CREUTZFELDT-JAKOB-DISEASE - CLINICAL-FEATURES, EPIDEMIOLOGY AND TESTS

Creutzfeldt-Jakob disease (CJD) is now recognized to occur in four mai n forms: classical sporadic, genetic, iatrogenic and new variant. Thes e 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 f orm of a normal cellular protein. Protein analysis and the study of th e gene which encodes for this protein have therefore played an importa nt role in understanding CJD. Diagnostic tests for CJD are discussed w ith particular reference to the detection of the 14-3-3 protein in the cerebrospinal fluid (CSF).