Case Report
Wernicke’s encephalopathy mimicking variant Creutzfeldt-Jakob disease

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Abstract

A 45-year-old man from tropical Australia was admitted with subacute social withdrawal, cognitive deterioration, reduced awareness and eventual mutism. Variant Creutzfeldt-Jakob disease was considered on the basis of WHO case definition criteria including typical clinical features and MRI showing symmetrical hyperintensity in the pulvinar (posterior) nuclei of the thalami. However, tonsillar biopsy was negative. Wernicke’s encephalopathy was established on the basis of low serum thiamine on admission and eventual clinical improvement on high-dose intravenous thiamine replacement, despite initial failure to respond to the standard dose of 100 mg daily intramuscularly.

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Case report

A 45-year-old man presented to the emergency department of the Cairns Base Hospital, Queensland, Australia, with subacute, severe progressive cognitive and behavioural impairment. The patient was unable to give a history, and background information was obtained over the course of several days from family members. They described 6 months of mood swings and increasing depression, progressing to social isolation and weight loss. Over the prior 2 weeks he had become progressively withdrawn, food

Discussion

This case illustrates some important issues. First, there is overlap between the clinical features and investigation findings of WE and vCJD. Although the case definition for vCJD is well-defined,1 the clinical features of WE are much more variable. MRI features are similar in both conditions. Second, the optimal treatment of acute WE remains ill-defined and the commonly used ‘standard’ therapy of thiamine 100 mg intramuscularly daily may not be sufficient in severe cases, where high-dose

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