Korsakow Syndrome – the Alcoholic Dementia

With long-term, heavy alcohol consumption, not only is a strained liver to be expected, but the addiction can permanently damage the brain. Similar to age-related dementia, Korsakoff syndrome can lead to severe impairment of memory and retention. What is Korsakoff’s disease, what are the symptoms and how is it treated? You can read that and more here.

Definition: What is Korsakoff Syndrome?

Korsakoff syndrome, also known as Korsakov’s disease, is damage to the brain with pronounced memory disorders, which is often the result of long-term alcohol addiction. Korsakoff syndrome often occurs after what is known as Wernicke encephalopathy – a brain disease caused by a vitamin B1 deficiency. This can also arise in connection with chronic alcohol abuse. Therefore, the occurrence of both diseases is often related, and one then speaks of Wernicke-Korsakov syndrome.

The cause of Korsakoff’s syndrome is the lack of vitamin B1. Vitamin deficiency affects the metabolic processes in the brain and, as a result of cell death, neurological functional disorders occur, which in Korsakoff syndrome are particularly noticeable in the impairment of short-term memory.

However, Korsakoff syndrome can also occur as part of other brain damage, for example, as a result of infections (herpes encephalitis), craniocerebral trauma, bleeding in the brain or after carbon monoxide poisoning.

 

Symptoms of Korsakoff Syndrome

The following symptoms make Korsakov’s disease noticeable:

• disorientation
• severe memory impairment
• decreased memory
• Inability to place memory content (e.g. biographical memories) in the correct context
• Confabulations: the unintentional invention of information content
• drive reduction
• withdrawal from the outside world
• distant or indifferent behaviour due to loss of ability to show feelings (flattening of effect)
• reduced critical ability

Main symptom disorientation

A particularly striking sign of Korsakoff syndrome is the pronounced disorientation. Those affected lose the ability to express themselves about the time and place of the situation or themselves. This can take on forms in which they no longer know what day or year it is, where they are, and how they got to this place or situation. Finally, knowledge about oneself, for example, who they are or what job they do, is also increasingly lost.

 

Amnestic Syndrome: When the ability to remember fails

The various forms of memory disorders are summarized in the so-called amnestic syndrome. People with Korsakov’s disease find it challenging to recall older memories from the past (retrograde amnesia) and remember new information (anterograde amnesia). Anterograde amnesia also makes it challenging to learn new cognitive skills. Typically, short-term memory is particularly affected in Korsakoff syndrome. After a few minutes, previously learned content can no longer be played back. Secondary memory and consciousness, on the other hand, are undisturbed.

Time lattice disturbances can also occur. Various memory contents can no longer be classified in the correct chronological order.

Since sufferers are often unaware of their memory impairment, they often tend to fill in the gaps in their memory with invented content. In medicine, this phenomenon is described as confabulation. However, it is not a matter of deliberately lying to others, but those affected believe in the truthfulness of their stories.

Even if Korsakoff’s syndrome is often referred to as alcohol dementia, the amnestic syndrome is not one of the dementia diseases since intellectual disorders do not occur or are not the main focus of the syndrome.

How does Korsakov’s disease affect social life?

Those affected by Korsakoff’s disease do not only suffer from the difficulties of memory disorders. If the frontal brain function is impaired, there can also be a lack of drive and reduced intensity of the feelings shown. Bystanders often perceive Korsakow sufferers as introspective and distant from what is happening in the outside world.

Less spontaneity, depression or aggressiveness are also often observed, which is probably due to long-term alcohol dependence.

Wernicke encephalopathy causes other symptoms.

Korsakoff syndrome often develops from untreated Wernicke encephalopathy, which is a neurological emergency caused by chronic vitamin B1 deficiency. In the case of previous Wernicke encephalopathy, the following additional symptoms can occur depending on the location of the damage in the brain:

• confusion
• disturbances of consciousness
• Eye movement disorders
• Change in gait pattern due to disturbances in the coordination of movement ( ataxia )
• Dysregulation of the autonomic nervous system: drop in core body temperature, drop in blood pressure

 

Causes: how does Korsakoff syndrome develop?

