Wernicke–Korsakoff Syndrome (WKS)

Excessive alcohol consumption can be the cause of a raft of serious health issues, including liver disease, bowel cancer and addiction.

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 n.d.)

Due to the 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).

The 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).

In Australia, one study found that though the prevalence of WKS has dropped significantly in recent years, it still remains higher than most other Western countries (Harper et al. 2012).

According to the National Organization for Rare Diseases (2005), WKS presents in roughly 1 - 2% of the general population in the United States and is more likely to occur in males rather than females, between the ages of 30 and 70 years.

The US Centers for Disease Control and Prevention (CDC) report that around 88,000 people die each year from complications related to alcohol abuse (Alcohol.Org Editorial Staff 2018).

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.

Though there is some dispute over whether WE and KS are two separate but related disorders, or simply different stages of the same disorder (NORD 2005), they often co-occur, thus forming Wernicke-Korsakoff syndrome.

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 2018).

Wernicke-Korsakoff Syndrome Presentation

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

Wernicke Encephalopathy Symptoms

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, dropping of the upper eyelids (ptosis).

(NORD 2005; Dementia Australia 2016)

From here, approximately 80 to 90% of individuals develop KS.

Korsakoff Syndrome Symptoms:

KS mostly presents in memory impairment such as:

• Memory loss,
• Inability to form new memories,
• Inability to learn new information,
• Personality changes,
• Confabulation,
• Hallucination,
• Lack of insight into the condition.

(NORD 2005; Dementia Australia 2016)

Causes of WKS

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 is not currently known why some individuals will and some will not, however genetics, as well as diet and other lifestyle factors are suspected to play a role (NORD 2005; Dementia Australia 2016).

The main cause for WKS is a deficiency of thiamine, or 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 2005). When thiamine levels are low, brain and nerve cell damage can occur.

It is also important to note that alcohol consumption is not the only cause of thiamine deficiency. WKS can also develop in individuals who are suffering from 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 2005; Dementia Australia 2016).

Wernicke-Korsakoff Syndrome Diagnosis

WKS diagnosis can be difficult as symptoms often present in similar ways to that of dementia (Dementia Australia 2016), and so should not be ruled out in high-risk populations.

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 2018).

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, indicative of WKS (NORD 2005).

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 to prevent further brain damage. Timely treatment can show noticeable improvement to 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 sufferers of WKS will unfortunately not fully recover, with about 25% of people who develop WKS requiring long-term treatment (alcohol.org).

Prevention strategies can include limiting the amount of alcohol consumption. The National Health & Medical Research Council of Australia recommended no more than two standards drinks a day as for both men and women. A healthy and balanced diet can also help to reduce the chances of developing WKS (Medline Plus 2018).

References

• Alcohol.Org Editorial Staff 2018, ‘Wernicke-Korsakoff Syndrome’, Alcohol.org, viewed 24 April 2019, https://www.alcohol.org/effects/wernicke-korsakoff-syndrome/
• Day, E, Bentham, PW, Callaghan, R, Kuruvilla, T & George, S 2013, ‘Thiamine for prevention and treatment of Wernicke-Korsakoff syndrome in people who abuse alcohol’, Cochrane, 1 July, viewed 24 April 2019, https://www.cochrane.org/CD004033/DEMENTIA_thiamine-for-prevention-and-treatment-of-wernicke-korsakoff-syndrome-in-people-who-abuse-alcohol
• Dementia Australia 2016, Alcohol related dementia and Wernicke-Korsakoff syndrome, Dementia Australia, viewed 24 April 2019, https://www.dementia.org.au/files/helpsheets/Helpsheet-AboutDementia18-AlcoholRelatedDementiaAndWernickeKorsakoffSyndrome_english.pdf
• Dementia Australia n.d., Alcohol related dementia, Dementia Australia, viewed 24 April 2019, https://livingwith.dementia.org.au/about-dementia/types-of-dementia/alcohol-related-dementia
• Harper, CG, Sheedy, DL, Lara, AI, Garrick, TM, Hilton, JM & Raisanen, J 2012, ‘Prevalence of Wernicke-Korsakoff syndrome in Australia: has thiamine fortification made a difference?’, Medical Journal of Australia, vol. 168, no. 11, pp. 542-5, viewed 24 April 2019, https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3391549/
• Isenberg-Grzeda, E, Kutner, HE & Nicolson, SE 2012, ‘Wernicke-Korsakoff-syndrome: under-recognized and under-treated’, Psychosomatics, vol. 53, no. 5, pp. 507-16, viewed 24 April 2019, https://www.ncbi.nlm.nih.gov/pubmed/23157990
• Medline Plus 2018, Wernicke-Korsakoff syndrome, Medline Plus, viewed 24 April 2019, https://medlineplus.gov/ency/article/000771.htm
• National Organization for Rare Diseases 2005, Wernicke-Korsakoff Syndrome, NORD, viewed 24 April 2019, https://rarediseases.org/rare-diseases/wernicke-korsakoff-syndrome/