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Wernicke encephalopathy

MedGen UID:
53073
•Concept ID:
C0043121
•
Disease or Syndrome
Synonyms: Beriberi, Cerebral; Cerebral Beriberi; Encephalopathies, Wernicke; Encephalopathy, Gayet Wernicke; Encephalopathy, Gayet-Wernicke; Encephalopathy, Wernicke; Encephalopathy, Wernicke's; Encephalopathy, Wernickes; Gayet Wernicke Encephalopathy; Gayet-Wernicke Encephalopathy; Wernicke Disease; Wernicke Encephalopathies; Wernicke Encephalopathy; Wernicke Polioencephalitis, Superior Hemorrhagic; Wernicke Superior Hemorrhagic Polioencephalitis; Wernicke Syndrome; Wernicke's Disease; Wernicke's Encephalopathy; Wernicke's Polioencephalitis, Superior Hemorrhagic; Wernicke's Superior Hemorrhagic Polioencephalitis; Wernicke's Syndrome
SNOMED CT: Wernicke disease (21007002); Wernicke's disease (21007002); Wernicke's encephalopathy (21007002)
 
Monarch Initiative: MONDO:0007020
Orphanet: ORPHA97354

Definition

An acute neurological disorder characterized by the triad of ophthalmoplegia, ataxia, and disturbances of mental activity or consciousness. Eye movement abnormalities include nystagmus, external rectus palsies, and reduced conjugate gaze. thiamine deficiency and chronic alcoholism are associated conditions. Pathologic features include periventricular petechial hemorrhages and neuropil breakdown in the diencephalon and brainstem. Chronic thiamine deficiency may lead to korsakoff syndrome. (Adams et al., Principles of Neurology, 6th ed, pp1139-42; Davis & Robertson, Textbook of Neuropathology, 2nd ed, pp452-3) [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVWernicke encephalopathy

Professional guidelines

PubMed

CLINICAL PRACTICE GUIDELINES FOR THE PERIOPERATIVE NUTRITION, METABOLIC, AND NONSURGICAL SUPPORT OF PATIENTS UNDERGOING BARIATRIC PROCEDURES - 2019 UPDATE: COSPONSORED BY AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS/AMERICAN COLLEGE OF ENDOCRINOLOGY, THE OBESITY SOCIETY, AMERICAN SOCIETY FOR METABOLIC & BARIATRIC SURGERY, OBESITY MEDICINE ASSOCIATION, AND AMERICAN SOCIETY OF ANESTHESIOLOGISTS - EXECUTIVE SUMMARY.
Mechanick JI, Apovian C, Brethauer S, Garvey WT, Joffe AM, Kim J, Kushner RF, Lindquist R, Pessah-Pollack R, Seger J, Urman RD, Adams S, Cleek JB, Correa R, Figaro MK, Flanders K, Grams J, Hurley DL, Kothari S, Seger MV, Still CD
Endocr Pract 2019 Dec;25(12):1346-1359. Epub 2019 Nov 4 doi: 10.4158/GL-2019-0406. PMID: 31682518
Thiamine in the treatment of Wernicke encephalopathy in patients with alcohol use disorders.
Latt N, Dore G
Intern Med J 2014 Sep;44(9):911-5. doi: 10.1111/imj.12522. PMID: 25201422
EFNS guidelines for diagnosis, therapy and prevention of Wernicke encephalopathy.
Galvin R, Bråthen G, Ivashynka A, Hillbom M, Tanasescu R, Leone MA; EFNS
Eur J Neurol 2010 Dec;17(12):1408-18. doi: 10.1111/j.1468-1331.2010.03153.x. PMID: 20642790

Recent clinical studies

Etiology

The importance of thiamine (vitamin B1) in humans.
Mrowicka M, Mrowicki J, Dragan G, Majsterek I
Biosci Rep 2023 Oct 31;43(10) doi: 10.1042/BSR20230374. PMID: 37389565Free PMC Article
CLINICAL PRACTICE GUIDELINES FOR THE PERIOPERATIVE NUTRITION, METABOLIC, AND NONSURGICAL SUPPORT OF PATIENTS UNDERGOING BARIATRIC PROCEDURES - 2019 UPDATE: COSPONSORED BY AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS/AMERICAN COLLEGE OF ENDOCRINOLOGY, THE OBESITY SOCIETY, AMERICAN SOCIETY FOR METABOLIC & BARIATRIC SURGERY, OBESITY MEDICINE ASSOCIATION, AND AMERICAN SOCIETY OF ANESTHESIOLOGISTS - EXECUTIVE SUMMARY.
Mechanick JI, Apovian C, Brethauer S, Garvey WT, Joffe AM, Kim J, Kushner RF, Lindquist R, Pessah-Pollack R, Seger J, Urman RD, Adams S, Cleek JB, Correa R, Figaro MK, Flanders K, Grams J, Hurley DL, Kothari S, Seger MV, Still CD
Endocr Pract 2019 Dec;25(12):1346-1359. Epub 2019 Nov 4 doi: 10.4158/GL-2019-0406. PMID: 31682518
Prion Disease.
Baldwin KJ, Correll CM
Semin Neurol 2019 Aug;39(4):428-439. Epub 2019 Sep 18 doi: 10.1055/s-0039-1687841. PMID: 31533183
Wernicke Encephalopathy-Clinical Pearls.
Sinha S, Kataria A, Kolla BP, Thusius N, Loukianova LL
Mayo Clin Proc 2019 Jun;94(6):1065-1072. doi: 10.1016/j.mayocp.2019.02.018. PMID: 31171116
Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management.
Sechi G, Serra A
Lancet Neurol 2007 May;6(5):442-55. doi: 10.1016/S1474-4422(07)70104-7. PMID: 17434099

