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 53 12 49 50 14 69
Korsakoff Syndrome 12 51 41
Transketolase Defect 53 69
Korsakoff's Syndrome 12 72
Alcohol-Induced Encephalopathy 53
Alcohol Induced Encephalopathy 69
Korsakoff's Psychosis 12
Korsakov's Psychosis 12
Korsakov Psychosis 12

Characteristics:

OMIM:

53
Inheritance:
autosomal recessive


HPO:

31
wernicke-korsakoff syndrome:
Inheritance autosomal recessive inheritance


Classifications:



Summaries for Wernicke-Korsakoff Syndrome

NIH Rare Diseases : 49 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.  Last updated: 7/26/2016

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

NINDS : 50 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 : 72 Korsakoff\'s syndrome is an amnestic disorder caused by thiamine deficiency usually associated with... more...

Description from OMIM: 277730

Related Diseases for Wernicke-Korsakoff Syndrome

Diseases related to Wernicke-Korsakoff Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 33)
# Related Disease Score Top Affiliating Genes
1 beriberi 30.9 SLC19A2 TKT
2 alzheimer disease 28.7 ACHE CHAT DLST SERPINA3
3 wernicke encephalopathy 11.9
4 cerebral beriberi 11.3
5 korsakoff's amnesic syndrome 11.3
6 cerebritis 10.3
7 neuronitis 10.3
8 encephalopathy 10.3
9 creutzfeldt-jakob disease 10.2
10 ataxia and polyneuropathy, adult-onset 10.2
11 bulimia nervosa 2 10.2
12 alcohol abuse 10.2
13 dementia 10.2
14 polyneuropathy 10.2
15 anterograde amnesia 10.2
16 multiple sclerosis 10.0
17 graves disease 1 10.0
18 diabetes mellitus 10.0
19 lymphoma 10.0
20 peritonitis 10.0
21 neuropathy 10.0
22 encephalitis 10.0
23 brainstem auditory evoked responses 10.0
24 cerebral atrophy 10.0
25 dysphagia 10.0
26 head injury 10.0
27 cenani-lenz syndactyly syndrome 10.0 ACHE CHAT
28 tendinosis 10.0 ACHE CHAT
29 amnestic disorder 9.9 ACHE CHAT
30 alpha-ketoglutarate dehydrogenase deficiency 9.9 DLST OGDH
31 megacolon 9.8 ACHE CHAT
32 vascular dementia 9.7 ACHE CHAT SERPINA3
33 supranuclear palsy, progressive, 1 9.7 ACHE CHAT

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:

53
Neurologic Central Nervous System:
ataxia
ocular muscle abnormalities
coma (if untreated)

Neurologic Peripheral Nervous System:
polyneuropathy

Head And Neck Eyes:
ophthalmoplegia
horizontal nystagmus
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:

31 (show all 9)
# Description HPO Frequency HPO Source Accession
1 ataxia 31 HP:0001251
2 ophthalmoplegia 31 HP:0000602
3 ptosis 31 HP:0000508
4 memory impairment 31 HP:0002354
5 psychosis 31 HP:0000709
6 coma 31 HP:0001259
7 polyneuropathy 31 HP:0001271
8 confusion 31 HP:0001289
9 horizontal nystagmus 31 HP:0000666

UMLS symptoms related to Wernicke-Korsakoff Syndrome:


ophthalmoplegia, ataxia

MGI Mouse Phenotypes related to Wernicke-Korsakoff Syndrome:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 adipose tissue MP:0005375 9.46 ACHE KIAA0753 OGDH TKT
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
Thiamine Approved, Investigational, Nutraceutical, Vet_approved Phase 4,Phase 3 59-43-8 1130
2
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3 59-30-3 6037
3 Vitamin B Complex Phase 4,Phase 3
4 Vitamins Phase 4,Phase 3
5 Micronutrients Phase 4,Phase 3
6 Trace Elements Phase 4,Phase 3
7 Thiamin Nutraceutical Phase 4,Phase 3
8 Folate Nutraceutical Phase 4,Phase 3
9 Vitamin B9 Nutraceutical Phase 4,Phase 3
10
Diphenhydramine Approved, Investigational Phase 3 58-73-1, 147-24-0 3100
11
Histamine Approved, Investigational Phase 3 51-45-6, 75614-87-8 774
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 3416-24-8 439213
29 Adjuvants, Immunologic
30 Antioxidants
31 Benphothiamine
32 Chelating Agents
33 Protective Agents
34 Tezosentan
35 Thioctic Acid
36 pyruvate Nutraceutical
37 Alpha-lipoic Acid Nutraceutical

