Tuesday, December 15, 2009

대사성 뇌질환 - 1. Wernicke-Korsakoff syndrome’s Treatment & Prognosis

Wernicke-Korsakoff syndrome’s Treatment & Prognosis

Treatment

Treatment consists of reversing the thiamine deficiency by giving supplemental thiamine, usually by starting with an initial intravenous or intramuscular dose, followed by supplemental oral doses. Some people think that it is important to start the thiamine treatment before giving any glucose, as the encephalopathy will be worsened by the glucose, however this is based only on case reports(Glucose administration promotes decarboxylation of pyruvate, a biochemical reaction which requires thiamine). By the time amnesia and psychosis have occurred, complete recovery is unlikely.

Nutritional

Individuals with signs of Wernicke's encephalopathy should be treated with thiamin immediately. In many cases, prompt administration of thiamin reverses the symptoms and prevents amnesia from developing.

Thiamin can be administered intravenously or directly into the digestive system. Unfortunately, thiamin is less effective in the chronic phase of the condition.

Based on autopsy findings suggesting the presence of Wernicke-Korsakoff syndrome in people with milder cognitive problems who do not show the classic signs of the disorder, researchers have examined the usefulness of thiamin treatment in people with alcohol dependence who are at risk of developing the syndrome.

Results suggest that thiamin treatment improves performance on memory tests in this group, and that higher thiamin doses are associated with better performance. These findings suggest that thiamin treatment can help prevent Wernicke-Korsakoff syndrome in heavy drinkers.

Medication

Recent reports suggest that donepezil and rivastigmine , drugs used to treat Alzheimer's disease , may improve memory in patients with Wernicke-Korsakoff syndrome. Both drugs prevent the breakdown of the neurotransmitter acetylcholine, which is important for the formation of memories.

Patients treated with these drugs showed improvements on memory tests and were more able to recognize hospital staff and family members. Although improvements appear to be rather modest, these drugs may be useful for patients who do not respond to thiamin.

Antidepressants that increase levels of serotonin may also be helpful, although the reasons why are not clear since these drugs are not effective with other memory disorders.

Conditioning

The fact that implicit memory is not affected by Wernicke-Korsakoff syndrome has led some researchers to explore the use of classical conditioning procedures in helping patients to remember specific people.

In classical conditioning, animals and people learn to associate a stimulus with an outcome. The most famous example is the pairing of a ringing bell with food. Dogs naturally salivate when given food.

In a famous experiment, Ivan Pavlov rang a bell immediately before serving food to dogs. After doing this repeatedly, Pavlov found that the dogs salivated upon hearing the bell ring even when the food was not presented. This form of learning does not rely on the hippocampus and cortex but appears to involve neurons in other parts of the brain.

Patients with Wernicke-Korsakoff syndrome who are given specific rewards for correctly choosing a picture of a face that matches a face they have seen previously are more able to choose the correct face than those who do not receive the rewards.

Although these individuals do not explicitly remember the face they saw previously, they are still able to make the correct choice. Training patients in this way could enable them to recognize familiar people and differentiate them from strangers.

Prognosis

The prognosis for full recovery from Wernicke-Korsakoff syndrome is poor. Once chronic Korsakoff's amnesia ensues, approximately 80% of patients will never fully recover the ability to learn and remember new information.

Because they cannot learn from experience, individuals with Wernicke-Korsakoff syndrome almost always require some form of custodial care. They are usually unable to work, although some can perform simple tasks they learned prior to onset of the condition if closely supervised.

Prevention

Wernicke-Korsakoff syndrome can be prevented with a nutritious diet containing sufficient thiamin. Because severe chronic alcoholism is the most common cause of thiamin deficiency, treatment of alcohol dependence is extremely important.

In order to prevent Wernicke-Korsakoff syndrome among people who are unable to stop drinking or among particularly vulnerable individuals like homeless drinkers, some researchers and clinicians have advocated supplementing alcoholic beverages with thiamin.