MRI of Creutzfeldt-Jakob disease: Imaging features and recommended MRI protocol

Creutzfeldt-Jakob Disease (CJD) is a rare, progressive and invariably fatal neurodegenerative disease characterized by specific histopathological feat ures. Of the four subtypes of CJD described, the commonest is sporadic CJD (sCJD). More recently, a new clinically distinct form of the disease affect ing younger patients, known as variant CJD (vCJD), has been identified, and this has been causally linked to the bovine spongiform encephalopathy (BSE ) agent in cattle. Characteristic appearances on magnetic resonance imaging (MRI) have been identified in several forms of CJD; sCJD may be associated with high signal changes in the putamen and caudate head and vCJD is usual ly associated with hyperintensity of the pulvinar (posterior nuclei) of the thalamus. These appearances and other imaging features are described in th is article. Using appropriate clinical and radiological criteria and tailor ed imaging protocols, MRI plays an important part in the in vivo diagnosis of this disease. (C) 2001 The Royal College of Radiologists.