Abstract
BackgroundPrion diseases are kinds of progressive, incurable neurodegenerative disorders. So far, survival time of the patients with these diseases in China is unclear.MethodsBased 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.ResultsMedian 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.ConclusionsSurvival 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.
Highlights
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of neurodegenerative disorders that afflict human and animals [1]
Another acquired CJD, namely variant Creutzfeldt-Jakob disease, is caused by consuming the beef or its products contaminated with the agent of bovine spongiform encephalopathy (BSE) [3]
Follow-up and Data Collection Suspected CJD cases referred to China CJD surveillance were diagnosed and subtyped according to the diagnostic criteria issued by Chinese Center for Disease Control and Prevention (CCDC), which was constituted based on the diagnostic criteria for CJD issued by WHO [6]
Summary
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a group of neurodegenerative disorders that afflict human and animals [1]. Sporadic human prion disease mainly indicates sporadic Creutzfeldt-Jakob disease (sCJD) which is the most common condition and its reasons are still unclear. Acquired Creutzfeldt-Jakob disease traditionally refers to the diseases transmitted by various iatrogenic procedures, such as treatment with human pituitary growth hormones, dura mater and cornea grafts, deep brain electrodes and neurosurgery [2]. Another acquired CJD, namely variant Creutzfeldt-Jakob disease (vCJD), is caused by consuming the beef or its products contaminated with the agent of bovine spongiform encephalopathy (BSE) [3]. Survival time of the patients with these diseases in China is unclear
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
