Wernicke-Korsakoff syndrome

Answer 1

Definition

Wernicke-Korsakoff syndrome is a brain disorder due to thiamine deficiency.

Alternative Names

Korsakoff psychosis; Alcoholic encephalopathy; Encephalopathy - alcoholic; Wernicke's disease

Causes, incidence, and risk factors

Wernicke's encephalopathy and Korsakoff syndrome are different conditions that are both due to brain damage caused by a lack of vitamin B1 (thiamine).

A lack of vitamin B1 is common in people with alcoholism. It is also common in persons whose bodies do not absorb food properly (malabsorption), such as sometimes occurs after obesity surgery.

Korsakoff syndrome, or Korsakoff psychosis, tends to develop as Wernicke's symptoms go away. Wernicke's encephalopathy causes brain damage in lower parts of the brain called the thalamus and hypothalamus. Korsakoff psychosis results from damage to areas of the brain involved with memory.

Symptoms

Symptoms of Wernicke's encephalopathy:

• Confusion
• Loss of muscle coordination (ataxia)
  • Leg tremor
• Vision changes
  • Abnormal eye movements (back and forth movements called nystagmus)
  • Double vision
  • Eyelid drooping

Symptoms of Korsakoff syndrome:

• Inability to form new memories
• Loss of memory, can be severe
• Making up stories (confabulation)
• Seeing or hearing things that aren't really there (hallucinations)

Note: There may also be symptoms of alcohol withdrawal.

Signs and tests

Examination of the nervous/muscular system may show damage to many nerve systems:

• Abnormal eye movement
• Decreased or abnormal reflexes
• Fast pulse(heart rate)
• Low blood pressure
• Low body temperature
• Muscle weakness and atrophy (loss of tissue mass)
• Problems with walk (gait) and coordination

The person may appear poorly nourished. The following tests are used to check a person's nutrition level:

• Serum albumin(relates to person's general nutrition)
• Serum vitamin B1 levels
• Transketolase activity in red blood cells (reduced in people with thiamine deficiency)

Blood or urine alcohol levels and liver enzymes may be high in people with a history of long-term alcohol abuse.

Other conditions that may cause thiamine deficiency include:

• AIDS
• Cancers that have spread throughout the body
• Extreme nausea and vomiting during pregnancy (hyperemesis gravidarum)
• Heart failure(when treated with long-term diuretic therapy)
• Long periods of intravenous (IV) therapy without receiving thiamine supplements
• Long-term dialysis
• Very high thyroid hormone levels (thyrotoxicosis)

A brain MRI may show changes in the tissue of the brain, but if Wernicke-Korsakoff syndrome is suspected, treatment should start immediately. Usually a brain MRI exam is not needed.

Treatment

The goals of treatment are to control symptoms as much as possible and to prevent the disorder from getting worse. Some people may need to stay in the hospital early in the condition to help control symptoms.

Monitoring and special care may be needed if the person is:

• Comatose
• Lethargic
• Unconscious

Thiamine may be given by injection into a vein or a muscle, or by mouth. It may improve symptoms of:

• Confusion or delirium
• Difficulties with vision and eye movement
• Lack of muscle coordination

Thiamine usually does not improve loss of memory and intellect that occur with Korsakoff psychosis.

Stopping alcohol use can prevent additional loss of brain function and damage to nerves. Eating a well-balanced, nourishing diet can help, but it is not a substitute for stopping alcohol use.

Support Groups

You can often ease the stress of illness by joining a support group where members share common experiences and problems. See alcoholism - support group.

Expectations (prognosis)

Without treatment, Wernicke-Korsakoff syndrome gets steadily worse and can be life threatening. With treatment, you can control symptoms (such as uncoordinated movement and vision difficulties), and slow or stop the disorder from getting worse.

Some symptoms -- especially the loss of memory and thinking skills -- may be permanent. Other disorders related to alcohol abuse may also occur.

Complications

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

In people at risk, Wernicke's encephalopathy may be caused by carbohydrate loading or glucose infusion. Always supplement with thiamine before glucose infusion to prevent this.

Calling your health care provider

Call your health care provider or go to the emergency room if you have symptoms of Wernicke-Korsakoff syndrome, or if you have been diagnosed with the condition and your symptoms get worse or return.

Prevention

Not drinking alcohol or drinking in moderation and getting enough nutrition reduce the risk of developing Wernicke-Korsakoff syndrome. If a heavy drinker will not quit, thiamine supplements and a good diet may reduce the chance of getting this condition, but do not eliminate the risk.

References

Brust JCM. Nutrition and alcohol-related neurologic disorders. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 443.

Answer 2

Definition

Wernicke-Korsakoff syndrome is a brain disorder due to thiamine deficiency.

