What is Wernicke-Korsakoff’s Syndrome?

Wernicke-Korsakoff’s Syndrome is caused by malnutrition, specifically a lack of Vitamin B1. It primarily affects people who are dependent on alcohol, but can be associated with chronic malnutrition due to eating disorders, gastric bands and AIDS, amongst others. For a list of other causes and risks, see below.

Wernicke’s Encephalopathy and Korsakoff’s Syndrome are two separate conditions, but they are often referred to together as Wernicke-Korsakoff’s Syndrome.

There is little doubt that Wernicke’s Encephalopathy can lead to Korsakoff’s Syndrome if not treated. It seems however that some people develop Korsakoff’s Syndrome independently without having an episode of Wernicke’s Encephalopathy.

Below we outline the symptoms of each separate condition:

What is Wernicke’s Encephalopathy?

Wernicke’s Encephalopathy (WE) is a type of fast-onset brain injury that classically causes three main problems:

  • Eye/vision issues
  • Confusion
  • Difficulty walking

It is caused specifically by a deficiency of thiamine.

Some believe that Wernicke’s Encephalopathy is the initial, onset phase and Korsakoff syndrome is the chronic, long-term state. Wernicke’s Encephalopathy often leads to Korsakoff Syndrome even after treatment. When not recognized or treated early, it can lead to permanent brain damage and even death.

In industrialized countries 90% of the cases of deficiency are associated with alcohol misuse (Thomson, 2000).
Failure to diagnose WE and institute adequate parenteral therapy results in death in 20% of patients; 75% will be left with permanent brain damage involving severe short-term memory loss [Korsakoff’s Psychosis (KP)]. Because of the close relationship between WE and KP, reference is often made to the Wernicke–Korsakoff Syndrome (WKS) as if it were a single entity.
Twenty five percent of patients with KP will be sufficiently affected to require long-term institutionalization. There has been a disturbing increase in KP in the UK over recent years (…) (Ramayya and Jauhar, 1997; Cook et al., 1998).
The Natural History and Pathophysiology of Wernicke’s Encephalopathy and Korsakoff’s Psychosis -Allan D. Thomson and E. Jane Marshall

Symptoms of Wernicke’s Encephalopathy

When thiamine is not present in high enough amounts, the body cannot perform the basic and necessary tasks of cell function and problems begin. Brain cells are particularly sensitive to low levels of thiamine because it is required in many cellular pathways needed for normal brain activity.

Symptoms of Wernicke’s encephalopathy can include:

  • disorientation, confusion or mild memory loss
  • nystagmus (involuntary, jerky eye movements or paralysis of the muscles that move the eyes)
  • poor balance or unsteadiness, or other signs of damage to a part of the brain called the cerebellum (a region involved in co-ordinating movement).

The person may also (but not necessarily) appear underweight or malnourished.

Is Wernicke’s Encephalopathy treatable?

Yes. If Wernicke’s encephalopathy is suspected, immediate medical treatment is vital. The person will need high doses of thiamine (and other B vitamins) injected slowly via a drip into a vein (usually Pabrinex). This treatment usually takes place in hospital. If treatment is done in time, most symptoms will be reversed in a few days. However, permanent brain damage may result if Wernicke’s encephalopathy is left untreated or is not treated properly or not treated in time. In some severe cases the person may die.

An accurate and timely diagnosis of Wernicke’s Encephalopathy is sadly not always made. A person who is dependent on alcohol may self-discharge or otherwise resist treatment. Because the symptoms of WE can include confusion and an inability to walk straight or look straight, a person may, for example, be misidentified as intoxicated rather than being in the middle of suffering a significant brain injury. If that person has turned up at A&E drunk on many occasions, the staff may automatically assume that he/she is drunk again and may send him/her away to sober up.  New guidelines issued by NICE in the last decade have improved the likelihood of accurate diagnosis in A&E but we still hear about cases where people have not been diagnosed before significant brain damage has already occurred, or worse.

If left untreated, it can progress to coma and then death.

