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Updated: Mar 1 2019

Wernicke-Korsakoff Syndrome

  • Snapshot
    • A 44-year-old male presents with confusion and gait instability. Medical history is significant for alcohol use disorder, requiring multiple hospitalizations for alcohol intoxication. He drinks one to two pints of vodka daily. On exam, he appears inattentive, has bilateral horizontal nystagmus, and his gait is slow and wide-based.
  • Introduction
    • Wernicke-Korsakoff (WK) syndrome
      • secondary to thiamine (B1) deficiency
      • bilateral necrosis of the mammillary bodies
        • dorsal medial nucleus of the thalamus is also involved
      • triad: confusion (encephalopathy), ataxia, ophthalmoplegia
        • complete triad not always present
          • occurs in ~30% of cases
        • cannot rule out WK on absence of triad
  • Pathophsiology
    • Certain metabolic enzymes use thiamine as a cofactor
      • e.g., pyruvate dehydrogenase, transketolase, alpha-ketoglutarate dehydrogenase
      • increased thiamine demand when metabolic demand is increased or increased glucose intake
        • must give thiamine before glucose administration
      • seen in chronic alcoholism, and other conditions of poor nutritions (e.g., malabsorption)
  • Epidemiology
    • Alcoholics are mostly affected
  • Presentation
    • Symptoms
      • Wernicke's encephalopathy
        • confusion
        • opthalmoplegia
        • ataxia
      • Korsakoff's psychosis
        • memory loss
        • confabulation
        • hallucination
        • personality change
  • Evaluation
    • Clinical diagnosis
      • primarily
    • Thiamine level
      • low
      • non-specific
    • Erythrocyte thiamine transketolase
      • can establish thiamine deficiency
  • Differential
    • Alcohol intoxication
    • Early Alzheimer's disease
  • Treatment
    • Intravenous thiamine
      • administer immediately
      • leads to improvement of reversible symptoms
  • Complications
    • lead to Korsakoff syndrome if Wernicke's encephalopathy is not treated
  • Prevention
    • adeqaute thiamine intake
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