scholarly journals Analysis of 22 years of surveillance for prion diseases in Slovenia, 1996 to 2017

2018 ◽  
Vol 57 (4) ◽  
pp. 227-233
Author(s):  
Nuška čakš Jager ◽  
Mara Popović ◽  
Mateja Blaško Markič ◽  
Alenka Kraigher
Keyword(s):  
Public Health ◽  
Prion Diseases ◽  
Interdisciplinary Approach ◽  
Diagnostic Value ◽  
Incidence Rates ◽  
Effective Response ◽  
Jakob Disease ◽  
Reporting Data ◽  
Sporadic Cjd ◽  
Completeness Of Reporting

Abstract Introduction The objective was to present the results of surveillance of prion diseases in Slovenia that was established in 1996 and then to assess the interdisciplinary approach according to the algorithm of case management and reporting data to the National Register at the National Institute of Public Health. Methods A descriptive study of Creutzfeldt-Jakob disease (CJD) recorded in the period from 1996 to 2017 was carried out. Results A total of 123 cases of prion disease were notified between 1996 and 2017. Out of these, 68 were recorded and confirmed by autopsy as sporadic CJD with an average incidence rate of 1,5 cases per million population per year. In one case a gene analysis showed mutation E200K in prion protein gene, PRNP. Two cases of the Gerstman-Sträussler Scheinker syndrome and one clinical case of fatal insomnia with new PRNP mutation, N181S, were notified. Diagnostic value of protein 14-3-3 analysis in the liquor reached 82% sensitivity and 71% specificity. 25 cases of notified clinically possible/probable CJD were disproved after autopsy. In eleven notified possible CJD cases the autopsy had not been performed. Variant CJD has not yet been proven in Slovenia. Conclusion Incidence rates were comparable with other European countries. Completeness of reporting and proper management of CJD cases according to the algorithm of reporting, management and case confirmation would need some improvement. A well-functioning surveillance system, including timely notifications, would enable an appropriate epidemiological investigation and an effective response to public health risks, thus the awareness of prion diseases should not decline.

scholarly journals Rapid and Sensitive RT-QuIC Detection of Human Creutzfeldt-Jakob Disease Using Cerebrospinal Fluid

mBio ◽  
2015 ◽  
Vol 6 (1) ◽  
Author(s):  
Christina D. Orrú ◽  
Bradley R. Groveman ◽  
Andrew G. Hughson ◽  
Gianluigi Zanusso ◽  
Michael B. Coulthart ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Protein Misfolding ◽  
Prion Diseases ◽  
Analytical Sensitivity ◽  
Enhanced Sensitivity ◽  
Highly Sensitive ◽  
Jakob Disease ◽  
Misfolding Diseases ◽  
Amyloid Diseases ◽  
Sporadic Cjd

ABSTRACT  Fast, definitive diagnosis of Creutzfeldt-Jakob disease (CJD) is important in assessing patient care options and transmission risks. Real-time quaking-induced conversion (RT-QuIC) assays of cerebrospinal fluid (CSF) and nasal-brushing specimens are valuable in distinguishing CJD from non-CJD conditions but have required 2.5 to 5 days. Here, an improved RT-QuIC assay is described which identified positive CSF samples within 4 to 14 h with better analytical sensitivity. Moreover, analysis of 11 CJD patients demonstrated that while 7 were RT-QuIC positive using the previous conditions, 10 were positive using the new assay. In these and further analyses, a total of 46 of 48 CSF samples from sporadic CJD patients were positive, while all 39 non-CJD patients were negative, giving 95.8% diagnostic sensitivity and 100% specificity. This second-generation RT-QuIC assay markedly improved the speed and sensitivity of detecting prion seeds in CSF specimens from CJD patients. This should enhance prospects for rapid and accurate ante mortem CJD diagnosis. IMPORTANCE A long-standing problem in dealing with various neurodegenerative protein misfolding diseases is early and accurate diagnosis. This issue is particularly important with human prion diseases, such as CJD, because prions are deadly, transmissible, and unusually resistant to decontamination. The recently developed RT-QuIC test allows for highly sensitive and specific detection of CJD in human cerebrospinal fluid and is being broadly implemented as a key diagnostic tool. However, as currently applied, RT-QuIC takes 2.5 to 5 days and misses 11 to 23% of CJD cases. Now, we have markedly improved RT-QuIC analysis of human CSF such that CJD and non-CJD patients can be discriminated in a matter of hours rather than days with enhanced sensitivity. These improvements should allow for much faster, more accurate, and practical testing for CJD. In broader terms, our study provides a prototype for tests for misfolded protein aggregates that cause many important amyloid diseases, such as Alzheimer’s, Parkinson’s, and tauopathies.

