TABLE 1

Diagnostic tests for Creutzfeldt-Jakob disease: Sensitivity and specificity

TestingSensitivitySpecificityDiagnostic criteriaNotes
Magnetic resonance imaging
DWI or FLAIR3083%83%At least 2 cortical regions affected (parietal–temporal–occipital) or both putamen and nucleus caudatum affectedRetrospective evaluation of pathology-proven CJD
DWI and FLAIR3191%95%2005 UCSF MRI criteria for CJD31Retrospective evaluation of clinically diagnosed prion disease, majority spontaneous CJD (83%); excellent interreader reliability (kappa 0.96)
DWI and FLAIR3296%93%2005 UCSF MRI criteria for CJD31Retrospective evaluation of clinically diagnosed prion disease, majority spontaneous CJD (79%)
DWI3392%94%High-intensity lesions in the striatum (caudate or putamen, or both), lesions in the thalamus including the pulvinar, and/or lesions along the cortical ribbon (cerebral or cerebellar)Retrospective evaluation of clinically diagnosed prion disease, majority spontaneous CJD (78%)
Electroencephalography32
64%91%1996 Steinhoff criteria27Retrospective evaluation of pathology-proven CJD
Cerebrospinal fluid studies34
14-3-3 protein83%63%Positive testRetrospective analysis of 111 neuropathologically confirmed sCJD cases
Total tau protein91% 46%Positive test
RT-QuIC92% 99%Positive test
  • CJD = Creutzfeldt-Jakob disease; DWI = diffusion-weighted imaging; FLAIR = fluid-attenuated inversion recovery; RT-QulC = real-time quaking-induced conversion; sCJD = sporadic Creutzfeldt-Jakob disease; UCSF = University of California, San Francisco