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| Image = Thiamine-2D-skeletal.png | | | Image = Thiamine-2D-skeletal.png | |
| Caption = [[Thiamine]] | | | Caption = [[Thiamine]] | |
| DiseasesDB = |
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| ICD10 = {{ICD10|E|51|2|e|50}}, {{ICD10|F|10||f|10}}6 |
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| ICD9 = {{ICD9|294.0}} |
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| ICDO = |
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| OMIM = 277730 |
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| MedlinePlus = 000771 |
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| MeshID = D020915 |
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| }} | | }} |
| {{SI}} | | {{Wernicke-Korsakoff syndrome}} |
| {{CMG}}; {{AE}} {{CZ}}; {{ADI}} | | {{CMG}}; {{AE}} {{CZ}}; {{ADI}} |
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| {{SK}} Alcohol induced encephalopathy; transketolase defect; Korsakoff psychosis; alcoholic encephalopathy | | {{SK}} Alcohol induced encephalopathy; transketolase defect; Korsakoff psychosis; alcoholic encephalopathy |
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| ==Overview== | | ==[[Wernicke-Korsakoff syndrome overview|Overview]]== |
| '''Wernicke-Korsakoff syndrome''' is a is a degenerative brain disorder cause by [[thiamine]] deficiency. This is usually secondary to [[alcohol abuse]].
| | ==[[Wernicke-Korsakoff syndrome historical perspective|Historical Perspective]]== |
| 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.
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| ==Historical Perspective== | | ==[[Wernicke-Korsakoff syndrome pathophysiology|Pathophysiology]]== |
| The syndrome is a combined manifestation of two eponymous disorders, Korsakoff's Psychosis and Wernicke's encephalopathy, named for Drs. [[Sergei Korsakoff]] and [[Carl Wernicke]].
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| ==Pathophysiology== | | ==[[Wernicke-Korsakoff syndrome causes|Causes]]== |
| Wernicke-Korsakoff syndrome results from thiamin deficiency. It is generally agreed that Wernicke's encephalpathy results from severe acute deficiency of [[thiamine]] ([[Vitamin B]]<sub>1</sub>), whilst Korsakoff's psychosis results from chronic deficiency of thiamin. The metabolically active form of thiamin is thiamin diphosphate which plays a major role as a [[Cofactor (biochemistry)|cofactor]] in glucose metabolism. The enzymes which are dependent on thiamin diphosphate are associated with the TCA Cycle and catalyse the [[pyruvate dehydrogenase|oxidation of pyruvate]],alphaketoglutarate and branched chain amino acids. Thus, anything that encourages glucose metabolism will exacerbate an existing clinical or [[sub-clinical]] thiamine deficiency.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.
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| There is an unreplicated study that has associated susceptiblity to this syndrome with a hereditary deficiency of [[transketolase]], an [[enzyme]] involved in thiamine metabolism.<ref>{{cite journal |author=Nixon P, Kaczmarek M, Tate J, Kerr R, Price J |title=An erythrocyte transketolase isoenzyme pattern associated with the Wernicke-Korsakoff syndrome |journal=Eur J Clin Invest |volume=14 |issue=4 |pages=278-81 |year=1984 |pmid=6434322}}</ref>
| | ==[[Wernicke-Korsakoff syndrome differential diagnosis|Differentiating Wernicke-Korsakoff Syndrome from other Diseases]]== |
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| ===Pathology=== | | ==[[Wernicke-Korsakoff syndrome epidemiology and demographics|Epidemiology and Demographics]]== |
| Wernicke-Korsakoff syndrome in alcoholics especially is associated with [[atrophy]]<ref>Kolb & Whishaw: ''Fundamentals of Human Neuropsychology'', 2003, pages 473-473</ref> of specific regions of the brain, especially the mamillary bodies. Other regions include the anterior region of the [[thalamus]] (accounting for amnesic symptoms), the medial dorsal [[thalamus]], the [[basal forebrain]], and median and dorsal [[raphe nuclei]].<ref>{{cite journal |author=Mann K, Agartz I, Harper C, Shoaf S et al|title=Neuroimaging in alcoholism: ethanol and brain damage |journal=Alcohol Clin Exp Res |volume=25 |issue=5 Suppl ISBRA |pages=104S-109S |year=2001 |pmid=11391058}}</ref> | |
| 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. Frequently, for unknown reasons, patients with Korsakoff's psychosis will exhibit marked degeneration of the mamillary bodies. The mechanism of this degeneration is unknown, but it supports current neurological theory that the mamillary bodies play a role in various "memory circuits" within the brain. An example of a memory circuit is the [[Papez circuit]].