scholarly journals Prospective Study Demonstrates Utility of EP-QuIC in Creutzfeldt–Jakob Disease Diagnoses

2020 ◽  
pp. 1-3
Author(s):  
Sharon L. R. Simon ◽  
Anne Peterson ◽  
Clark Phillipson ◽  
Jonathan M. Walker ◽  
Meika Richmond ◽  
...  
Keyword(s):  
Public Health ◽  
Cerebrospinal Fluid ◽  
Prospective Study ◽  
Prion Diseases ◽  
Health Agency ◽  
Surrogate Markers ◽  
Predictive Values ◽  
The Public ◽  
End Point ◽  
Jakob Disease

ABSTRACT: Prospectively acquired Canadian cerebrospinal fluid samples were used to assess the performance characteristics of three ante-mortem tests commonly used to support diagnoses of Creutzfeldt–Jakob disease. The utility of the end-point quaking-induced conversion assay as a test for Creutzfeldt–Jakob disease diagnoses was compared to that of immunoassays designed to detect increased amounts of the surrogate markers 14-3-3γ and hTau. The positive predictive values of the end-point quaking-induced conversion, 14-3-3γ, and hTau tests conducted at the Prion Diseases Section of the Public Health Agency of Canada were 96%, 68%, and 66%, respectively.

Sporadic Creutzfeldt-Jakob disease in older people

2009 ◽  
Vol 19 (3) ◽  
pp. 207-215
Author(s):  
N Shah ◽  
A Agbobu ◽  
I Costello ◽  
A Beri ◽  
K Minhas
Keyword(s):  
Older People ◽  
Age Of Onset ◽  
Prion Proteins ◽  
Prion Diseases ◽  
Human Prion Disease ◽  
Degenerative Disorders ◽  
Abnormal Accumulation ◽  
Jakob Disease ◽  
The Uk ◽  
Sporadic Cjd

SummaryPrion diseases are rare degenerative disorders of the nervous system caused by abnormal accumulation and/or metabolism of prion proteins. They are invariably fatal. Creutzfeldt-Jakob disease (CJD) is the most common human prion disease and the sporadic form accounts for 85% of cases, whilst about 15% are genetic and 1% is iatrogenic. Variant CJD (vCJD) was mostly seen in the UK and France and mainly affected young people. Sporadic CJD (sCJD) occurs throughout the world with mean age of onset in the seventh decade. It typically presents with rapidly declining cognition that may get confused with other forms of dementia. Thus identification of sCJD in older people depends on a judicious awareness of the clinical features. Here we present a clinical, pathological, therapeutic and diagnostic review of sCJD.

scholarly journals A field on fire: The biochemistry of mad cows

2005 ◽  
Vol 27 (4) ◽  
pp. 6-8
Author(s):  
David R. Brown
Keyword(s):  
Bovine Spongiform Encephalopathy ◽  
Prion Protein ◽  
Prion Disease ◽  
Prion Diseases ◽  
Human Diseases ◽  
Spongiform Encephalopathy ◽  
Related Disease ◽  
Jakob Disease ◽  
Sporadic Cjd

