MCID: WRN002
MIFTS: 52

Wernicke-Korsakoff Syndrome

Categories: Rare diseases, Metabolic diseases

Aliases & Classifications for Wernicke-Korsakoff Syndrome

MalaCards integrated aliases for Wernicke-Korsakoff Syndrome:

Name: Wernicke-Korsakoff Syndrome 57 12 53 54 15 73
Korsakoff Syndrome 12 55 44
Transketolase Defect 57 73
Korsakoff's Syndrome 12 76
Alcohol-Induced Encephalopathy 57
Alcohol Induced Encephalopathy 73
Korsakoff's Psychosis 12
Korsakov's Psychosis 12
Korsakov Psychosis 12

Characteristics:

OMIM:

57
Inheritance:
autosomal recessive


HPO:

32
wernicke-korsakoff syndrome:
Inheritance autosomal recessive inheritance


Classifications:



Summaries for Wernicke-Korsakoff Syndrome

NIH Rare Diseases : 53 Wernicke-Korsakoff syndromeis 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. 

MalaCards based summary : Wernicke-Korsakoff Syndrome, also known as korsakoff syndrome, is related to beriberi and alzheimer disease, and has symptoms including ataxia and ophthalmoplegia. An important gene associated with Wernicke-Korsakoff Syndrome is TKT (Transketolase), and among its related pathways/superpathways are Metabolism and Carbon metabolism. The drugs Folic Acid and Thiamine have been mentioned in the context of this disorder. Affiliated tissues include brain, heart and eye, and related phenotypes are ptosis and ataxia

NINDS : 54 Wernicke's encephalopathy is a degenerative brain disorder caused by the lack of thiamine (vitamin B1). It may result from alcohol abuse, dietary deficiencies, prolonged vomiting, eating disorders, or the effects of chemotherapy. B1 deficiency causes damage to the brain's thalamus and hypothalamus. Symptoms include mental confusion, vision problems, coma, hypothermia, low blood pressure, and lack of muscle coordination (ataxia). Korsakoff syndrome (also called Korsakoff's amnesic syndrome) is a memory disorder that results from vitamin B1 deficiency and is associated with alcoholism. Korsakoff's syndrome damages nerve cells and supporting cells in the brain and spinal cord, as well as the part of the brain involved with memory. Symptoms include amnesia, tremor, coma, disorientation, and vision problems, The disorder's main features are problems in acquiring new information or establishing new memories, and in retrieving previous memories. Although Wernicke's and Korsakoff's are related disorders, some scientists believe them 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 disorder progressing to a "chronic" or long-lasting stage.

Wikipedia : 76 Alcoholic Korsakoff syndrome (AKS), Korsakoff syndrome is an amnestic disorder caused by thiamine... more...

Description from OMIM: 277730

Related Diseases for Wernicke-Korsakoff Syndrome

Graphical network of the top 20 diseases related to Wernicke-Korsakoff Syndrome:



Diseases related to Wernicke-Korsakoff Syndrome

Symptoms & Phenotypes for Wernicke-Korsakoff Syndrome

Symptoms via clinical synopsis from OMIM:

57
Neurologic Central Nervous System:
ataxia
ocular muscle abnormalities
coma (if untreated)

Neurologic Peripheral Nervous System:
polyneuropathy

Head And Neck Eyes:
horizontal nystagmus
ophthalmoplegia
ptosis (rare)
lateral rectus palsy

Neurologic Behavioral Psychiatric Manifestations:
confusion
impaired memory
disorientation
agitated delirium
korsakoff's psychosis
more

Clinical features from OMIM:

277730

Human phenotypes related to Wernicke-Korsakoff Syndrome:

32 (show all 9)
# Description HPO Frequency HPO Source Accession
1 ptosis 32 HP:0000508
2 ataxia 32 HP:0001251
3 memory impairment 32 HP:0002354
4 psychosis 32 HP:0000709
5 coma 32 HP:0001259
6 horizontal nystagmus 32 HP:0000666
7 ophthalmoplegia 32 HP:0000602
8 polyneuropathy 32 HP:0001271
9 confusion 32 HP:0001289

UMLS symptoms related to Wernicke-Korsakoff Syndrome:


ataxia, ophthalmoplegia

MGI Mouse Phenotypes related to Wernicke-Korsakoff Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 adipose tissue MP:0005375 9.46 KIAA0753 OGDH TKT ACHE
2 behavior/neurological MP:0005386 9.43 ACHE CHAT DLST OGDH SLC19A2 TKT
3 growth/size/body region MP:0005378 9.1 ACHE CHAT DLST KIAA0753 OGDH TKT