Korsakoff’s syndrome

As mentioned, Wernicke’s Encephalopathy is often the condition that occurs before Korsakoff’s Syndrome develops. As Korsakoff’s Syndrome symptoms increase, Wernicke’s encephalopathy symptoms tend to subside. If Wernicke’s encephalopathy is successfully treated with thiamine intravenously, Korsakoff’s Syndrome may not develop at all.

Korsakoff’s syndrome (KS) is characterized by memory loss and problems carrying out and managing day-to-day functions. There is often difficulty learning new information. The person may also unintentionally make up information that bridges the gaps in their memory- this is called confabulation. (Find out more about that here: Confabulation)

Confabulation has been described as the formation of “fictitious memories.” It is not a deliberate deception, the individual’s brain is attempting to fill memory gaps subconsciously.

Problems with short-term memory can lead to difficulty in making new memories and recalling recent events. An individual with KS may undergo personality changes, showing either apathy and a lack of concern, or displaying talkative and repetitive behavior. KS may gradually improve over time, but it is estimated that in about 25 percent of cases the condition is permanent.

Causes and risks

Scientists don’t yet know exactly how Korsakoff’s syndrome damages the brain. Research has shown that severe thiamine deficiency disrupts several biochemicals that play key roles in carrying signals among brain cells and in storing and retrieving memories. These disruptions destroy brain cells and cause widespread microscopic bleeding and scar tissue. Most cases of Korsakoff’s Syndrome result from alcohol misuse. Scientists don’t yet know why heavy drinking causes severe thiamine deficiency in some alcoholics, while others may be affected primarily by alcohol’s effects on the liver, stomach, heart, intestines or other body systems.

Researchers have identified several genetic variations that may increase susceptibility to Korsakoff syndrome. Poor nutrition also may raise risk.

Korsakoff syndrome also can be caused by anorexia, overly stringent dieting, fasting, starvation or weight-loss surgery; uncontrolled vomiting; AIDS; kidney dialysis; chronic infection; or cancer that has spread throughout the body.

Wernicke-Korsakoff syndrome is most common among people with alcohol addiction. Thiamine deficiency is a common consequence of alcoholism.

Individuals whose bodies do not absorb nutrients properly, who are malnourished, or who fast for a long time may experience thiamine deficiency as well.

Heavy drinking is often accompanied by poor diet, but alcohol also interferes with the proper absorption of nutrients from the digestive system. Thiamine is needed by the body to convert food into energy. It is stored in small quantities in the liver, but only for up to 18 days.

WE can affect people who do not consume alcohol, but who have other problems with absorption or intake of vitamin B1; for instance, individuals who have:

  • recently undergone surgery for obesity – portion size and, consequently, nutrient supply is limited
  • kidney dialysis
  • hyperemesis – severe and persistent vomiting
  • anorexia – and other eating disorders
  • severe diets or fasting
  • AIDS
  • chronic infection
  • cancer that has spread throughout the body

How common is Korsakoff’s Syndrome?

The exact prevalence of WKS is unknown. Autopsies have found brain lesions matching symptoms of WKS in 0.4-2.8 percent of the general population in Western countries, including the United States.

Other figures suggest a prevalence of 1-2 percent in the general population, and 12-14 percent in those who consume large amounts of alcohol.

Left untreated, Wernicke-Korsakoff syndrome is fatal. Death can occur due to coma, complications of dementia, lung infections, irreversible brain damage, and wide-spread infections.

Can a person with Korsakoff’s Syndrome get better?

That depends on many things. In our experience, the earlier a person is diagnosed, and the earlier a person gets the right kind of support, the better the long-term outcome.
The Upstreet Project team is committed to providing outstanding support, care and rehabilitation facilities for adults with a primary diagnosis of Korsakoff’s Syndrome, hepatic encephalopathy, or other forms of dementia or brain damage resulting from long term alcohol or substance misuse. We also support individuals with related mental and physical health conditions. We achieve this through an holistic social care model, encouraging and enabling physical activity, mental stimulation and life skills development in order to empower our clients to make healthier choices for their future.

If you would like to know more about how we might be able to support you, or your relative or friend, please click here: How can we help?