Wernicke's Encephalopathy

Aka: Wernicke's Encephalopathy, Wernicke Encephalopathy, Wernickes Encephalopathy, Acute Thiamine Deficiency

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II. History

  1. First described in 1881 by Dr. Carl Wernicke (polish neurologist) who recognized the classic triad

III. Cause

  1. Acute Thiamine Deficiency associated with Alcoholism
    1. Contrast with Chronic Thiamine deficiency (Beriberi)
  2. May also occur with malnourishment (similar causes of Beriberi)
    1. Hyperemesis Gravidarum
    2. Bariatric Surgery
    3. Chemotherapy
    4. Antiretroviral therapy
    5. Anorexia Nervosa
    6. Elderly with constrained diet
    7. AIDS

IV. Signs

  1. Classic Triad (full triad present in less than one third of cases)
    1. Mnemonic: WACO (Wernicke's Ataxia Confusion Ophthalmoplegia)
    2. Ataxia
      1. Wide based unsteady gait
      2. Other coordination testing may be normal (e.g. normal Finger-Nose-Finger)
    3. Mental status changes or Altered Level of Consciousness
      1. Confusion or Delirium
      2. Apathy
      3. Inattention
      4. Drowsiness
      5. Decreased speech
      6. Short Term Memory deficit
    4. Ophthalmoplegia
      1. Horizontal Nystagmus is most common (but Vertical Nystagmus may also be present)
      2. Lateral Rectus Palsy (internal Strabismus, Conjugate Gaze Palsy, variably present)
  2. Other findings
    1. See Beriberi
    2. Hypothermia
    3. Hyperhidrosis
    4. Neuropsychiatric symptoms (psychomotor slowing, Seizures, depression, Dysphagia)

V. Differential Diagnosis

  1. See Ataxia
  2. See Delirium
  3. See Altered Level of Consciousness
  4. See Ophthalmoplegia or Nystagmus

VI. Labs

  1. See Lab Markers of Malnutrition
  2. See Delirium

VII. Imaging

  1. CT Head
    1. Initial imaging to exclude acute cause (e.g. Intracranial Hemorrhage)
  2. MRI Brain
    1. Mammillary Body T2 contrast enhancement (2 brightly enhancing ovoid bodies at anterior Midbrain)
      1. Mammillary Body enhancement present in 58% of those with Wernicke's Encephalopathy
    2. Other regions with variable involvement
      1. Medial Thalamus
      2. Periventricular Third Ventricle
      3. Periaqueduct
      4. Tectal Plate
      5. Dorsal Medulla
    3. References
      1. Zuccoli (2007) Am J Neuroradiol 28(7): 1328-31 [PubMed]

VIII. Complications

  1. Associated Mortality
  2. Korsakoff's Disease (Wernicke-Korsakoff Syndrome or Korsakoff's Psychosis)
    1. Life-long neurologic Impairment
    2. Associated with learning difficulties, memory difficulties, confabulation (invented memories)

IX. Management

  1. Emergent Thiamine Dosing
    1. Start immediately without delay
    2. Glucose or dextrose given before Thiamine can precipitate encephalopathy
    3. Initial
      1. Thiamine 500 mg IV every 8 hours for 3 days (or more)
    4. Next
      1. Thiamine 50-100 mg orally, IV or IM daily
  2. Other management
    1. Magnesium

X. Resources

  1. Wernicke Encephalopathy (NIH Stat Pearls)
    1. https://www.ncbi.nlm.nih.gov/books/NBK470344/

XI. References

  1. Broder (2020) Crit Dec Emerg Med 34(9): 18-9
  2. Rendon et al. (2017) Crit Dec Emerg Med 31(6): 15-21

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