Wernicke–Korsakoff Syndrome

Abstract

Wernicke–Korsakoff syndrome refers to a life‐threatening encephalopathy due to inadequate supply of thiamine (vitamin B1) to the brain. It is a combination of Wernicke encephalopathy (WE), an acute neuropsychiatric disorder fully responsive to prompt and adequate thiamine replacement, and Korsakoff syndrome (KS), the chronic, irreversible form of WE that does not remit with thiamine treatment. Thiamine deficiency can occur in any condition of unbalanced nutrition that lasts for 2–3 weeks. These include poor nutrition, chronic alcohol misuse, loss of thiamine owing to recurrent vomiting or malabsorption, increased thiamine requirements owing to chronic diseases, prolonged and excessive carbohydrate intake and gastrointestinal surgical procedures, in particular, patients after bariatric surgery. The diagnosis is clinical and is mainly supported by the dramatic improvement of neurological signs to parenteral thiamine. Neuroimaging studies are useful to confirm the diagnosis of both WE and KS. Patients with suspected thiamine deficiency should be treated immediately with parenteral thiamine at the doses deemed to be effective in the treatment of WE and prevention of KS.

Key Concepts:

  • Thiamine deficiency can occur in a myriad of clinical settings that include malnutrition, malabsorption and increased metabolism and in patients following bariatric surgery.

  • A thiamine deficiency that lasts for 2 to 3 weeks leads to Wernicke encephalopathy, an acute, life‐threatening disorder that is a medical emergency.

  • Wernicke encephalopathy is fully responsive to prompt and adequate thiamine replacement.

  • Untreated or inadequately treated Wernicke encephalopathy may lead to Korsakoff syndrome, a chronic amnesic encephalopathy that does not remit with thiamine treatment.

  • The goal of treatment of Wernicke encephalopathy is the immediate administration of high doses of parenteral thiamine deemed sufficient to prevent Korsakoff syndrome.

Keywords: thiamine deficiency; Wernicke encephalopathy; Korsakoff syndrome; Wernicke–Korsakoff syndrome; encephalopathy; vitamin B1

Figure 1.

Schematic representation of clinical manifestations related to severe, short‐term thiamine deficiency (acute deficiency) and to mild/moderate, prolonged thiamine deficiency (chronic deficiency). TPP=thiamine pyrophosphate.

Figure 2.

Brain MRI, coronal sections. (a, b) Coloured areas indicate some of the brain regions frequently involved in WE and KS: medial thalami, in red; mammillary bodies, in blue; anterior thalamus, in yellow and hippocampal regions, in green. Brain areas typically involved in WE are the medial thalami and mammillary bodies, whereas in KS, the mammillary bodies, thalamus anterior and hippocampal regions.

close

References

Antunez E, Estruch R, Cardenal C et al. (1998) Usefulness of CT and MR imaging in the diagnosis of acute Wernicke's encephalopathy. American Journal of Roentgenology 171: 1131–1137.

Bates CI and Heseker H (1994) Human bioavailability of vitamins. Nutrition Research Reviews 7: 93–127.

Beshlawi I, Zadjali SA, Bashir W et al. (2014) Thiamine responsive megaloblastic anemia: the puzzling phenotype. Pediatric Blood & Cancer 61: 528–531.

Bleggi‐Torres LF, de Medeiros BC, Werner B et al. (2000) Neuropathological findings after bone marrow transplantation: an autopsy study of 180 cases. Bone Marrow Transplantation 25: 301–307.

Boldorini R, Vago L, Lechi A, Tedeschi F and Trabattoni GR (1992) Wernicke's encephalopathy: occurrence and pathological aspects in a series of 400 AIDS patients. Acta Biomedica de l'Ateneo Parmense 63: 43–49.

Brown G (2014) Defects of thiamine transport and metabolism. Journal of Inherited Metabolic Disease. doi:10.1007/s10545‐014‐9712‐9.

Campbell ACP and Russel WR (1941) Wernicke's encephalopathy: the clinical features and their probable relationship to vitamin B deficiency. QJM 10: 41–64.

Day E, Bentham PW, Callagham R, Kuruvilla T and Georges S (2013) Thiamine for prevention and treatment of Wernicke‐Korsakoff syndrome in people who abuse alcohol. Cochrane Database of Systematic Reviews 7: 1–20.

De la Monte SM and Kril JJ (2014) Human alcohol‐related neuropathology. Acta Neuropathologica 127: 71–90.

Hanninen S, Darling P, Sole M, Barr A and Keith M (2006) The prevalence of thiamine deficiency in hospitalized patients with congestive heart failure. Journal of the American College of Cardiology 47: 354–361.