Diagnosis

Prion Disease.
Baldwin KJ, Correll CM
Semin Neurol 2019 Aug;39(4):428-439. Epub 2019 Sep 18 doi: 10.1055/s-0039-1687841. PMID: 31533183
Wernicke Encephalopathy-Clinical Pearls.
Sinha S, Kataria A, Kolla BP, Thusius N, Loukianova LL
Mayo Clin Proc 2019 Jun;94(6):1065-1072. doi: 10.1016/j.mayocp.2019.02.018. PMID: 31171116
Review of thiamine deficiency disorders: Wernicke encephalopathy and Korsakoff psychosis.
Chandrakumar A, Bhardwaj A, 't Jong GW
J Basic Clin Physiol Pharmacol 2018 Oct 2;30(2):153-162. doi: 10.1515/jbcpp-2018-0075. PMID: 30281514
EFNS guidelines for diagnosis, therapy and prevention of Wernicke encephalopathy.
Galvin R, Bråthen G, Ivashynka A, Hillbom M, Tanasescu R, Leone MA; EFNS
Eur J Neurol 2010 Dec;17(12):1408-18. doi: 10.1111/j.1468-1331.2010.03153.x. PMID: 20642790
Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management.
Sechi G, Serra A
Lancet Neurol 2007 May;6(5):442-55. doi: 10.1016/S1474-4422(07)70104-7. PMID: 17434099

Therapy

CLINICAL PRACTICE GUIDELINES FOR THE PERIOPERATIVE NUTRITION, METABOLIC, AND NONSURGICAL SUPPORT OF PATIENTS UNDERGOING BARIATRIC PROCEDURES - 2019 UPDATE: COSPONSORED BY AMERICAN ASSOCIATION OF CLINICAL ENDOCRINOLOGISTS/AMERICAN COLLEGE OF ENDOCRINOLOGY, THE OBESITY SOCIETY, AMERICAN SOCIETY FOR METABOLIC & BARIATRIC SURGERY, OBESITY MEDICINE ASSOCIATION, AND AMERICAN SOCIETY OF ANESTHESIOLOGISTS - EXECUTIVE SUMMARY.
Mechanick JI, Apovian C, Brethauer S, Garvey WT, Joffe AM, Kim J, Kushner RF, Lindquist R, Pessah-Pollack R, Seger J, Urman RD, Adams S, Cleek JB, Correa R, Figaro MK, Flanders K, Grams J, Hurley DL, Kothari S, Seger MV, Still CD
Endocr Pract 2019 Dec;25(12):1346-1359. Epub 2019 Nov 4 doi: 10.4158/GL-2019-0406. PMID: 31682518
Ataxia.
Kuo SH
Continuum (Minneap Minn) 2019 Aug;25(4):1036-1054. doi: 10.1212/CON.0000000000000753. PMID: 31356292Free PMC Article
Wernicke Encephalopathy-Clinical Pearls.
Sinha S, Kataria A, Kolla BP, Thusius N, Loukianova LL
Mayo Clin Proc 2019 Jun;94(6):1065-1072. doi: 10.1016/j.mayocp.2019.02.018. PMID: 31171116
Thiamine in the treatment of Wernicke encephalopathy in patients with alcohol use disorders.
Latt N, Dore G
Intern Med J 2014 Sep;44(9):911-5. doi: 10.1111/imj.12522. PMID: 25201422
EFNS guidelines for diagnosis, therapy and prevention of Wernicke encephalopathy.
Galvin R, Bråthen G, Ivashynka A, Hillbom M, Tanasescu R, Leone MA; EFNS
Eur J Neurol 2010 Dec;17(12):1408-18. doi: 10.1111/j.1468-1331.2010.03153.x. PMID: 20642790

