Additional file 1: of Epidemiological characteristics of human prion diseases

Multilingual abstracts in the six official working languages of the United Nations. (PDF 201 kb

Analysis of the compliance and the related influence factors in the follow-up process of surveillance for Creutzfeldt-Jakob disease in China

<div><p>Creutzfeldt-Jakob disease (CJD) is a kind of rare, rapidly progressive fatal central nervous system disorders. In China, the surveillance for CJD has started since 2006. As one of the major issues in CJD surveillance, 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 in China CDC, it is frequently encountered that some interviewed family members do not cooperate well during follow-up. To screen the possible factors influence on the compliances of the interviewees during CJD follow-up, 11 independent variables from patient aspect and 4 variables from interviewee aspect were selected and a questionnaire was prepared. Based on 199 suspected sporadic CJD cases reported to CJD surveillance center in 2013, 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, continual medical treatment after diagnosis, medical treatment mode, 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 provide for the first time the factors related with the compliances of the interviewed family members of the suspected CJD patients during follow-up process, which supplies useful clue for us to improve CJD follow-up process and increase the capacity of CJD surveillance.</p></div

Table_1_Different reactive profiles of calmodulin in the CSF samples of Chinese patients of four types of genetic prion diseases.DOCX

Background and purposeCalmodulin (CaM) levels exhibit significant elevation in the brain tissue of rodent and cell line models infected with prion, as well as in the cerebrospinal fluid (CSF) samples from patients diagnosed with sporadic Creutzfeldt-Jakob disease (sCJD). However, the status of CSF CaM in patients with genetic prion diseases (gPrDs) remains unclear. This study aims to assess the characteristics of CSF CaM in Chinese patients presenting four subtypes of gPrDs.MethodsA total of 103 CSF samples from patients diagnosed with T188K-gCJD, E200K-gCJD, D178N-FFI, P102L-GSS were included in this study, along with 40 CSF samples from patients with non-prion diseases (non-PrDs). The presence of CSF CaM and 14-3-3 proteins was assessed using Western blots analysis, while levels of CSF 14-3-3 and total tau were measured using enzyme-linked immunosorbent assays (ELISAs). Statistical methods including multivariate logistic regression were employed to evaluate the association between CSF CaM positivity and relevant clinical, laboratory, and genetic factors.ResultsThe positive rates of CSF CaM were significantly higher in cases of T188K-gCJD (77.1%), E200K-gCJD (86.0%), and P102-GSS (90.9%) compared to non-PrD cases (22.5%). In contrast, CSF CaM positivity was slightly elevated in D178N-FFI (34.3%). CSF CaM positivity was remarkably high in patients who tested positive for CSF 14-3-3 by Western blot and exhibited high levels of total tau (≥1400 pg/ml) as measures by ELISA. Multivariate logistic regression analysis confirmed a significant association between CSF CaM positivity and specific mutations in PRNP, as well as with CSF 14-3-3 positivity. Furthermore, the diagnostic performance of CaM surpassed that of 14-3-3 and tau when analyzing CSF samples from T188K-gCJD and E200K-gCJD patients.ConclusionWestern blot analysis reveals significant variations in the positivity of CSF CaM among the four genotypes of gPrD cases, demonstrating a positive correlation with 14-3-3 positivity and elevated tau levels in CSF.</p

The Kaplan-Meier survival curves for deceased patients.

<p>(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.</p

Clinical Examinations and the Median Survival Times (Months) of the Fatal Cases of Various Prion Diseases.

a<p>Periodic sharp curve complexes.</p>b<p>Electroencephalograms.</p>c<p>Magnatic Resonance Imaging releases high signal in caudate/putamen.</p

Sociodemographic Features and the Median Survival Times (Months) of the Fatal Cases of Various Prion Diseases.

<p>Abbreviations: sCJD = sporadic Creutzfeldt-Jakob disease; fCJD = familial CJD; GSS = Gerstmann-straussler-Sheinker syndrome; FFI = fatal familial insomnia.</p>a<p>Difference in median survival time among multiple-group were tested by using Breslow (Wilcoxon) method.</p>b<p>Difference in median survival time between two groups were tested by using log-rank (Mantel-Cox) method.</p

Additional file 2: Table S1. of Low activity of complement in the cerebrospinal fluid of the patients with various prion diseases

The relevant characteristics of CSF samples between the group of probable sCJD and non-CJD. (DOCX 24 kb

The presenting symptoms of the probable and possible sCJD patients.

1<p>including sleeping turbulence, depression, anxiety and stress etc.</p>2<p>when the dementia persists longer than one year progressively without other manifestations.</p

The geographic distribution of probable and possible sCJD cases based on the permanent residences.

<p>The numbers of probable and possible sCJD cases in each province were showed as X (X), respectively.</p

Additional file 4: Table S3. of Low activity of complement in the cerebrospinal fluid of the patients with various prion diseases

Analysis of CH50 values in CSF from various prion diseases according to the intervals from disease onset to sampling. (DOCX 20 kb