- Korsakoff's syndrome
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MeshID = D020915Korsakoff's syndrome or "Korsakov's syndrome" (Korsakoff's psychosis, amnesic-confabulatory syndrome), is a brain disorder caused by the lack of thiamine (vitamin B1) in the
brain . Thesyndrome is named afterSergei Korsakoff , the neuropsychiatrist who popularized the theory.ymptoms
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 inconversation
# lack ofinsight
#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 themammillary bodies of the hypothalamus as well as generalizedcerebral atrophy . [Kolb & Whishaw: "Fundamentals of Human Neuropsychology", 2003, pages 473-473]When
Wernicke's encephalopathy accompanies Korsakoff's syndrome, the combination is called theWernicke-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 toneurons ;gliosis which is a result of damage to supporting cells of thecentral nervous system ; andhemorrhage or bleeding in mammillary bodies. Damage to thedorsomedial nucleus of thethalamus is also associated with this disorder.igns
*
Ataxia
*Apathy
* Retrograde and anterograde amnesia
*Confabulation
* Tremors
*Paralysis of muscles controlling the eye
* Lack of insight to the condition
*Coma Treatment
It was once assumed that anyone suffering from Korsakoff's syndrome would eventually need full time care. However, this is not the case and with proper treatment there is every chance that a person will remain independent and maintain a reasonable quality of life. [ [http://www.korsakoffs.com] ] Treatment involves the replacement or suppletion of
thiamine byintravenous (IV) orintramuscular (IM) injection, together with propernutrition andhydration . However, the amnesia and brain damage caused by the disease does not always respond tothiamine 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 severemalnutrition . Alcoholism is often an indicator of poor nutrition, which in addition toinflammation of the stomach lining, causes thiamine deficiency. [http://www.alzheimers.org.uk/site/scripts/documents_info.php?categoryID=200171&documentID=98] As well asalcohol abuse , causes include dietary deficiencies, prolonged vomiting,eating disorder s, or the effects ofchemotherapy . It can also occur in pregnant women who have a form of extreme morning sickness known ashyperemesis gravidarum . [http://www.healthatoz.com/healthatoz/Atoz/common/standard/transform.jsp?requestURI=/healthatoz/Atoz/ency/korsakoffs_syndrome.jsp Korsakoff's syndrome ] ]Mercury poisoning can also cause it. It has also been caused bycentipede ("mukade") bites in Japan [ [http://www.ncbi.nlm.nih.gov/pubmed/2022129?dopt=Citation Centipede bites in Japan. [Cutis. 1991 - PubMed Result ] ] .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 ).However long term memory prior to the disorder can remain intact.Prevention
The most effective method of preventing Korsakoff's syndrome is to avoid B vitamin/thiamine deficiency. In Western nations the most common cause of such deficiency is alcoholism. Thiamine was introduced to alcoholic beverages in the U.S. for a time, but this does not appear to have an effect on Korsakoff's syndrome, as the cause of Korsakoff's syndrome in chronic alcoholics is not a deficiency of thiamine in the diet, but rather a reduction in the body's ability to absorb thiamine in the intestine. In the U.S., government mandates to adding thiamine to alcoholic beverages have been blocked for this reason and also by political groups asserting that such supplementation would encourage alcohol use.Fact|date=March 2008
Also, alcohol is, by itself, neurotoxic. It will cause neural damage taken in excess, especially in the
hippocampus . The body responds to alcohol ingestion by releasing cortisol as a neuroprotective mechanism (animals that have been adrenalectomized may be killed by a fraction of the dose of alcohol that an unadrenalectomized animal may tolerate). Cortisol, specifically, has been shown to cause irreversible damage to thehippocampus when present in large amounts for extended periods of time. [ [http://www.tianeptine.com/cortisol-stress.html Tree shrews, cortisol, and the hippocampus ] ] Alcohol in excess may be causal in and of itself in Korsakoff dementia regardless of thiamine addition to spirits. Alcohol toxicity is cumulative; cessation of its use, or never having used it, will reduce risk of korsakoff and other dementias.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 ".Other cases include that of artist
Charles Blackman [ [http://www.theage.com.au/news/arts/artists-wonderland-is-back-in-town/2006/07/28/1153816384482.html Artist's wonderland is back in town - Arts - Entertainment - theage.com.au ] ] and entertainerGraham Kennedy . [ [http://web.archive.org/web/20050619210114/http://www.bulletin.ninemsn.com.au/bulletin/site/articleIDs/6D71054423628356CA25700D0005E365 Bulletin - Graham Kennedy ] ]In popular culture
*In the fictional show "House", episode "Histories", the clinic patient suffers from Korsakoff's as a result of malnutrition.
*In the novel "Mona Lisa Overdrive ", the character Slick wasincarcerated in a "chemo-penal unit" with "induced Korsakov's" (sic).
*In the British movieThe Wrong Box ,Peter Sellers plays a doctor suffering from Korsakoff's syndrome, who is reduced to signing reports of more than questionable medical content, while telling everybody of his former lavish Mayfair office, where only the Lords and Counts used to be his clientèle.References
External links
* [http://www.untitledtheater.com/plays/strangers-linguish.html "Strangers"] is a play by [http://www.untitledtheater.com/Edwardbio.htm Edward Einhorn] based on the syndrome.
* [http://www.merck.com/mrkshared/mmanual/home.jsp The Merck Manual] : [http://www.merck.com/mrkshared/mmanual/section14/chapter169/169.jsp Function And Dysfunction Of The Cerebral Lobes] - [http://www.merck.com/mrkshared/mmanual/section14/chapter169/169e.jsp Amnesias - Korsakoff's syndrome]
* [http://www.healthatoz.com/healthatoz/Atoz/ency/korsakoffs_syndrome.jsp Simple description of Korsakoff's syndrome]
* [http://en.citizendium.org/wiki/Korsakoff%27s_syndrome Citizendium's article on Korsakoff's syndrome]
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