Found programs: National Natural Science Foundation of China (No.81630062);National Key Research and Development Program of China (No.2020YFE0205700)
Authors:Zhang Weiwei; Liang Donglin; Zhou Donghua; Wang Yuan; Xiao Kang; Zhou Wei; Dong Xiaoping; Shi Qi
Keywords:sporadic Creutzfeldt-Jakob disease;Prion;survival analysis;influencing factors;Cox proportional hazards regression model
DOI:DOI:10.19405/j.cnki.issn1000-1492.2024.10.021
〔Abstract〕 Objective To investigate the survival time of patients diagnosed with sporadic Creutzfeldt-Jakob disease in China between 2020 and 2022 and explore the associated factors influencing survival. Methods A retrospective analysis was conducted on clinically diagnosed cases with complete information on sporadic Creutzfeldt-Jakob disease diagnosed by the China Creutzfeldt-Jakob disease surveillance network from 2020 to 2022, baseline information of patients was obtained from the case files, telephone follow-up was used to obtain the treatment and survival status of the patients after the diagnosis, life-table method was used for estimating the survival rate, Kaplan-Meier method was used for calculating the median survival time and the 95%CI, Cox regression model was used for univariate and multivariate analyses were used to screen for factors influencing survival time. Results The median survival time of the 300 patients was 5 months(95%CI: 4.165-5.835). Univariate analysis revealed that factors such as age at onset, regional distribution, presence of corticobasal or extrapyramidal symptoms as initial manifestations, number of initial symptoms, presence of corticobasal or extrapyramidal functional abnormalities, number of major clinical manifestations, presence of typical electroencephalogram findings, and use of nasal feeding during the course of the disease were potential factors influencing survival time(P<0.1). Multivariate analysis showed that the risk of death in patients with onset age>65 years was 1.350 times higher than in patients with onset age ≤65 years(P=0.021, 95.0%CI: 1.046-1.742). Patients without pyramidal or extrapyramidal dysfunction had a 0.674-fold lower risk of death compared to those with these symptoms(P=0.020, 95.0%CI: 0.483-0.939). Patients who did not receive nasal feeding had a 1.817-fold higher risk of death compared to those who did(P<0.001, 95.0%CI: 1.406-2.349). Conclusion Age at onset, the presence of pyramidal or extrapyramidal functional abnormalities, and the use of nasal feeding during the disease course are factors influencing the survival time of patients clinically diagnosed with sCJD.