Wernicke-Korsakoff syndrome is a brain disorder due to thiamine deficiency.
Wernicke’s encephalopathy and Korsakoff syndrome are believed to be two stages of the same condition.
Wernicke’s encephalopathy is caused by damaging changes in the brain, usually due to a lack of vitamin B1 (thiamine).
A lack of vitamin B1 is common in people with alcoholism. Heavy alcohol use affects the breakdown of thiamine in the body. Even if someone who drinks alcohol heavily follows a well-balanced diet, most of the thiamine is not absorbed.
Korsakoff syndrome, or Korsakoff psychosis, tends to develop as Wernicke’s symptoms go away. Korsakoff psychosis involves damage to areas of the brain involved with memory.
* Inability to form new memories
* Loss of memory, can be severe
* Loss of muscle coordination (ataxia)
o Unsteady, uncoordinated walking
* Making up stories (confabulation)
* Seeing or hearing things that aren’t really there (hallucinations)
* Vision changes
o Abnormal eye movements
o Double vision
o Eyelid drooping
Note: There may also be symptoms of alcohol withdrawal.
Wernicke’s encephalopathy is diagnosed when patients seek medical attention and have the classic trio of signs: mental confusion, eye movement disorders, and ataxia. The diagnosis of Korsakoff syndrome is given when anterograde amnesia is present in an individual with a history of chronic, heavy drinking or malnutrition. When Korsakoff syndrome follows Wernicke’s encephalopathy, the entire Wernicke-Korsakoff syndrome diagnosis is appropriate. The diagnosis is supported by neuroimaging or autopsy findings showing degeneration of the thalamus and mammillary bodies and loss of brain volume in the area surrounding the fourth ventricle – a fluid-filled cavity near the brainstem.
Although DSM-IV-TR criteria for alcohol-induced persisting amnestic disorder apply to most people with Wernicke-Korsakoff syndrome, there are some differences between the two diagnoses. Despite research findings suggesting that severe amnesia is not a necessary symptom of Wernicke-Korsakoff syndrome, the DSM-IVTR requires the presence of either anterograde or retrograde amnesia for a diagnosis of alcohol-induced persisting amnestic disorder. One additional cognitive symptom is also required. Symptoms listed in the DSM-IV-TR include language disturbance (aphasia), inability to carry out motor activities (apraxia), inability to recognize objects (agnosia), or deficits in planning, initiation, organization and abstraction (executive functions). Individuals with Wernicke-Korsakoff syndrome frequently demonstrate problems with executive functions that contribute to the symptoms of confabulation and apathy. Aphasia, apraxia, and agnosia are not common signs of Wernicke-Korsakoff syndrome.
The DSM-IV-TR also requires that memory impairment must significantly impair a person’s ability to perform normal activities and functions, and it must represent a decline from a previous level of functioning. Amnesia cannot occur exclusively during states of delirium , alcohol intoxication, or withdrawal, or be exclusively associated with a dementia. Both of the these requirements are consistent with the usual presentation of Wernicke-Korsakoff syndrome.
Finally, the DSM-IV-TR requires evidence that amnesia is caused by use of alcohol. Such evidence can include an extensive history of heavy drinking; or physical examination or laboratory findings revealing other signs of heavy alcohol use, such as abnormal liver function tests. Despite this DSM-IV-TR requirement, Wernicke-Korsakoff’s syndrome can occur in the absence of heavy alcohol use. Emergence of the disorder in people without alcoholism is much less common today than it was in the past, however, since vitamins are now added to many foods. In practice, most people who show the hallmark symptoms of Wernicke-Korsakoff syndrome also qualify for the DSM-IV-TR diagnosis.
Exams and Tests
Examination of the nervous/muscular system may show damage to many nerve systems:
* Decreased or abnormal reflexes
* Problems with walk (gait) and coordination
* Muscle weakness and atrophy (loss of tissue mass)
* Abnormal eye movement
* Low blood pressure
* Low body temperature
* Fast pulse (heart rate)
The person may appear poorly nourished. The following tests are used to check a person’s nutrition level:
* Serum B1 levels
* Transketolase activity
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:
* 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 in rare cases shows changes in the tissue of the brain.
* Psychosis Polyneurotica
* Wernicke Disease
* Wernicke Syndrome
* Korsakoff Psychosis
* Korsakoff Amnesic Syndrome
* Korsakoff Syndrome
* Wernicke Encephalopathy
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:
Thiamine (vitamin B1) 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 does not usually improve loss of memory and intellect that occur with Korsakoff psychosis.
Stopping alcohol use can prevent loss of brain function and damage to nerves. Eat a well-balanced, nourishing diet.
You can often ease the stress of illness by joining a support group where members share common experiences and problems.
* 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.
When to Contact a Medical Professional
Call your health care provider if you have symptoms of Wernicke-Korsakoff syndrome, or if you have been diagnosed with the condition and your symptoms get worse or return.
Also call if new symptoms develop, especially symptoms of alcohol withdrawal. Alcohol withdrawal can be fatal, so call the local emergency number (such as 911) or go to the emergency room if any severe symptoms occur.
Symptoms of alcohol withdrawal include:
* Delirium or confusion
* Fast heart rate
* Jumpiness or nervousness
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 help prevent this condition, but not if damage has already occurred.
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.
Reviewed By: Luc Jasmin, MD, PhD, Departments of Anatomy & Neurological Surgery, University of California, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.