Wernicke's encephalopathy is an acute neurological disorder caused by thiamine (vitamin B1) deficiency. It is characterized by the classic triad of ocular abnormalities, cerebellar dysfunction, and confusion. It most commonly occurs in alcoholics and has a prevalence of 1.3%. Diagnosis involves evaluating clinical presentation and administering thiamine intravenously, with monitoring for clinical improvement. Prompt treatment with high dose intravenous thiamine is required to prevent the condition from progressing to Korsakoff's syndrome.
3. Introduction
• Described at 19th century
• Acute / subacute neuropsychiatric syndrome
• Thiamine (B1) deficiency
• Classic triad: ocular signs (nystagmus / ophtalmoplegia), cerebellar dysfunction and confusion.
• Prevalence: 1,3 % (0,4-2,8%)
• Alcohol-related
• Rare, catastrophic, clinically complex, often delayed in diagnosis
• If become persist and irreversible Korsakoff syndrome
Sechi, G., & Serra, A. (2007). Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management. The Lancet Neurology, 6(5), 442-455.
Galvin, R., Bråthen, G., Ivashynka, A., Hillbom, M., Tanasescu, R., & Leone, M. A. (2010). EFNS guidelines for diagnosis, therapy and prevention of Wernicke encephalopathy. European Journal of Neurology, 17(12), 1408-1418.
4. Epidemiology
Sechi, G., & Serra, A. (2007). Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management. The Lancet Neurology, 6(5), 442-455.
5. Etiology
Sechi, G., & Serra, A. (2007). Wernicke's encephalopathy:
new clinical settings and recent advances in diagnosis and
management. The Lancet Neurology, 6(5), 442-455.
6. Pathophysiology
Sechi, G., & Serra, A. (2007). Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management. The Lancet Neurology, 6(5), 442-455.
7. Clinical presentation
Sechi, G., & Serra, A. (2007). Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management. The Lancet Neurology, 6(5), 442-455.
8. Diagnosis
EEG: Diffuse slow waves in the theta range
Sechi, G., & Serra, A. (2007). Wernicke's encephalopathy: new clinical settings and recent
advances in diagnosis and management. The Lancet Neurology, 6(5), 442-455.
Whenever WE is suspected a blood sample for measurement of
total thiamine should be drawn immediately before
administration of thiamine and sent for HPLC analysis
Galvin, R., Bråthen, G., Ivashynka, A., Hillbom, M., Tanasescu, R., & Leone, M. A.
(2010). EFNS guidelines for diagnosis, therapy and prevention of Wernicke
encephalopathy. European Journal of Neurology, 17(12), 1408-1418.
9. Differential Diagnosis
• Paramedian thalamic infarction (top-of-the-basilar syndrome),
• Ventriculoencephalitis,
• Miller-Fisher syndrome,
• Primary cerebral lymphoma,
• Behçet’s disease,
• Multiple sclerosis,
• Leigh’s disease,
• Variant Creutzfeldt-Jakob disease,
• Paraneoplastic encephalitis,
• Severe hypophosphataemia,
• Acute intoxication from methyl bromide,
• Chronic intoxication from bromvalerylurea
Weidauer S, Nichtweiss M, Lanfermann H, Zanella FE. Wernicke’s encephalopathy: MR fi ndings and clinical presentation. Eur Radiol 2003; 13: 1001–09.
Brechtelsbauer DL, Urbach H, Sommer T, Blumcke I, Woitas R, Solymosi L. Cytomegalovirus encephalitis and primary cerebral lymphoma mimicking Wernicke’s encephalopathy. Neuroradiology 1997; 39: 19–22.
Zurkirchen MA, Misteli M, Conen D. Reversible neurological complications in chronic alcohol abuse with hypophosphatemia. Scweiz Med Wochenschr 1994; 124: 1807–12.
Squier MV, Thompson J, Rajgopalan B. Case report: neuropathology of methyl bromate intoxication. Neuropathol Appl Neurobiol 1992; 6: 579–84.
10. Management
• Medical emergency
• Thiamine: 3 x 200 mg (im or iv)
• Thiamine should be given before any carbohydrate, and a normal diet should be instituted
immediately after thiamine
• Treatment should be continued until there is no further improvement in signs and symptoms
Galvin, R., Bråthen, G., Ivashynka, A., Hillbom, M., Tanasescu, R., & Leone, M. A. (2010). EFNS guidelines for diagnosis, therapy and prevention of Wernicke encephalopathy. European Journal of Neurology, 17(12), 1408-1418.
11. Prevention
• Supplementation of thiamine to food may prevent the development of WE
• 200 mg thiamine before carbohydrates are started in all subjects with a risk condition managed
at the Emergency Room
• After bariatric surgery & hunger strikers
Galvin, R., Bråthen, G., Ivashynka, A., Hillbom, M., Tanasescu, R., & Leone, M. A. (2010). EFNS guidelines for diagnosis, therapy and prevention of Wernicke encephalopathy. European Journal of Neurology, 17(12), 1408-1418.
12. Summary
• Any condition of unbalanced nutrition that lasts for 2–3 weeks can lead to thiamine depletion
and Wernicke’s encephalopathy with damage in selective diencephalic and brainstem areas
• Chronic alcoholism & gastrointestinal surgery
• Difficult to diagnose
• Diagnosis: dramatic response after parenteral thiamine & MRI