Wernicke-Korsakoff Syndrome

Two statesmen having drunk their fill. Tim Bobbin (i.e. John Collier) inv. et pinx. T. Sanders sculp. Source: National Library of Medicine.

Wernicke-Korsakoff syndrome is a degenerative brain disorder caused by a deficiency of thiamine (vitamin B1). The syndrome most commonly occurs as a result of chronic alcohol abuse, but may also be caused by an inadequate dietary intake of thiamine, prolonged vomiting, eating disorders, or the effects of chemotherapy. Wernicke-Korsakoff Syndrome is considered to have two stages: acute, and chronic.

Other names

• Alcoholic encephalopathy
• Cerebral beriberi

Types

Wernicke encephalopathy

Wernicke's encephalopathy represents the acute phase of Wernicke-Korsakoff syndrome. Symptoms include mental confusion, vision impairment, stupor, coma, hypothermia, hypotension, and ataxia (uncoordinated muscle movement).

Korsakoff psychosis

Korsakoff syndrome, or Korsakoff psychosis, tends to develop as Wernicke's symptoms go away. This disorder involves damage to areas of the brain involved with memory. Patients often attempt to hide their poor memory by creating detailed, believable stories about experiences or situations. This is not usually a deliberate attempt to deceive because the patient often believes what he is saying to be true.

The main features of Korsakoff's amnesic syndrome are the impairments in acquiring new information or establishing new memories, and in retrieving previous memories. Korsakoff psychosis is considered to be the chronic phase of Wernicke-Korsakoff syndrome.

Signs and Symptoms

Common signs and symptoms of Wernicke-Korsakoff syndrome include:

• Mental confusion
• Vision impairment
• Visual changes
• Ataxia (loss of muscle coordination)
• Unsteady, uncoordinated walking
• Difficulties with memory (loss of memory, inability to form new memories)

Late-stage Wernicke-Korsakoff syndrome may result in stupor, coma or even death.

Symptoms of alcohol withdrawl may also be present.

Causes

Wernicke-Korsakoff syndrome may result from a variety of conditions that affect thiamine levels in the body such as:

• Alcohol abuse (heavy alcohol use interferes with the break down of thiamine in the body, so even if someone with alcoholism follows a well-balanced diet, most of the thiamine is not absorbed)
• Dietary deficiencies (fortified breads, cereals, pasta, whole grains (especially wheat germ), lean meats (especially pork), fish, dried beans, peas, and soybeans)
• A high intake of foods such as milled rice, raw freshwater fish, raw shellfish, and ferns, tea, coffee, and betel nuts or processed food with a content high in sulfite. These products deplete thiamine.
• Eating disorders
• Prolonged vomiting (such as that of hyperemesis gravidarum (continuous nausea and vomiting during pregnancy) or from chemotherapy
• AIDS
• Thyrotoxicosis (very high thyroid hormonelevels)
• Cancers that have spread throughout the body
• Long-term dialysis
• Congestive heart failure, when treated with long-term diuretic therapy

Thiamine is an essential cofactor in most organisms and is required at several stages of metabolism, such as intracellular glucose metabolism, and is also a modulator of neuronal and neuro-muscular transmission. Thiamine acts as a coenzyme for transketolase (TK) and for the pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase complexes, enzymes which play a fundamental role for intracellular glucose metabolism. Lack of thiamine or defects in its intracellular transport can cause a number of severe disorders ^[1]

Diagnosis

Physical examination

Examination of the nervous/muscular system may show damage to multiple nerve systems. Reflexes may be decreased or abnormal. The person's walk (gait) and coordination will be tested. Muscles may be weak and may show atrophy (loss of tissue mass).

Examination of the eyes shows abnormalities of eye movement. Blood pressure and body temperature measurement may be low. Pulse (heart rate) may be rapid.

Laboratory tests

The person may appear malnourished. The following tests are used to check a person's nutrition level and may confirm a malnourished state.

• Serum B1 levels (may be low)
• Pyruvate level (may be high because vitamin B is required for the oxidation of pyruvate)
• Transketolase activity is decreased.
• Blood or urine alcohol levels and liver enzymes may be high if the person has a history of chronic (long-term) alcohol abuse.

Magnetic resonance imaging (MRI) of the brain rarely shows changes in the tissue of the brain indicating Wernicke-Korsakoff syndrome.

Treatment

The goals of treatment are to control symptoms as much as possible and to prevent progression of the disorder. Hospitalization is required for initial control of symptoms.

If the person is lethargic, unconscious, or comatose, monitoring and care appropriate to the condition may be required. The airway should be monitored and protected as appropriate.

Thiamine (vitamin B1) may improve symptoms of confusion or delirium, difficulties with vision and eye movement, and muscle incoordination. Vitamin B1 may be given by injection into a vein or a muscle, or by mouth. Thiamine does not generally improve loss of memory and intellect associated with Korsakoff psychosis.

