Citation, DOI and article data
Wernicke encephalopathy, also referred as Wernicke-Korsakoff syndrome, is a form of thiamine (vitamin B1) deficiency, and is typically seen in alcoholics.
On imaging, it is commonly seen on MRI as areas of symmetrical increased T2/FLAIR signal involving the mammillary bodies, dorsomedial thalami, tectal plate, periaqueductal area and/or around the third ventricle.
It was originally described as characterized by the triad of:
- acute confusion
- ophthalmoplegia (most commonly horizontal nystagmus and conjugate gaze palsies)
Wernicke encephalopathy can evolve into the chronic form of thiamine deficiency known as Korsakoff psychosis, characterized by:
- memory loss (global amnesia)
The two terms are often concatenated to form Wernicke-Korsakoff syndrome.
Thiamine deficiency results from malnutrition or malabsorption, which can occur for a number of reasons 6,7:
- alcohol abuse (up to 90% in industrialised countries 5)
- prolonged total parenteral nutrition without supplementation
- postbariatric surgery
- hyperemesis gravidarum
- gastrointestinal malignancy
- chronic dialysis
In acute stages, hemorrhage, necrosis, and edema may be present. In chronic stages, atrophic changes may be present especially involving the mamillary bodies.
- T2/FLAIR: symmetrically increased signal intensity in the
- T1 C+ (Gd): contrast enhancement can also be seen in the same regions, most commonly of the mammillary bodies 4
- DWI/ADC: restricted diffusion can also be seen in the same regions 4
- MR spectroscopy: may show decreased or normal NAA with the notable presence of lactate 4
Treatment and prognosis
Treatment of acute Wernicke-Korsakoff syndrome is with intravenous thiamine hydrochloride, along with other vitamins/minerals, and treatment of the underlying cause (e.g. alcohol cessation). Untreated, there is high mortality of up to 20% 9,10.
History and etymology
It is named after:
- Carl Wernicke: German neurologist and psychiatrist (1848-1905) 2
- Sergei Korsakoff: Russian neuropsychiatrist (1854-1900) 2
General imaging differential considerations include:
- 1. Zuccoli G, Gallucci M, Capellades J et-al. Wernicke encephalopathy: MR findings at clinical presentation in twenty-six alcoholic and nonalcoholic patients. AJNR Am J Neuroradiol. 2007;28 (7): 1328-31. doi:10.3174/ajnr.A0544 - Pubmed citation
- 2. Richard Langton Gregory. The Oxford Companion to the Mind. (2018) ISBN: 9780198662242
- 3. Zuccoli G, Pipitone N. Neuroimaging findings in acute Wernicke's encephalopathy: review of the literature. AJR Am J Roentgenol. 2009;192 (2): 501-8. doi:10.2214/AJR.07.3959 - Pubmed citation
- 4. Degnan AJ, Levy LM. Neuroimaging of rapidly progressive dementias, part 2: prion, inflammatory, neoplastic, and other etiologies. AJNR Am J Neuroradiol. 2014;35 (3): 424-31. doi:10.3174/ajnr.A3455 - Pubmed citation
- 6. Thomson AD, Marshall EJ. The natural history and pathophysiology of Wernicke's Encephalopathy and Korsakoff's Psychosis. Alcohol Alcohol. 2006;41 (2): 151-8. doi:10.1093/alcalc/agh249 - Pubmed citation
- 7. Hegde AN, Mohan S, Lath N et-al. Differential diagnosis for bilateral abnormalities of the basal ganglia and thalamus. Radiographics. 2011;31 (1): 5-30. Radiographics (full text) - doi:10.1148/rg.311105041 - Pubmed citation
- 8. Nelson JS, Mena H, Parisi JE et-al. Principles and Practice of Neuropathology. Oxford University Press, USA. ISBN:B005LQBYFI. Read it at Google Books - Find it at Amazon
- 8. Loh Y, Watson WD, Verma A, Krapiva P. Restricted diffusion of the splenium in acute Wernicke's encephalopathy. (2005) Journal of neuroimaging : official journal of the American Society of Neuroimaging. 15 (4): 373-5. doi:10.1177/1051228405279037 - Pubmed
- 9. Thomson AD, Guerrini I, Marshall EJ. Wernicke’s Encephalopathy: Role of Thiamine. PRACTICAL GASTROENTEROLOGY. JUNE 2009. Available from: http://www.medicine.virginia.edu/clinical/departments/medicine/divisions/digestive-health/nutrition-support-team/nutrition-articles/ThomsonArticle.pdf
- 10. Sabatini JS, Schutz-Pereira GL, Feltrin F, Teive HAG, Camargo CHF. Wernicke's encephalopathy with chorea: Neuroimaging findings. Dement Neuropsychol. 2016;10(4):370‐372. doi:10.1590/s1980-5764-2016dn1004020 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5619281/