Wernicke-Korsakoff syndrome is a neurodegenerative disorder. It is the result of a deficiency of vitamin B-1, also known as thiamine.
A common cause of Wernicke-Korsakoff syndrome (WKS) is the heavy, regular consumption of alcohol over an extended period, but stomach surgery, severe intestinal problems, cancer, and malnutrition can also lead to its development.
Researchers disagree on whether WKS consists of two separate but related disorders, or if its symptoms are the spectrum of a single disorder.
Some believe Wernicke’s to be the initial, onset phase and Korsakoff syndrome to be the chronic, long-term state. Wernicke’s encephalopathy often leads to Korsakoff syndrome even after treatment.
Various difficulties in thought processing and changes in the nervous system characterize WKS. Without early treatment, it can lead to permanent brain damage and death.
In this article, we look at the symptoms, causes, and treatment of WKS.
Most people with WKS first develop Wernicke’s encephalopathy first, and later develop Korsakoff syndrome. Below we outline the symptoms of each condition:
Wernicke’s encephalopathy (WE) is a type of brain injury that classically causes three main problems:
- vision problems
- difficulty walking
A deficiency of thiamine causes WE. Every cell in the body requires thiamine, and humans cannot produce it, so all sources of the vitamin must be dietary. The heart, brain, kidney, and liver require especially high levels of thiamine.
When thiamine is not available in high enough amounts, the body cannot perform basic and necessary cell functions. This leads to the health problems that constitute WE.
Brain cells are particularly sensitive to low levels of thiamine, which is why many of the symptoms of WE present as neurological.
An individual with WE may experience:
- altered mental status
- jerky or involuntary eye movements
- droopy upper eyelids
- double vision
- poor balance and difficulty walking
Often, individuals with WE may appear malnourished and underweight. They may also experience low blood pressure, heart problems, and low body temperature.
The symptoms of WE vary between people, which makes it difficult to diagnose. The coordination issues and physical effects of WE can also resemble those of alcohol intoxication, so heavy drinking may mask WE.
Without treatment, WE might progress to coma, and it can be fatal in some people.
Memory loss and problems managing day-to-day tasks are key features of Korsakoff syndrome (KS).
People with KS often experience difficulties in learning new information. The person may also unintentionally make up information that bridges the gaps in their memory. Doctors refer to this as confabulation.
Some people have described confabulation as forming false memories, but these do not form part of a deliberate deception on the part of the person with KS. The brain unconsciously attempts to fill gaps in memory.
Problems with short-term memory can lead problems with making new memories and recalling recent events. An individual with KS may undergo personality changes, showing apathy and a lack of concern or displaying talkative and repetitive behavior.
The symptoms of KS may gradually improve over time, but an estimated 25 percent of people with KS experience a permanent form of the condition.
WE and KS relate to each other in the following ways:
- WE often occurs before KS develops.
- As KS symptoms increase, WE symptoms tend to decrease.
- If a person successfully receives treatment for WE, KS may not develop.
WKS is most common among people with alcohol use disorder. Thiamine deficiency is a common effect of heavy, regular alcohol consumption.
Individuals whose bodies do not absorb nutrients efficiently, 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.
Other risk factors for thiamine deficiency include:
- recent surgery for obesity, as this may limit portion size and nutrient absorption
- kidney dialysis
- hyperemesis, or severe and persistent vomiting
- anorexia nervosa, bulimia, and other eating disorders
- extreme diets or fasting
- stage-3 HIV
- chronic infection
- cancer that has spread throughout the body
How common is WKS?
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.
If a doctor suspects Wernicke-Korsakoff, the person with symptoms requires intravenous (IV) thiamine. This will require hospitalization.
The medical team will also monitor and treat other symptoms and complications.
Thiamine supplementation is the most effective way to treat the common symptoms, such as vision problems, eye movements, difficulties with coordination, and confusion.
Memory and cognition problems are less likely to show signs of improvement and may require further management, but prompt thiamine treatment may prevent further deterioration.
Intravenous treatment usually continues until the physician observes no further improvements. Different hospitals may treat thiamine deficiency differently, because not enough data is available to support one particular dosing schedule.
After this, the individual with WKS will receive oral doses of thiamine as a regular supplement.
To prevent the progression of the syndrome, a person with WKS will have to abstain completely from alcohol and follow a well-balanced diet. If alcohol is not the cause, treatment for the underlying medical problem is necessary.
Studies have suggested that about 25 percent of people with Korsakoff syndrome completely recover, around half make a partial recovery, and around 25 percent remain the same.
Wernicke-Korsakoff syndrome is fatal without treatment.
To prevent Wernicke-Korsakoff syndrome, it is important to have a well-balanced, healthy diet, and not to abuse alcohol.
After leaving hospital, many patients will need support. Those who have problems with alcohol abuse often return to unstable living conditions and the possibility of drinking again, with an accompanying poor diet.
Anyone who has signs of a malabsorption problem should seek medical advice to prevent thiamine deficiency.
Foods rich in thiamine
Foods high in thiamine, or vitamin B-1, include:
- enriched rice
- soy beans
- lima beans
- yeast extract
- some seeds, including sunflower, chia, pumpkin, and squash
- nuts, including macadamia, pistachio, and brazil nuts
- whole grains
Other types of brain damage that are related to chronic alcohol consumption include cerebellar degeneration, alcohol-related brain damage, alcoholic dementia, stroke, and hepatic encephalopathy.
If patients present with other symptoms of alcohol abuse, the physician should consider Wernicke-Korsakoff syndrome as a possibility.
WKS is brain damage that occurs due to vitamin-B1 deficiency. Two medical issues make up WKS: Wernicke’s encephalopathy (WE) and Korsakoff syndrome (KS).
Scientists continue to debate whether they are two separate conditions or one syndrome with two distinct phases. The three main symptoms of WE are vision problems, problems with walking, and confusion.
In KS, people experience severe memory loss and difficulty with day-to-day tasks. They may create false memories to bridge gaps in real memory in a process known as confabulation.
WE occurs before KS, and when KS symptoms start, WE symptoms tend to slow down. If a person receives successful treatment for WE, they can prevent KS.
Regular, heavy alcohol use can lead to thiamin deficiency, as can people who absorb nutrients less efficiently and those who are malnourished. Thiamin supplementation and abstinence from alcohol are the ways to treat WKS.
A diet containing the recommended amount of thiamin can help prevent WKS. Foods such as lima beans, oranges, and fortified rice are plentiful sources.