MCID: WRN002
MIFTS: 53

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 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
Wernickekorsakoff Syndrome 76
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 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 Carbon metabolism and Amino Acid metabolism. 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 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 : 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 delirium 32 HP:0031258

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 behavior/neurological MP:0005386 9.73 ACHE CHAT DLST OGDH SLC19A2 TKT
2 growth/size/body region MP:0005378 9.7 ACHE CHAT DLST KIAA0753 LEFTY2 OGDH
3 adipose tissue MP:0005375 9.62 ACHE KIAA0753 OGDH TKT
4 mortality/aging MP:0010768 9.5 ACHE CHAT KIAA0753 LEFTY2 OGDH SLC19A2
5 nervous system MP:0003631 9.1 ACHE CHAT DLST LEFTY2 SLC19A2 TKT

Drugs & Therapeutics for Wernicke-Korsakoff Syndrome

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

(show all 46)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Thiamine Approved, Investigational, Nutraceutical, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 59-43-8, 70-16-6 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 Trace Elements Phase 4,Phase 2,Phase 3,Not Applicable
5 Vitamin B9 Phase 4,Phase 2,Phase 3,Not Applicable
6 Vitamin B Complex Phase 4,Phase 2,Phase 3,Not Applicable
7 Thiamin Phase 4,Phase 2,Phase 3,Not Applicable
8 Folate Phase 4,Phase 2,Phase 3,Not Applicable
9 Vitamins Phase 4,Phase 2,Phase 3,Not Applicable
10
Magnesium Sulfate Approved, Investigational, Vet_approved Phase 2, Phase 3 7487-88-9 24083
11
Ethanol Approved Phase 2, Phase 3 64-17-5 702
12
Histamine Approved, Investigational Phase 3 51-45-6, 75614-87-8 774
13
Promethazine Approved, Investigational Phase 3 60-87-7 4927
14
Diphenhydramine Approved, Investigational Phase 3 147-24-0, 58-73-1 3100
15 Anticonvulsants Phase 2, Phase 3
16 calcium channel blockers Phase 2, Phase 3
17 Calcium, Dietary Phase 2, Phase 3
18 Anesthetics Phase 2, Phase 3,Phase 3
19 Analgesics Phase 2, Phase 3
20 Peripheral Nervous System Agents Phase 2, Phase 3,Phase 3
21 Central Nervous System Depressants Phase 2, Phase 3,Phase 3
22 Tocolytic Agents Phase 2, Phase 3
23 Anti-Arrhythmia Agents Phase 2, Phase 3
24 Neurotransmitter Agents Phase 3
25 Histamine Antagonists Phase 3
26 Histamine H1 Antagonists Phase 3
27 Anti-Allergic Agents Phase 3
28 Anesthetics, Local Phase 3
29 Hypnotics and Sedatives Phase 3
30 Dermatologic Agents Phase 3
31 Antiemetics Phase 3
32 Autonomic Agents Phase 3
33 Gastrointestinal Agents Phase 3
34
Histamine Phosphate Phase 3 51-74-1 65513
35 Antipruritics Phase 3
36
Glucosamine Approved, Investigational Not Applicable 3416-24-8 439213
37 Tezosentan Investigational Not Applicable 180384-57-0
38 pyruvate Not Applicable
39 Antioxidants Not Applicable
40 Adjuvants, Immunologic Not Applicable
41 Immunologic Factors Not Applicable
42 Thioctic Acid Not Applicable
43 Protective Agents Not Applicable
44 Benphothiamine Not Applicable
45 Chelating Agents Not Applicable
46 Alpha-lipoic Acid Not Applicable

Interventional clinical trials:

(show all 15)
# 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 Not yet recruiting NCT03755518 Phase 3 FEDRATINIB
5 Does Thiamine Help Vomiting and Nausea in Pregnancy? Terminated NCT00861523 Phase 3 thiamine & promethazine
6 From Uncomplicated Alcoholism to Korsakoff's Syndrome Unknown status NCT01455207 Not Applicable
7 Study of Dry Beriberi in Mayotte, Comoro Archipelago Unknown status NCT00934349
8 Thiamin (Vitamin B1) Levels in Eating Disorder Adolescent Patients Unknown status NCT02164487
9 Reactive Oxygen Species in the Pathogenesis of Diabetic Complications Completed NCT00703989 Not Applicable
10 Models of Disordered Knowledge and Memory Systems in Dementia and Related Disorders Completed NCT00004557
11 Systemic Inflammatory Response: Thiamine and Magnesium Status (Sir TaM Study) Recruiting NCT03554668
12 Alzheimer's Autism and Cognitive Impairment Stem Cell Treatment Study Recruiting NCT03724136 Not Applicable
13 Thiamine Responsive Disorders (TRD) Among Infants in Lao PDR Not yet recruiting NCT03626337 Thiamine 100 MG/ML
14 Trial of Thiamine Supplementation in Cambodia Not yet recruiting NCT03616288 Not Applicable
15 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:

