Case ReportWernicke’s encephalopathy mimicking variant Creutzfeldt-Jakob disease
Section snippets
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 Over the prior 2 weeks he had become progressively withdrawn, food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
References (4)
- et al.
The pulvinar sign on magnetic imaging in variant Creutzfeldt-Jakob disease
Lancet
(2000) - World Health Organization. The revision of the variant Creutzfeldt-Jakob (vCJD) case definition. Report of a WHO...
Cited by (9)
Prion Diseases
2018, Neurologic ClinicsCitation Excerpt :Another common, yet less specific MRI finding is the double hockey stick sign, in which bilateral medial-dorsal and posterior thalami are bright on T2- or diffusion-weighted sequences (Fig. 4).109,118 The double hockey stick sign also has been reported on T2- and/or diffusion-weighted MRI in a variety of conditions, including sJCD,119,120 metabolic disorders such as Wernicke’s encephalopathy,111,112 paraneoplastic limbic encephalitis,115,116 and thalamic infarcts. As vJCD progresses, the pulvinar sign and other MRI findings may disappear in late stages of disease.118,121
Variant Creutzfeldt-Jakob disease: Neuropsychological profile in an extended series of cases
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