Korsakoff's syndrome

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**Korsakoff's syndrome**
*Classification & external resources*

Thiamine
ICD-10	F 10.6
ICD-9	291.1, 294.0
DiseasesDB	14107
eMedicine	med/2405
MeSH	D020915

Korsakoff's syndrome (Korsakoff's psychosis, amnesic-confabulatory syndrome), is a degenerative brain disorder caused by the lack of thiamine (vitamin B₁) in the brain. The syndrome is named after Sergei Korsakoff, the neuropsychiatrist who popularized the theory.

1 Symptoms
- 1.1 Indications
2 Treatment
3 Causes
4 Case studies
5 In popular culture
6 References
7 External links

[edit] Symptoms

There are six major symptoms of Korsakoff's syndrome: anterograde and retrograde amnesia, or severe memory loss; confabulation, that is, invented memories which are then taken as true due to gaps in memory sometimes associated with blackouts; meager content in conversation; lack of insight, and 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 (nodes on the underside of the brain associated with memory) 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.

The disease involves neuronal loss, that is, damage to neurotransmitters; gliosis which is damage to cells in the Central Nervous System; and hemorrhage or bleeding in mammillary bodies. Damage to the dorsomedial nucleus of the thalamus is also associated with this disorder.

[edit] Indications

Ataxia
Apathy
Retrograde amnesia and anterograde amnesia
Confabulation (inventing events to compensate for gaps in memory)
Tremors
Paralysis of muscles controlling the eye
Lack of insight to the condition
Coma

[edit] Treatment

Treatment involves replacing the thiamine by Intravenous (IV) or intramuscular (IM) injection, and providing proper nutrition and hydration. However, the amnesia and brain damage caused by the disease does not respond to thiamine replacement therapy. In some cases, drug therapy is recommended to the patient. If treatment is successful, signs will show within two years though recovery is slow and often incomplete.

[edit] Causes

Conditions resulting in the vitamin deficiency and its effects include chronic alcoholism, and severe malnutrition. Alcoholism is often an indicator of poor nutrition, which in addition to inflammation of stomach lining, causes thiamine deficiency.^[2] As well as alcohol abuse, 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.^[3] Mercury poisoning can also cause it.

[edit] Case studies

A famous case study is recounted by Oliver Sacks in "The Lost Mariner", which can be found in The Man Who Mistook His Wife for a Hat.

Another case is that of the Australian artist Charles Blackman.^[4]

[edit] In popular culture

Korsakoff's syndrome appeared in the fictional Fox Network series House where a young woman in the emergency room was giving interns different stories as to what happened to her, so naturally the interns thought she was lying. However, Dr. House discovered this was due to the effects of Korsakoff's syndrome when she fabricated a final story involving images on his pin and clipboard. The patient wasn't lying, but because of her lack of memory she mistook her guesses for reality.