Objective To investigate the clinical and imaging features of sporadic CreutzfeldtJakob disease （sCJD） and to follow up the survival period， in order to guide the clinical early detection of the disease. Methods A retrospective analysis of 9 patients with sCJD admitted to the Department of Neurology， Peking University Third Hospital from January 2002 to December 2020 was carried out. Results The onset age of the 9 patients was （62±10） years. The most common symptoms were akinetic mutismin 7 cases （7/9）， myoclonic seizures 6 cases （6/9）， slow to response 5 cases （5/9）， ataxia 4 cases （4/9）， mental behavior disorder and confusion 3 cases （3/9）， and walking instability 3 cases （3/9）. The first symptoms were mainly sluggish reaction， mental behavior abnormal and confusion. There are six cases（6/9）with cortical ribboning sign on diffusion weighted imaging，positive cerebral spinal fluid 14-3-3 protein 4 cases （4/9）， basal ganglion abnormal high signal 3 case （3/9）， and electroencephalogram three-phase wave 1 case （1/9）. The median survival time was 4 months. In the 24-months follow-up， 2 patients （2/9） survived. Conclusions In this study， the majority of patients with sCJD are middle-aged and elderly， and the first symptom is not specific. The occurrence of magnetic resonance diffusion-weight imaging "cortical ribboning sign" and basal ganglion abnormal high signal is frequent. The median survival time was short.