What is Wernicke Korsakoff Syndrome?

Overview

Wernicke-Korsakoff syndrome (WKS) is a brain disorder that can result from a deficiency in vitamin B1 (thiamine). This syndrome encompasses two interconnected conditions: Wernicke’s encephalopathy (WE) and Korsakoff syndrome. Typically, individuals experience the symptoms of Wernicke’s encephalopathy first. The manifestations of WKS may involve confusion, changes in vision and mental alterations, such as an inclination towards exaggerated storytelling. The primary cause of WKS is alcoholism. Additionally, WKS can be associated with inadequate dietary intake or other medical conditions that hinder the absorption of vitamin B1. This article will go over the symptoms, causes, risk factors and treatment options for WKS. 

Causes of wernicke korsakoff syndrome

Alcoholism stands as the primary factor contributing to WKS.¹ Less frequently, WKS can arise from conditions that hinder the absorption of essential nutrients. Nutritional intake and absorption may be compromised in the following circumstances:

• Gastric bypass surgery restricts food portions, making it challenging to meet nutritional requirements adequately
• Stomach cancer, which can impede the absorption of vital nutrients in the stomach
• Colon cancer can lead to pain in the abdomen, deterring individuals from consuming regular meals
• Eating disorders can prevent a person from meeting dietary requirements 

Alcoholism is the foremost cause of WKS because individuals affected by this condition generally exhibit poor dietary habits. Furthermore, alcohol impedes the absorption and storage of vitamin B1. 

Signs and symptoms of wernicke korsakoff syndrome

WE presents itself as brain lesions caused by a deficiency in vitamin B1.² Prominent symptoms associated with WE include:

• Double vision
• Drooping of the upper eyelid (ptosis)
• Involuntary eye movements (up-and-down or side-to-side)
• Loss of muscle coordination (ataxia), which can affect walking
• Confused mental state leading to combative or violent behaviour

Subsequently, WE can progress into Korsakoff's syndrome, which manifests as various memory-related issues. Individuals with WKS may experience memory loss or have difficulty forming new memories.

If you have WKS, you might also exhibit the following symptoms:

• Anterograde and retrograde amnesia
• Difficulty understanding what is being said or presented
• Challenges in contextualising words
• Hallucinations
• Excessive storytelling (confabulation)³

Management and treatment for wernicke korsakoff syndrome

Staring treatment immediately is crucial for individuals with WKS. Timely intervention has the potential to delay or stop the progression of the disease, even having the ability to reverse certain brain abnormalities. Treatment often begins with hospitalisation, where doctors will monitor you in order to ensure that your digestive system is effectively absorbing nutrients. 

The treatment approach for WKS typically involves:⁴

• Administration of vitamin B1 through an intravenous (IV) line in the arm or hand
• Oral supplementation of vitamin B1
• Adoption of a balanced diet to maintain adequate levels of vitamin B1
• Treatment for alcoholism, if applicable

Following the diagnosis, your doctor will likely administer vitamin B1 intravenously (through your veins). Prompt treatment can potentially reverse many of the neurological symptoms associated with WKS. 

In some cases, a negative reaction may occur during vitamin B1 treatment, especially in individuals with alcoholism. These reactions can vary and may include symptoms of alcohol withdrawal, such as insomnia, sweating, and mood swings. Other possible side effects encompass hallucinations, confusion and agitation. 

Diagnosis

Diagnosing WKS can be quite challenging.⁵ If you are affected by WKS, you may experience mental confusion, which can impede effective communication with your doctor from happening. Hence, this confusion may lead doctors to overlook the possibility of an underlying physical disorder.

The diagnostic process for WKS typically involves the following assessments:

Assessment for signs of alcoholism 

The doctor may initially evaluate signs of alcoholism by checking your blood alcohol levels and conducting liver function tests to identify liver damage that can be indicative of alcoholism. Examination of vital signs, eye movements, reflexes, blood pressure, and body temperature may also be part of diagnosing chronic alcoholism.

