Wernicke-Korsakoff Syndrome – Symptoms, Treatment, Diagnosis

What is Wernicke-Korsakoff Syndrome?

Wernicke-Korsakoff Syndrome is due to nutritional deficiency (particularly of thiamine or Vitamin B1) that causes neurologic manifestations such as visual changes, ataxia, and memory impairment. This is usually seen among alcoholic patients since alcohol impairs the absorption of Vitamin B1.

Wernicke-Korsakoff Syndrome Affecting the Wernicke Area in the Brain

Distinction Between Wernicke encephalopathy and Korsakoff’s Psychosis

Although this condition is taken as one syndrome, it is actually a combination of two separate conditions that very often occur together: Wernicke’s encephalopathy and Korsakoff’s psychosis.  Wernicke’s encephalopathy is easier and faster to cure than Korsakoff’s psychosis, while the latter may take quite some time to handle. Another distinction between the two is each of them presents a myriad of signs and symptoms unique to each other.

Wernicke-Korsakoff Syndrome Symptoms

Since Wernicke- korsakoff Syndrome is a combination of two separate conditions, the signs and symptoms would also be different for each.

Wernicke’s Encephalopathy

• Nystagmus or the involuntary movement of the eye
• Anisocoria or the unequal pupil size
• Ataxia or the lack of coordination
• Pupillary reflexes that are sluggish
• Confusion
• Ophthalmoplegia or impaired movement of the eye

Korsakoff’s Psychosis

• Hallucinations
• Anterograde amnesia loss of memories that happen after an amnesic event
• Retrograde amnesia or loss of memories that happened prior to the amnesia
• Confabulation or false memories

Causes

Vitamin B1 Deficiency

The primary cause of Wernicke- korsakoff Syndrome is the lack in Vitamin B1 or thiamine. Thiamine or Vitamin B1 is known to participate in significant processes in the body. that is why its deficiency affects almost all organs in the body, however the brain and the heart are systems most affected by this deficiency.

Thiamine is known to participate in glucose metabolism, carbohydrate metabolism and energy production. These three processes are essential in the maintenance of brain and nervous system functions. Thiamine is also known to contribute to neural health by enhancing the signals between neurons found in the brain and all over the body.

Pathogenesis of Thiamine Deficiency and Wernicke- korsakoff Syndrome

Since thiamine or Vitamin B1 is an essential co-enzyme to glucose metabolism, a lack of it greatly affects this process. Glucose is the main “brain food”, this is the nutrient that fuels the brain cells to work and function properly. Glucose is produced through glucose metabolism, and without thiamine aiding in this process, the brain is rid of its essential nutrient.

Furthermore, B Vitamins such as Thiamine also exert therapeutic effects on the brain. these compounds enhance neural transmission from neuron to neuron, thus promoting good brain function.

Vitamin B1 Deficiency can be a result of the following conditions:

• Severe malnutrition
• Alcoholism
• Prolonged Intravenous therapy
• Chronic conditions requiring prolonged stay in an intensive care unit
• Gastric stapling

Wernicke-Korsakoff Syndrome and Alcoholism

In Western countries Wernicke- korsakoff Syndrome is seen to be a common problem. Chronic alcoholism impairs the absorption of B Vitamins such as Thiamine; the lack of thiamine then leads to this condition. Furthermore, chronic alcoholism also causes changes in the brain structure. Atrophy or infarction of brain areas are seen among chronic alcoholics. The structural change worsens the nutritional deficiency and the syndrome. Atrophy (decrease in size) and infarction (tissue death) happens in brain areas such as the mammillary bodies, the thalamus, and basal forebrain. The affectation of such areas would explain the different behavioral and memory changes seen in Wernicke- korsakoff Syndrome.

Wernicke-Korsakoff Syndrome and Severe Malnutrition

Asian countries whose primary diet comprises of solely refined rice can suffer from malnutrition and in turn Wernicke- korsakoff Syndrome. However, this is not often seen as vitamin B deficiency in this case is in the form of beri-beri. This is also a nervous system disorder with muscle weakness as its main characterisitic.

Wernicke-Korsakoff Syndrome Diagnosis

This condition can be diagnosed with the following methods:

• Neuropyschological Assessment

A complete and comprehensive neuropsychological assessment can confirm or diagnose Wernicke- korsakoff Syndrome. This involves assessment for anterograde and retrograde amnesia, confabulation (which is a characteristic of the disease) and ataxia and nystagmus.

• Imaging Studies such as MRI and CT Scan

This is to confirm for the atrophy and degeneration of the mammillary bodies.

Wernicke-Korsakoff Syndrome Treatment

Vitamin B1 Supplementation

This is the mainstay treatment for Wernicke- korsakoff Syndrome. Supplementation is initially done intravenously then followed by intramuscular doses then oral doses. While on Vitamin B1 supplementation, the individual is not yet given high amounts of glucose. This is because deficiency might still not be corrected during the initial phase, and high amounts of glucose can exacerbate the encephalopathy.

Symptom and Supportive Management such as:

Constant Reorientation

Patients who manifest Wernicke’s encephalopathy often have confusion, to aid to these patients with Wernicke- korsakoff Syndrome must be constantly oriented to their environment, the time and the person they are with. Keeping a calendar nearby and frequently feeding the patient information such as time, place and persons around can manage this problem.

Assistive Devices

Assistive devices are also helpful, especially for those with ataxia or uncoordinated movement.

Visual management

Wernicke- korsakoff Syndrome patients also suffer from vision problems because of weakness of the muscles controlling the eye. They may only have central vision or double vision. Patients must be instructed to close when eye if double vision is experienced, and family members must position themselves directly in from of the patient if central vision is only present.

Once Korsakoff’s psychosis settles in, complete recovery from the condition becomes difficult and the individual might need intense neuropsychiatric management.

Prognosis

When Wernicke- korsakoff Syndrome is not treated immediately prognosis becomes poor. A person’s recovery and the likelihood he/she would assume normal functioning becomes unlikely when the Korsakoff’s psychosis settles in.

Complications

When untreated Wernicke- korsakoff Syndrome can lead to:

• Coma
• Permanent brain damage
• Cognitive and behavioral changes
• Psychosis
• Permanent amnesia and memory loss
• Death if untreated

Prevention

To prevent this condition an individual must:

• Good supplement of Vitamin B1 by eating grains, nuts, whole grains, oatmeals, asoaragus, eggs., and potatoes
• Managing Chronic alcoholism