Alternative Names

Korsakoff psychosis; Alcoholic encephalopathy; Encephalopathy - alcoholic; Wernicke's disease

Causes, incidence, and risk factors

Wernicke's encephalopathy and Korsakoff syndrome are different conditions that are both due to brain damage caused by a lack of vitamin B1 (thiamine).

A lack of vitamin B1 is common in people with alcoholism. It is also common in persons whose bodies do not absorb food properly (malabsorption), such as sometimes occurs after obesity surgery.

Korsakoff syndrome, or Korsakoff psychosis, tends to develop as Wernicke's symptoms go away. Wernicke's encephalopathy causes brain damage in lower parts of the brain called the thalamus and hypothalamus. Korsakoff psychosis results from damage to areas of the brain involved with memory.

Symptoms

Symptoms of Wernicke's encephalopathy:

• Confusion
• Loss of muscle coordination (ataxia)
  • Leg tremor
• Vision changes
  • Abnormal eye movements (back and forth movements called nystagmus)
  • Double vision
  • Eyelid drooping

Symptoms of Korsakoff syndrome:

• Inability to form new memories
• Loss of memory, can be severe
• Making up stories (confabulation)
• Seeing or hearing things that aren't really there (hallucinations)

Note: There may also be symptoms of alcohol withdrawal.

Signs and tests

Examination of the nervous/muscular system may show damage to many nerve systems:

• Abnormal eye movement
• Decreased or abnormal reflexes
• Fast pulse(heart rate)
• Low blood pressure
• Low body temperature
• Muscle weakness and atrophy (loss of tissue mass)
• Problems with walk (gait) and coordination

The person may appear poorly nourished. The following tests are used to check a person's nutrition level:

• Serum albumin(relates to person's general nutrition)
• Serum vitamin B1 levels
• Transketolase activity in red blood cells (reduced in people with thiamine deficiency)

Blood or urine alcohol levels and liver enzymes may be high in people with a history of long-term alcohol abuse.

Other conditions that may cause thiamine deficiency include:

• AIDS
• Cancers that have spread throughout the body
• Extreme nausea and vomiting during pregnancy (hyperemesis gravidarum)
• Heart failure(when treated with long-term diuretic therapy)
• Long periods of intravenous (IV) therapy without receiving thiamine supplements
• Long-term dialysis
• Very high thyroid hormone levels (thyrotoxicosis)

A brain MRI may show changes in the tissue of the brain, but if Wernicke-Korsakoff syndrome is suspected, treatment should start immediately. Usually a brain MRI exam is not needed.

Treatment

The goals of treatment are to control symptoms as much as possible and to prevent the disorder from getting worse. Some people may need to stay in the hospital early in the condition to help control symptoms.

Monitoring and special care may be needed if the person is:

• Comatose
• Lethargic
• Unconscious

Thiamine may be given by injection into a vein or a muscle, or by mouth. It may improve symptoms of:

• Confusion or delirium
• Difficulties with vision and eye movement
• Lack of muscle coordination

Thiamine usually does not improve loss of memory and intellect that occur with Korsakoff psychosis.

Stopping alcohol use can prevent additional loss of brain function and damage to nerves. Eating a well-balanced, nourishing diet can help, but it is not a substitute for stopping alcohol use.

Support Groups

You can often ease the stress of illness by joining a support group where members share common experiences and problems. See alcoholism - support group.

Expectations (prognosis)

Without treatment, Wernicke-Korsakoff syndrome gets steadily worse and can be life threatening. With treatment, you can control symptoms (such as uncoordinated movement and vision difficulties), and slow or stop the disorder from getting worse.

Some symptoms -- especially the loss of memory and thinking skills -- may be permanent. Other disorders related to alcohol abuse may also occur.

Complications

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

In people at risk, Wernicke's encephalopathy may be caused by carbohydrate loading or glucose infusion. Always supplement with thiamine before glucose infusion to prevent this.

Calling your health care provider

Call your health care provider or go to the emergency room if you have symptoms of Wernicke-Korsakoff syndrome, or if you have been diagnosed with the condition and your symptoms get worse or return.

Prevention

Not drinking alcohol or drinking in moderation and getting enough nutrition reduce the risk of developing Wernicke-Korsakoff syndrome. If a heavy drinker will not quit, thiamine supplements and a good diet may reduce the chance of getting this condition, but do not eliminate the risk.

References

Brust JCM. Nutrition and alcohol-related neurologic disorders. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 443.

Reviewed By

Review Date: 02/06/2010

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Luc Jasmin, MD, PhD, Department of Neurosurgery at Cedars-Sinai Medical Center, Los Angeles, and Department of Anatomy at UCSF, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.