Wernicke-Korsakoff syndrome: Difference between revisions
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==Background== | ==Background== | ||
*Wernicke’s Encephalopathy | *Wernicke’s Encephalopathy: '''acute''' neurologic symptoms caused by [[thiamine deficiency]] | ||
*Korsakoff’s Psychosis | *Korsakoff’s Psychosis: '''chronic''' neurologic symptoms caused by thiamine deficiency | ||
*Wernicke-Korsakoff Syndrome (WKS): presence of | *Wernicke-Korsakoff Syndrome (WKS): presence of Wernicke's Encephalopathy + Korsakoff's Psychosis simultaneously | ||
===Epidemiology=== | ===Epidemiology=== | ||
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*Other symptoms: | *Other symptoms: | ||
**[[Hypotension]], tachycardia, ECG abnormalities | **[[Hypotension]], tachycardia, ECG abnormalities | ||
**[[ | **[[Dyspnea]] on exertion, [[CHF]] symptoms | ||
**Hypothermia, | **Hypothermia, | ||
**Dry/wet [[beriberi]] | **Dry/wet [[beriberi]] | ||
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*Antero/retrograde amnesia | *Antero/retrograde amnesia | ||
*Confabulation, confusion, apathy | *Confabulation, confusion, apathy | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
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==Evaluation== | ==Evaluation== | ||
*Clinical diagnosis | *Clinical diagnosis | ||
*Wernicke's Encephalopathy | *Wernicke's Encephalopathy - at least 2 of the following:<ref>Isenberg-Grzeda E, Kutner HE, Nicolson SE. Wernicke-Korsakoff-syndrome: under-recognized and under-treated. Psychosomatics. 2012 Nov-Dec;53(6):507-16.</ref>: | ||
**Nutritional deficiency | **Nutritional deficiency | ||
**Ocular findings (ophthalmoplegia, nystagmus) | **Ocular findings (ophthalmoplegia, nystagmus) | ||
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**Treatment can take days to weeks to work if at all (despite accurate diagnosis) | **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 | **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 | ||
==Disposition== | ==Disposition== | ||
*Admit | *Admit | ||
==Prevention== | |||
{{Vitamin prophylaxis for ETOH}} | |||
==See Also== | ==See Also== | ||
{{Ethanol DDX}} | |||
==Video== | |||
{{#widget:YouTube|id=6MhVkxiZdck}} | |||
==References== | ==References== | ||
<references/> | <references/> | ||
[[Category:Neurology]] | [[Category:Neurology]] | ||
Revision as of 23:06, 7 November 2017
Background
- Wernicke’s Encephalopathy: acute neurologic symptoms caused by thiamine deficiency
- Korsakoff’s Psychosis: chronic neurologic symptoms caused by thiamine deficiency
- Wernicke-Korsakoff Syndrome (WKS): presence of Wernicke's Encephalopathy + Korsakoff's Psychosis 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)
- Deficiency leads to lactic acidosis, altered brain metabolism
- Lipid metabolism (including myelin sheath formation)
- Alterations in myelination leads to peripheral neuropathy
- Energy production pathways (Kreb's cycle, pentose phosphate pathway, alpha-ketoglutarate dehydrogenase, pyruvate dehydrogenase)
- 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
- Nystagmus/ophthalmoplegia
- Other symptoms:
- Hypotension, tachycardia, ECG abnormalities
- Dyspnea on exertion, CHF symptoms
- Hypothermia,
- Dry/wet beriberi
- Coma
Korsakoff’s Psychosis
- Antero/retrograde amnesia
- Confabulation, confusion, apathy
Differential Diagnosis
- Ethanol toxicity
- Alcohol use disorder
- Alcohol withdrawal
- Electrolyte/acid-base disorder
Vitamin deficiencies
- Vitamin A deficiency
- Vitamin B deficiencies
- Vitamin B1 deficiency (Thiamine)
- Vitamin B3 deficiency (Pellagra)
- Vitamin B9 deficiency (Folate)
- Vitamin B7 deficiency (Biotin)
- Vitamin B12 deficiency
- Vitamin C deficiency (Scurvy)
- Vitamin D deficiency (Rickets)
- Vitamin E deficiency
- Vitamin K deficiency
- Zinc deficiency
Evaluation
- Clinical diagnosis
- Wernicke's Encephalopathy - at least 2 of the following:[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
Disposition
- Admit
Prevention
Vitamin Prophylaxis for Chronic alcoholics
- At risk for thiamine deficiency, but no symptoms: thiamine 100mg PO q day
- Give multivitamin PO; patient at risk for other vitamin deficiencies
Banana bag
The majority of chronic alcoholics do NOT require a banana bag[2][3]
- Thiamine 100mg IV
- Folate 1mg IV (cheaper PO)
- Multivitamin 1 tab IV (cheaper PO)
- Magnesium sulfate 2mg IV
- Normal saline as needed for hydration
See Also
- Ethanol toxicity
- Alcohol use disorder
- Alcohol withdrawal
- Electrolyte/acid-base disorder
Video
{{#widget:YouTube|id=6MhVkxiZdck}}
References
- ↑ Isenberg-Grzeda E, Kutner HE, Nicolson SE. Wernicke-Korsakoff-syndrome: under-recognized and under-treated. Psychosomatics. 2012 Nov-Dec;53(6):507-16.
- ↑ Krishel, S, et al. Intravenous Vitamins for Alcoholics in the Emergency Department: A Review. The Journal of Emergency Medicine. 1998; 16(3):419–424.
- ↑ Li, SF, et al. Vitamin deficiencies in acutely intoxicated patients in the ED. The American Journal of Emergency Medicine. 2008; 26(7):792–795.