Creutzfeldt‐Jakob disease: Clinical features, epidemiology and tests

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Abstract
Creutzfeldt‐Jakob disease (CJD) is now recognized to occur in four main forms: classical sporadic, genetic, iatrogenic and new variant. These are briefly described. The evidence that new variant disease is due to bovine spongiform encephalopathy (BSE) is reviewed. In CJD, disease and infectivity are associated with the accumulation of an abnormal form of a normal cellular protein. Protein analysis and the study of the gene which encodes for this protein have therefore played an important role in understanding CJD. Diagnostic tests for CJD are discussed with particular reference to the detection of the 14‐3‐3 protein in the cerebrospinal fluid (CSF).