Key features

  • This brain disorder is characterized by a lack of thiamine (vitamin B-1). It involves two separate phases:
    1. Wernicke encephalopathy is the first, acute phase.
    2. Korsakoff psychosis is the long-lasting, chronic stage.
  • Symptoms of Korsakoff syndrome include:
    • Confusion, permanent gaps in memory and problems with learning new information.
    • Individuals may have a tendency to "confabulate" or "make up" information they can't remember. They are not necessarily "lying," but may actually believe the invented explanation.
    • Unsteadiness, muscle weakness and lack of coordination.
  • The most common risk factor is alcoholism, but the syndrome can also be associated with AIDS, cancers that have spread through the body, very high levels of thyroid hormone and certain other conditions. Recent research suggests that APOE-e4 – a variant of a gene that produces the protein apolipoprotein E – may be associated with a higher risk of Wernicke-Korsakoff in individuals who drink heavily. APOE-e4 is also linked to a higher risk of developing Alzheimer's.

Treatment

  • If the condition is caught early and drinking stops, treatment with high-dose thiamine may reverse some, but usually not all, damage.
  • In later stages, treatment will arrest progression of damage but may not reverse it.
  • A history of Korsakoff syndrome increases susceptibility to any future brain degeneration and raises the likelihood of later developing dementia.
  • The syndrome has become increasingly rare since fortification of foods with B-vitamins.
Pathology
Thiamine helps brain cells produce energy from sugar. When levels of thiamine become too low, cells are unable to generate enough energy to function properly. Such loss of function leads to the cognitive symptoms of Korsakoff syndrome.

For your patients and families: Korsakoff Syndrome