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Optimum Thiamine Intervention (OpTIn) Trial Recruiting NCT02788552 Phase 4 Thiamine Hydrochloride
2 Does Thiamine Help Vomiting and Nausea in Pregnancy? Terminated NCT00861523 Phase 3 thiamine & promethazine
3 From Uncomplicated Alcoholism to Korsakoff's Syndrome Unknown status NCT01455207
4 Study of Dry Beriberi in Mayotte, Comoro Archipelago Unknown status NCT00934349
5 Thiamin (Vitamin B1) Levels in Eating Disorder Adolescent Patients Unknown status NCT02164487
6 Reactive Oxygen Species in the Pathogenesis of Diabetic Complications Completed NCT00703989
7 Models of Disordered Knowledge and Memory Systems in Dementia and Related Disorders Completed NCT00004557
8 The Effect of tDCS on Subcortical Brain Functioning Enrolling by invitation NCT01602276

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:

38
Brain, Heart, Eye, Spinal Cord, Hypothalamus, Thalamus, Liver

Publications for Wernicke-Korsakoff Syndrome

Articles related to Wernicke-Korsakoff Syndrome:

(show top 50) (show all 145)
# Title Authors Year
1
Wernicke-Korsakoff syndrome complicated by subacute beriberi neuropathy in an alcoholic patient. ( 29128624 )
2018
2
Wernicke-Korsakoff syndrome as a rare phenotype of sporadic Creutzfeldt-Jakob disease. ( 29380664 )
2018
3
A Case of Bariatric Surgery-related Wernicke-Korsakoff Syndrome with Persisting Anterograde Amnesia. ( 28430846 )
2017
4
Repetitive Transcranial Magnetic Stimulation for Wernicke-Korsakoff Syndrome: A Case Report. ( 28289650 )
2017
5
Wernicke-Korsakoff Syndrome ( 28613480 )
2017
6
Wernicke-Korsakoff syndrome: recognition and treatment. ( 28351256 )
2017
7
Long-Term Mortality of Patients with an Alcohol-Related Wernicke-Korsakoff Syndrome. ( 28340112 )
2017
8
Nonalcoholic Thiamine-Related Encephalopathy (Wernicke-Korsakoff Syndrome) Among Inpatients With Cancer: A Series of 18 Cases. ( 26791514 )
2016
9
Cancer-related Wernicke-Korsakoff syndrome. ( 27299273 )
2016
10
Olmesartan-induced Enteropathy Manifesting as Wernicke-Korsakoff Syndrome. ( 27980272 )
2016
11
Psychiatric Emergencies for Clinicians: Emergency Department Management of Wernicke-Korsakoff Syndrome. ( 27553920 )
2016
12
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
13
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
14
Wernicke-Korsakoff syndrome in patients with cancer: a systematic review. ( 27300674 )
2016
15
Wernicke-Korsakoff syndrome not related to alcohol use: a systematic review. ( 25589780 )
2015
16
Wernicke-Korsakoff Syndrome as a Consequence of Delusional Food Refusal: A Case Study. ( 26705268 )
2015
17
A Case of Wernicke-Korsakoff Syndrome Treated 1 Year After the Onset of Symptoms. ( 27057403 )
2015
18
Spatial cognitive deficits in an animal model of Wernicke-Korsakoff syndrome are related to changes in thalamic VDAC protein concentrations. ( 25766938 )
2015
19
Neural Correlate of Anterograde Amnesia in Wernicke-Korsakoff Syndrome. ( 25148770 )
2014
20
Wernicke-Korsakoff syndrome in a patient with self-neglect associated with severe depression. ( 25057374 )
2014
21
Unusual presentation of uncommon disease: anorexia nervosa presenting as wernicke-korsakoff syndrome-a case report from southeast Asia. ( 24963430 )
2014
22
Thiamine for prevention and treatment of Wernicke-Korsakoff Syndrome in people who abuse alcohol. ( 23818100 )
2013
23
Isolated prospective confabulation in Wernicke-Korsakoff syndrome: a case for reality filtering. ( 22512690 )
2013
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
