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Wernicke-Korsakoff syndrome
From WikEM
(Redirected from Wernicke disease)
Contents
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)
- 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
Wernicke-Korsakoff Syndrome
- Findings of both Wernicke and Korsakoff
Differential Diagnosis
- Ethanol toxicity
- Alcohol withdrawal
- Electrolyte/acid-base disorder
- Wernicke-Korsakoff syndrome
- Beer potomania syndrome
- Alcoholic ketoacidosis
Vitamin deficiencies
- Vitamin A deficiency
- Vitamin B deficiencies
- Vitamin B1 deficiency (Thiamine)
- Beriberi
- Wernicke-Korsakoff syndrome
- Vitamin B3 deficiency (Pellagra)
- Vitamin B9 deficiency (Folate)
- Vitamin B7 deficiency (Biotin)
- Vitamin B12 deficiency
- Vitamin B1 deficiency (Thiamine)
- Vitamin C deficiency (Scurvy)
- Vitamin D deficiency (Rickets)
- Vitamin E deficiency
- Vitamin K deficiency
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 PO; patient at risk for other vitamin deficiencies
Disposition
- Admit
See Also
- Alcohol (ETOH) Intoxication
- Alcoholic ketoacidosis
- Alcohol withdrawal: Inpatient management
- Alcohol withdrawal: Outpatient management
- Alcohol withdrawal seizures
- Beriberi
- Delerium tremens
- Thiamine
- Thiamine deficiency
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
- ↑ 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 Rreview. 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.
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