Hoorn RK, Flikweert JP and Westerink D (1975) Vitamin B‐1, B‐2 and B‐6 deficiencies in geriatrics patients, measures by coenzyme stimulation of enzyme activities. Clinica Chimica Acta 61: 151–162.

Isenberg‐Grzeda E, Kutner HE and Nicolson SE (2012) Wernicke‐Korsakoff syndrome: under‐recognized and under‐treated. Psychosomatics 53: 507–516.

Jung Y‐C, Chanraud S and Sullivan EV (2012) Neuroimaging of Wernicke's encephalopathy and Korsakoff's syndrome. Neuropsychology Review 22: 170–180.

Kono S, Miyajima H, Yoshida K et al. (2009) Mutations in a thiamine‐transporter gene and Wernicke's‐like encephalopathy. New England Journal of Medicine 360: 1792–1794.

Kopelman MD, Thomson AD, Guerrini I and Marshall EJ (2009) The Korsakoff syndrome: clinical aspects, psychology and treatment. Alcohol and Alcoholism 44: 148–154.

Kumar N (2010) Neurologic presentations of nutritional deficiencies. Neurologic Clinics 28: 107–170.

Le Berre A‐P, Pitel A‐L, Chanraud S et al. (2014) Chronic alcohol consumption and its effect on nodes of pontocerebellar and limbic circuitry: comparison of effects in France and United States. Human Brain Mapping. doi:10.1002/hbm.22500.

Lishman WA (1981) Cerebral disorder in alcoholism: syndromes of impairment. Brain 104: 1–20.

Malamud N and Skillicorn SA (1956) Relationship between the Wernicke and the Korsakoff syndrome: a clinicopathologic study of 70 cases. A.M.A. Archives of Neurology and Psychiatry 76: 585–596.

Manzo L, Locatelli C, Candura SM and Costa LG (1994) Nutrition and alcohol neurotoxicity. Neurotoxicology 15: 555–565.

Ramayya A and Jauhar P (1997) Increasing incidence of Korsakoff's psychosis in the East End of Glasgow. Alcohol and Alcoholism 32(suppl.): 281–285.

Said HM (2011) Intestinal adsorption of water‐soluble vitamins in health and disease. Biochemical Journal 437: 357–372.

Sechi G and Serra A (2007) Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management. Lancet Neurology 6: 442–455.

Shankar P, Boylan M and Sriram K (2010) Micronutrient deficiencies after bariatric surgery. Nutrition 26: 1031–1037.

Thomson AD (2000) Mechanisms of vitamin deficiency in chronic alcohol misusers and the development of Wernicke‐Korsakoff syndrome. Alcohol and Alcoholism 35(suppl. 1): 2–7.

Thomson AD, Cook CCH, Touquet R and Henry JA (2002) The Royal College of Physicians report on alcohol: guidelines for managing Wernicke's encephalopathy in the accident and emergency department. Alcohol and Alcoholism 37: 513–521.

Thomson AD, Guerrini I and Marshall EJ (2012) The evaluation and treatment of Korsakoff's syndrome. Out of sight, out of mind? Neuropsychology Review 22: 81–92.

Torvik A, Lindboe CF and Rodge S (1982) Brain lesions in alcoholics. A neuropathological study with clinical correlations. Journal of the Neurological Sciences 56: 233–248.

Victor M, Adams RD and Collins GH (1971) The Wernicke‐Korsakoff syndrome: a clinical and pathological study of 245 patients, 82 with post‐mortem examinations. Contemporary Neurology Series 7: 1–206.

Wernicke C (1881) Die acute haemorrhagische polioencephalitis superior. In: Wernicke C (ed.) Lehrbuch der Gehirnkrankheiten fϋr Aerzle und Studirende, pp. 229–242. Kassel: Bd H, Fisher Verlag.

Zuccoli G, Santa cruz D, Bertolini M et al. (2009) MR imaging findings in 56 patients with Wernicke's encephalopathy: nonalcoholics may differ from alcoholics. American Journal of Neuroradiology 30: 171–176.

Further Reading

Kim TE, Lee EJ, Young JB, Shin DJ and Kim JH (2014) Wernicke encephalopathy and ethanol-related syndromes. Seminars in Ultrasound CT and MRI 35: 85–96.

Latt N and Dore G (2014) Thiamine in the treatment of Wernicke encephalopathy in patients with alcohol use disorders. Internal Medicine Journal 44: 911–915.

Contact Editor close
Submit a note to the editor about this article by filling in the form below.

* Required Field

How to Cite close
Sechi, Gian Pietro, Fancellu, Laura, Doneddu, Pietro, and Sechi, Elia(Nov 2014) Wernicke–Korsakoff Syndrome. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1002/9780470015902.a0025810]