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 211)
# 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
Imbalance between cognitive systems in alcohol-dependence and Korsakoff syndrome: An exploration using the Alcohol Flanker Task. ( 29509075 )
2018
6
Is physical rehabilitation effective for Wernicke-Korsakoff syndrome? A case report. ( 29994923 )
2018
7
Wernicke-Korsakoff Syndrome in End-Stage Renal Disease: A Case Report. ( 30009401 )
2018
8
Impaired affective prosody decoding in severe alcohol use disorder and Korsakoff syndrome. ( 29679843 )
2018
9
Korsakoff syndrome from retrochiasmatic suprasellar lesions: rapid reversal after relief of cerebral compression in 4 cases. ( 28574307 )
2018
10
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
11
Korsakoff Syndrome in Non-alcoholic Psychiatric Patients. Variable Cognitive Presentation and Impaired Frontotemporal Connectivity. ( 29910747 )
2018
12
Wernicke-Korsakoff syndrome: recognition and treatment. ( 28351256 )
2017
13
A Case of Bariatric Surgery-related Wernicke-Korsakoff Syndrome with Persisting Anterograde Amnesia. ( 28430846 )
2017
14
Long-Term Mortality of Patients with an Alcohol-Related Wernicke-Korsakoff Syndrome. ( 28340112 )
2017
15
Repetitive Transcranial Magnetic Stimulation for Wernicke-Korsakoff Syndrome: A Case Report. ( 28289650 )
2017
16
Personal semantic and episodic autobiographical memories in Korsakoff syndrome: A comparison of interview methods. ( 27829317 )
2017
17
Prevalence and severity of behavioural symptoms in patients with Korsakoff syndrome and other alcohol-related cognitive disorders: a systematic review. ( 27918121 )
2017
18
Source Memory in Korsakoff Syndrome: Disentangling the Mechanisms of Temporal Confusion. ( 28160301 )
2017
19
Psychiatric Emergencies for Clinicians: Emergency Department Management of Wernicke-Korsakoff Syndrome. ( 27553920 )
2016
20
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
21
Nonalcoholic Thiamine-Related Encephalopathy (Wernicke-Korsakoff Syndrome) Among Inpatients With Cancer: A Series of 18 Cases. ( 26791514 )
2016
22
Olmesartan-induced Enteropathy Manifesting as Wernicke-Korsakoff Syndrome. ( 27980272 )
2016
23
Cancer-related Wernicke-Korsakoff syndrome. ( 27299273 )
2016
24
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
25
Wernicke-Korsakoff syndrome in patients with cancer: a systematic review. ( 27300674 )
2016
26
Characteristic Neuroimaging Abnormalities of Korsakoff Syndrome. ( 27571012 )
2016
27
Non-alcoholic Korsakoff syndrome in psychiatric patients with a history of undiagnosed Wernicke's encephalopathy. ( 27772780 )
2016
28
A Case of Wernicke-Korsakoff Syndrome Treated 1 Year After the Onset of Symptoms. ( 27057403 )
2015
29
Wernicke-Korsakoff syndrome not related to alcohol use: a systematic review. ( 25589780 )
2015
30
Wernicke-Korsakoff Syndrome as a Consequence of Delusional Food Refusal: A Case Study. ( 26705268 )
2015
31
Spatial cognitive deficits in an animal model of Wernicke-Korsakoff syndrome are related to changes in thalamic VDAC protein concentrations. ( 25766938 )
2015
32
What does a comparison of the alcoholic Korsakoff syndrome and thalamic infarction tell us about thalamic amnesia? ( 25218758 )
2015
33
Contrast enhancement in the mammillary bodies: An easily missed sign of Korsakoff syndrome. ( 29213992 )
2015
34
Wernicke-Korsakoff syndrome in a patient with self-neglect associated with severe depression. ( 25057374 )
2014
35
Unusual presentation of uncommon disease: anorexia nervosa presenting as wernicke-korsakoff syndrome-a case report from southeast Asia. ( 24963430 )
2014
36
Neural Correlate of Anterograde Amnesia in Wernicke-Korsakoff Syndrome. ( 25148770 )
2014
37
Patients with Korsakoff syndrome in nursing homes: characteristics, comorbidity, and use of psychotropic drugs. ( 24308344 )
2014
38
Revisiting the continuum hypothesis: toward an in-depth exploration of executive functions in korsakoff syndrome. ( 25071526 )
2014
39
Biomarkers of delirium as a clue to diagnosis and pathogenesis of Wernicke-Korsakoff syndrome. ( 23790077 )
2013
40
Wernicke-korsakoff syndrome in primary peritoneal cancer. ( 24474922 )
2013
41
Thiamine for prevention and treatment of Wernicke-Korsakoff Syndrome in people who abuse alcohol. ( 23818100 )
2013
42
Isolated prospective confabulation in Wernicke-Korsakoff syndrome: a case for reality filtering. ( 22512690 )
2013
43
Use of an electronic cognitive aid by a person with Korsakoff syndrome. ( 23883173 )
2013
44
Evolution of Wernicke-Korsakoff syndrome in self-neglecting alcoholics: preliminary results of relation with Wernicke-delirium and diabetes mellitus. ( 22332852 )
2012
45
Brain and behavioral pathology in an animal model of Wernicke's encephalopathy and Wernicke-Korsakoff Syndrome. ( 22192411 )
2012
46
Wernicke-Korsakoff-syndrome: under-recognized and under-treated. ( 23157990 )
2012
47
Translational rodent models of Korsakoff syndrome reveal the critical neuroanatomical substrates of memory dysfunction and recovery. ( 22528861 )
2012
48
Anterograde episodic memory in Korsakoff syndrome. ( 22644546 )
2012
49
Beyond alcoholism: Wernicke-Korsakoff syndrome in patients with psychiatric disorders. ( 22134191 )
2011
50
Wernicke-Korsakoff syndrome in the course of thyrotoxicosis - a case report. ( 21528481 )
2011

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 platelet alpha granule lumen GO:0031093 8.96 LEFTY2 SERPINA3
2 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 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
Content
Loading form....