Evolution of Wernicke-Korsakoff syndrome in self-neglecting alcoholics: preliminary results of relation with Wernicke-delirium and diabetes mellitus. ( 22332852 )
2012
27
Wernicke-Korsakoff-syndrome: under-recognized and under-treated. ( 23157990 )
2012
28
Brain and behavioral pathology in an animal model of Wernicke's encephalopathy and Wernicke-Korsakoff Syndrome. ( 22192411 )
2012
29
Wernicke-Korsakoff syndrome in the course of thyrotoxicosis - a case report. ( 21528481 )
2011
30
Teaching neuroimages: bilateral anterior thalami and fornix macrohemorrhage in Wernicke-Korsakoff syndrome. ( 22123785 )
2011
31
Stage-dependent alterations of progenitor cell proliferation and neurogenesis in an animal model of Wernicke-Korsakoff syndrome. ( 21440532 )
2011
32
Beyond alcoholism: Wernicke-Korsakoff syndrome in patients with psychiatric disorders. ( 22134191 )
2011
33
Complete recovery from undertreated Wernicke-Korsakoff syndrome following aggressive thiamine treatment. ( 20364001 )
2010
34
Cholinergic dysfunction and amnesia in patients with Wernicke-Korsakoff syndrome: a transcranial magnetic stimulation study. ( 19960210 )
2010
35
Anorexia nervosa and Wernicke-Korsakoff syndrome: a case report. ( 20646296 )
2010
36
Neuroimaging of the Wernicke-Korsakoff syndrome. ( 19066199 )
2009
37
Wernicke-Korsakoff syndrome (WKS) in Australia: no room for complacency. ( 18584388 )
2008
38
Severe dysphagia as the presenting symptom of Wernicke-Korsakoff syndrome in a non-alcoholic man. ( 18379741 )
2008
39
Correlation between phosphorylation level of a hippocampal 86kDa protein and extinction of a behaviour in a model of Wernicke-Korsakoff syndrome. ( 17395279 )
2007
40
A clinical and radiographic variant of Wernicke-Korsakoff syndrome in a nonalcoholic patient. ( 17159109 )
2006
41
Wernicke-Korsakoff syndrome. ( 16369858 )
2006
42
Eating avoidance disorder and Wernicke-Korsakoff syndrome following gastric bypass: an under-diagnosed association. ( 16197798 )
2005
43
Acetylcholinesterase inhibitors for the treatment of Wernicke-Korsakoff syndrome--three further cases show response to donepezil. ( 15596427 )
2005
44
Cholinergic parameters and the retrieval of learned and re-learned spatial information: a study using a model of Wernicke-Korsakoff Syndrome. ( 15922063 )
2005
45
Functional MRI study of diencephalic amnesia in Wernicke-Korsakoff syndrome. ( 15817513 )
2005
46
Wernicke-Korsakoff syndrome associated with hyperemesis gravidarum. ( 16209289 )
2005
47
Direct genomic PCR sequencing of the high affinity thiamine transporter (SLC19A2) gene identifies three genetic variants in Wernicke Korsakoff syndrome (WKS). ( 16015585 )
2005
48
Increased cerebellar PET glucose metabolism corresponds to ataxia in Wernicke-Korsakoff syndrome. ( 14998834 )
2004
49
Thiamine for Wernicke-Korsakoff Syndrome in people at risk from alcohol abuse. ( 14974055 )
2004
50
Repeated clinical episodes of Wernicke-Korsakoff syndrome. ( 15298591 )
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 9.58 DLST OGDH TKT
2 generation of precursor metabolites and energy GO:0006091 9.37 DLST OGDH
3 tricarboxylic acid cycle GO:0006099 9.32 DLST OGDH
4 phosphatidylcholine biosynthetic process GO:0006656 9.26 ACHE CHAT
5 cellular nitrogen compound metabolic process GO:0034641 9.16 DLST OGDH
6 neurotransmitter biosynthetic process GO:0042136 8.96 ACHE CHAT
7 lysine catabolic process GO:0006554 8.62 DLST OGDH

Sources for Wernicke-Korsakoff Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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