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| ==Causes== | | ==[[Wernicke-Korsakoff syndrome risk factors|Risk Factors]]== |
| * [[Malnourishment]]
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| * [[Chronic alcoholism]]
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| * Prolonged intravenous therapy
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| * [[gastric bypass|Gastric stapling]]
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| * [[intensive care unit| ICU]] stays
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| * Chronic intake of polished rice
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| * Large dose of [[glucose]] in [[thiamine]] deficient individuals <ref>{{cite journal |author=Zimitat C, Nixon P, |title= Glucose loading precipitates encephalopathy in thiamine-deficient rats. |journal= Metabolic Brain Disease |volume=14 |issue=1 |pages=1-10 |year=2000 }}</ref>
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| ==Epidemiology and Demography== | | ==[[Wernicke-Korsakoff syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| * In France, a country with one of the highest per capita consumptions of alcohol, the prevalence of the WKS was found to be only 0.4% in a small retrospective autopsy study.<ref>http://www.ncbi.nlm.nih.gov/pubmed/7596325</ref>
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| * Prevalence rates in Australia are highest 2.8%.<ref>http://www.ncbi.nlm.nih.gov/pubmed/7596325</ref>
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| * Per capita consumption of alcohol is not corelating with the prevalence of the syndrome in many countries.<ref>http://www.ncbi.nlm.nih.gov/pubmed/7596325</ref>
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| ===Age===
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| * It is more common in middle aged persons.
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| ===Gender===
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| * It is more common in men.
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| ==Diagnosis== | | ==Diagnosis== |
| | | [[Wernicke-Korsakoff syndrome history and symptoms|History and Symptoms]] | [[Wernicke-Korsakoff syndrome physical examination|Physical Examination]] | [[Wernicke-Korsakoff syndrome laboratory findings|Laboratory Findings]] | [[Wernicke-Korsakoff syndrome MRI|MRI]] | [[Wernicke-Korsakoff syndrome other imaging findings|Other Imaging Findings]] | [[Wernicke-Korsakoff syndrome other diagnostic studies|Other Diagnostic Studies]] |
| ===Symptoms===
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| [[Korsakoff's syndrome|Korsakoff's psychosis]] is characterized by | |
| * confusion
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| * [[anterograde amnesia|anterograde]] and [[retrograde amnesia|retrograde]] amnesia.
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| * [[confabulation]]
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| Other important symptoms of [[Korsakoff's syndrome|Korsakoff's psychosis]] are
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| * meager content in [[conversation]]
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| * lack of insight
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| * [[apathy]] - the patients lose interest in things quickly and generally appear indifferent to change.
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| [[Wernicke's encephalopathy]] is characterized by
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| *[[nystagmus]]
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| *[[ophthalmoplegia]]
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| *[[anisocoria]]
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| *[[ataxia]]
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| *sluggish pupillary reflexes
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| *[[coma]] and [[death]] if untreated
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| === Physical Examination ===
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| ==== Appearance of the Patient ====
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| * Lethargic and disturbed consciousness
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| ==== Eyes ====
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| * May have jaundice due to liver damage
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| * [[Nystagmus]]
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| * [[Anisocoria]]
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| * Sluggish pupilary reflex
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| * [[opthalmoplegia]]
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| ==== Abdomen ====
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| * [[Organomegaly ]] may be noticed
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| ==== Neurologic ====
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| * [[Tremors]]
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| * [[Ataxia]]
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| Diagnosis of Wernicke-Korsakoff syndrome is by clinical impression and can sometimes be confirmed with formal Neuropsychological assessment. Wernicke's [[encephalopathy]] typically presents with [[ataxia]] and [[nystagmus]], and Korsakoff's psychosis with anterograde and retrograde [[amnesia]] and confabulation upon relevant lines of questioning.
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| ==Treatment== | | ==Treatment== |
| Treatment consists of reversing the thiamine deficiency by giving supplemental thiamine, usually by starting with an initial intravenous or intramuscular dose followed by supplemental oral doses. It is important to start the thiamine treatment before giving any glucose as the encephalopathy will be worsened by the glucose. (Glucose administration promotes dehydrogenation of [[pyruvate]], a biochemical reaction which consumes thiamine.) By the time [[amnesia]] and [[psychosis]] have occurred, complete recovery is unlikely.
| | [[Wernicke-Korsakoff syndrome medical therapy|Medical Therapy]] | [[Wernicke-Korsakoff syndrome primary prevention|Primary Prevention]] | [[Wernicke-Korsakoff syndrome secondary prevention|Secondary Prevention]] | [[Wernicke-Korsakoff syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Wernicke-Korsakoff syndrome future or investigational therapies|Future or Investigational Therapies]] |
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| ==References== | | == Case Studies == |
| {{Reflist|2}}
| | [[Wernicke-Korsakoff syndrome case study one|Caes #1]] |
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| [[Category:Neurological disorders]] | | [[Category:Neurological disorders]] |
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| [[Category:Neurology]] | | [[Category:Neurology]] |
| [[Category:Malnutrition]] | | [[Category:Malnutrition]] |
| [[Category:Overview complete]]
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| [[Category:Alcohol abuse]] | | [[Category:Alcohol abuse]] |
| | [[Category:Emergency medicine]] |
| | [[Category:Disease]] |
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| [[de:Wernicke-Korsakow-Syndrom]] | | [[de:Wernicke-Korsakow-Syndrom]] |
| [[fr:Syndrome de Wernicke-Korsakoff]] | | [[fr:Syndrome de Wernicke-Korsakoff]] |
