Wernicke-Korsakoff syndrome

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Background

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

Epidemiology

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

Causes

  • Anything that causes thiamine (vitamin B1) deficiency: poor dietary intake, malabsorption, increased metabolic requirement
    • Chronic alcoholism, dieting/fasting/starvation, anorexia, vomiting/diarrhea, unbalanced TPN, GI surgery, malignancy, dialysis, AIDS, IBD, pancreatitis, liver disease, thyrotoxicosis

Pathophysiology

  • Brain lesions/atrophy occurs: mamillary bodies (nearly all cases), thalamus, periaqueductal gray matter, 3rd/4th ventricle, cerebellum, frontal lobe

Diagnosis

WE/KP/WKS = clinical diagnoses


Wernicke’s Encephalopathy

  • Classic triad: encephalopathy, oculomotor dysfunction, gait ataxia
  • werNICke mnemonic:
    • N: Nystagmus/ophthalmoplegia
    • I: Incoordination/ataxia
    • C: Confusion/memory impairment
  • Other sx: hypotension, tachycardia, EKG abnormalities, DOE, CHF sx, hypothermia, coma, dry/wet Beriberi


Korsakoff’s Psychosis

  • Sx: anterograde/retrograde amnesia, confabulation, confusion, apathy


Wernicke-Korsakoff Syndrome

  • Sx: combination of WE and KP

Treatment

If suspect WE/KP/WKS: thiamine 500 mg IV over 30 min TID x 2 days, then 500 mg IV/IM q day for 5 days, then 100 mg PO q day until pt no longer at risk


If suspect at risk for thiamine deficiency but no sx: thiamine 100 mg PO q day


  • If you suspect WE/KP/WKS then treat it! Diagnosis is clinical and difficult to confirm, treatment is simple/inexpensive/effective, there is little risk to treatment, and the risk of morbidity/mortality from not treating is high
  • Give magnesium; hypomagnesemic state may be resistant to thiamine administration
  • Give multivitamin; pt at risk for other vitamin deficiencies
  • For chronic alcoholics always consider banana bag: thiamine 100 mg + magnesium 2-4 g + folate 1 mg + multivitamin; all in 1L NS or D5W

See Also