Drugs & Therapeutics for Wernicke-Korsakoff Syndrome

Drugs for Wernicke-Korsakoff Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 37)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Not Applicable 59-30-3 6037
2
Thiamine Approved, Investigational, Nutraceutical, Vet_approved Phase 4,Phase 3,Not Applicable 59-43-8, 70-16-6 1130
3 Micronutrients Phase 4,Phase 3,Not Applicable
4 Trace Elements Phase 4,Phase 3,Not Applicable
5 Vitamin B Complex Phase 4,Phase 3,Not Applicable
6 Vitamins Phase 4,Phase 3,Not Applicable
7 Folate Nutraceutical Phase 4,Phase 3,Not Applicable
8 Thiamin Nutraceutical Phase 4,Phase 3,Not Applicable
9 Vitamin B9 Nutraceutical Phase 4,Phase 3,Not Applicable
10
Diphenhydramine Approved, Investigational Phase 3 147-24-0, 58-73-1 3100
11
Histamine Approved, Investigational Phase 3 75614-87-8, 51-45-6 774
12
Promethazine Approved, Investigational Phase 3 60-87-7 4927
13 Anesthetics Phase 3
14 Anesthetics, Local Phase 3
15 Anti-Allergic Agents Phase 3
16 Antiemetics Phase 3
17 Antipruritics Phase 3
18 Autonomic Agents Phase 3
19 Central Nervous System Depressants Phase 3
20 Dermatologic Agents Phase 3
21 Gastrointestinal Agents Phase 3
22 Histamine Antagonists Phase 3
23 Histamine H1 Antagonists Phase 3
24
Histamine Phosphate Phase 3 51-74-1 65513
25 Hypnotics and Sedatives Phase 3
26 Neurotransmitter Agents Phase 3
27 Peripheral Nervous System Agents Phase 3
28
Glucosamine Approved, Investigational, Nutraceutical Not Applicable 3416-24-8 439213
29 Tezosentan Investigational Not Applicable 180384-57-0
30 Adjuvants, Immunologic Not Applicable
31 Antioxidants Not Applicable
32 Benphothiamine Not Applicable
33 Chelating Agents Not Applicable
34 Protective Agents Not Applicable
35 Thioctic Acid Not Applicable
36 pyruvate Nutraceutical Not Applicable
37 Alpha-lipoic Acid Nutraceutical Not Applicable

Interventional clinical trials:

(show all 11)
# Name Status NCT ID Phase Drugs
1 Preoperative Parenteral Thiamine Supplementation in Patients Undergoing Heart Surgery Unknown status NCT01524315 Phase 4 Vitamin B1-ratiopharm;Placebo
2 Optimum Thiamine Intervention (OpTIn) Trial Recruiting NCT02788552 Phase 4 Thiamine Hydrochloride
3 Alcohol: Thiamine and or Magnesium 1 Recruiting NCT03466528 Phase 2, Phase 3 Magnesium Sulfate;Pabrinex
4 Does Thiamine Help Vomiting and Nausea in Pregnancy? Terminated NCT00861523 Phase 3 thiamine & promethazine
5 From Uncomplicated Alcoholism to Korsakoff's Syndrome Unknown status NCT01455207 Not Applicable
6 Study of Dry Beriberi in Mayotte, Comoro Archipelago Unknown status NCT00934349
7 Thiamin (Vitamin B1) Levels in Eating Disorder Adolescent Patients Unknown status NCT02164487
8 Reactive Oxygen Species in the Pathogenesis of Diabetic Complications Completed NCT00703989 Not Applicable
9 Models of Disordered Knowledge and Memory Systems in Dementia and Related Disorders Completed NCT00004557
10 Systemic Inflammatory Response: Thiamine and Magnesium Status (Sir TaM Study) Recruiting NCT03554668
11 The Effect of tDCS on Subcortical Brain Functioning Enrolling by invitation NCT01602276 Not Applicable

Search NIH Clinical Center for Wernicke-Korsakoff Syndrome

Cochrane evidence based reviews: korsakoff syndrome

Genetic Tests for Wernicke-Korsakoff Syndrome

Anatomical Context for Wernicke-Korsakoff Syndrome

MalaCards organs/tissues related to Wernicke-Korsakoff Syndrome:

41
Brain, Heart, Eye, Spinal Cord, Hypothalamus, Thalamus, Liver

Publications for Wernicke-Korsakoff Syndrome

Articles related to Wernicke-Korsakoff Syndrome:

(show top 50) (show all 148)
# Title Authors Year
1
What can go wrong in hyperemesis gravidarum: Wernicke-Korsakoff syndrome in Bulawayo, Zimbabwe. ( 29744060 )
2018
2
Wernicke-Korsakoff syndrome complicated by subacute beriberi neuropathy in an alcoholic patient. ( 29128624 )
2018
3
Wernicke-Korsakoff syndrome as a rare phenotype of sporadic Creutzfeldt-Jakob disease. ( 29380664 )
2018
4
Non-alcoholic Wernicke-Korsakoff syndrome heralding non-Hodgkin lymphoma progression. ( 29582179 )
2018
5
Wernicke-Korsakoff syndrome: recognition and treatment. ( 28351256 )
2017
6
A Case of Bariatric Surgery-related Wernicke-Korsakoff Syndrome with Persisting Anterograde Amnesia. ( 28430846 )
2017
7
Long-Term Mortality of Patients with an Alcohol-Related Wernicke-Korsakoff Syndrome. ( 28340112 )
2017
8
Repetitive Transcranial Magnetic Stimulation for Wernicke-Korsakoff Syndrome: A Case Report. ( 28289650 )
2017
9
Wernicke-Korsakoff Syndrome ( 28613480 )
2017
10
Psychiatric Emergencies for Clinicians: Emergency Department Management of Wernicke-Korsakoff Syndrome. ( 27553920 )
2016
11
Vitamin B1-deficient mice show impairment of hippocampus-dependent memory formation and loss of hippocampal neurons and dendritic spines: potential microendophenotypes of Wernicke-Korsakoff syndrome. ( 27576603 )
2016
12
Nonalcoholic Thiamine-Related Encephalopathy (Wernicke-Korsakoff Syndrome) Among Inpatients With Cancer: A Series of 18 Cases. ( 26791514 )
2016
13
Olmesartan-induced Enteropathy Manifesting as Wernicke-Korsakoff Syndrome. ( 27980272 )
2016
14
Cancer-related Wernicke-Korsakoff syndrome. ( 27299273 )
2016
15
Severe Infections are Common in Thiamine Deficiency and May be Related to Cognitive Outcomes: A Cohort Study of 68 Patients With Wernicke-Korsakoff Syndrome. ( 27498674 )
2016
16
Wernicke-Korsakoff syndrome in patients with cancer: a systematic review. ( 27300674 )
2016
17
A Case of Wernicke-Korsakoff Syndrome Treated 1 Year After the Onset of Symptoms. ( 27057403 )
2015
18
Wernicke-Korsakoff syndrome not related to alcohol use: a systematic review. ( 25589780 )
2015
19
Wernicke-Korsakoff Syndrome as a Consequence of Delusional Food Refusal: A Case Study. ( 26705268 )
2015
20
Spatial cognitive deficits in an animal model of Wernicke-Korsakoff syndrome are related to changes in thalamic VDAC protein concentrations. ( 25766938 )
2015
21
Wernicke-Korsakoff syndrome in a patient with self-neglect associated with severe depression. ( 25057374 )
2014
22
Unusual presentation of uncommon disease: anorexia nervosa presenting as wernicke-korsakoff syndrome-a case report from southeast Asia. ( 24963430 )
2014
23
Neural Correlate of Anterograde Amnesia in Wernicke-Korsakoff Syndrome. ( 25148770 )
2014
24
Biomarkers of delirium as a clue to diagnosis and pathogenesis of Wernicke-Korsakoff syndrome. ( 23790077 )
2013
25
Wernicke-korsakoff syndrome in primary peritoneal cancer. ( 24474922 )
2013
26
Thiamine for prevention and treatment of Wernicke-Korsakoff Syndrome in people who abuse alcohol. ( 23818100 )
2013
27
Isolated prospective confabulation in Wernicke-Korsakoff syndrome: a case for reality filtering. ( 22512690 )
2013
28
Evolution of Wernicke-Korsakoff syndrome in self-neglecting alcoholics: preliminary results of relation with Wernicke-delirium and diabetes mellitus. ( 22332852 )
2012
29
Brain and behavioral pathology in an animal model of Wernicke's encephalopathy and Wernicke-Korsakoff Syndrome. ( 22192411 )
2012
30
Wernicke-Korsakoff-syndrome: under-recognized and under-treated. ( 23157990 )
2012
31
Beyond alcoholism: Wernicke-Korsakoff syndrome in patients with psychiatric disorders. ( 22134191 )
2011
32
Wernicke-Korsakoff syndrome in the course of thyrotoxicosis - a case report. ( 21528481 )
2011
33
Teaching neuroimages: bilateral anterior thalami and fornix macrohemorrhage in Wernicke-Korsakoff syndrome. ( 22123785 )
2011
34
Stage-dependent alterations of progenitor cell proliferation and neurogenesis in an animal model of Wernicke-Korsakoff syndrome. ( 21440532 )
2011
35
Anorexia nervosa and Wernicke-Korsakoff syndrome: a case report. ( 20646296 )
2010
36
Complete recovery from undertreated Wernicke-Korsakoff syndrome following aggressive thiamine treatment. ( 20364001 )
2010
37
Cholinergic dysfunction and amnesia in patients with Wernicke-Korsakoff syndrome: a transcranial magnetic stimulation study. ( 19960210 )
2010
38
Neuroimaging of the Wernicke-Korsakoff syndrome. ( 19066199 )
2009
39
Severe dysphagia as the presenting symptom of Wernicke-Korsakoff syndrome in a non-alcoholic man. ( 18379741 )
2008
40
Wernicke-Korsakoff syndrome (WKS) in Australia: no room for complacency. ( 18584388 )
2008
41
Correlation between phosphorylation level of a hippocampal 86kDa protein and extinction of a behaviour in a model of Wernicke-Korsakoff syndrome. ( 17395279 )
2007
42
Wernicke-Korsakoff syndrome. ( 16369858 )
2006
43
A clinical and radiographic variant of Wernicke-Korsakoff syndrome in a nonalcoholic patient. ( 17159109 )
2006
44
Direct genomic PCR sequencing of the high affinity thiamine transporter (SLC19A2) gene identifies three genetic variants in Wernicke Korsakoff syndrome (WKS). ( 16015585 )
2005
45
Eating avoidance disorder and Wernicke-Korsakoff syndrome following gastric bypass: an under-diagnosed association. ( 16197798 )
2005
46
Acetylcholinesterase inhibitors for the treatment of Wernicke-Korsakoff syndrome--three further cases show response to donepezil. ( 15596427 )
2005
47
Cholinergic parameters and the retrieval of learned and re-learned spatial information: a study using a model of Wernicke-Korsakoff Syndrome. ( 15922063 )
2005
48
Functional MRI study of diencephalic amnesia in Wernicke-Korsakoff syndrome. ( 15817513 )
2005
49
Wernicke-Korsakoff syndrome associated with hyperemesis gravidarum. ( 16209289 )
2005
50
Wernicke-Korsakoff syndrome and galantamine. ( 15232056 )
2004

