Wernicke’s encephalopathy mimicking variant Creutzfeldt-Jakob disease

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.