Korsakoff's syndrome

Korsakoff's syndrome (also called Korsakoff's dementia, Korsakov's syndrome, Korsakoff's psychosis, or amnesic-confabulatory syndrome) is a neurological disorder caused by the lack of thiamine (vitamin B₁) in the brain. Its onset is linked to chronic alcohol abuse and/or severe malnutrition. The syndrome is named after Sergei Korsakoff, the neuropsychiatrist who popularized the theory.

Symptoms

There are six major symptoms of Korsakoff's syndrome:

1. anterograde amnesia
2. retrograde amnesia, severe memory loss
3. confabulation, that is, invented memories which are then taken as true due to gaps in memory sometimes associated with blackouts
4. meager content in conversation
5. lack of insight
6. apathy - the patients lose interest in things quickly and generally appear indifferent to change.

These symptoms are caused by a deficiency of thiamine (vitamin B₁), which is thought to cause damage to the medial thalamus and possibly to the mammillary bodies of the hypothalamus as well as generalized cerebral atrophy.^[1]

When Wernicke's encephalopathy accompanies Korsakoff's syndrome, the combination is called the Wernicke-Korsakoff syndrome. Korsakoff's is a continuum of Wernicke's encephalopathy, though a recognised episode of Wernicke's is not always obvious.

Korsakoff's involves neuronal loss, that is, damage to neurons; gliosis which is a result of damage to supporting cells of the central nervous system; and hemorrhage or bleeding in mammillary bodies. Damage to the dorsomedial nucleus or anterior group of the thalamus (limbic-specific nuclei) is also associated with this disorder.

Signs

• Apathy
• Ataxia
• Coma
• Confabulation
• Paralysis of muscles controlling the eye
• Retrograde and anterograde amnesia
• Tremor
• Anosognosia - Lack of insight to or awareness of the condition

Treatment

It was once assumed that anyone suffering from Korsakoff's syndrome would eventually need full time care. This is still often the case, but rehabilitation can help regain some, often limited, level of independence.^[2] Treatment involves the replacement or supplementation of thiamine by intravenous (IV) or intramuscular (IM) injection, together with proper nutrition and hydration. However, the amnesia and brain damage caused by the disease does not always respond to thiamine replacement therapy. In some cases, drug therapy is recommended. If treatment is successful, improvement will become apparent within two years although recovery is slow and often incomplete.

Causes

Conditions resulting in the vitamin deficiency and its effects include chronic alcoholism and severe malnutrition. Alcoholism may be an indicator of poor nutrition, which in addition to inflammation of the stomach lining, causes thiamine deficiency.^[3] Other causes include dietary deficiencies, prolonged vomiting, eating disorders, or the effects of chemotherapy. It can also occur in pregnant women who have a form of extreme morning sickness known as hyperemesis gravidarum.^[4] Mercury poisoning can also lead to Korsakoff's syndrome.^[5] It has also been caused by centipede (mukade) bites in Japan.^[6]

There is no specific treatment because the previous thiamine deficiency produces irreversible damage to the medial thalamic nuclei and mammillary bodies. Mammillary body atrophy may be visible on high-resolution MRI.^[7]

Prevention

The most effective method of preventing Korsakoff's syndrome is to avoid B vitamin/thiamine deficiency. In Western nations, the most common causes of such a deficiency are alcoholism and weight disorders.

Case studies

A famous case study is recounted by Oliver Sacks in "The Lost Mariner" and "A Matter of Identity", which can be found in The Man Who Mistook His Wife for a Hat. Other cases include German entertainer Harald Juhnke, artist Charles Blackman,^[8] and entertainer Graham Kennedy.^[9]

In popular culture

• Strangers is a play by Edward Einhorn based on the syndrome.
• In the Half-Life 2 mod Korsakovia, by thechineseroom, the player character Christopher is under treatment for Korsakoff's Syndrome.
• In the television show House, season 1 episode "Histories," Dr. House diagnoses a patient with Korsakoff's syndrome after she gives different explanations for a sprained wrist to different interns who independently take her history.
• Internet comedy sketch group Derrick Comedy features a man with Korsakoff's syndrome in their video "Progression of a Mad Hatter".
• In the episode "Keeping the Faith" of the television show Royal Pains first aired on 06/17/10, Korsakoff's syndrome is offered as a diagnosis for a female who had previously undergone a stomach reduction surgery.
• In the novel Now Wait for Last Year by Philip K. Dick, The main character's wife is diagnosed with the syndrome after years of drug and alcohol abuse.
• It is probable that Boston Corbett, the man who shot John Wilkes Booth, suffered from Korsakoff's syndrome later in life due to his pre-and-post war profession as a hatter, and his eventual madness.
• Davey Volner winner of the 81st Glascock Prize wrote a poem titled "Korsakoff's Syndrome".^[10]
• Dr. Butz, the attending physician in the movie, Critical Care (1997), requests the etiology and symptoms of Korsakoff's syndrome of his resident, Dr. Ernst.
• In the novel Mona Lisa Overdrive by William Gibson, as punishment for stealing cars prior to the events in the novel, the character Slick Henry was subjected to a process that induced Korsakoff's syndrome in him. He features the episodic memory loss characteristic of the syndrome, and builds cybernetic sculptures as therapy.

References

External links

• The Merck Manual: Function And Dysfunction Of The Cerebral Lobes - Amnesias - Korsakoff's syndrome
• Simple description of Korsakoff's syndrome
• An Amnesic Patient With An Extraordinary Distorted Memory