Wernicke-Korsakoff syndrome: Difference between revisions

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


==Clinical Features==
==Clinical Features==
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*Classic triad: [[encephalopathy]], oculomotor dysfunction, gait [[ataxia]]
*Classic triad: [[encephalopathy]], oculomotor dysfunction, gait [[ataxia]]
*wer'''NIC'''ke mnemonic:  
*wer'''NIC'''ke mnemonic:  
**'''N'''ystagmus/ophthalmoplegia
**[[nystagmus|'''N'''ystagmus]]/ophthalmoplegia
***Ocular findings may also include bilateral 6th nerve palsy, conjugate gaze palsy, pupillary abnormality, retinal hemorrhage, ptosis.   
***Ocular findings may also include bilateral 6th nerve palsy, conjugate gaze palsy, pupillary abnormality, retinal hemorrhage, ptosis.   
**'''I'''ncoordination/ataxia
**'''I'''ncoordination/[[ataxia]]
**'''C'''onfusion/memory impairment
**[[confusion|'''C'''onfusion]]/memory impairment
*Other symptoms:  
*Other symptoms:  
**[[Hypotension]], tachycardia, ECG abnormalities
**[[Hypotension]], [[tachycardia]], ECG abnormalities
**[[Dyspnea]] on exertion, [[CHF]] symptoms
**[[Dyspnea]] on exertion, [[CHF]] symptoms
**Hypothermia,
**[[Hypothermia]]
**Dry/wet [[beriberi]]
**Dry/wet [[beriberi]]
**[[Coma]]
**[[Coma]]
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==Evaluation==
==Evaluation==
[[File:MRI FLAIR sequence Wernicke Encephalopathy.jpg|thumb|Axial MRI FLAIR image showing hyperintense signal in the mesial dorsal thalami, a common finding in Wernicke encephalopathy]]
===Workup===
===Diagnosis===
*Clinical diagnosis
*Clinical diagnosis
*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>:
*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>:
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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
===Vitamin Prophylaxis for Alcoholics===
*For the majority of chronic alcoholics, you should NOT administer a [[banana bag]]<ref>Krishel, S, et al. Intravenous Vitamins for Alcoholics in the Emergency Department: A Review. The Journal of Emergency Medicine. 1998; 16(3):419–424.</ref><ref>Li, SF, et al. Vitamin deficiencies in acutely intoxicated patients in the ED. The American Journal of Emergency Medicine. 2008; 26(7):792–795.</ref>
**At risk for thiamine deficiency, but no symptoms: thiamine 100mg PO q day
**Give multivitamin PO; patient at risk for other vitamin deficiencies


==Disposition==
==Disposition==
*Admit
*Admit
*<25% of patients show full recovery, 50% show partial recovery, the remainder show no response despite treatment<ref>https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/korsakoff-syndrome</ref>
==Prevention==
{{Vitamin prophylaxis for ETOH}}


==See Also==
==See Also==

Latest revision as of 22:46, 18 October 2023

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)
    • 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 deficiency types

Clinical Features

Wernicke’s Encephalopathy

Korsakoff’s Psychosis

  • Antero/retrograde amnesia
  • Confabulation, confusion, apathy

Differential Diagnosis

Ethanol related disease processes

Vitamin deficiencies

Evaluation

Axial MRI FLAIR image showing hyperintense signal in the mesial dorsal thalami, a common finding in Wernicke encephalopathy

Workup

Diagnosis

  • 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
  • <25% of patients show full recovery, 50% show partial recovery, the remainder show no response despite treatment[2]

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[3][4]

See Also

Ethanol related disease processes

Video

{{#widget:YouTube|id=6MhVkxiZdck}}

References

  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. https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/korsakoff-syndrome
  3. Krishel, S, et al. Intravenous Vitamins for Alcoholics in the Emergency Department: A Review. The Journal of Emergency Medicine. 1998; 16(3):419–424.
  4. Li, SF, et al. Vitamin deficiencies in acutely intoxicated patients in the ED. The American Journal of Emergency Medicine. 2008; 26(7):792–795.