Korsakoff's syndrome
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| Korsakoff's syndrome | |
 | |
|---|---|
| Thiamine | |
| ICD-10 | F106 |
| ICD-9 | 291.1, 294.0 |
| DiseasesDB | 14107 |
| eMedicine | med/2405 |
| MeSH | D020915 |
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Overview
Korsakoff's syndrome (Korsakoff's psychosis, amnesic-confabulatory syndrome) or Wernicke's encephalopathy 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.
It may result from alcohol abuse, dietary deficiencies, prolonged vomiting, eating disorders, or the effects of chemotherapy. Symptoms include mental confusion, vision impairment, stupor, coma, hypothermia, hypotension, and ataxia. Korsakoff's amnesic syndrome-a memory disorder-also results from a deficiency of thiamine, and is associated with alcoholism. The heart, vascular, and nervous system are involved. Symptoms include amnesia, confabulation, attention deficit, disorientation, and vision impairment. The main features of Korsakoff's amnesic syndrome are the impairments in acquiring new information or establishing new memories, and in retrieving previous memories. Although Wernicke's and Korsakoff's may appear to be two different disorders, they are generally considered to be different stages of the same disorder, which is called Wernicke-Korsakoff syndrome. Wernicke's encephalopathy represents the "acute" phase of the disorder, and Korsakoff's amnesic syndrome represents the "chronic" phase.
Symptoms
There are six major symptoms of Korsakoff's syndrome:
- anterograde amnesia and
- retrograde amnesia, 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
- 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.
Signs
- Ataxia
- Apathy
- Retrograde and anterograde amnesia
- Confabulation
- Tremors
- Paralysis of muscles controlling the eye
- Lack of insight to the condition
- Coma
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.[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.
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)
References
- ↑ Kolb & Whishaw: Fundamentals of Human Neuropsychology, 2003, pages 473-473
- ↑ http://www.alzheimers.org.uk/Facts_about_dementia/What_is_dementia/info_korsakoffs.htm
- ↑ http://www.healthatoz.com/healthatoz/Atoz/common/standard/transform.jsp?requestURI=/healthatoz/Atoz/ency/korsakoffs_syndrome.jsp
External links
- Strangers is a play by Edward Einhorn based on the syndrome.
- The Merck Manual: Function And Dysfunction Of The Cerebral Lobes - Amnesias - Korsakoff's syndrome
- Simple description of Korsakoff's syndrome
de:Korsakow-Syndrom it:Sindrome di Korsakov nl:Syndroom van Korsakov