Variations for Wernicke-Korsakoff Syndrome

Expression for Wernicke-Korsakoff Syndrome

Search GEO for disease gene expression data for Wernicke-Korsakoff Syndrome.

Pathways for Wernicke-Korsakoff Syndrome

GO Terms for Wernicke-Korsakoff Syndrome

Cellular components related to Wernicke-Korsakoff Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 oxoglutarate dehydrogenase complex GO:0045252 8.62 DLST OGDH

Biological processes related to Wernicke-Korsakoff Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 metabolic process GO:0008152 9.65 DLST OGDH TKT
2 generation of precursor metabolites and energy GO:0006091 9.46 DLST OGDH
3 phosphatidylcholine biosynthetic process GO:0006656 9.43 ACHE CHAT
4 tricarboxylic acid cycle GO:0006099 9.4 DLST OGDH
5 cellular nitrogen compound metabolic process GO:0034641 9.37 DLST OGDH
6 2-oxoglutarate metabolic process GO:0006103 9.32 DLST OGDH
7 neurotransmitter biosynthetic process GO:0042136 9.26 ACHE CHAT
8 lysine catabolic process GO:0006554 9.16 DLST OGDH
9 succinyl-CoA metabolic process GO:0006104 8.96 DLST OGDH
10 histone succinylation GO:0106077 8.62 DLST OGDH

Sources for Wernicke-Korsakoff Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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