Study On The Characteristics Of The Surveiled Creutzfeldt-Jakob Disease Patients In2013in China And Analysis Of The Compliance And Related Influence Factors In The Follow-up Process Of Surveillance

Transmissible spongiform encephalopathies (TSE) is a kind of fatal neurodegenerative disease affecting human and many other mammalian hosts which was caused by prion. Prion, the infection factor of the disease, is considered as a self-duplicate infection protein without nucleic acid, thereby, TSE is also called prion disease. More than20kinds of spontaneous or infected TSEs have been identified so far. Human TSEs are classified as Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (FFI), Gerstmann-Straussler-Scheinker syndrome (GSS) and Kuru. Animal prion diseases include bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep, chronic wasting disease in deer and elk(CWD), transmissible mink encephalopathy in minks(TME), transmissible feline encephalopathy (FSE) in feline catamount and so on. According to pathogenesis. CJD can be divided to four subtypes: sporadic CJD, genetic CJD or familial CJD. iatrogenic CJD and variant CJD. This study is divided into two parts. Part one is the description of the epidemiological and clinical characteristics of the CJD patients reported through the national CJD surveillance system in2013. Part two is the analysis of the possible factors influencing on the compliances of the interviewees via telephone during CJD follow-up based on the cases from January to September in2013of the surveillance system.Part I:Study on the characteristics of the surveiled Creutzfeldt-Jakob disease patients in2013in ChinaTo describe the epidemiological and clinical characteristics of Creutzfeldt-Jakob disease (CJD) patients in mainland of China, the clinical and epidemical information of the reported suspect CJD patients from China CJD surveillance network was analyzed. Blood and cerebral spinal fluid (CSF) specimens from these patients were collected. Western blot assay was used for detecting14-3-3protein in CSF. PCR and sequencing assay were used for analyzing the potential mutation of PRNP gene and the polymorphism of amino acid (aa)129and219.Totally,299suspect CJD cases were reported to CJD surveillance center in China CDC. About116(38.80%) probable and25(8.36%) possible sporadic CJD (sCJD) patients,9(3.01%) genetic CJD (gCJD) cases and3FFI (1.00%) cases were identified. No seasonal-, geographic-or occupational-related events were observed among these cases. The median age of probably and possibly diagnosed sCJD cases was61(32,86) and53(21,87) year-old respectively. The male to female ratio of the probable CJD patients and possible CJD was0.87:1and0.79:1. Rapidly progressive dementia was the main foremost symptom. In probable CJD cases, the proportion of appearance of four and three major clinical manifestations was higher than that of possible CJD cases. Generally, the patients of probably sCJD with typical abnormalities in EEG, MRI and CSF14-3-3showed more clinical symptoms. Among the tested283blood samples of suspect CJD cases,278cases were M/M and5were M/V in codon129alleles,277cases were E/E and6were E/K in codon219alleles. In Conclusion, CJD occurred sporadically in China in2013. rapidly progressive dementia was the main foremost symptom. Probable CJD patients showed more major clinical manifestations than that of the possible ones. The seasonal and geography distribution, occupation, the ratio of gender and the mean onset age of the CJD cases in2013were consistent with the general characteristics of sCJD in the world. Additionally,12genetic human prion disease were diagnosed in2013. D178R E200K and T188K gCJD were the most commonly identified genetic prion diseases in Han Chinese.Part â…¡:Analysis of the compliance and the related influence factors in the follow-up process of surveillance for Creutzfeldt-Jakob diseaseThe surveillance for CJD was set up from2006in China. As one of the major issues in CJD surveillance routine work, the follow-up process via telephone plays important role in CJD diagnosis and surveillance. Although the follow-up process was conducted by the experiential staffs from CJD surveillance center, some family members of the CJD cases still do not cooperate well with the staffs. To screen the possible factors influence on the compliances of the interviewees during CJD follow-up, thirteen independent variables from patient aspect and five variables from interviewees were selected and a questionnaire was prepared. Based on199suspected sporadic CJD cases reported to CJD surveillance center in2013from January to September, a telephone-inquiring was conducted and the degree of compliances of the interviewees were given as good, fair or poor. After screened with univariate analysis and evaluated ordinal logistic regression analysis, several indictors, such as the patient gender, CJD diagnosis, numbers of clinical symptoms, medical treatment mode, continual medical treatment after diagnosis, as well as the relationship with the patient and CJD knowledge of the interviewees, showed influence on the compliance in CJD follow-up process significantly. The data here provides for the first time the factors related with the compliances of the interviewee during follow-up process, which supplies useful clue for us to improve CJD follow-up of CJD surveillance.