Wernicke-Korsakoff syndrome

Información en español Title

Categories:

Nervous System Diseases

Summary Summary

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.^[1] 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. It 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.^[1]^[2]^[3] Studies indicate that there may be some genetic predisposition for the disease. Treatment involves supplementing the diet with thiamine. Wernicke encephalopathy is an acute syndrome and requires emergency treatment to prevent death and neurologic complications. In cases where the diagnosis is not confirmed, patients should still be treated while additional evaluations are completed.^[2]^[3]

Last updated: 7/26/2016

Symptoms Symptoms

Although these conditions 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 and Korsakoff's amnesic syndrome represents the "chronic" phase.^[1] The symptoms of Wernicke encephalopathy (WE) commonly include:^[1]^[2]^[4]

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).

Other symptoms may include:^[1]^[3]^[4]

Korsakoff syndrome (KS) is seen most frequently in people who abuse alcohol after they experience an episode of WE. Most patients with KS show typical WE lesions in the brain.^[5] The main features of Korsakoff's syndrome are impairments in acquiring new information or establishing new memories, and in retrieving previous memories.^[1]^[4]

The symptoms include:^[1]^[2]^[3]^[4]

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.

Attention and social behavior are relatively preserved. Affected subjects are able to carry on a socially appropriate conversation that may seem normal to an unsuspecting spectator. Patients with KS are as a rule unaware of their illness. Some patients may have severe cognitive function and global dementia.^[5]

In people with memory impairment, lesions in an area of the brain known as the anterior thalamus are commonly found. The shrinking of the mamillary bodies is also specific for WE, so when damaged or very small mamillary bodies are found in a demented patient, it is thought that the dementia may be due to alcohol abuse and malnutrition.^[5]

Last updated: 7/26/2016

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 10 |

Medical Terms	Other Names	Learn More: HPO ID
Percent of people who have these symptoms is not available through HPO
Ataxia		0001251
Autosomal recessive inheritance		0000007
Coma		0001259
Delirium		0031258
Horizontal nystagmus		0000666
Memory impairment	Forgetfulness Memory loss Memory problems Poor memory [ more ]	0002354
Ophthalmoplegia	Eye muscle paralysis	0000602
Polyneuropathy	Peripheral nerve disease	0001271
Psychosis		0000709
Ptosis	Drooping upper eyelid	0000508

Showing of 10 |

Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 2/1/2019

Do you have updated information on this disease? We want to hear from you.

Cause Cause

Wernicke- Korsakoff is caused by thiamine deficiency. Thiamine is a cofactor for several key enzymes important in energy metabolism. Thiamine requirements depend on metabolic rate, with the greatest need during periods of high metabolic demand and high glucose intake.^[3]^[4]

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.^[2]

Thiamine deficiency in alcohol abusers results from a combination of inadequate dietary intake, reduced gastrointestinal absorption, decreased hepatic storage, and impaired utilization.^[4]

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.^[3]

Last updated: 7/26/2016

Treatment Treatment

The goals of treatment are to control symptoms as much as possible and to prevent progression of the disorder. Some people may need to be hospitalized initially to control the symptoms.^[4] Wernicke encephalopathy is an acute syndrome and requires emergency treatment to prevent death and neurologic problems. For this reason, even if the diagnosis is not confirmed, patients should be treated while additional evaluations are completed.^[2]^[3]

Treatment involves replacement of thiamine and providing proper nutrition and hydration.^[1]^[3] Intravenous thiamine is the treatment of choice. After the initial dose, daily doses of thiamine are usually recommended. Supplementation of electrolytes, particularly magnesium and potassium (often low in people with alcoholism), may be required in addition to thiamine. In those who are chronically malnourished, the remainder of the B vitamins should also be supplemented. Supplementation can be tapered as the patient resumes normal intake and shows improvement.^[3]

Because long-term alcohol use is the most common cause for Wernicke-Korsakoff syndrome, avoiding alcohol provides the best chance for recovery. Referral to an alcohol recovery program should be part of the treatment regimen.^[3]

Due to difficulties with movement, patients should be provided with assistance when walking during the initial phase of treatment. Patients may require physical therapy to assist with movement. Walking difficulties may be permanent, depending on the severity at initial presentation and the timeliness of therapy.^[3]

Patients with Korsakoff syndrome rarely recover. Many patients require at least some form of supervision and social support, either at home or in a chronic care facility.^[5]

Last updated: 7/26/2016

Prognosis Prognosis

Most symptoms (such as uncoordinated movement and vision difficulties) can be reversed if detected and treated promptly. Without treatment, these disorders can be disabling and life-threatening.^[1]^[4] Patients with Korsakoff syndrome rarely recover. Many patients require at least some form of supervision and social support, either at home or in a chronic care facility.^[5]

Last updated: 7/26/2016

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
ClinicalTrials.gov lists trials that are related to Wernicke-Korsakoff syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Alzheimer's Association
225 N. Michigan Ave., Fl. 17
Chicago, IL 60601-7633
Toll-free: 1-800-272-3900 (24/7 Helpline)
TTY: 1-866-403-3073
Fax: 1-866-699-1246
E-mail: info@alz.org
Website: https://www.alz.org/

Organizations Providing General Support

Alcoholics Anonymous
P.O. Box 459
Grand Central Station
New York, NY 10163
Telephone: 212-870-3400
E-mail: http://www.aa.org/
Family Caregiver Alliance
785 Market Street
Suite 750
San Francisco, CA 94103
Toll-free: 800-445-8106
Telephone: 415-434-3388
Fax: 415-434-3508
E-mail: info@caregiver.org
Website: http://www.caregiver.org

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Wernicke-Korsakoff syndrome. Click on the link to view a sample search on this topic.

News & Events News & Events

News

NIH Hosts Rare Disease Day Event, Twitter Chat
January 24, 2019

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

Does Wernicke-Korsakoff syndrome affect a person's ability to walk, feed themselves, and move? What are the odds of an individual who has been an alcoholic for over 30 years recovering from this condition? See answer

Have a question? Contact a GARD Information Specialist.