MCID: WRN002
MIFTS: 52

Wernicke-Korsakoff Syndrome

Categories: Mental diseases, Metabolic diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Wernicke-Korsakoff Syndrome

MalaCards integrated aliases for Wernicke-Korsakoff Syndrome:

Name: Wernicke-Korsakoff Syndrome 58 12 77 54 55 15 17 74
Korsakoff Syndrome 12 56 45
Transketolase Defect 58 74
Korsakoff's Syndrome 12 77
Alcohol-Induced Encephalopathy 58
Alcohol Induced Encephalopathy 74
Korsakoff's Psychosis 12
Korsakov's Psychosis 12
Korsakov Psychosis 12

Characteristics:

OMIM:

58
Inheritance:
autosomal recessive


HPO:

33
wernicke-korsakoff syndrome:
Inheritance autosomal recessive inheritance


Classifications:



Summaries for Wernicke-Korsakoff Syndrome

NIH Rare Diseases : 54 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 amnestic disorder, 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 Thiamine and Folic Acid have been mentioned in the context of this disorder. Affiliated tissues include brain, eye and spinal cord, and related phenotypes are ptosis and ataxia

Disease Ontology : 12 A nutritional deficiency disease that is characterized by ophthalmoplegia, ataxia, change in mental status and acute onset of severe memory impairment without any dysfunction in intellectual abilities, and has material basis in thiamine deficiency.

NINDS : 55 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 : 77 Korsakoff syndrome is an amnestic disorder caused by thiamine (vitamin B1) deficiency 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 48)
# Related Disease Score Top Affiliating Genes
1 beriberi 31.1 SLC19A2 TKT
2 amnestic disorder 30.7 ACHE CHAT
3 vascular dementia 29.8 ACHE CHAT SERPINA3
4 wernicke encephalopathy 12.3
5 korsakoff's amnesic syndrome 11.5
6 cerebral beriberi 11.5
7 alcohol dependence 10.5
8 encephalopathy 10.5
9 creutzfeldt-jakob disease 10.4
10 anorexia nervosa 10.4
11 lymphoma 10.4
12 dementia 10.4
13 polyneuropathy 10.4
14 anterograde amnesia 10.4
15 alzheimer disease 10.2
16 multiple sclerosis 10.2
17 lymphoma, hodgkin, classic 10.2
18 graves disease 1 10.2
19 lymphoma, non-hodgkin, familial 10.2
20 alcohol abuse 10.2
21 diabetes mellitus 10.2
22 end stage renal failure 10.2
23 retrograde amnesia 10.2
24 subacute delirium 10.2
25 neuropathy 10.2
26 encephalitis 10.2
27 brainstem auditory evoked responses 10.2
28 depression 10.2
29 cerebral atrophy 10.2
30 dysphagia 10.2
31 head injury 10.2
32 discrimination, two-point, reduction in 10.1
33 diabetes insipidus 10.1
34 binswanger's disease 10.1
35 cenani-lenz syndactyly syndrome 9.9 ACHE CHAT
36 aging 9.9
37 langerhans cell histiocytosis 9.9
38 leukemia 9.9
39 liver disease 9.9
40 choreatic disease 9.9
41 histiocytosis 9.9
42 tendinosis 9.9 ACHE CHAT
43 alpha-ketoglutarate dehydrogenase deficiency 9.9 DLST OGDH
44 schizophrenia 9.9
45 hypoascorbemia 9.9
46 human immunodeficiency virus type 1 9.9
47 supranuclear palsy, progressive, 1 9.8 ACHE CHAT
48 megacolon 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

Human phenotypes related to Wernicke-Korsakoff Syndrome:

33 (show all 9)
# Description HPO Frequency HPO Source Accession
1 ptosis 33 HP:0000508
2 ataxia 33 HP:0001251
3 memory impairment 33 HP:0002354
4 ophthalmoplegia 33 HP:0000602
5 psychosis 33 HP:0000709
6 coma 33 HP:0001259
7 horizontal nystagmus 33 HP:0000666
8 polyneuropathy 33 HP:0001271
9 delirium 33 HP:0031258

Symptoms via clinical synopsis from OMIM:

58
Neurologic Central Nervous System:
ataxia
ocular muscle abnormalities
coma (if untreated)

Neurologic Behavioral Psychiatric Manifestations:
confusion
impaired memory
disorientation
agitated delirium
korsakoff's psychosis
more
Head And Neck Eyes:
ophthalmoplegia
horizontal nystagmus
ptosis (rare)
lateral rectus palsy

