Wernicke-Korsakoff syndrome

ウェルニッケ・コルサコフ症候群

WordNet

1. a pattern of symptoms indicative of some disease
2. a complex of concurrent things; "every word has a syndrome of meanings"
3. German neurologist best known for his studies of aphasia (1848-1905) (同)Karl Wernicke

PrepTutorEJDIC

1. (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/08/16 02:24:58」(JST)

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Wernicke–Korsakoff syndrome
Classification and external resources
Thiamine
ICD-10	E51.2, F10.6
ICD-9	294.0
OMIM	277730
MedlinePlus	000771
eMedicine	med/2405
MeSH	D020915

Wernicke–Korsakoff syndrome (also called wet brain, Korsakoff's psychosis, alcoholic encephalopathy, Wernicke's disease, and encephalopathy - alcoholic) is a manifestation of thiamine (vitamin B₁) deficiency, or beriberi. This is usually secondary to alcohol abuse. It mainly causes vision changes, ataxia and impaired memory.

Korsakoff's syndrome and Wernicke's encephalopathy

The syndrome is a combined manifestation of two eponymous disorders, Korsakoff's syndrome and Wernicke's encephalopathy, named after Sergei Korsakoff and Carl Wernicke respectively.

Wernicke's encephalopathy is characterized by:

1. confusion
2. nystagmus (involuntary eye movement)
3. ophthalmoplegia (impaired eye movement)
4. anisocoria (unequal size of pupils)
5. ataxia (lack of muscle coordination)
6. sluggish pupillary reflexes
7. coma and death if untreated

Korsakoff's psychosis is characterized by:

1. anterograde amnesia (inability to form new memories)
2. retrograde amnesia (loss of existing memories)
3. confabulation (false perceptions or memories)
4. hallucinations

Causes

Wernicke-Korsakoff syndrome is usually found in chronic alcoholics. Wernicke-Korsakoff syndrome results from thiamine deficiency. It is generally agreed that Wernicke's encephalopathy results from severe acute deficiency of thiamine (vitamin B₁), whilst Korsakoff's psychosis is a chronic neurologic sequela after Wernicke's encephalopathy. The metabolically active form of thiamine is thiamine diphosphate which plays a major role as a cofactor or coenzyme in glucose metabolism. The enzymes which are dependent on thiamine diphosphate are associated with the citric acid cycle (also known as the Kreb's cycle), and catalyze the 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 syndrome in the United States is usually found in malnourished chronic alcoholics, though it is also found in patients who undergo prolonged intravenous (IV) therapy without vitamin B₁ supplementation, gastric stapling, intensive care unit (ICU) stays or hunger strikes. In some regions, physicians have observed thiamine 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.

Wernicke-Korsakoff syndrome in alcoholics particularly is associated with atrophy/infarction 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, the median and dorsal raphe nuclei, and the cerebellum.

One as-yet-unreplicated study has associated susceptibility to this syndrome with a hereditary deficiency of transketolase, an enzyme that requires thiamine as a coenzyme.

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, secondary to thiamine deficiency and subsequent cytotoxic edema in Wernicke's encephalopathy, patients will have marked degeneration of the mamillary bodies. Thiamine (vitamin B1) is an essential coenzyme in carbohydrate metabolism and is also a regulator of osmotic gradient. Its deficiency may cause swelling of the intracellular space and local disruption of the blood-brain barrier. Brain tissue is very sensitive to changes in electrolytes and pressure and edema can be cytotoxic. In Wernicke's this occurs specifically in the mamillary bodies, medial thalami, tectal plate, and periaqueductal areas. Sufferers may also exhibit a dislike for sunlight and so may wish to stay indoors with the lights off. 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 beginning with an initial intravenous or intramuscular dose, followed by supplemental oral doses. Some people think that it is important to start the thiamine treatment before giving any glucose, as the encephalopathy will be worsened by the glucose; however, this is based only on case reports. (Glucose administration promotes decarboxylation of pyruvate, a biochemical reaction which requires thiamine.) By the time amnesia and psychosis have occurred, complete recovery is extremely unlikely.^{[citation needed]}

Reference in Popular Culture

In the opening episode of the TNT TV series Perception, a character is poisoned with a thiamin-depleting drug that leads to her developing Wernicke-Korsakoff syndrome.

