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Wernicke's syndrome (Wernicke's encephalopathy)

A condition frequently observed in alcoholics often coexistent with Korsakoff's syndrome. Etiology: 1) thiamine deficiency a) alcoholism b) vitamin-free fluid intake (parenteral nutrition) c) hyperemesis gravidarum d) bariatric surgery [2] e) prolonged fasting f) malnutrition &/or malabsorption syndromes - cachexia - diarrhea - sprue g) HIV1 infection 2) infection is the most common precipitating event in patients with thiamine deficiency 3) superior hemorrhagic polio-encephalitis Pathology: 1) develops after 4-6 weeks of thiamine deficiency 2) carbohydrate metabolism exhausts thiamine stores in: a) thalamus (dorsal, medial) b) brainstem c) mammillary bodies 3) transketolase insufficiency results from thiamine deficiency 4) gliosis in mammillary bodies & periaqueductal gray Clinical manifestations: 1) classic triad in 16% [3] a) ophthalmoplegia 1] disturbances in ocular motility - ocular bobbing on primary gaze - no ocular bobbing on horizontal gaze 2] pupillary alterations 3] nystagmus b) ataxia c) confusion (encephalopathy) 1] global 2] listlessness 3] inattentiveness/lack of concentration 2) amnesia, dementia 3) confabulation 4) autonomic insufficiency - hypertension (rare) 5) tremors 6) anxious insomnia, fear of dark 7) evidence of malnutrition: cachexia, diarrhea (see etiology) Radiology: - MRI neuroimaging with fluid attenutated inversion recovery - hyperintensity of the mammillary bodies & periaqueductal gray matter Complications: - administration of dextrose without first treating thiamine deficiency can precipitate further neurologic injury including infarction of mammillary bodies resulting in severe memory impairment (see Korsakoff's syndrome) - progression to Korsakoff's syndrome Differential diagnosis: - synthetic cannabinoid toxicity Management: 1) thiamine a) 100 mg IV/IM PRIOR to carbohydrate loading b) then 100 mg PO QD c) treat until opthalmoplegia resolves 2) rehydration 3) correct electrolyte imbalances 4) multivitamins including folate 5) prognosis: a) may clear in days to weeks b) may progress to Korsakoff's syndrome

Related

Korsakoff's syndrome (amnesic psychosis) thiamine (vitamin B1, Betalin) Wernicke's aphasia; fluent aphasia; garbled speech

Specific

Wernicke-Korsakoff syndrome

General

encephalopathy syndrome

Database Correlations

OMIM 277730

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 15, 16. American College of Physicians, Philadelphia 2009, 2012
  2. Aasheim ET. Wernicke encephalopathy after bariatric surgery: a systematic review. Ann Surg. 2008 Nov;248(5):714-20 PMID: 18948797
  3. Geriatric Review Syllabus, 8th edition (GRS8) Durso SC and Sullivan GN (eds) American Geriatrics Society, 2013 - Geriatric Review Syllabus, 11th edition (GRS11) Harper GM, Lyons WL, Potter JF (eds) American Geriatrics Society, 2022
  4. Donnino MW, Vega J, Miller J, Walsh M. Myths and misconceptions of Wernicke's encephalopathy: what every emergency physician should know. Ann Emerg Med. 2007 Dec;50(6):715-21. Epub 2007 Aug 3. Review. PMID: 17681641
  5. Galvin R, Brathen G, Ivashynka A et al EFNS guidelines for diagnosis, therapy and prevention of Wernicke encephalopathy. Eur J Neurol. 2010 Dec;17(12):1408-18. PMID: 20642790
  6. NEJM Knowledge+ Psychiatry - Kaineg B, Hudgins PA. Images in clinical medicine. Wernicke's encephalopathy. N Engl J Med. 2005 May 12;352(19):e18. PMID: 15888690 Free article. https://www.nejm.org/doi/pdf/10.1056/NEJMicm040862 - Sechi G, Serra A. Wernicke's encephalopathy: new clinical settings and recent advances in diagnosis and management. Lancet Neurol. 2007 May;6(5):442-55. PMID: 17434099 Review.