摘要Creutzfeldt-Jakob disease (CJD), which is caused by prion scrapie protein, is a rare, chronic, transmissible and fatal disease. Clinical manifestations of CJD include rapidly progressive dementia, cerebellar ataxia, visual disturbance, as well as pyramidal and extrapyramidal tract signs. Four subtypes of CJD have been reported, including sporadic, familial or genetic, iatrogenic and variant. Given the infectiousness and high mortality of the disease, it is imperative that earlier and more accurate diagnostic methods are developed. In the past years, 14-3-3 protein testing and periodic sharp wave complexes in electroencephalogram have been widely used in CJD clinical diagnosis; and the abnormal hyper-intensity in diffusion weighted imaging has also been used. Recently, there has been a focus on the diagnostic value of 18F-fluorodeoxyglucose positron emission tomography/computed tomography. New findings of potential biomarkers in cerebrospinal fluid and decreases in diffusion tensor imaging measures have emerged as having an association with CJD. Magnetic resonance spectroscopy has also drawn attention as an emerging method for diagnosis. In this review, the progress in auxiliary examinations of CJD is discussed and the potential, future diagnostic methods are introduced.