What are the other Names for this Condition? (Also known as/Synonyms)

• Alcohol-Induced Encephalopathy
• Gayet-Wernicke Syndrome
• Transketolase Defect

What is Wernicke-Korsakoff Syndrome? (Definition/Background Information)

• Wernicke-Korsakoff Syndrome is a brain disorder, due to thiamine deficiency that has been associated with both Wernicke's encephalopathy and Korsakoff syndrome
• The term refers to two different syndromes, each representing a different stage of the disease. Wernicke's encephalopathy represents the "acute" phase and Korsakoff's syndrome represents the "chronic" phase. However, they are used interchangeable in many sites
• Wernicke's encephalopathy is characterized by confusion, abnormal stance and gait (ataxia), and abnormal eye movements (nystagmus). Korsakoff's syndrome is observed in a small number of patients
• Wernicke-Korsakoff Syndrome is a type of dementia, characterized by memory loss and confabulation (filling in of memory gaps with data the patient can readily recall) and involvement of the heart, vascular, and nervous system
• Wernicke-Korsakoff Syndrome mainly results from chronic alcohol use, but also from dietary deficiencies, prolonged vomiting, eating disorders, systemic diseases (cancer, AIDS, infections), bariatric surgery, transplants, or the effects of chemotherapy
• Studies indicate that there may be some genetic predisposition for Wernicke-Korsakoff Syndrome. Treatment involves supplementing the diet with thiamine
• Wernicke encephalopathy is an acute syndrome and requires emergency treatment to prevent death and neurologic complications

(Source: Wernicke-Korsakoff Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Wernicke-Korsakoff Syndrome? (Age and Sex Distribution)

• Wernicke-Korsakoff Syndrome is a rare disorder and affects 1-2% of the population in the United States
• The onset of symptoms typically occurs in adulthood
• Both genders may be affected by this disorder
• Worldwide, individuals of all races and ethnicities may be affected. However, Europeans are more susceptible to this disorder than Asians or Africans

What are the Risk Factors for Wernicke-Korsakoff Syndrome? (Predisposing Factors)

The risk factors for Wernicke-Korsakoff Syndrome may include the following:

• Chronic alcohol abuse
• A possible inherited metabolic disorder, with resultant thiamine deficiency
• Dietary thiamine deficiency
• Prolonged vomiting
• Bariatric surgery
• Organ/tissue transplantation
• Cancers
• Chemotherapy for cancer
• HIV infection and/or AIDS

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Wernicke-Korsakoff Syndrome? (Etiology)

Wernicke-Korsakoff Syndrome is caused by thiamine deficiency.

• Thiamine is a cofactor for several key enzymes important in energy metabolism
• Because of the role of thiamine in cerebral energy utilization, it has been proposed that its deficiency initiates neuronal injury by inhibiting metabolism in brain regions with high metabolic requirements and high thiamine turnover
• Thiamine deficiency in alcohol abusers results from a combination of inadequate dietary intake, reduced gastrointestinal absorption, decreased hepatic storage, and impaired utilization
• Some studies indicate that there may be a genetic predisposition for the disease
• Variants in the high affinity thiamine transporter gene have also been implicated.  The SLC19A2 gene provides instructions for making a protein called thiamine transporter 1
• This protein is located on the surface of cells, where it works to bring vitamin B1 (thiamine) into cells

(Source: Wernicke-Korsakoff Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

What are the Signs and Symptoms of Wernicke-Korsakoff Syndrome?

Although Wernicke-Korsakoff Syndrome may appear to be two different disorders, they are generally considered to be different stages of Wernicke-Korsakoff Syndrome. Wernicke's encephalopathy represents the "acute" phase; Korsakoff's amnesic syndrome represents the "chronic" phase of the disorder.

The signs and symptoms of Wernicke encephalopathy (WE) commonly include:  

• Encephalopathy (profound disorientation or mental confusion, inability to think clearly and indifference)
• Vision problems (including double vision, abnormal eye movements (nystagmus) and eyelid drooping)
• Loss of muscle coordination (ataxia)

The signs and symptoms of Korsakoff syndrome include:

• Loss of memory and inability to form new memories
• Making of stories (confabulation)
• Seeing or hearing things that are not really there (hallucinations)
• Disorientation
• Vision impairment

Other signs and symptoms of Wernicke-Korsakoff Syndrome may include:

• Delirium tremor
• Hypothermia
• Hypotension
• Ataxia 
• Confusion
• Horizontal nystagmus 
• Memory impairment
• Ophthalmoplegia
• Polyneuropathy
• Psychosis
• Ptosis

(Source: Wernicke-Korsakoff Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Wernicke-Korsakoff Syndrome Diagnosed?

Wernicke-Korsakoff Syndrome is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Wernicke-Korsakoff Syndrome?

The complications of Wernicke-Korsakoff Syndrome may include:

• Difficulty walking
• Chronic impairment of short-term memory 
• Progressive neurological problems
• Behavioral problems
• Coma

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Wernicke-Korsakoff Syndrome Treated?

The treatment for Wernicke-Korsakoff Syndrome may include the following:

• Hospitalization to control symptoms, for acute Wernicke encephalopathy
• Thiamine supplementation
• Supplementation of electrolytes for proper electrolyte balance
• Vitamin B supplements in those who are severely malnourished
• Devices for walking assistance to improve mobility
• Enrolling in programs for recovery from alcohol abuse

How can Wernicke-Korsakoff Syndrome be Prevented?

• The following are some methods for the prevention of Wernicke-Korsakoff Syndrome:
  • Abstinence from alcohol
  • Seeking treatment for alcohol abuse
  • Eating nutritious food rich in vitamins (such as thiamine) and minerals
  • Proper hydration
  • Prompt treatment at the onset of symptoms
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Wernicke-Korsakoff Syndrome

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

What is the Prognosis of Wernicke-Korsakoff Syndrome? (Outcomes/Resolutions)

• The prognosis of Wernicke-Korsakoff Syndrome is dependent upon the severity of the signs and symptoms, associated complications and timely treatment 
• With a timely treatment, the symptoms such as uncoordinated movements and impaired vision may be reversible
• If the condition progresses to the chronic Korsakoff syndrome, the prognosis is generally guarded; the affected individuals may require supervision for their regular day-to-day activities and chores
• Without treatment, the condition can lead to permanent disabilities and can possibly be fatal
• The prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Wernicke-Korsakoff Syndrome:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/