Korsakoff syndrome develops based on a long-standing vitamin B1 deficiency (thiamine deficiency), which is often caused by long-term alcohol dependence. It is not necessarily the alcohol itself that has the damaging effect on the brain, but the lack of a balanced diet with foods containing vitamin B1 caused by alcohol consumption. In addition, the alcohol also reduces the activity of enzymes that bring the vitamin B1 into its practical form.

The lack of the thiamine vitamin can also be caused by an absorption disorder independent of the alcohol. This can be caused by malignant tumours, surgical interventions in the gastrointestinal system or anorexia due to malnutrition or malnutrition. Excessive vomiting also decreases thiamine absorption in the gut, as does parenteral nutrition of ICU patients via IV fluids.

Why is vitamin B1 so crucial for the brain?

Thiamine pyrophosphate, the active form of thiamine, acts as an “assistant” enzyme in carbohydrate metabolism. Due to a lack of the vitamin, the body can no longer break down carbohydrates to the usual extent. Disorders of the cerebral energy metabolism and the destruction of essential neurons in the brain occur—the affected brain regions atrophy.

In addition to the thalamic core areas of the brain, the corpora mammillaria, which is essential for memory and learning processes, is particularly affected, a part of the hypothalamus that begins to shrink due to haemorrhaging.

Diagnosis of Korsakoff’s syndrome

The main focus of the diagnosis is the clinical picture of the symptoms typical of Korsakov’s disease and the presence of risk factors causing a vitamin B1 deficiency, such as long-term alcohol abuse. A detailed anamnesis with the person concerned and possibly the relatives or nursing staff usually provides information.

Various neuropsychological tests are carried out to get a picture of the person’s neurological status. These tests include, among other things, biographical questions and test knowledge of current events. The person being examined must remember lists of words and be able to recall them later. This allows the doctor to recognize which forms of memory disorders are present and distinguish them from similar diseases such as Alzheimer’s dementia.

MRI (magnetic resonance imaging) can also show reduced mamillary bodies, characteristic of regression . However, inconspicuous findings here do not rule out Korsakov’s disease, so the diagnosis is mainly based on the symptomatic picture and the anamnesis.

The causative vitamin B1 deficiency can also be detected by taking a blood sample through a reduced thiamine concentration.

 

Treatment of Wernicke-Korsakov Syndrome

There is no disease-specific therapy for a pronounced Korsakoff syndrome. However, previous Wernicke encephalopathy can be treated with high-dose intravenous thiamine. Treatment should be started as soon as there is suspicion since the untreated progression of the disease to Korsakoff syndrome dramatically reduces the chances of recovery.

In the future, switching to oral vitamin administration is possible. In addition, rehabilitation training is carried out to improve neuropsychological functions.

An essential therapy component to prevent further damage to cognitive performance is to change eating habits towards a balanced diet that covers all essential nutrients and vitamins. Thiamine is found in pork, potatoes, whole grains, oatmeal, and nuts, among other things.

How long can you live with Korsakoff Syndrome?

Korsakoff syndrome does not have an excellent long-term prognosis, but life expectancy depends heavily on the extent of the brain damage that has already occurred. Immediate abstinence from alcohol and switching to a balanced diet may help short-term memory problems to resolve, but full recovery cannot be guaranteed.

The mortality rate with optimal therapy for Wernicke-Korsakov disease is 15 to 20 per cent—an improvement after therapy occurs in around one-seventh of those affected.

Caring for Korsakoff patients

Since the diagnosis is often delayed or made too late, permanent impairments can lead to severe limitations in independence in everyday life, so those affected need care from relatives or nursing homes. Managing end-stage Korsakoff disease is often not easy, and caregivers usually need help from staff trained in the disease.

In addition to the specific symptoms of Korsakoff’s syndrome, those affected often have to contend with other organ-specific consequences as a result of long-term alcohol abuse. Liver damage, such as fatty liver and liver cirrhosis, is typical, but alcohol-related polyneuropathy can also develop.