Korsakoff's syndrome
332008
221306486
2008-06-23T23:14:43Z
Vendettax
1669120
/* Treatment */ adding "always" to the sentence for clarity, it sounded like that's what the editor was saying, if this is not true please remove it.
{{Infobox_Disease |
Name = {{PAGENAME}} |
Image = Thiamine-2D-skeletal.png |
Caption = [[Thiamine]] |
DiseasesDB = 14107 |
ICD10 = {{ICD10|F|10||f|10}}6 |
ICD9 = {{ICD9|291.1}}, {{ICD9|294.0}} |
ICDO = |
OMIM = |
MedlinePlus = |
eMedicineSubj = med |
eMedicineTopic = 2405 |
MeshID = D020915 |
}}
'''Korsakoff's syndrome''' ('''Korsakoff's psychosis''', '''amnesic-confabulatory syndrome'''), is a brain disorder caused by the lack of [[thiamine|thiamine (vitamin B<sub>1</sub>)]] in the [[brain]]. The [[syndrome]] is named after [[Sergei Korsakoff]], the [[Neuropsychiatry|neuropsychiatrist]] who popularized the theory.
==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 [[Blackout (Alcohol Related Amnesia)|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|thiamine (vitamin B<sub>1</sub>)]], 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]].<ref>Kolb & Whishaw: ''Fundamentals of Human Neuropsychology'', 2003, pages 473-473</ref>
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 body|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 always 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.<ref>http://www.alzheimers.org.uk/Facts_about_dementia/What_is_dementia/info_korsakoffs.htm</ref> As well as [[alcohol abuse]], causes include dietary deficiencies, prolonged vomiting, [[eating disorder]]s, or the effects of [[chemotherapy]]. It can also occur in pregnant women who have a form of extreme morning sickness known as [[hyperemesis gravidarum]].<ref>[http://www.healthatoz.com/healthatoz/Atoz/common/standard/transform.jsp?requestURI=/healthatoz/Atoz/ency/korsakoffs_syndrome.jsp Korsakoff's syndrome<!-- Bot generated title -->]</ref> [[Mercury poisoning]] can also cause it. It has also been caused by [[centipede]] (''mukade'') bites in Japan<ref>[http://www.ncbi.nlm.nih.gov/pubmed/2022129?dopt=Citation Centipede bites in Japan. [Cutis. 1991] - PubMed Result<!-- Bot generated title -->]</ref>.
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]])
==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.<ref>
http://www.healthatoz.com/healthatoz/Atoz/common/standard/transform.jsp?requestURI=/healthatoz/Atoz/ency/korsakoffs_syndrome.jsp
</ref> 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 the hippocampus when present in large amounts for extended periods of time.<ref>
http://www.tianeptine.com/cortisol-stress.html
</ref> 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]]<ref>[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<!-- Bot generated title -->]</ref> and entertainer [[Graham Kennedy]].<ref>[http://web.archive.org/web/20050619210114/http://www.bulletin.ninemsn.com.au/bulletin/site/articleIDs/6D71054423628356CA25700D0005E365]</ref>
==In popular culture==
*In the fictional show ''[[House (TV series)|House]]'', episode "[[Histories (House episode)|Histories]]", the clinic patient suffers from Korsakoff's as a result of malnutrition.
*In the novel ''[[Mona Lisa Overdrive]]'', the character Slick was [[incarcerated]] in a "chemo-penal unit" with "induced Korsakov's" (sic).
*In the British movie [[The 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==
{{reflist}}
== 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]
{{Mental and behavioural disorders}}
[[Category:Memory disorders]]
[[Category:Neurology]]
[[Category:Malnutrition]]
[[Category:Syndromes]]
[[de:Korsakow-Syndrom]]
[[es:Síndrome de Korsakoff]]
[[fr:Syndrome de Korsakoff]]
[[hr:Korsakovljeva psihoza]]
[[it:Sindrome di Korsakoff]]
[[lt:Korsakovo sindromas]]
[[nl:Syndroom van Korsakov]]
[[ja:コルサコフ症候群]]
[[pl:Zespół Korsakowa]]
[[pt:Síndrome de Korsakoff]]
[[ru:Синдром Корсакова]]