55 Kenosia Avenue
Danbury, CT 06810
Phone: 203.744.0100
Toll Free: 1.800.999.6673
http://rarediseases.org

Wernicke-Korsakoff Syndrome

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Copyright 1986, 1992, 1998, 2005

Synonyms of Wernicke-Korsakoff Syndrome

Disorder Subdivisions

General Discussion

Wernicke syndrome and Korsakoff syndrome are related disorders that often occur due to a deficiency of thiamine (vitamin B1). Wernicke's syndrome, also known as Wernicke encephalopathy, is a neurological disease characterized by the clinical triad of confusion, the inability to coordinate voluntary movement (ataxia), and eye (ocular) abnormalities. Korsakoff's syndrome is a mental disorder characterized by disproportionate memory loss in relation to other mental aspects. When these two disorders occur together, the term Wernicke-Korsakoff syndrome is used. In the United States, most cases occur in alcoholics.

Some researchers believe Wernicke and Korsakoff syndromes are separate yet related disorders; others believe them to be different stages of the same disorder or disease spectrum. Wernicke syndrome is considered the acute phase with a shorter duration and more serious symptoms. Korsakoff syndrome is considered the chronic phase and is a long-lasting condition.

Symptoms

There is disagreement in the medical literature as to whether or not an episode of Wernicke syndrome always precedes the development of Korsakoff syndrome. Wernicke syndrome is characterized by the clinical triad of mental status changes (e.g., confused state), the inability to coordinate voluntary movement (ataxia), and eye abnormalities. Affected individuals may not display all three symptoms.

The confusion or disorientation associated with Wernicke syndrome develops over a few days or weeks and is the main mental problem associated with the disorder. Affected individuals may experience lethargy, inattentiveness, drowsiness and indifference. In extreme cases, delirium may occur. If left untreated, affected individuals may develop stupor or loss of consciousness (coma).

Ataxia most often affects how a person walks (gait). In some cases, affected individuals may have a slow, unsteady gait; in the acute stage of the disease, ataxia may prevent an affected individual from standing or walking without assistance.

Ocular abnormalities associated with Wernicke syndrome include double vision, rapid, involuntary eye movements (nystagmus), paralysis of certain eye muscles (ophthalmaplegia), and in rare cases drooping of the upper eyelids (ptosis).

Additional symptoms associated with Wernicke-Korsakoff syndrome include disease affecting many nerves (polyneuropathy), especially those outside of the central nervous system (peripheral neuropathy). Peripheral neuropathy may cause weakness of the arms and legs and contribute to difficult walking. A variety of cardiovascular abnormalities may also occur in individuals with Wernicke-Korsakoff syndrome including a rapid heartbeat (tachycardia), low blood pressure upon standing (postural hypotension), and loss of consciousness (syncope).

Approximately 80-90 percent of individuals with Wernicke syndrome develop Korsakoff syndrome. The symptoms of Korsakoff syndrome often develop as the mental symptoms of Wernicke syndrome begin to lessen.

Korsakoff syndrome is characterized by memory impairment, specifically short-term memory loss (i.e., the inability to form new memories). In some cases, affected individuals may also have random loss of long-term memories. In rare cases, individuals may create imaginary events to fill in gaps in their memory (confabulation).

Causes

Wernicke-Korsakoff syndrome is caused by a deficiency of thiamine (vitamin B1). In the United States the majority of causes occur in alcoholics. Alcohol reduces the absorption of thiamine by the body, diminishes stores of thiamine in the liver, and hampers the activity of the enzyme that converts thiamine into an active state.

The disorder may also occur due to malnutrition. Causes of malnutrition that may result in Wernicke-Korsakoff syndrome include starvation, eating disorders (e.g., anorexia), prolonged or chronic vomiting as found in certain disorders such as hyperemesis gravidarum. In addition, chronic disorders such as cancer, AIDS, disorders of the stomach (gastropathies), and kidney disorders requiring dialysis may also cause Wernicke-Korsakoff syndrome.

In some cases, hereditary factors may cause some individuals to have a genetic predisposition to developing Wernicke-Korsakoff syndrome. However, more research is necessary to determine what role, if any, genetics plays in the development of the disorder.

The symptoms of Wernicke-Korsakoff syndrome occur due to a deficiency of thiamine. Although thiamine is a nutrient required by all the tissues of the body, thiamine deficiency affects the cells of the nervous and cardiovascular systems to a greater degree than the cells of other organ systems.