Neurologic Peripheral Nervous System:
polyneuropathy

Clinical features from OMIM:

277730

UMLS symptoms related to Wernicke-Korsakoff Syndrome:


ataxia, ophthalmoplegia

MGI Mouse Phenotypes related to Wernicke-Korsakoff Syndrome:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.43 ACHE CHAT DLST OGDH SLC19A2 TKT
2 adipose tissue MP:0005375 9.33 ACHE OGDH 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 49)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Thiamine Approved, Investigational, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 70-16-6, 59-43-8 1130
2
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 59-30-3 6037
3 Micronutrients Phase 4,Phase 2,Phase 3,Not Applicable
4 Vitamins Phase 4,Phase 2,Phase 3,Not Applicable
5 Vitamin B Complex Phase 4,Phase 2,Phase 3,Not Applicable
6 Trace Elements Phase 4,Phase 2,Phase 3,Not Applicable
7 Nutrients Phase 4,Phase 2,Phase 3,Not Applicable
8 Folate Phase 4,Phase 2,Phase 3,Not Applicable
9 Vitamin B9 Phase 4,Phase 2,Phase 3,Not Applicable
10 Thiamin Phase 4,Phase 2,Phase 3,Not Applicable
11
Magnesium Sulfate Approved, Investigational, Vet_approved Phase 2, Phase 3 7487-88-9 24083
12
Ethanol Approved Phase 2, Phase 3 64-17-5 702
13
Histamine Approved, Investigational Phase 3 51-45-6 774
14
Promethazine Approved, Investigational Phase 3 60-87-7 4927
15
Diphenhydramine Approved, Investigational Phase 3 147-24-0, 58-73-1 3100
16
Calcium Approved, Nutraceutical Phase 2, Phase 3 7440-70-2 271
17 Anticonvulsants Phase 2, Phase 3
18 Peripheral Nervous System Agents Phase 2, Phase 3,Phase 3
19 Tocolytic Agents Phase 2, Phase 3
20 Anti-Arrhythmia Agents Phase 2, Phase 3
21 Calcium, Dietary Phase 2, Phase 3
22 Anesthetics Phase 2, Phase 3,Phase 3
23 Central Nervous System Depressants Phase 2, Phase 3,Phase 3
24 Analgesics Phase 2, Phase 3
25 Hormones Phase 2, Phase 3
26 calcium channel blockers Phase 2, Phase 3
27 Dermatologic Agents Phase 3
28
Histamine Phosphate Phase 3 51-74-1 65513
29 Histamine H1 Antagonists Phase 3
30 Hypnotics and Sedatives Phase 3
31 Anti-Allergic Agents Phase 3
32 Histamine Antagonists Phase 3
33 Gastrointestinal Agents Phase 3
34 Anesthetics, Local Phase 3
35 Neurotransmitter Agents Phase 3
36 Antiemetics Phase 3
37 Antipruritics Phase 3
38 Autonomic Agents Phase 3
39
Glucosamine Approved, Investigational Not Applicable 3416-24-8 439213
40 Tezosentan Investigational Not Applicable 180384-57-0
41 pyruvate Not Applicable
42 Immunologic Factors Not Applicable
43 Adjuvants, Immunologic Not Applicable
44 Protective Agents Not Applicable
45 Thioctic Acid Not Applicable
46 Antioxidants Not Applicable
47 Alpha-lipoic Acid Not Applicable
48 Chelating Agents Not Applicable
49 Benphothiamine Not Applicable

Interventional clinical trials:

(show all 16)
# 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 An Efficacy and Safety Trial of Fedratinib in Subjects With DIPSS, Intermediate or High-Risk Primary Myelofibrosis, Post-Polycythemia Vera Myelofibrosis, or Post-Essential Thrombocythemia Myelofibrosis and Previously Treated With Ruxolitinib Recruiting NCT03755518 Phase 3 FEDRATINIB
5 An Efficacy and Safety Study of Fedratinib Compared to Best Available Therapy in Subjects With DIPSS-intermediate or High-risk Primary Myelofibrosis, Post-polycythemia Vera Myelofibrosis, or Post-essential Thrombocythemia Myelofibrosis and Previously Treated With Ruxolitinib Not yet recruiting NCT03952039 Phase 3 FEDRATINIB;Best Available Therapy (BAT)
6 Does Thiamine Help Vomiting and Nausea in Pregnancy? Terminated NCT00861523 Phase 3 thiamine & promethazine
7 From Uncomplicated Alcoholism to Korsakoff's Syndrome Unknown status NCT01455207 Not Applicable
8 Study of Dry Beriberi in Mayotte, Comoro Archipelago Unknown status NCT00934349
9 Thiamin (Vitamin B1) Levels in Eating Disorder Adolescent Patients Unknown status NCT02164487
10 Reactive Oxygen Species in the Pathogenesis of Diabetic Complications Completed NCT00703989 Not Applicable
11 Models of Disordered Knowledge and Memory Systems in Dementia and Related Disorders Completed NCT00004557
12 Systemic Inflammatory Response: Thiamine and Magnesium Status (Sir TaM Study) Recruiting NCT03554668
13 Alzheimer's Autism and Cognitive Impairment Stem Cell Treatment Study Recruiting NCT03724136 Not Applicable
14 Thiamine Responsive Disorders (TRD) Among Infants in Lao PDR Not yet recruiting NCT03626337 Thiamine 100 MG/ML
15 Trial of Thiamine Supplementation in Cambodia Not yet recruiting NCT03616288 Not Applicable
16 The Effect of tDCS on Subcortical Brain Functioning Terminated 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:

42
Brain, Eye, Spinal Cord, Hypothalamus, Thalamus, Testes, T Cells

Publications for Wernicke-Korsakoff Syndrome

Articles related to Wernicke-Korsakoff Syndrome:

