Wernicke–Korsakoff Syndrome (WKS)


Published: 05 May 2022

Note: While Wernicke-Korsakoff syndrome has a variety of causes related to thiamine (vitamin B1) deficiency, this Article primarily focuses on alcohol as a causative factor.

Excessive alcohol consumption can be the cause of a raft of serious health issues, including liver disease, bowel cancer and addiction - in 2020 alone there were 1,452 alcohol-related deaths in Australia (AIHW 2022).

Among these, Wernicke-Korsakoff syndrome (WKS) - the composite term for the dual presence of Wernicke encephalopathy (WE) and alcoholic Korsakoff syndrome - is a form of alcohol-related brain injury associated with long-term alcoholic behaviour (Dementia Australia 2016).

Due to its similarity in symptoms to other conditions, such as dementia, WKS can often go unrecognised or under-treated and should be at the top of mind for healthcare professionals and nurses, particularly those working in aged care and drug and alcohol settings (Isenberg-Grzeda et al. 2012).

What is Wernicke-Korsakoff Syndrome?

WKS refers to the condition where both Wernicke encephalopathy (WE) and Korsakoff syndrome (KS) are present in the one individual.

WKS is most commonly referred to as an alcohol-related brain injury, developed as a result of long-term alcohol abuse leading to severe thiamine deficiency. However, it can also develop in a handful of other conditions that lead to a thiamine deficiency (Alcohol.Org Editorial Staff 2020).

WKS is usually preceded by a sudden and acute phase of WE, followed by the development of a chronic alcoholic KS phase (Dementia Australia 2016).

Prevalence of Wernicke-Korsakoff Syndrome

WKS most commonly affects males who are over the age of 45 years and who have a long history of alcohol abuse (Dementia Australia 2016).

It presents in roughly 3% of the general population in Australia (Akhouri et al. 2021).

Symptoms of Wernicke Encephalopathy

WE symptoms are categorised into the following clinical triad:

  1. Changes in mental state: confusion, lethargy, inattentiveness, drowsiness, indifference, delirium, loss of consciousness.
  2. Ataxia (the inability to coordinate voluntary movements): leg tremor, poor balance, staggering, inability to stand or walk, loss of muscle coordination, slow and unsteady gait.
  3. Ocular disturbances: jerky eye movement, paralysis of eye muscles, double vision, rapid involuntary eye movement, drooping of the upper eyelids (ptosis).

(NORD 2021; Dementia Australia 2016)

From here, approximately 80 to 90% of individuals develop KS (NORD 2021).

Symptoms of Korsakoff Syndrome:

KS mostly presents in memory impairment such as:

  • Memory loss
  • Inability to form new memories
  • Inability to learn new information
  • Personality changes
  • Confabulation (creation of false memories)
  • Hallucinations
  • Lack of insight into the condition.

(NORD 2021; Dementia Australia 2016)

Causes of Wernicke–Korsakoff Syndrome

While the most common cause of WKS is heavy and long-term alcohol abuse, it is important to note that not all heavy drinkers will develop WKS.

It's not currently known why some individuals will and some will not, however, genetics, as well as diet and other lifestyle factors, are suspected to be involved (NORD 2021; Dementia Australia 2016).

The main cause of WKS is a deficiency in thiamine (vitamin B1). Heavy alcohol consumption reduces the absorption and further creation of thiamine by the body and diminishes that which is stored in the liver (NORD 2021). When thiamine levels are low, brain and nerve cell damage can occur.

Thiamine molecular structure | Image
Thiamine (vitamin B1).

It's also important to note that alcohol consumption is not the only cause of thiamine deficiency. WKS can also develop in individuals who are experiencing a lack of thiamine due to other chronic conditions such as cancer or AIDS; prolonged treatments such as dialysis or IV therapy; or severe malnutrition, such as starvation (NORD 2021; Dementia Australia 2016).

Diagnosis of Wernicke-Korsakoff Syndrome

WKS diagnosis can be difficult there are a variety of conditions that present similarly, including:

  • Anoxic encephalopathy
  • Alzheimer's disease
  • Temporal lobe epilepsy
  • Concussive head injury
  • Dementia with Lewy bodies
  • Herpes simplex virus
  • Temporal lobe infarction
  • Transient global amnesia
  • Third ventricle tumour.

(Akhouri et al. 2022)

A diagnosis of WKS is made based on a holistic clinical evaluation involving the patient’s history of drinking, mental status examination and physical tests (Alcohol.Org Editorial Staff 2020).

Testing for WKS can include blood tests to rule out other disorders and to measure protein and thiamine levels in the patient. CT or MRI scans can also be requested. Brain scans can reveal changes in the brain, such as a degeneration of the mammillary bodies, that are indicative of WKS (NORD 2021).

MRI comparison of a healthy brain and with abnormal hyperintense signal in the mesial dorsal thalami | Image
MRI of a healthy brain v with abnormal hyperintense signal in the mesial dorsal thalami, indicative of Wernicke Encephalopathy (CC Jto410).

Treatment of Wernicke-Korsakoff Syndrome

The goals of WKS treatment are twofold: to control the symptoms and to prevent the disorder from worsening.

If the syndrome is detected early, withdrawal and abstinence from alcohol, as well as nutritional interventions, can show a halt to the progress of WKS (Day et al. 2013).

Immediate thiamine supplementation via intravenous injection is also recommended to restore thiamine levels and prevent further brain damage. Timely treatment can show noticeable improvement in mental and physical health within two or three weeks (Alcohol.Org Editorial Staff 2018).

Providing WKS is recognised and treated early, significant recovery can be made. However, in some cases, mental state changes, particularly memory impairment, can continue for several years.

Some people will unfortunately not fully recover, with about 25% of people who develop WKS requiring long-term treatment (Akhouri et al. 2022).

Prevention strategies can include limiting alcohol consumption. A healthy and balanced diet can also help to reduce the chances of developing WKS (Medline Plus 2020).


Test Your Knowledge

Question 1 of 3

In relation to Wernicke-Korsakoff syndrome, what does the term 'ataxia' refer to?


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