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

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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Related Studies

Ontology: Wernicke Encephalopathy (C0043121)

Definition (MSHCZE) Neurologická porucha vznikající při těžkém nedostatku vitaminu B1, např. u alkoholiků nebo po resekci žaludku. Projevuje se poruchami vědomí (deliriem), obrnou okohybných svalů (s diplopií), poruchami chůze, ataxií, demencí aj. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (PSY) Use APHASIA for Wernicke's aphasia.
Definition (MSH) An acute neurological disorder characterized by the triad of ophthalmoplegia, ataxia, and disturbances of mental activity or consciousness. Eye movement abnormalities include nystagmus, external rectus palsies, and reduced conjugate gaze. THIAMINE DEFICIENCY and chronic ALCOHOLISM are associated conditions. Pathologic features include periventricular petechial hemorrhages and neuropil breakdown in the diencephalon and brainstem. Chronic thiamine deficiency may lead to KORSAKOFF SYNDROME. (Adams et al., Principles of Neurology, 6th ed, pp1139-42; Davis & Robertson, Textbook of Neuropathology, 2nd ed, pp452-3)
Concepts Disease or Syndrome (T047)
MSH D014899
ICD10 E51.2
SnomedCT 21007002, 190626005, 190627001
English Wernicke's Encephalopathy, Encephalopathy, Wernicke's, Wernicke's Syndrome, Wernicke encephalopathy, WERNICKE ENCEPH, ENCEPH GAYET WERNICKE, ENCEPH WERNICKES, WERNICKES SUPERIOR HEMORRHAGIC POLIOENCEPH, WERNICKES ENCEPH, GAYET WERNICKE ENCEPH, WERNICKES DIS, WERNICKE POLIOENCEPH SUPERIOR HEMORRHAGIC, ENCEPH WERNICKE, WERNICKE SUPERIOR HEMORRHAGIC POLIOENCEPH, WERNICKE DIS, Wernicke's disease (diagnosis), Wernicke's syndrome, Beriberi, Cerebral, Cerebral Beriberi, Wernicke Disease, Wernicke's Disease, Encephalopathy, Gayet Wernicke, Encephalopathy, Gayet-Wernicke, Gayet Wernicke Encephalopathy, Gayet-Wernicke Encephalopathy, Encephalopathies, Wernicke, Encephalopathy, Wernicke, Encephalopathy, Wernickes, Wernicke Encephalopathies, Wernicke Polioencephalitis, Superior Hemorrhagic, Wernicke Superior Hemorrhagic Polioencephalitis, Wernicke's Polioencephalitis, Superior Hemorrhagic, Wernicke's Superior Hemorrhagic Polioencephalitis, Wernicke Syndrome, Wernicke disease, Wernicke Encephalopathy [Disease/Finding], Wernicke Encephalopathy, wernicke syndrome, wernicke encephalopathy, disease wernickes, encephalopathy wernicke's, wernickes syndrome, wernickes, encephalopathy wernickes, wernicke's disease, wernicke, wernicke's encephalopathy, wernicke's syndrome, Wernicke encephalopathy (disorder), Wernicke's encephalopathy, Wernicke's disease, Wernicke's disease (disorder), Wernicke; disease or syndrome (manifestation), Wernickes Syndrome, wernickes encephalopathy, Encephalopathy;wernickes
Dutch Wernicke-syndroom, Wernicke; ziekte of syndroom, Wernicke-encefalopathie, Beriberi, cerebrale, Encefalopathie van Wernicke, Encefalopathie, Wernicke, Pseudo-encefalitis, Wernicke, encefalopathie van
German Wernicke-Syndrom, Wernicke Enzephalopathie, Wernicke-Enzephalopathie, Beriberi, zerebrale, Enzephalopathie, Wernicke-, Pseudoencephalitis haemorrhagica superior
Portuguese Síndrome de Wernicke, Encefalopatia de Wernicke, Beribéri Cerebral
Spanish Síndrome de Wernicke, encefalopatía de Wernicke, enfermedad de Wernicke (trastorno), enfermedad de Wernicke, Beriberi Cerebral, Encefalopatía de Wernicke
Japanese ウェルニッケ症候群, ウェルニッケショウコウグン, ウェルニッケノウショウ, Wernicke脳症, Wernicke脳障害, ウェルニッケ脳症, ウエルニッケ脳症, ヴェルニッケエンセファロパシー, ヴェルニッケ脳障害, 脚気-脳, 脳症-ウェルニッケ, 脳脚気
Swedish Wernickes encefalopati
Czech beri-beri mozková, Wernickeho syndrom, Wernickeova encefalopatie, Wernickeho encefalopatie
Finnish Wernicken oireyhtymä
Italian Sindrome di Wernicke, Encefalopatia di Gayet-Wernicke, Beriberi cerebrale, Malattia di Wernicke, Polioencefalite emorragica superiore di Wernicke., Encefalopatia di Wernicke
Russian POLIENTSEFALIT GEMORRAGICHESKII, VERNIKE ENTSEFALOPATIIA, ВЕРНИКЕ ЭНЦЕФАЛОПАТИЯ, ПОЛИЭНЦЕФАЛИТ ГЕМОРРАГИЧЕСКИЙ
Korean 베르니케 뇌병증
Polish Encefalopatia Wernickiego
Hungarian Wernicke-encephalopathia, Wernicke-syndroma
Norwegian Wernickes syndrom, Encefalopati, Gayet-Wernickes, Gayet-Wernickes encefalopati, Wernickes encefalopati, Encefalopati, Wernickes, Wernickes sykdom, Beriberi, cerebral, Wernickes polioencefalitt, øvre hemoragisk, Wernickes øvre hemoragisk polioencefalitt, Cerebral beriberi
French Encéphalopathie de Wernicke, Encéphalopathie de Gayet-Wernicke, Maladie de Gayet-Wernicke, Syndrome de Wernicke