Prognosis

Ataxia.
Kuo SH
Continuum (Minneap Minn) 2019 Aug;25(4):1036-1054. doi: 10.1212/CON.0000000000000753. PMID: 31356292Free PMC Article
Wernicke Encephalopathy.
Jenkins PF
Am Orthopt J 2015;65:104-8. doi: 10.3368/aoj.65.1.104. PMID: 26564935
Alcoholic metabolic emergencies.
Allison MG, McCurdy MT
Emerg Med Clin North Am 2014 May;32(2):293-301. Epub 2014 Feb 19 doi: 10.1016/j.emc.2013.12.002. PMID: 24766933
Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management.
Sechi G, Serra A
Lancet Neurol 2007 May;6(5):442-55. doi: 10.1016/S1474-4422(07)70104-7. PMID: 17434099
Wernicke-Korsakoff syndrome.
Zubaran C, Fernandes JG, Rodnight R
Postgrad Med J 1997 Jan;73(855):27-31. doi: 10.1136/pgmj.73.855.27. PMID: 9039406Free PMC Article

Clinical prediction guides

What is the optimum thiamine dose to treat or prevent Wernicke's encephalopathy or Wernicke-Korsakoff syndrome? Results of a randomized controlled trial.
Dingwall KM, Delima JF, Binks P, Batey R, Bowden SC
Alcohol Clin Exp Res 2022 Jun;46(6):1133-1147. Epub 2022 May 10 doi: 10.1111/acer.14843. PMID: 35428992Free PMC Article
Wernicke's encephalopathy in women with hyperemesis gravidarum - Case series and literature review.
Rane MA, Boorugu HK, Ravishankar U, Tarakeswari S, Vadlamani H, Anand H
Trop Doct 2022 Jan;52(1):98-100. Epub 2021 Jun 24 doi: 10.1177/00494755211014396. PMID: 34167385
Wernicke's encephalopathy after cardiac surgery.
Nishimura Y
Asian Cardiovasc Thorac Ann 2018 May;26(4):302-304. Epub 2018 Apr 18 doi: 10.1177/0218492318772693. PMID: 29667898
Safety and Efficacy of Fedratinib in Patients With Primary or Secondary Myelofibrosis: A Randomized Clinical Trial.
Pardanani A, Harrison C, Cortes JE, Cervantes F, Mesa RA, Milligan D, Masszi T, Mishchenko E, Jourdan E, Vannucchi AM, Drummond MW, Jurgutis M, Kuliczkowski K, Gheorghita E, Passamonti F, Neumann F, Patki A, Gao G, Tefferi A
JAMA Oncol 2015 Aug;1(5):643-51. doi: 10.1001/jamaoncol.2015.1590. PMID: 26181658
Wernicke encephalopathy and Creutzfeldt-Jakob disease.
Bertrand A, Brandel JP, Grignon Y, Sazdovitch V, Seilhean D, Faucheux B, Privat N, Brault JL, Vital A, Uro-Coste E, Pluot M, Chapon F, Maurage CA, Letournel F, Vespignani H, Place G, Degos CF, Peoc'h K, Haïk S, Hauw JJ
J Neurol 2009 Jun;256(6):904-9. Epub 2009 Mar 1 doi: 10.1007/s00415-009-5038-1. PMID: 19252796

Recent systematic reviews

Diagnosis and treatment of Wernicke's encephalopathy: A systematic literature review.
Cantu-Weinstein A, Branning R, Alamir M, Weleff J, Do M, Nero N, Anand A
Gen Hosp Psychiatry 2024 Mar-Apr;87:48-59. Epub 2024 Feb 1 doi: 10.1016/j.genhosppsych.2024.01.005. PMID: 38306946
Wernicke's Encephalopathy in Acute and Chronic Kidney Disease: A Systematic Review.
Oudman E, Wijnia JW, Severs D, Oey MJ, van Dam M, van Dorp M, Postma A
J Ren Nutr 2024 Mar;34(2):105-114. Epub 2023 Oct 13 doi: 10.1053/j.jrn.2023.10.003. PMID: 37838073
Wernicke Encephalopathy in schizophrenia: a systematic review.
Oudman E, Wijnia JW, Oey MJ, van Dam MJ, Postma A
Int J Psychiatry Clin Pract 2021 Sep;25(3):233-237. Epub 2020 Sep 21 doi: 10.1080/13651501.2020.1819333. PMID: 32957818
Preventing Wernicke's encephalopathy in anorexia nervosa: A systematic review.
Oudman E, Wijnia JW, Oey MJ, van Dam MJ, Postma A
Psychiatry Clin Neurosci 2018 Oct;72(10):774-779. Epub 2018 Jul 31 doi: 10.1111/pcn.12735. PMID: 29984541
Wernicke Encephalopathy: a Future Problem Even After Sleeve Gastrectomy? A Systematic Literature Review.
Kröll D, Laimer M, Borbély YM, Laederach K, Candinas D, Nett PC
Obes Surg 2016 Jan;26(1):205-12. doi: 10.1007/s11695-015-1927-9. PMID: 26476834

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