(show top 50) (show all 223)
# Title Authors Year
1
Wernicke-Korsakoff syndrome: Focus on low-threshold diagnosis and prompt treatment in the primary care setting. ( 30823858 )
2019
2
Awareness and its relationships with neuropsychiatric symptoms in people with Korsakoff syndrome or other alcohol-related cognitive disorders living in specialized nursing homes. ( 30854733 )
2019
3
Smartwatch aids time-based prospective memory in Korsakoff syndrome: a case study. ( 30966873 )
2019
4
Future Thinking in Korsakoff Syndrome. ( 31044226 )
2019
5
Imbalance between cognitive systems in alcohol-dependence and Korsakoff syndrome: An exploration using the Alcohol Flanker Task. ( 29509075 )
2018
6
Wernicke-Korsakoff syndrome complicated by subacute beriberi neuropathy in an alcoholic patient. ( 29128624 )
2018
7
Wernicke-Korsakoff syndrome as a rare phenotype of sporadic Creutzfeldt-Jakob disease. ( 29380664 )
2018
8
Non-alcoholic Wernicke-Korsakoff syndrome heralding non-Hodgkin lymphoma progression. ( 29582179 )
2018
9
What can go wrong in hyperemesis gravidarum: Wernicke-Korsakoff syndrome in Bulawayo, Zimbabwe. ( 29744060 )
2018
10
Is physical rehabilitation effective for Wernicke-Korsakoff syndrome? A case report. ( 29994923 )
2018
11
Wernicke-Korsakoff Syndrome in End-Stage Renal Disease: A Case Report. ( 30009401 )
2018
12
Vitamin Deficiency and Systemic Failure: The Case For Greater Focus On Wernicke-Korsakoff Syndrome ( 30560643 )
2018
13
Impaired affective prosody decoding in severe alcohol use disorder and Korsakoff syndrome. ( 29679843 )
2018
14
Korsakoff syndrome from retrochiasmatic suprasellar lesions: rapid reversal after relief of cerebral compression in 4 cases. ( 28574307 )
2018
15
Neuropsychiatric Symptoms in People With Korsakoff Syndrome and Other Alcohol-Related Cognitive Disorders Living in Specialized Long-Term Care Facilities: Prevalence, Severity, and Associated Caregiver Distress. ( 29079031 )
2018
16
Korsakoff Syndrome in Non-alcoholic Psychiatric Patients. Variable Cognitive Presentation and Impaired Frontotemporal Connectivity. ( 29910747 )
2018
17
Crossmodal processing of emotions in alcohol-dependence and Korsakoff syndrome. ( 28885888 )
2017
18
A Case of Bariatric Surgery-related Wernicke-Korsakoff Syndrome with Persisting Anterograde Amnesia. ( 28430846 )
2017
19
Repetitive Transcranial Magnetic Stimulation for Wernicke-Korsakoff Syndrome: A Case Report. ( 28289650 )
2017
20
Long-Term Mortality of Patients with an Alcohol-Related Wernicke-Korsakoff Syndrome. ( 28340112 )
2017
21
Wernicke-Korsakoff syndrome: recognition and treatment. ( 28351256 )
2017
22
Personal semantic and episodic autobiographical memories in Korsakoff syndrome: A comparison of interview methods. ( 27829317 )
2017
23
Prevalence and severity of behavioural symptoms in patients with Korsakoff syndrome and other alcohol-related cognitive disorders: a systematic review. ( 27918121 )
2017
24
Source Memory in Korsakoff Syndrome: Disentangling the Mechanisms of Temporal Confusion. ( 28160301 )
2017
25
Non-alcoholic Korsakoff syndrome in psychiatric patients with a history of undiagnosed Wernicke's encephalopathy. ( 27772780 )
2016
26
Nonalcoholic Thiamine-Related Encephalopathy (Wernicke-Korsakoff Syndrome) Among Inpatients With Cancer: A Series of 18 Cases. ( 26791514 )
2016
27
Cancer-related Wernicke-Korsakoff syndrome. ( 27299273 )
2016
28
Wernicke-Korsakoff syndrome in patients with cancer: a systematic review. ( 27300674 )
2016
29
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
30
Psychiatric Emergencies for Clinicians: Emergency Department Management of Wernicke-Korsakoff Syndrome. ( 27553920 )
2016
31
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
32
Olmesartan-induced Enteropathy Manifesting as Wernicke-Korsakoff Syndrome. ( 27980272 )
2016
33
Characteristic Neuroimaging Abnormalities of Korsakoff Syndrome. ( 27571012 )
2016
34
Neural Correlate of Anterograde Amnesia in Wernicke-Korsakoff Syndrome. ( 25148770 )
2015
35
Wernicke-Korsakoff syndrome not related to alcohol use: a systematic review. ( 25589780 )
2015
36
Spatial cognitive deficits in an animal model of Wernicke-Korsakoff syndrome are related to changes in thalamic VDAC protein concentrations. ( 25766938 )
2015
37
Wernicke-Korsakoff Syndrome as a Consequence of Delusional Food Refusal: A Case Study. ( 26705268 )
2015
38
A Case of Wernicke-Korsakoff Syndrome Treated 1 Year After the Onset of Symptoms. ( 27057403 )
2015
39
What does a comparison of the alcoholic Korsakoff syndrome and thalamic infarction tell us about thalamic amnesia? ( 25218758 )
2015
40
Contrast enhancement in the mammillary bodies: An easily missed sign of Korsakoff syndrome. ( 29213992 )
2015
41
Unusual presentation of uncommon disease: anorexia nervosa presenting as wernicke-korsakoff syndrome-a case report from southeast Asia. ( 24963430 )
2014
42
Wernicke-Korsakoff syndrome in a patient with self-neglect associated with severe depression. ( 25057374 )
2014
43
Patients with Korsakoff syndrome in nursing homes: characteristics, comorbidity, and use of psychotropic drugs. ( 24308344 )
2014
44
Revisiting the continuum hypothesis: toward an in-depth exploration of executive functions in korsakoff syndrome. ( 25071526 )
2014
45
Isolated prospective confabulation in Wernicke-Korsakoff syndrome: a case for reality filtering. ( 22512690 )
2013
46
Biomarkers of delirium as a clue to diagnosis and pathogenesis of Wernicke-Korsakoff syndrome. ( 23790077 )
2013
47
Thiamine for prevention and treatment of Wernicke-Korsakoff Syndrome in people who abuse alcohol. ( 23818100 )
2013
48
Wernicke-korsakoff syndrome in primary peritoneal cancer. ( 24474922 )
2013
49
Executive functioning in chronic alcoholism and Korsakoff syndrome. ( 23656524 )
2013
50
Use of an electronic cognitive aid by a person with Korsakoff syndrome. ( 23883173 )
2013

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 generation of precursor metabolites and energy GO:0006091 9.4 DLST OGDH
2 tricarboxylic acid cycle GO:0006099 9.37 DLST OGDH
3 phosphatidylcholine biosynthetic process GO:0006656 9.32 ACHE CHAT
4 2-oxoglutarate metabolic process GO:0006103 9.26 DLST OGDH
5 neurotransmitter biosynthetic process GO:0042136 9.16 ACHE CHAT
6 succinyl-CoA metabolic process GO:0006104 8.96 DLST OGDH
7 histone succinylation GO:0106077 8.62 DLST OGDH

Sources for Wernicke-Korsakoff Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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