References

See also

• Alcoholic dementia
• Korsakoff syndrome
• Wernicke encephalopathy
• Alcoholism
• Dementia
• Malabsorption

UpToDate Contents

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• 1. ウェルニッケ脳症 wernicke encephalopathy
• 2. アルコールの慢性神経学的合併症の概要 overview of the chronic neurologic complications of alcohol
• 3. 持続的なアルコール依存症患者における栄養状態 nutritional status in patients with sustained heavy alcohol use
• 4. 水溶性ビタミンの概要 overview of water soluble vitamins
• 5. 周術期におけるアルコールの有害な使用の同定およびマネージメント identification and management of unhealthy alcohol use in the perioperative period

English Journal

• Destination Memory in Korsakoff's Syndrome.

• El Haj M1, Kessels RP2,3,4, Matton C5, Bacquet JE5, Urso L6, Cool G5, Guidez F5, Potier S6, Nandrino JL1, Antoine P1.
• Alcoholism, clinical and experimental research.Alcohol Clin Exp Res.2016 Apr 18. doi: 10.1111/acer.13070. [Epub ahead of print]
• BACKGROUND: Context memory, or the ability to remember the context in which an episodic event has occurred (e.g., where and when an event took place), has been found to be compromised in Korsakoff's syndrome. This study examined whether a similar deficit would be observed for destination memory, tha
• PMID 27087276

• Exercise leads to the re-emergence of the cholinergic/nestin neuronal phenotype within the medial septum/diagonal band and subsequent rescue of both hippocampal ACh efflux and spatial behavior.

• Hall JM1, Savage LM2.
• Experimental neurology.Exp Neurol.2016 Apr;278:62-75. doi: 10.1016/j.expneurol.2016.01.018. Epub 2016 Jan 30.
• Exercise has been shown to improve cognitive functioning in a range of species, presumably through an increase in neurotrophins throughout the brain, but in particular the hippocampus. The current study assessed the ability of exercise to restore septohippocampal cholinergic functioning in the pyrit
• PMID 26836322

• Challenges in Diagnosis and Treatment of Wernicke Encephalopathy: Report of 2 Cases.

• Infante MT1, Fancellu R2, Murialdo A3, Barletta L4, Castellan L4, Serrati C3.
• Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition.Nutr Clin Pract.2016 Apr;31(2):186-90. doi: 10.1177/0884533615621753. Epub 2016 Feb 11.
• BACKGROUND: Wernicke encephalopathy (WE) is a medical emergency caused by thiamine deficiency, characterized by cerebellar ataxia, ophthalmoplegia, and cognitive disturbances that may progress to Korsakoff amnesia. We describe 2 patients with WE who needed high-dose and long-term treatment with thia
• PMID 26869612

Japanese Journal

• アルコールと神経障害

• 鈴木 裕
• 日大醫學雜誌 70(3), 150-154, 2011-06-01
• NAID 10029405296

• チアミン欠乏によって生じる中枢神経障害 : Wernicke-Korsakoff症候群

• 荒木 彩,平嶋 浩子,車田 麻美 [他],濱田 俊
• ビタミン 84(11), 510-516, 2010-11-25
• … In humans, a clinical syndrome based on the brain damage induced by thiamin deficiency is known as Wernicke-Korsakoff syndrome (WKS). …
• NAID 110007989341

Related Links

• Wernicke-Korsakoff syndrome: MedlinePlus Medical ...

Wernicke encephalopathy and Korsakoff syndrome are different conditions. Both are due to brain damage caused by a lack of vitamin B1. Lack of vitamin B1 is common in people with alcoholism. It is also common in ...

• Wernicke-Korsakoff Syndrome - Diseases & Conditions ...

Wernicke-Korsakoff Syndrome. When persistent learning and memory deficits are present in patients with Wernicke encephalopathy (a clinical triad that classically, but not always, consists of confusion, ataxia, and ...

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リンク元	「ウェルニッケ・コルサコフ症候群」「Wernicke-Korsakoff症候群」
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「ウェルニッケ・コルサコフ症候群」

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英

Wernicke-Korsakoff syndrome

同

ウェルニッケ・コルサコフ脳症 Wernicke-Korsakov encephalopathy、ウェルニッケ・コルサコフ病 [[[Wernicke-Korsakoff disease]]

関

ウェルニッケ脳症、ビタミンB1、コルサコフ症候群

「Wernicke-Korsakoff症候群」

　　[★]

英

Wernicke-Korsakoff syndrome

関

ウェルニッケ・コルサコフ症候群

「syndrome」

　　[★]

• n.

• 症候群