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1
Wernicke’s Encephalopathy AMITESHWAR SINGH
INTERN, KMC MANIPAL.
Dr Carl WernickeA Polish neurologist, who
described this neuropsychiatric
syndrome in 1881 as a triad of acute mental
confusion, ataxia, and ophthalmoplegia.
2
3Etiopathogenesis Deficiency of vitamin B1 (thiamine) An essential coenzyme in several
biochemical pathways in the brain, Krebs cycle and the pentose phosphate
pathway α-ketoglutarate dehydrogenase, pyruvate
dehydrogenase, and transketolase. Mainly involved in the glucose
metabolism and cerebral energy utilization
Inhibition of metabolism in brain regions with higher metabolic
demands and high thiamine turnover.
4THIAMINE DEFICIENCY
Failure of thiamine-dependent cellular systems
Energy production drops, and neuronal damage ensues
BRAIN TISSUE INJURY
Reduced production of
succinate
Failure of GABA metabolism
5Causes Chronic alcoholism – Malnutrition –
reduced thiamine uptake and utilization Prolonged starvation Hyperemesis Gravidarum Bariatric Surgery (Bariatric BeriBeri) Malabsorption Syndromes Infants on formula diet deficient in
thiamine
Epidemiology
The incidence can be as high as 12.5% in a population of alcoholics.
The prevalence approximately 2%.
The male-to-female ratio is 1.7 : 1Average age at onset is 50 years.
6
History
The classical triad of symptoms – only 1/3rd of cases
Evidence of long-term alcohol abuse or malnutrition
Other symptoms could include memory disturbance, hypothermia with hypotension, and delirium tremens.
Special clinical scenarios as previously mentioned
7
8Physical examination
Ocular abnormalities – nystagmus , bilateral lateral rectus palsies, conjugate gaze palsies, sluggish pupils, ptosis, and anisocoria
Encephalopathy – global confusional state, disinterest, inattentiveness, or agitation; Coma is rare.
Gait ataxia – cerebellar damage, and vestibular paresis
Peripheral neuropathy – foot drop, and decreased proprioception
9Differential Diagnosis
Psychosis Normal pressure hydrocephalus Cerebrovascular accident Chronic hypoxia Closed-head injury Hepatic encephalopathy Postictal state
10
Work-Up CBC – R/o severe anemias and leukemias
as causes of altered mental status Blood glucose levels - Exclude
hypoglycemia/ hyperglycemia O2 Saturation and ABG - Exclude
hypoxia/ hypercarbia; Metabolic Acidosis may be+
Toxic drug screening - Excludes some causes of drug-induced altered mental status.
Lumbar puncture (LP) - Exclude CNS infections, if indicated
11
CT/MRI – exclude ICSOLs etc.No particular Changes in acute
presentation Chronic encephalopathy - excessive
mamillary body and cerebellar shrinkage
Erythrocyte transketolase activity assay, Thiamine assay – very specific tests – not widely available – reserved for diagnostic dilemmas
12
Treatment Emergency department care – Parenteral
thiamine –Requirement in chronic alcoholics may be as high as 500mg single dose or multiple daily doses.
Never start on Dextrose Parenteral magnesium sulfate as thiamine therapy
ineffective in presence of hypomagnesemia In-Patient care – Watch for complications –
Korsakoff psychosis – Alcohol withdrawal – Congestive heart failure – Lactic acidosis
Out-Patient Care – Thiamine 100 mg PO daily, start alcohol cessation program, Advise on importance of balanced diet.
13
Wernicke-Korsakoff Syndrome
85% of the survivors of the acute phase of Wernicke encephalopathy who remain untreated go on to develop Wernicke-Korsakoff syndrome.
It manifests with memory loss and confabulation.
About 20% eventually recover completely during long-term follow-up care.
14
Conclusion
Wernicke encephalopathy must be considered as a medical emergency, as it is associated with significant morbidity and mortality.
Because the condition is potentially reversible, early diagnosis is important.
The diagnosis is clinical and is mainly supported by the dramatic improvement of neurological signs on parenteral thiamine therapy.