Affected Populations

Wernicke-Korsakoff syndrome occurs in 1-2 percent of the general population in the United States. The disorder affects slightly more males than females and is evenly distributed between ages 30-70.

Related Disorders

N/A

Standard Therapies

Diagnosis
A diagnosis of Wernicke-Korsakoff syndrome is made based upon a thorough clinical evaluation, a detailed patient history and a variety of specialized tests including routine laboratory screens and liver function tests that can rule out other disorders with similar presentations. Test that measure thiamine and erythrocyte transketolase acitivity (both which are reduced in Wernicke-Korsakoff syndrome) may also help in attaining a diagnosis. Computed tomography (CT) scanning and magnetic resonance imaging (MRI) may be necessary to rule out tumors, infarcts, and bleeding (hemorrhaging). CT scans and MRIs may also reveal brain changes that are indicative of Wernicke-Korsakoff syndrome (e.g., shrunken mammillary bodies).

The mammillary bodies are a pair of small round structures found within the brain that form part of the limbic system. The limbic system is a group of structures within the brain that are involved with emotions and in the formation of memory.

Treatment
The immediate administration of thiamine (replacement therapy) is used to treat individuals with Wernicke-Korsakoff syndrome. Thiamine is injected directly into the intravenously because abdominal absorption of thiamine is hampered in affected individuals. Mental status changes, vision abnormalities and ataxia all usually improve upon administration of thiamine. Thiamine administration may continue daily for several months.

Abstinence from alcohol and proper dietary changes are recommended. Some affected individuals may benefit from psychological methods designed to treat mental and emotional disorders (psychotherapy).

If the disorder is caught early and treatment started promptly a significant recovery can be made. In some cases, confusion and mental problems may take a year to resolve. In severe cases, brain damage may cause lasting problems with memory and gait.

Other treatment is symptomatic and supportive.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Organizations related to Wernicke-Korsakoff Syndrome

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.

References

TEXTBOOKS
Sahoo S, Pearl PL. Korsakoff Syndrome. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:546.

Rowland LP, ed. Merritt's Neurology. 10th ed. Lippincott Williams & Wilkins. Philadelphia, PA. 2000:.

Bennett JC, Plum F., eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:2039-40.

Fauci AS, et al., eds. Harrison's Principles of Internal Medicine, 14th Ed. New York, NY: McGraw-Hill, Inc; 1998:2455-6.

Adams RD, et al., eds. Principles of Neurology. 6th ed. New York, NY: McGraw-Hill, Companies; 1997:1138-45.

Gabbard GO, ed. Treatments of Psychiatric Disorders, 2nd ed. Washington, DC: American Psychiatric Association; 1995:201-2.

JOURNAL ARTICLES
Caulo M, Van Hecke J, Toma L, et al., Functional MRI study of diencephalic amnesia in Wernikce-Korsakoff syndrome. Brain. 2005;Epub ahead of print.

Cochrane M, Cochrane A, Jauhar P, Ashton E. Acetylcholinesterase inhibitors for the treatment of Wernicke-Korsakoff syndrome - three further cases show response to donepezil. Alcohol Alcohol. 2005;40:151-4.

Day E, Bentham P, Callaghan R, Kuruvilla T, George S. Thiamine for Wernicke-Korsakoff syndrome in people at risk from alcohol abuse. Cochrane Database Syst Rev. 2004;1:CD004033.

Martin PR, Singleton CK, Hiller-Sturmhofel S. The role of thiamine deficiency in alcoholic brain disease. Alcohol Res Health. 2003;27:134-42.

Singleton CK, Martin PR. Molecular mechanisms of thiamine utilization. Curr Mol Med. 2001;1:197-207.

FROM THE INTERNET
McKusick VA., ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No:277730; Last Update:3/17/2004. Available at: http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=277730 Accessed on: April 27, 2005.

National Institute of Neurological Disorders and Stoke. Wernicke-Korsakoff Syndrome Information Page. February 9, 2005. Available at:
http://www.ninds.nih.gov/disorders/wernicke_korsakoff/wernicke-korsakoff.htm Accessed On: April 27th, 2005.

DeAngelo A. Wernicke-Korsakoff Syndrome. eMedicine Journal. 2002;3:10pp. Available at http://www.emedicine.com. Accessed On: April 27th, 2005.

Report last updated: 2008/05/05 00:00:00 GMT+0