Korsakoff's syndrome: Difference between revisions

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{{Infobox_Disease |
#REDIRECT [[Wernicke-Korsakoff syndrome]]
  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 |
}}
{{SI}}
 
{{EH}}
 
'''Korsakoff's syndrome''' ('''Korsakoff's psychosis''', '''amnesic-confabulatory syndrome'''), is a degenerative 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 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</ref> [[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]].<ref>http://www.theage.com.au/news/arts/artists-wonderland-is-back-in-town/2006/07/28/1153816384482.html</ref>
 
 
 
==References==
<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]
 
{{Mental and behavioural disorders}}
 
[[Category:Memory disorders]]
[[Category:Neurology]]
[[Category:Malnutrition]]
[[Category:Syndromes]]
 
[[de:Korsakow-Syndrom]]
[[es:Síndrome de Korsakoff]]
[[fr:Syndrome de Korsakoff]]
[[it:Sindrome di Korsakov]]
[[nl:Syndroom van Korsakov]]
[[ja:コルサコフ症候群]]
[[pl:Zespół Korsakowa]]
[[pt:Síndrome de Korsakoff]]
[[ru:Синдром Корсакова]]
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Latest revision as of 16:28, 5 August 2011