Difference between revisions of "Wernicke-Korsakoff syndrome"

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==Background==
 
==Background==
*Wernicke’s Encephalopathy (WE): ACUTE neuro/cardiovascular sx caused by thiamine deficiency
+
*Wernicke’s Encephalopathy: '''acute''' neurologic symptoms caused by [[thiamine deficiency]]
*Korsakoff’s Psychosis (KP): CHRONIC neurologic symptoms caused by thiamine deficiency
+
*Korsakoff’s Psychosis: '''chronic''' neurologic symptoms caused by thiamine deficiency
*Wernicke-Korsakoff Syndrome (WKS): presence of WE + KP simultaneously
+
*Wernicke-Korsakoff Syndrome (WKS): presence of Wernicke's Encephalopathy + Korsakoff's Psychosis simultaneously
  
 
===Epidemiology===
 
===Epidemiology===
*Only 20% identified before death, failure of dx leads to 20% mortality and 75% permanent damage
+
*Only 20% identified before death, failure of diagnosis leads to 20% mortality and 75% permanent damage
  
 
===Pathophysiology===
 
===Pathophysiology===
*Brain lesions/atrophy occurs: mamillary bodies (nearly all cases), thalamus, periaqueductal gray matter, 3rd/4th ventricle, cerebellum, frontal lobe
+
*Thiamine plays critical role in:
*Thiamine is a cofactor for enzymes required in:
+
**Energy production pathways (Kreb's cycle, pentose phosphate pathway, alpha-ketoglutarate dehydrogenase, pyruvate dehydrogenase)
**krebs cycle
+
***Deficiency leads to [[lactic acidosis]], altered brain metabolism
**Pentose phosphate pathway
+
**Lipid metabolism (including myelin sheath formation)
**Alpha-ketoglutarate dehydrogenase, pyruvate dehydrogenase.
+
***Alterations in myelination leads to peripheral neuropathy
*Because thiamine is an important cofactor in critical pathways for energy production, deficiency results in lactic acidosis and alteration of brain metabolism
+
*Brain lesions/atrophy usually occur in: mamillary bodies (nearly all cases), thalamus, periaqueductal gray matter, 3rd/4th ventricle, cerebellum, frontal lobe
*Thiamine is also important for lipid metabolism and may affect myelin sheath formation.  This may explain peripheral neuropathy symptoms in dry beriberi.
 
  
 
===Causes===
 
===Causes===
*Anything that causes thiamine (vitamin B1) deficiency: poor dietary intake, malabsorption, increased metabolic requirement
+
*Thiamine (vitamin B1) deficiency caused by
**Chronic alcoholism, dieting/fasting/starvation, anorexia, vomiting/diarrhea, unbalanced TPN, GI surgery, malignancy, dialysis, AIDS, IBD, pancreatitis, liver disease, thyrotoxicosis
+
**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
 +
 
 +
{{Thiamine deficiency types}}
  
 
==Clinical Features==
 
==Clinical Features==
 
===Wernicke’s Encephalopathy===
 
===Wernicke’s Encephalopathy===
*Classic triad: encephalopathy, oculomotor dysfunction, gait ataxia
+
*Classic triad: [[encephalopathy]], oculomotor dysfunction, gait [[ataxia]]
*werNICke mnemonic:  
+
*wer'''NIC'''ke mnemonic:  
**N: Nystagmus/ophthalmoplegiaOcular findings may also include bilateral 6th nerve palsy, conjugate gaze palsy, pupillary abnormality, retinal hemorrhage, ptosis.   
+
**[[nystagmus|'''N'''ystagmus]]/ophthalmoplegia
**I: Incoordination/ataxia
+
***Ocular findings may also include bilateral 6th nerve palsy, conjugate gaze palsy, pupillary abnormality, retinal hemorrhage, ptosis.   
**C: Confusion/memory impairment
+
**'''I'''ncoordination/[[ataxia]]
*Other sx: hypotension, tachycardia, ECG abnormalities, DOE, CHF sx, hypothermia, coma, dry/wet Beriberi
+
**[[confusion|'''C'''onfusion]]/memory impairment
 +
*Other symptoms:  
 +
**[[Hypotension]], [[tachycardia]], ECG abnormalities
 +
**[[Dyspnea]] on exertion, [[CHF]] symptoms
 +
**[[Hypothermia]]
 +
**Dry/wet [[beriberi]]
 +
**[[Coma]]
  
 
===Korsakoff’s Psychosis===
 
===Korsakoff’s Psychosis===
*Sx: anterograde/retrograde amnesia, confabulation, confusion, apathy
+
*Antero/retrograde amnesia
 
+
*Confabulation, confusion, apathy
===Wernicke-Korsakoff Syndrome===
 
*Sx: combination of WE and KP
 
  
 
==Differential Diagnosis==
 
==Differential Diagnosis==
 +
{{Ethanol DDX}}
 
{{Vitamin deficiencies DDX}}
 
{{Vitamin deficiencies DDX}}
  
==Diagnosis==
+
==Evaluation==
WE/KP/WKS = clinical diagnoses.
+
===Workup===
  
Wernicke's Encephalopathy diagnosis requires at least 2 out of the following 4 clinical criteria:
+
===Diagnosis===
# Nutritional deficiency
+
*Clinical diagnosis
#Ocular findings (ophthalmoplegia, nystagmus)
+
*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>:
# Ataxia
+
**Nutritional deficiency
# Mental status change
+
**Ocular findings (ophthalmoplegia, nystagmus)
 +
**Ataxia
 +
**Mental status change
  
==Treatment==
+
==Management==
''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''
+
''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
*Suspected 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
+
**Give [[magnesium]]; hypomagnesemic state may be resistant to thiamine administration
**Give magnesium; hypomagnesemic state may be resistant to thiamine administration
 
 
**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===
+
==Disposition==
*For the majority of chronic alcoholics, you should not administer a banana bag (thiamine 100 mg + magnesium 2-4 g + folate 1 mg + multivitamin; all in 1L NS or D5W)<ref>Krishel, S, et al. Intravenous Vitamins for Alcoholics in the Emergency Department: A Rreview. 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>
+
*Admit
**At risk for thiamine deficiency but no symptoms: thiamine 100 mg PO q day
+
*<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>
**Give multivitamin; pt at risk for other vitamin deficiencies
+
 
 +
==Prevention==
 +
{{Vitamin prophylaxis for ETOH}}
  
 
==See Also==
 
==See Also==
*[[Alcohol (ETOH) Intoxication]]
+
{{Ethanol DDX}}
*[[Alcoholic ketoacidosis]]
+
 
*[[Alcohol withdrawal: Inpatient management]]
+
==Video==
*[[Alcohol withdrawal: Outpatient management]]
+
{{#widget:YouTube|id=6MhVkxiZdck}}
*[[Alcohol withdrawal seizures]]
 
*[[Beriberi]]
 
*[[Delerium tremens]]
 
*[[Thiamine]]
 
*[[Thiamine deficiency]]
 
  
 
==References==
 
==References==
# 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.
 
# 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
 
 
<references/>
 
<references/>
  
==Video==
 
{{#widget:YouTube|id=6MhVkxiZdck}}
 
 
[[Category:Neurology]]
 
[[Category:Neurology]]

Latest revision as of 19:31, 23 February 2021

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

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

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.