 introduced into your diet.

You may have to put B1 into your body through an IV or directly into your muscles for several months. It depends on the severity of your condition.

In the short-term, introducing thiamine these ways three times daily for 2–3 days can produce the start of an effective and positive response. 

The good news is that many mental and confusion issues, vision abnormalities, and ataxia all usually improve when thiamine is introduced.

Some people may benefit from psychotherapy to help manage associated mental and emotional disorders. 

The other key is to steer clear of all alcohol. That can be a tough demon to tame and will require a lifetime of support.

Wernicke’s disease can often be reversed, but Korsakoff syndrome typically can’t be. When you are diagnosed early and begin treatment immediately, you can make a full recovery, although it could take up to a full year.

The length of the recovery will depend on how early you started treatment and how diligent you are in following your treatment protocol.

Those with Korsakoff syndrome have a reduced tolerance for alcohol. That puts them at a potentially higher risk for more alcohol-related health problems.

If symptoms don't diminish over time, the only option might be some form of long-term care in an assisted living facility.

To prevent WKS, avoid alcohol and eat a balanced diet rich in B-1. High levels of thiamine are found in several types of food, including lean pork, peas, rice, spinach, oranges, milk, firm tofu, and salmon, among many others.

Here are some additional foods you might want to consider that contain high levels of B1:

Top 10 Foods Highest in Thiamin (Vitamin B1)

20 Vitamin B1 Foods You Should Include in Your Diet

The World’s Healthiest Foods – Vitamin B1 

Prognosis of Korsakoff Syndrome

Make no mistake about it, if a person drinks heavily and suffers from poor nutrition, the development of Wernicke-Korsakoff syndrome can eventually kill them.

The prognosis for someone with WKS depends heavily on how soon treatment begins and how much brain damage, if any, has been done.

The effects of WKS can be stopped if a person stops drinking alcohol, switches to a healthy diet, and starts taking vitamin supplements. When thiamine is introduced, it can help prevent further brain damage from taking place.

Improvements generally take place over two years when these changes are introduced. But some symptoms, such as memory loss and thinking skills, may be permanent ⁹.

Unfortunately, there are some cases where WKS is too far advanced, and no recovery takes place, leading to long-term care.

Otherwise, when changes are made, people with WKS are reported to have an average life expectancy.

What to do Next: Living with Korsakoff's Syndrome 

If treated early enough, long-term effects from Korsakoff syndrome may be non-existent going forward. But in other cases, damage can be permanent or even life-threatening.

The best thing to do is to stay vigilant, especially if a friend or family member exhibits signs and symptoms of this disorder. Due to diminished mental capacity, they may need help to get an immediate treatment that minimizes health risks.

There are also several possible resources to turn to for more information and help in understanding all aspects of Korsakoff syndrome. These include:

Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Phone: (301) 251-4925

Toll-free: (888) 205-2311

Website: http://rarediseases.info.nih.gov/GARD/

Mental Health America

2000 N. Beauregard Street, 6th Floor

Alexandria, VA 22314-2971 USA

Phone: (703) 684-7722

Toll-free: (800) 969-6642

Email: [email protected]

Website: http://www.mentalhealthamerica.net/

Family Caregiver Alliance
National Center on Caregiving
(415) 434-3388 | (800) 445-8106
Website: www.caregiver.org
Email: [email protected]
FCA CareJourney: www.caregiver.org/carejourney
Family Care Navigator: www.caregiver.org/family-care-navigator

NIH/Institute on Alcohol Abuse and Alcoholism

5635 Fishers Lane

MSC 9304

Bethesda, MD 20892-9304

Phone: (301) 443-3860

Email: [email protected]

Website: http://www.niaaa.nih.gov/

NIH/National Institute of Diabetes, Digestive & Kidney Diseases

Office of Communications & Public Liaison

Bldg 31, Rm 9A06

Bethesda, MD 20892-2560

Phone: (301) 496-3583

Email: [email protected]

Website: http://www2.niddk.nih.gov/

NIH/National Institute of Mental Health

Health Science Writing, Press and Dissemination Branch

6001 Executive Boulevard

Bethesda, MD 20892-9663

Phone: (301) 443-4513

Toll-free: (866) 615-6464

Email: [email protected]

Website: http://www.nimh.nih.gov/index.shtml

Substance Abuse and Mental Health Services Administration

U.S. Dept of Health and Human Serv

1 Choke Cherry Road

Rockville, MD 20857

Phone: (240) 276-2000

Toll-free: (877) 726-4727

Email: [email protected]

Website: http://www.samhsa.gov/

Alcohol.org

866-233-1131