Conventional wisdom holds that thiamine should be given before intravenous glucose when treating Wernicke-Korsakoff syndrome. Because thiamine is involved in the metabolism of glucose, there is concern that if glucose were given first, the body's thiamine would be futher depleted and symptoms would worsen.

Prevention

Abstinence or moderate alcohol use and adequate nutrition reduce the risk of developing Wernicke-Korsakoff syndrome. Total abstinence from alcohol is required to prevent progressive loss of brain function and damage to peripheral nerves.

A well-balanced, nourishing diet and thiamine supplementation is recommended for anyone at risk for Wernicke-Korsakoff syndrome.

Related Problems

Complications

Complications of Wernicke-Korsakoff syndrome include:

• Permanent loss of memory
• Permanent loss of cognitive skills
• Injury caused by falls
• Difficulty with personal or social interaction
• Alcohol withdrawal state
• Permanent alcoholic neuropathy
• Shortened life span

Related disorders

Symptoms of alcohol withdrawal (which may co-exist with the symptoms of Wernicke-Korsakoff syndrome include:

• Delirium or confusion
• Agitation
• Jumpiness or nervousness
• Insomnia
• Hallucinations
• Palpitations
• Heart rate that is faster than normal

Clinical Trials

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Research

• Confabulation (false memory) appears to be as much a feature in Korsakoff syndrome as the associated memor defecits. Screening for confabulation in patients with Korsakoff syndrome may be a helpful diagnostic tool. ^[2]
• Confabulation can be either spontaneous or provoked. There did not appear to be an association between spontaneous confabulation, provoked confabulation and false memory in a group of patients with Korsakoff syndrome. ^[3]
• A group of patients with Wernicke-Korsakoff syndrome were given rivastigmine, an achetylcholinesterase inhibitor to try to improve memory function. No significant difference in memory function was seen with the drug. ^[4]
• An errorless learning approach using a procedural memory task (i.e. learning of actual routes) in patients with Korsakoff syndrome and amnesia was compared to trial-and-error learning. No significant differences were found in the two approaches to learning. ^[5]

Controversy

Adding thiamine to alcoholic beverages and flour in an attempt to prevent Wernicke-Korsakoff syndrome has been discussed. ^[6] and has been the subject of some debate. ^[7]

Expected Outcome

If treated promptly, most symptoms of the syndrome can be reversed. However, improvement in memory function is slow.

Without prompt treatment, Wernicke-Korsakoff syndrome can be life-threatening.

History

Karl Wernicke, a German psychiatrist and neurologist, first described an illness that consisted of paralysis of eye movements, ataxia, and mental confusion in the 1880s. S.S. Korsakoff, a Russian psychiatrist, described memory disturbance in conjunction with peripheral neuropathy in alcoholic patients in a series of papers published in the late 1880s. The two disorders were believed to be part of the same disease process, which was named for both men.

References

1. ↑ Beltramo E, Berrone E, Tarallo S, Porta M. Effects of thiamine and benfotiamine on intracellular glucose metabolism and relevance in the prevention of diabetic complications. Acta Diabetol. 2008 Sep;45(3):131-41. Epub 2008 Jun 26. Abstract
2. ↑ Borsutzky S, Fujiwara E, Brand M, Markowitsch HJ. Confabulations in alcoholic Korsakoff patients. Neuropsychologia. 2008 Jul 15. [Epub ahead of print] Abstract
3. ↑ Kessels RP, Kortrijk HE, Wester AJ, Nys GM. Confabulation behavior and false memories in Korsakoff's syndrome: role of source memory and executive functioning. Psychiatry Clin Neurosci. 2008 Apr;62(2):220-5. Abstract
4. ↑ Luykx HJ, Dorresteijn LD, Haffmans PM, Bonebakker A, Kerkmeer M, Hendriks VM. Rivastigmine in Wernicke-Korsakoff's syndrome: five patients with rivastigmine showed no more improvement than five patients without rivastigmine. Alcohol Alcohol. 2008 Jan-Feb;43(1):70-2. Epub 2007 Nov 5. Abstract
5. ↑ Kessels RP, van Loon E, Wester AJ. Route learning in amnesia: a comparison of trial-and-error and errorless learning in patients with the Korsakoff syndrome. Clin Rehabil. 2007 Oct;21(10):905-11. Abstract
6. ↑ Centerwall BS, Criqui MH. Prevention of the Wernicke-Korsakoff syndrome: a cost-benefit analysis. N Engl J Med. 1978 Aug 10;299(6):285-9. Abstract
7. ↑ Bond HW, Homewood J. Wernicke's encephalopathy and Korsakoff's psychosis: to fortify or not to fortify? Neurotoxicol Teratol. 1991 Jul-Aug;13(4):353-5. Abstract

External Links

Family Caregiver Alliance

National Institute on Alcohol Abuse and Alcoholism