The clinical and laboratory diagnosis of sCJD and vCJD

Clinical diagnosis of sporadic CJD is usually confirmed by a typical EEG pattern and an increased level of protein 14-3-3 in the CSF, with specificity of 74% and 84%, respectively. However, both these tests are often negative in vCJD patients. Recently MR imaging has substantially improved the diagnosis of sCJD and vCJD. In sCJD patients hyperintense signal in T2-weighted sequences was found to be present in the neostriatum (the caudate nucleus and putamen) with specificity of 93%, while in vCJD cases signal hyperintensity was found in pulvinar thalami. The "pulvinar sign" was evidenced to be highly sensitive (79%) and specific (100%) for the diagnosis of the latter form of CJD.