Wernicke-Korsakoff Syndrome

Wernicke-Korsakoff syndrome is a brain damage disorder which results from vitamin B1 (thiamine) deficiency. Thiamine is a water-soluble vitamin that is essential for the activity of several enzymes associated with energy metabolism. Thiamine is found naturally in meats such as pork and fish, and in whole grains. It is also added to many breads and cereals. The body is able to store between 30-50mg of thiamine. However, this can get depleted within 4-6 weeks if the diet is deficient, and the body’s thiamine-storing ability is reduced in those with alcohol-related liver damage. Thus, thiamine deficiency is common in people who have a poor dietary intake of vitamin-rich foods, alcohol use disorder, and in those who have malabsorption issues, which can occur with chronic illness or after bariatric surgery.

Wernicke syndrome, or Wernicke encephalopathy, and Korsakoff syndrome are two different conditions that often occur together. Wernicke encephalopathy is a neurological disease that, if not identified and treated early, can lead to death or a chronic and irreversible syndrome known as Korsakoff syndrome, which is associated with permanent brain damage. Symptoms of Wernicke encephalopathy include confusion, ataxia, vision changes and eye abnormalities. Symptoms of Korsakoff syndrome include amnesia, hallucinations and permanent brain damage. When the two syndromes occur together, the term Wernicke-Korsakoff syndrome is used, and most cases are caused by chronic consumption of alcohol. Wernicke-Korsakoff syndrome is difficult to diagnose and is based on clinical impression, and sometimes confirmed with magnetic resonance imaging (MRI) and neuropsychological assessments.

As Wernicke encephalopathy is considered a medical emergency, treatment should be started as soon as possible when the disease is suspected. Prompt treatment can prevent the disorder from developing into Wernicke-Korsakoff syndrome, or at least reduce its severity. Studies have shown that parenteral thiamine administration dramatically reduces Wernicke-Korsakoff syndrome-related mortality. However, the optimum thiamine dose is debated. Studies have shown that a dose of 200mg-500mg of thiamine delivered intravenously (IV) or intramuscularly (IM) three times daily for three to five days is suitable. This is followed by either thiamine 300mg either IV or IM daily for one to two weeks, or thiamine 100mg orally three times daily for one to two weeks. Whilst the patient is receiving the high dose of thiamine therapy, serum electrolytes, blood pressure, and renal function should be monitored. Serum magnesium, fluid, and electrolyte abnormalities should be corrected if abnormal.

Therapy with parenteral thiamine is generally considered safe. However, there is a risk of allergic reactions, so resuscitative measures, such as adrenaline and steroids, should be at the ready in case of anaphylaxis. Maintenance therapy of thiamine 100mg orally daily should continue once the high-dose thiamine course is completed.

Treatment with thiamine may improve symptoms of confusion or delirium, eye issues and lack of muscle coordination, but generally does not improve the loss of memory and intellect that occurs with Korsakoff syndrome. The patient is strongly encouraged to eat a well-balanced, healthy diet and to stop alcohol use in order to prevent further loss of brain function and nerve damage.