Assessment for signs of nutritional deficiencies 

The doctor will look for clinical signs that can present in people with vitamin B1 deficiency. This may involve blood tests to measure thiamine levels and assess overall nutritional health. Nutritional tests may include a serum albumin test, to evaluate protein levels in the blood, as low albumin levels can indicate nutritional deficiencies and issues with the kidneys or liver. Additionally, a serum vitamin B1 test may be conducted to check vitamin B1 levels. Enzyme activity in red blood cells (RBCs) can also be examined, with low enzyme activity suggesting vitamin B1 deficiency.

Other diagnostic tests

Imaging tests may be recommended to detect characteristic organ damage that is associated with WKS. Thismay include performing an electrocardiogram (ECG or EKG) before and after administering vitamin B1. A computed tomography (CT)  scan can be utilised to detect brain lesions related to Wernicke's encephalopathy (WE), while MRI scans are employed to observe brain changes associated with the condition. Additionally, a neuropsychological test may be employed to assess the extent of cognitive impairments.These diagnostic measures aim to determine the presence and severity of WKS and provide a comprehensive understanding of the individual's condition. 

FAQs

How can I prevent wernicke korsakoff syndrome?

Prevention of WKS can be achieved by abstaining from alcohol consumption and by maintaining a balanced diet that is abundant in vitamin B1. 

To ensure an adequate intake of vitamin B1, it is recommended to include the following foods in your diet:

• Lean pork
• Fish
• Rice
• Peas
• Spinach  
• Whole wheat bread 
• Oranges
• Milk
• Sunflower seeds
• Green beans
• Yoghurt

By avoiding alcohol and incorporating these vitamin B1-rich foods into your diet, you can minimise the risk of developing WKS and promote your overall well-being.

How common is wernicke korsakoff syndrome?

Among individuals with alcohol use disorder, the prevalence of WKS has been reported to range from 1% to 2%. It is important to note that WKS is underdiagnosed and often difficult to recognise, which may cause its actual occurrence to be underestimated. The specific prevalence of WKS in the United Kingdom (UK) is not readily available as there is limited data specifically focused on the UK population. However, it is recognised that WKS occurs worldwide and is primarily associated with chronic alcohol misuse. The prevalence of WKS in the UK is likely to be influenced by factors such as alcohol consumption patterns, healthcare access and awareness of the syndrome among healthcare professionals. 

Who is at risk of developing wernicke korsakoff syndrome?

The risk factors associated with WKS are linked to one's diet and lifestyle. The primary risk factors for developing WKS ismalnourishment and prolonged misuse of alcohol. Additional risk factors for WKS includes:

• Limited access to adequate medical care and proper nutrition due to financial constraints
• Undergoing kidney dialysis, which can diminish the absorption of vitamin B1
• Having AIDS, which increases the likelihood of developing conditions that lead to a deficiency in vitamin B1

By addressing these risk factors through improved nutrition, responsible alcohol consumption and appropriate medical care, the chances of developing WKS can be minimised. 

When should I see a doctor?

If you experience double vision, drooping upper eyelids, involuntary eye movements, loss of muscle coordination and memory loss, especially if you are abusing alcohol, contact your general practitioner (GP). 

Summary

Wernicke-Korsakoff syndrome (WKS) is a brain disorder resulting from a deficiency invitamin B1. It consists of two conditions: Wernicke's encephalopathy and Korsakoff syndrome. WKS is primarily caused by chronic alcohol misuse, but it can also be linked to malnutrition and other medical conditions. Symptoms include confusion, vision changes, loss of muscle coordination, and memory issues. Treatment involves administering vitamin B1 intravenously or orally, maintaining a balanced diet, and addressing alcoholism. Diagnosis can be challenging if the affected individual presents with mental confusion, but it typically involves assessing signs of alcoholism and nutritional deficiencies, and conducting imaging tests.