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What is Korsakoff’s Syndrome?

Korsakoff’s syndrome is a neurological disorder.  It is also known as Korsakoff’s dementia, Korsakoff’s psychosis, or amnesic-confabulatory syndrome).  It was first described by Sergei Korsakoff, a Russian neuropsychiatrist, in the late 19th century.  When it is accompanied by Wernicke’s encephalopathy, it is known as the Wernicke-Korsakoff syndrome.

The primary characteristics of Korsakoff’s syndrome include:

1.  anterograde amnesia, or difficulty recalling the recent past

2.  retrograde amnesia, or difficulty recalling memories stored before the onset of the disease

3.  confabulation, in which the person “makes up” information they cannot remember.  They are not lying, but actually believe what their brain tells them is true.

4.  conversation that appears to be relatively normal, except that the person will often not recall that the interaction has taken place

5.  poor insight into or awareness of the condition, or anosognosia

6.  apathy

7.  ataxia, or an unsteady gait

8.  coma

9.  paralysis of muscles controlling the eye

10.  tremor

The syndrome develops when the brain becomes deficient in thiamine, or Vitamin B1, most often due to chronic alcoholism and severe malnutrition.  It can also be caused by prolonged vomiting, eating disorders, the effects of chemotherapy, a form of extreme morning sickness in pregnant women, mercury poisoning, or the bite of Japanese centipedes.

One theory is that this causes damage to the dorsomedial nucleus of the thalamus and mamillary bodies of the hypothalamus, along with generalized cerebral atrophy.  It involves damage to neurons, gliosis which results from damage to the supporting cells of the central nervous system, and bleeding in the mamillary bodies.  Cortical dysfunction may arise as a result of thiamine deficiency, alcohol neurotoxicity, and/or structural damage to the diencephalon.  Research has shown that there is a decrease in glucose metabolism in the frontal, parietal, and cingulate portions of the brains of persons with Korsakoff’s.

Spatial, verbal, and procedural memory usually remains intact in persons with Korsakoff’s syndrome.  They do have impaired executive functions, potentially causing behavioral problems and interfering with the person’s ability to perform daily activities.  It was originally theorized that the person with Korsakoff’s used confabulation to fill in gaps left by memory impairments, but research has shown that confabulation and amnesia do not always occur together.  The person may actually show one or more of three different types of confabulation:  provoked, spontaneous, and false memories.  In other words, the persons may be led to believe that certain things have happened when they have not.

Diagnosis of Korsakoff’s syndrome is similar to many other forms of dementia, in which other possible reasons for the characteristics displayed by the person must first be ruled out.  The symptoms may also be masked by other signs often found in a person who abuses alcohol, including intoxication or withdrawal, infection, and head injury.  There are no specific brain imaging studies or lab tests that will confirm the presence of the disorder while the person afflicted with it is still living, although research is beginning to suggest some possibilities for the future.

Several factors may place a person at risk for development of Korsakoff’s.  These include AIDS, chemotherapy, dialysis, extreme dieting, age, and genetic factors.  It is found in approximately 3% of the population.

Korsakoff’s syndrome is a degenerative disease, and the person who is afflicted with it will eventually require total care.  It is sometimes possible for the person to recover some degree of independence, following replacement or supplementation of thiamine by intravenous or intramuscular injection, along with hydration and proper nutrition.  It is estimated that only about 20% of cases are reversible, with treatment lasting from three to twelve months and recovery taking up to two years.

There have been a number of cases of Korsakoff’s syndrome recounted in the literature and the popular media.  Oliver Sacks describes a case study in his book The Man Who Mistook His Wife For a Hat.  Others include German actor Harald Juhnke, Australian artist Charles Blackman, and Australian actor Graham Kennedy.  It is speculated that Boston Corbett, the man who shot John Wilkes Boothe, developed the disorder in later life.  The syndrome is also given as a possible diagnosis for characters in the television series “House,”  “Royal Pains,” “A Gifted Man,” and “Perception,” as well as several other movies, books, and plays.

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