Analyses of the survival time and the influencing factors of chinese patients with prion diseases based on the surveillance data from 2008-2011

Abstract

Background: Prion diseases are kinds of progressive, incurable neurodegenerative disorders. So far, survival time of the patients with these diseases in China is unclear.

Methods: Based upon the surveillance data from Chinese Creutzfeldt-Jakob disease (CJD) surveillance network from January 2008 to December 2011, a retrospective follow-up survey was performed. The survival times of Chinese patients with prion diseases and the possible influencing factors were analyzed.

Results: Median survival time of 121 deceased patients was 7.1 months, while those for sporadic CJD (sCJD), familial CJD (fCJD) and fatal familial insomnia (FFI) cases were 6.1, 3.1 and 8.2 months, respectively. 74.0% of sCJD patients, 100% of fCJD cases and 91.7% FFI cases died within one year. The general socio-demographic factors, abnormalities in clinical examinations, clinical manifestations, and social factors did not significantly influence the survival times of Chinese prion patients.

Conclusions: Survival time of Chinese patients with prion diseases was comparable with that of many Western countries, but obviously shorter than that of Japan. Patients with acute onset and rapid progression had significantly short survival times.

Figure 1. Summarization of the follow-up results of surveillance cases which initially diagnosed as various subtypes of prion disease from January 2008 to December 2011.

Figure 2. The Kaplan-Meier survival curves for deceased patients.

(A) Survival time for deceased patients. B) Survival time for deceased patients stratified by subtypes of prion disease. X-axis represents survival time (months) and Y-axis represents survival probability. Graphic symbol shows the median survival time (50% percentile) of the entire and distinct subtype disease of deceased patients.