Korsakoff's syndrome

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Korsakoff's syndrome
Classification and external resources
Thiamine
ICD-10 F10.6
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.

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 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 of the thalamus is also associated with this disorder.

Indications

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.

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 the stomach lining, causes thiamine deficiency.[1] 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.[1] Mercury poisoning can also cause it.

Due to malnutrition and a lack of Thiamine, the hippocampus begins to decay, leaving holes that disallows one's rehearsed information within short term memory to transfer to long term memory (anterograde amnesia)

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.[1]

In popular culture

References


External links

de:Korsakow-Syndromfr:Syndrome de Korsakoff

it:Sindrome di Korsakov nl:Syndroom van Korsakov ja:コルサコフ症候群

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .

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