Wernicke-Korsakoff syndrome 311354 222478126 2008-06-29T15:36:45Z DOI bot 6652755 Citation maintenance. Formatted: title. You can [[WP:DOI|use this bot]] yourself! Please [[User:DOI_bot/bugs|report any bugs]]. {{Infobox_Disease | Name = {{PAGENAME}} | Image = Thiamine-2D-skeletal.png | Caption = [[Thiamine]] | DiseasesDB = | ICD10 = {{ICD10|E|51|2|e|50}}, {{ICD10|F|10||f|10}}6 | ICD9 = {{ICD9|294.0}} | ICDO = | OMIM = 277730 | MedlinePlus = 000771 | eMedicineSubj = med | eMedicineTopic = 2405 | MeshID = D020915 | }} '''Wernicke-Korsakoff syndrome''' (also called Korsakoff psychosis, alcoholic encephalopathy, Wernicke's disease, and encephalopathy - alcoholic)<ref> http://www.nlm.nih.gov/medlineplus/ency/article/000771.htm </ref> is a manifestation of [[thiamine]] deficiency, or [[beri-beri]]. This is usually secondary to [[alcohol abuse]]. ==Korsakoff's Psychosis and Wernicke's encephalopathy== The syndrome is a combined manifestation of two eponymous disorders, Korsakoff's Psychosis and Wernicke's encephalopathy, named after Drs. [[Sergei Korsakoff]] and [[Carl Wernicke]]. [[Wernicke's encephalopathy]] is characterized by #confusion. #[[nystagmus]] #[[ophthalmoplegia]] #[[anisocoria]] #[[ataxia]] #sluggish pupillary reflexes #[[coma]] and [[death]] if untreated [[Korsakoff's syndrome|Korsakoff's psychosis]] is characterized by #[[anterograde amnesia|anterograde]] and [[retrograde amnesia|retrograde]] amnesia. #[[confabulation]]. ==Causes== {{alcohealth}} Wernicke-Korsakoff syndrome results from thiamine deficiency. It is generally agreed that Wernicke's Encephalopathy results from severe acute deficiency of [[thiamine]] ([[Vitamin B]]<sub>1</sub>), whilst Korsakoff's Psychosis is a chronic neurologic sequela after Wernicke's Encephalopathy. The metabolically active form of thiamin is thiamin diphosphate which plays a major role as a [[Cofactor (biochemistry)|cofactor]] Coenzyme 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. As stated above, Wernicke-Korsakoff in the United States is usually found in malnourished [[chronic alcoholic]]s, though it is also found in patients who undergo prolonged [[intravenous]] (IV) therapy without ([[Vitamin B]]<sub>1</sub>) suplementation, [[gastric bypass|gastric stapling]] or [[intensive care unit]] (ICU) stays. In some regions, physicians have observed thiamin deficiency brought about by severe malnutrition, particularly in diets consisting mainly of [[polished rice]], which is thiamine deficient. The resulting nervous system ailment is called [[Beriberi]]. In individuals with sub-clinical thiamine deficiency, a large dose of glucose (either as sweet food etc or glucose infusion), can precipitate the onset of overt encephalopathy. <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 |doi= 10.1023/A:1020653312697 }}</ref><ref name="pmid18034292">{{cite journal |author=Navarro D, Zwingmann C, Chatauret N, Butterworth RF |title=Glucose loading precipitates focal lactic acidosis in the vulnerable medial thalamus of thiamine-deficient rats |journal=Metab Brain Dis |volume=23 |issue=1 |pages=115–22 |year=2008 |month=March |pmid=18034292 |doi=10.1007/s11011-007-9076-z |url=}}</ref> Wernicke-Korsakoff syndrome in alcoholics especially is associated with [[atrophy]] 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> One as-yet-unreplicated study 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 |doi=10.1111/j.1365-2362.1984.tb01181.x}}</ref> ==Diagnosis and findings== 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. 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]]. ==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. ==References== <references/> [[Category:Neurological disorders]] [[Category:Psychiatry]] [[Category:Syndromes]] [[Category:Alcohol abuse]] [[de:Wernicke-Korsakow-Syndrom]] [[fr:Syndrome de Wernicke-Korsakoff]] [[is:Wernicke-Korsakoff heilkenni]] [[nl:Syndroom van Wernicke]] [[pt:Síndrome de Wernicke-Korsakoff]] [[ru:Синдром Гайе-Вернике]] [[sq:Sindroma Wernicke-Korsakoff]]