Wernicke-Korsakoff syndrome

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Background

  • Wernicke’s Encephalopathy (WE): ACUTE neuro/cardiovascular symptoms caused by thiamine deficiency
  • Korsakoff’s Psychosis (KP): CHRONIC neurologic symptoms caused by thiamine deficiency
  • Wernicke-Korsakoff Syndrome (WKS): presence of WE + KP simultaneously

Epidemiology

  • Only 20% identified before death, failure of diagnosis leads to 20% mortality and 75% permanent damage

Pathophysiology

  • Thiamine plays critical role in:
    • Energy production pathways (Kreb's cycle, pentose phosphate pathway, alpha-ketoglutarate dehydrogenase, pyruvate dehydrogenase)
    • Lipid metabolism (including myelin sheath formation)
      • Alterations in myelination leads to peripheral neuropathy
  • Brain lesions/atrophy usually occur in: mamillary bodies (nearly all cases), thalamus, periaqueductal gray matter, 3rd/4th ventricle, cerebellum, frontal lobe

Causes

  • Thiamine (vitamin B1) deficiency caused by
    • Insufficient intake (e.g. chronic alcoholism, starvation/anorexia, severe vomiting/diarrhea, unbalanced TPN)
    • Malabsorption (post-gastrectomy, IBD, pancreatitis
    • Increased metabolic requirements (malignancy, thyrotoxicosis)
    • Thiamine losses (hemodialysis)
    • Miscellaneous: AIDS, liver disease

Clinical Features

Wernicke’s Encephalopathy

  • Classic triad: encephalopathy, oculomotor dysfunction, gait ataxia
  • werNICke mnemonic:
    • Nystagmus/ophthalmoplegia
      • Ocular findings may also include bilateral 6th nerve palsy, conjugate gaze palsy, pupillary abnormality, retinal hemorrhage, ptosis.
    • Incoordination/ataxia
    • Confusion/memory impairment
  • Other symptoms:

Korsakoff’s Psychosis

  • Antero/retrograde amnesia
  • Confabulation, confusion, apathy

Wernicke-Korsakoff Syndrome

  • Findings of both Wernicke and Korsakoff

Differential Diagnosis

Ethanol related disease processes

Vitamin deficiencies

Evaluation

  • Clinical diagnosis
  • Wernicke's Encephalopathy: at least 2 of:[1]:
    • Nutritional deficiency
    • Ocular findings (ophthalmoplegia, nystagmus)
    • Ataxia
    • Mental status change

Management

If you suspect, then treat! Confirming diagnosis is difficult, treatment is low risk and effective, and morbidity/mortality is high if untreated

  • Thiamine 500mg IV over 30 min TID x 2 days, then 500mg IV/IM q day for 5 days, then 100mg PO q day until patient no longer at risk
    • Give magnesium; hypomagnesemic state may be resistant to thiamine administration
    • Treatment can take days to weeks to work if at all (despite accurate diagnosis)
    • Give thiamine BEFORE glucose in patients requiring glucose who are at risk for thiamine deficiency; glucose without thiamine can precipitate/worsen WE by driving thiamine intracellularly

Vitamin Prophylaxis for Alcoholics

  • For the majority of chronic alcoholics, you should not administer a banana bag (thiamine 100mg + magnesium 2-4 g + folate 1mg + multivitamin; all in 1L NS or D5W)[2][3]
    • At risk for thiamine deficiency but no symptoms: thiamine 100mg PO q day
    • Give multivitamin; patient at risk for other vitamin deficiencies

Disposition

  • Admit

See Also

References

  1. Donnino, Michael, et al. “Myths and misconceptions of wernicke’s encephalopathy: what every emergency physician should know.” Annals of emergency medicine. 2007. Vol 50, no 6. Pages 715-721.
  2. Sechi, GianPietro; Serra, Alessandro. “Wernicke’s encephalopathy: new clnical settings and recent advances in diagnosis and management.” Neurology. Vol 6, May 2007. Pages 442-455
  1. Isenberg-Grzeda E, Kutner HE, Nicolson SE. Wernicke-Korsakoff-syndrome: under-recognized and under-treated. Psychosomatics. 2012 Nov-Dec;53(6):507-16.
  2. Krishel, S, et al. Intravenous Vitamins for Alcoholics in the Emergency Department: A Rreview. The Journal of Emergency Medicine. 1998; 16(3):419–424.
  3. Li, SF, et al. Vitamin deficiencies in acutely intoxicated patients in the ED. The American Journal of Emergency Medicine. 2008; 26(7):792–795.

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