Details of Disease

General Information of Disease (ID: DISBLQ1T)

• Disease Name: Wernicke-Korsakoff Syndrome
• Synonyms: Wernicke encephalopathy; transketolase defect; alcohol-induced encephalopathy; Korsakov psychosis; Korsakoff's psychosis; Korsakoff's syndrome; Korsakov's psychosis; Wernicke-Korsakoff syndrome; Korsakoff syndrome
• Disease Class: 5B55-5B5F: Vitamin deficiency
• Definition: Wernicke-Korsakoff syndrome is a brain disorder, due to thiamine deficiency that has been associated with both Wernicke's encephalopathy and Korsakoff syndrome. The term refers to two different syndromes, each representing a different stage of the disease. Wernicke's encephalopathy represents the 'acute' phase and Korsakoff's syndrome represents the 'chronic' phase. However, they are used interchangeable in many sites. Wernicke's encephalopathy is characterized by confusion, abnormal stance and gait (ataxia), and abnormal eye movements (nystagmus). Korsakoff's syndrome is observed in a small number of patients. It is a type of dementia, characterized by memory loss and confabulation (filling in of memory gaps with data the patient can readily recall) and involvement of the heart, vascular, and nervous system. Wernicke-Korsakoff syndrome mainly results from chronic alcohol use, but also from dietary deficiencies, prolonged vomiting, eating disorders, systemic diseases (cancer, AIDS, infections), bariatric surgery, transplants, or the effects of chemotherapy. Studies indicate that there may be some genetic predisposition for the disease.Treatment involves supplementing the diet with thiamine. Wernicke encephalopathy is an acute syndrome and requires emergency treatment to prevent death and neurologic complications. In cases where the diagnosis is not confirmed, patients should still be treated while additional evaluations are completed.|Editor note: todo check
• Disease Hierarchy:
  DIS6SVEE: Syndromic disease
  DIS1UUNY: Malnutrition
  DISBLQ1T: Wernicke-Korsakoff Syndrome
• ICD Code: ICD-11: ICD-11: 5B5A.1
• Disease Identifiers:
  MONDO ID: MONDO_0010198
  MESH ID: D020915
  UMLS CUI: C0349464
  OMIM ID: 277730
  MedGen ID: 83883
  SNOMED CT ID: 69482004

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 1 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Thiamine Hydrochloride	DM3185A	Approved	Small molecular drug	[1]

This Disease is Treated as An Indication in 1 Investigative Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
[3H]thiamine	DMSHW2E	Investigative	Small molecular drug	[2]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 2 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
SLC19A2	TT2A1DZ	Strong	Genetic Variation	[3]
ALDH2	TTFLN4T	Definitive	Genetic Variation	[4]

This Disease Is Related to 1 DME Molecule(s)

Gene Name	DME ID	Evidence Level	Mode of Inheritance	REF
ADH1B	DEEN9RD	Definitive	Genetic Variation	[4]

References

• [1] Drugs@FDA. U.S. Food and Drug Administration. U.S. Department of Health & Human Services. 2015
• [2] A standard database for drug repositioning. Sci Data. 2017 Mar 14;4:170029.
• [3] Direct genomic PCR sequencing of the high affinity thiamine transporter (SLC19A2) gene identifies three genetic variants in Wernicke Korsakoff syndrome (WKS).Am J Med Genet B Neuropsychiatr Genet. 2005 Aug 5;137B(1):17-9. doi: 10.1002/ajmg.b.30194.
• [4] Alcohol and aldehyde dehydrogenase genotypes in Korsakoff syndrome.Alcohol Clin Exp Res. 2000 Mar;24(3):337-40.