What is Wernicke-Korsakoff Syndrome?

The Wernicke-Korsakoff syndrome is a consequence of a deficiency of thiamine (vitamin B) and can be caused by years of excessive alcohol consumption. Colloquially it is also simply called Korsakoff. A well-known patient was Ramses Shaffy. What are the causes and characteristics of this and how is it treated?

Wernicke-Korsakoff syndrome

  • Wernicke-Korsakoff syndrome
  • Symptoms of Korsakoff’s psychosis
  • Symptoms of Wernicke’s encephalopathy
  • Causes
  • Therapy

 

Wernicke-Korsakoff syndrome

The Wernicke-Korsakoff syndrome is a combination of two different syndromes: Korsakoff’s psychosis and Wernicke’s encephalopathy , named after its discoverers Dr. Sergei Korsakoff and Dr. Carl Wernicke. The condition is quite well known because it is relatively common in people with a long history of alcoholism. For example, singer Ramses Shaffy suffered from the condition.

Symptoms of Korsakoff’s psychosis

Korsakoff’s psychosis is characterized by the following symptoms:

  • Confusion
  • Memory loss (particularly short-term memory is affected)
  • Overconfidence and disregard for others
  • Flattening of the emotional experience
  • Confabulation (telling exaggerated, fantasized or untrue stories)
  • Disorientation

 

Symptoms of Wernicke’s encephalopathy

Wernicke’s encephalopathy is characterized by the following symptoms:

  • Nystagmus (uncontrolled movement of the eyes)
  • Ophthalmoparesis (weakness or paralysis of the eye muscle)
  • Poor reflexes of the eye muscle
  • Anisocoria (difference in size between the two pupils)
  • Ataxia (disorders of balance and movement coordination)
  • Coma and death if not treated

 

Causes

Wernicke-Korsakoff syndrome is the result of a deficiency of thiamine (vitamin B). It is almost certain that Wernicke’s encephalopathy is caused by a severe acute deficiency of thiamine, while Korsakoff’s psychosis is a chronic and neurological condition post-Wernicke’s encephalopathy. The metabolically active form of thiamine is thiamine diphosphate, which plays an important role as a cofactor in glucose metabolism.
Wernicke-Korsakoff occurs mainly in chronic alcoholics, but it also occurs in people who are on a drip for a long time without vitamin B being administered. In some regions, thiamine deficiency can be caused by long-term consumption of polished rice that is poor in thiamine, which can lead to Beriberi, which affects the cardiovascular system. The condition can then manifest itself overnight.
The Wernicke-Korsakoff syndrome in alcoholics is associated with atrophy of specific locations in the brain, especially the corpus mamillare . Other sites affected are the posterior region of the thalamus (which explains the history), the medial dorsal thalamus, anterior lobe, and the dorsal raphe nuclei .

Therapy

Treatment consists of counteracting the thiamine deficiency by giving thiamine supplements, often with intravenous or intramuscular injections, followed by an oral dose. It is important to initiate thiamine therapy before giving glucose because the encephalopathy is worsened by glucose. If the anamnesis and psychoses have already started, complete recovery is virtually impossible. Many patients are therefore admitted to a care or nursing home. The Netherlands also has several specialized Korsakov clinics and Erasmus University has developed a special care program for patients.

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