Wernicke-Korsakoff Syndrome

What is Wernicke-Korsakoff Syndrome

Wernicke-Korsakoff syndrome (WKS) is a brain disorder that is characterized by memory loss and a serious mental condition that impairs thinking and emotions (psychosis). The term actually refers to two conditions that often occur together.

Wernicke encephalopathy happens first. This disease damages the hypothalamus and thalamus portions of the brain. As the disease gets worse, Wernicke encephalopathy is followed by Korsakoff syndrome (Korsakoff psychosis). This condition can permanently damage the areas of the brain that are responsible for memory.

What are the causes of Wernicke-Korsakoff Syndrome ?

This condition is caused by a lack of vitamin B1 (thiamine). This deficiency can result from:

• Alcoholism.
• Poor nutrition.
• Certain medical conditions.

What increases the risk of Wernicke-Korsakoff Syndrome ?

This condition is more likely to develop in:

• People who have had severe alcoholism for 5 years or longer.
• People who have poor nutrition.
• People who have AIDS (acquired immunodeficiency syndrome).
• People who have a history of chronic infections.
• People who have anorexia nervosa.
• People who receive kidney dialysis.
• People who have advanced cancer.

What are the signs or symptoms?

Symptoms vary based on what stage of the disease you have.

Symptoms of Wernicke encephalopathy include:

• Uncoordinated muscles.
• Abnormal eye movements.
• Confusion.
• Loss of other mental abilities.
• Double vision.
• Eyelid drooping.
• Symptoms of alcohol withdrawal.

As the disease gets worse, symptoms of Korsakoff syndrome may develop. These include:

• Severe memory loss.
• Inability to form new memories.
• Hallucinations.
• Tendency to make up stories.

How is this diagnosed?

There is no specific test to diagnose this condition. It may be diagnosed based on:

• Your symptoms and medical history. Your health care provider may suspect this condition if you abuse alcohol.
• A physical exam.
• Blood tests to check your thiamine level and to look for other signs of malnutrition.
• Tests to check for memory loss.
• Imaging studies to look for changes to the brain and other body parts. These may include MRI and CT scans.

How is this treated?

Treatment for this condition needs to start early. If the condition is diagnosed and treated in the early stages, it can be reversed. If the condition is left untreated, it can cause permanent brain damage. Treatment may include:

• Thiamine replacement therapy. You may be given thiamine through an IV tube or by mouth.
• Treatment for alcoholism.
• Medicine and mental health counseling for brain-related symptoms of chronic dementia.

Follow these instructions at home:

• Take medicines only as told by your health care provider.
• Do not drink alcohol. Get treatment for alcoholism if needed.
• Eat a nutritious diet.
• Keep all follow-up visits as told by your health care provider. This is important, especially for mental health counseling.
• Get caregiver support if necessary.

Contact a health care provider if:

• You have confusion or memory problems.
• You have severe alcoholism.

Get help right away if:

• Your symptoms become severe.
• You have serious thoughts about hurting yourself or others.

Detailed Info on Wernicke Korsakoff Syndrome

Korsakoff psychosis (KP) is a disorder of declarative learning and memory that results from thiamine deficiency, largely (90%) secondary to chronic alcohol abuse in industrialized nations. It is classically, but not always, seen following the presentation of Wernicke encephalopathy (see “ Wernicke Syndrome ”).

Synonyms

• Korsakoff syndrome
• Wernicke-Korsakoff syndrome
• Alcoholic polyneuritic psychosis
• KPF

Epidemiology & Demographics

• •The incidence of KP is declining as a result of improved patient nutrition and awareness by health professionals
• •More common in males
• •Age of onset evenly distributed between ages 30 and 70 yr

Physical Findings & Clinical Presentation

• •Impaired anterograde memory as a core feature, and commonly retrograde memory is impaired to a varying extent as well.
• •Remote memory is relatively less impaired on neuropsychiatric testing.
• •Confabulation (the fabrication of false memories to fill memory gaps) is common.
• •Severe declarative amnesia and concomitant deficits in executive function result in compromised autonomy.

Etiology

KP is the result of thiamine deficiency. Thiamine deficiency is commonly seen in chronic alcoholism and other malnourished populations (anorexia, bariatric surgery, malignancy). It may also occur from prolonged infusion of dextrose-containing fluids without thiamine repletion.

Differential Diagnosis

• •Stroke, trauma, or tumor affecting the temporal lobes or hippocampi
• •Cerebral anoxia
• •Transient global amnesia
• •Dementia of multiple causes

Workup

A high index of suspicion should be maintained in chronic alcoholism and malnourished states.

Laboratory Tests

• •CBC
• •Serum chemistries
• •Erythrocyte thiamine transketolase concentration before and after replacement with thiamine pyrophosphate

Imaging Studies

MRI brain may show nonspecific T₂ hyperintensities in the diencephalon and mesencephalon but is not pathognomonic. However, MRI/CT may be helpful in ruling out other diagnoses.

Nonpharmacologic Therapy

A supervised environment may be required.

• •Acute: Treatment should be initiated in all patients with clinical suspicion of Wernicke encephalopathy (WE). Thiamine supplementation should precede glucose administration in all patients at risk for WE lest they develop Korsakoff syndrome.
• •Alcoholics with WE: Treat with 500 mg of thiamine hydrochloride (dissolved in 100 ml of normal saline) infused intravenously over 30 min three times daily for 2 to 3 days.
• •If a response is observed, continue with 250 mg of thiamine hydrochloride intravenously or intramuscularly daily until clinical improvement ceases.
• •Nonalcoholics with WE: Treat with 200 mg of thiamine hydrochloride (dissolved in 100 ml of normal saline) infused intravenously over 30 minutes three times daily for 2 to 3 days.
• •Parenteral magnesium must be infused concurrently with thiamine.
• •Side effects of parenteral thiamine hydrochloride may include generalized pruritus, transient local irritation, or, rarely, anaphylactic or anaphylactoid reactions.

Chronic Treatment

Recommended oral dose after completed parenteral treatment for WE: Thiamine 30 to 100 mg twice daily for long-term thiamine replacement.

• •Individuals with KP rarely recover; therefore, one must consider the need for additional support and supervision within the home versus placement in a long-term care facility.

Referral

• •Neurology to confirm the diagnosis and rule out other causes
• •Neuropsychiatric testing to assess baseline functioning and further deterioration

Pearls & Considerations

• •KP is frequently misdiagnosed or underdiagnosed!
• •Memory problems may persist even in “recovered” patients.
• •Replace thiamine in patients at risk even if clinical symptoms are not evident.
• •Prolonged use of dextrose-containing IV fluids without supplemental thiamine may precipitate KP.
• •Strengthening of procedural memory and encouragement of procedural learning can help restore some of the patient’s autonomy.

Seek Additional Information

• Oudman E., et al.: Procedural learning and memory rehabilitation in Korsakoff’s syndrome: a review of the literature. Neuropsychol Rev 2015; 25: pp. 134-148.