Survival Analysis Of Patients With Creutzfeldt-Jakob Disease In China From2008-2011and The Surveillance Of Creutzfeldt-Jakob Disease In China,2012

Part Ⅰ:Survival analysis of patients with Creutzfeldt-Jakob disease in China from2008-2011Prion diseases are kinds of progressive, incurable neurodegenerative disorders. So far, the survival time of the patients with these diseases in China is unclear. Based upon the surveillance data from Chinese Creutzfeldt-Jakob disease (CJD) surveillance network from January2008to December2011, a retrospective follow-up survey was performed. Through telephone follow-up, the family members of the patients registered in the national CJD monitoring network were interviwed, and the living conditions and survival times of the patients with prion diseases were collected and analyzed. Totally,196cases were included in this stduy,. The major conponents of follow-up or retrospective collection from the database of CJD surveillance included the foremost symptom and the onset time, the typical clinical manifestations during disease progression, the results of laboratory testing and clinical examinations, and the time of death. Among the196cases of prion diseases,48cases were lost after discharge from hospitals, nine were diagnosed as other diseases lately,18patients are still alive at the time of interview (all of them are the case registered in2011). Finally,121cases were enrolled into the study. The survival times of Chinese patients with prion diseases and the possible influencing factors were analyzed.Median survival time of121deceased patients was7.1(1.0-23.3) months, while those for sporadic CJD (sCJD), familial CJD (fCJD) and fatal familial insomnia (FFI) cases were6.1(1.0-23.3),3.13.1(1.0-9.1) and8.1(6.1-14.2) months, respectively.74.0%of sCJD patients,100%of fCJD cases and91.7%FFI cases died within one year. The survival times of various subtypes of prion diseases semmed to be different, that the average survival time of fCJD patients was significantly shorter and that of FFI cases. Gender and onset age of the cases did not showed significant effect on the survival times. FFI cases were relatively younger at onset and had a longer median survival time. The first symptom and typical clinical manifestations (myoclonus, visual or cerebella disturbance, pyramidal or extrapyramidal disfunction and akinetic mutism), the results of clinical examinations (EEG, MRI) and14-3-3protein in CSF test did not affect significantly on the survival time. However, in fCJD group,4cases with typical MRI changes had significantly shorter survival time than other5cases without typical MRI.images. The testing time of CSF14-3-3protein,the patient’s permanent place of residence (urban or rural), the continuous medical care t after diagnosis, the final place of death (at home or in hospital) also had no significant effect on the survival timeIn all, 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.Part II:surveillance of Creutzfeldt-Jakob disease in China,2012To describe epidemiological and clinical characteristics of the surveilled Creutzfeldt-Jakob disease (CJD) patients in China2012, the clinical and epidemical information of 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 detecting PrPSc in brain tissue and14-3-3protein in CSF, and PCR and sequencing assay were used for analyzing the polymorphism of129amino acid and mutation of PRNP gene.One difinited,63probable and29possible sporadic CJD patients were identified. Additionally,5FFI cases,8genetic CJD cases including one V1801, E196A and R208H, two E200K and three T188K, were definitely diagnosed. No time-geographic-or occupational-related events were observed among these cases. The median age of probably and possibly diagnosed sCJD was59(39,79) and53(36,84), respectively. The male to female ratio of the probable CJD patients was0.91:1while that of the possible CJD patients was1.41:1. Rapidly progressive dementia was the main foremost symptom, presenting in46.74%of the CJD patients. Probable CJD patients showed more clinical manifestations than that of the possible ones. The patients with positive results in the examinationts of CSF14-3-3, EEG and MRI usually displayed more typical clinical manifestations.In all, among the reported suspected CJD cases in2012, sCJD cases are predominant. The general features of sCJD cases in2012are consistent with that in our previous surveilance data. Additioanlly,13genetic human prion disease are diagnosed. FFI and T188K gCJD are the most commonly identified genetic prion diseases in Han Chinese.