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Creutzfeldt-Jakob Disease
  1. Donald Collie
  1. Department of Neuroradiology and UK, CJD Surveillance Unit, Western General, Hospital, Edinburgh EH4 2XU, UK; E-mail: dac{at}skull.dcn.ed.ac.uk

    Abstract

    Introduction

    Creutzfeldt-Jakob Disease (CJD) is a rare fatal neurological disease, with four distinct forms. Sporadic CJD (sCJD) is the most common and is characterized by a rapidly progressive dementing illness, associated with EEG abnormalities and CSF protein changes (14-3-3 protein). In addition, there are characteristic magnetic resonance imaging (MRI) changes, consisting of basal ganglia (putamen and caudate head) hyperintensity. Although these abnormal appearances overlap with other diseases, their presence is diagnostically useful. In variant CJD (vCJD), the form of CJD associated with bovine spongiform encephalopathy (BSE), the pulvinar sign – a characteristic appearance of bilateral hyperintensity in the posterior nuclei of the thalamus – is highly specific for the diagnosis of variant CJD in the appropriate clinical context. This article reviews these and other MRI appearances of CJD.

    Background

    The transmissible spongiform encephalopathies (TSE) are a group of diseases affecting man and other animals, characterized by a fatal progressive neurological illness, characteristic

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