Variant Creutzfeldt-Jakob disease: update

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Variant Creutzfeldt-Jakob disease: an update.

Variant Creutzfeldt-Jakob disease (vCJD) is a novel human prion disease caused by the bovine spongiform encephalopathy agent. Most cases have occurred in the UK, with smaller numbers in 11 other countries. All definite vCJD cases have occurred in methionine homozygotes at codon 129 in the prion protein gene. Following oral infection, the vCJD agent appears to replicate in lymphoid tissues durin...

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Variant Creutzfeldt-Jakob disease.

Current evidence indicates that variant Creutzfeldt-Jakob disease is caused by the transmission of bovine spongiform encephalopathy to humans. The clinical and investigative features of variant CJD are relatively distinct from sporadic CJD and the neuropathological appearances are novel. The number of cases of vCJD in the UK may have peaked, but the total future number of cases of vCJD is uncer...

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Genetic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease.

The ‘‘pulvinar sign’’ on MRI brain scan is defined as hyperintensity of the posterior thalamus relative to the signal intensity of the anterior putamen. In the appropriate clinical context, the pulvinar sign and hyperintensity in the dorsomedial thalamic nuclei (the hockey stick sign) are sensitive and specific features of variant Creutzfeldt– Jakob disease (vCJD). Variant CJD is an acquired fo...

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Sporadic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease.

BACKGROUND The determination of the form of prion disease and early diagnosis are important for prognostic, public health, and epidemiologic reasons. OBJECTIVE To describe a patient with sporadic Creutzfeldt-Jakob disease (sCJD) who had a clinical history and initial electroencephalogram and magnetic resonance imaging findings consistent with variant CJD (vCJD). RESULTS Results of a repeate...

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New variant Creutzfeldt-Jakob disease.

New variant Creutzfeldt-Jakob disease is a novel human prion disorder with characteristic clinical and neuropathological features, which results from exposure to the bovine spongiform encephalopathy agent. The probably lengthy incubation period makes it difficult to predict future new variant Creutzfeldt-Jakob disease case numbers; further studies are required to clarify risk factors and the po...

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ژورنال

عنوان ژورنال: Gut

سال: 2005

ISSN: 0017-5749

DOI: 10.1136/gut.2004.048231