Prion diseases are neurodegenerative diseases1 that have been linked together because they may potentially have the same cause. These include the diseases scrapie of sheep and BSE (bovine spongiform encephalopathy) of cattle, and also several human diseases that include sporadic CJD (Creutzfeldt-Jakob) disease and a variety of inherited forms. The inherited forms of prion diseases are linked to mutations within the gene for the prion protein. Around 85% of all human cases of prion disease are sporadic CJD, which is a disease affecting people of around 60 years of age. The cause of this disease remains unknown. Unfortunately, the name of this disease causes some confusion, as it is similar to vCJD (variant CJD), a related disease of much younger people.

scholarly journals Further Characterization of Glycoform-Selective Prions of Variably Protease-Sensitive Prionopathy

Pathogens ◽  
2021 ◽  
Vol 10 (5) ◽  
pp. 513
Author(s):  
Weiguanliu Zhang ◽  
Xiangzhu Xiao ◽  
Mingxuan Ding ◽  
Jue Yuan ◽  
Aaron Foutz ◽  
...  
Keyword(s):  
Conformational Transition ◽  
Prion Diseases ◽  
Lag Time ◽  
Biological Properties ◽  
Proteinase K ◽  
Step Process ◽  
Altered Glycosylation ◽  
Jakob Disease ◽  
Sporadic Cjd

Prion is an infectious protein (PrPSc) that is derived from a cellular glycoprotein (PrPC) through a conformational transition and associated with a group of prion diseases in animals and humans. Characterization of proteinase K (PK)-resistant PrPSc by western blotting has been critical to diagnosis and understanding of prion diseases including Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker (GSS) disease in humans. However, formation as well as biochemical and biological properties of the glycoform-selective PrPSc in variably protease-sensitive prionopathy (VPSPr) remain poorly understood. Here we reveal that formation of the ladder-like PrPSc in VPSPr is a PK-dependent two-step process, which is enhanced by basic pH. Two sets of PrPSc fragments can be identified with antibodies directed against an intermediate or a C-terminal domain of the protein. Moreover, antibodies directed against specific PrP glycoforms reveal faster electrophoretic migrations of PrP fragments mono-glycosylated at residue 181 and 197 in VPSPr than those in sporadic CJD (sCJD). Finally, RT-QuIC assay indicates that PrPSc-seeding activity is lower and its lag time is longer in VPSPr than in sCJD. Our results suggest that the glycoform-selective PrPSc in VPSPr is associated with altered glycosylation, resulting in different PK-truncation and aggregation seeding activity compared to PrPSc in sCJD.

scholarly journals Bank vole prion protein extends the use of RT-QuIC assays to detect prions in a range of inherited prion diseases

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Tze How Mok ◽  
Akin Nihat ◽  
Connie Luk ◽  
Danielle Sequeira ◽  
Mark Batchelor ◽  
...  
Keyword(s):  
Prion Protein ◽  
Bank Vole ◽  
Prion Diseases ◽  
Post Mortem ◽  
Prion Strains ◽  
Wide Range ◽  
Jakob Disease ◽  
Recombinant Prion Protein ◽  
First Time ◽  
Sporadic Cjd

AbstractThe cerebrospinal fluid (CSF) real-time quaking-induced conversion assay (RT-QuIC) is an ultrasensitive prion amyloid seeding assay for diagnosis of sporadic Creutzfeldt–Jakob disease (CJD) but several prion strains remain unexplored or resistant to conversion with commonly used recombinant prion protein (rPrP) substrates. Here, bank vole (BV) rPrP was used to study seeding by a wide range of archived post-mortem human CSF samples from cases of sporadic, acquired and various inherited prion diseases in high throughput 384-well format. BV rPrP substrate yielded positive reactions in 70/79 cases of sporadic CJD [Sensitivity 88.6% (95% CI 79.5–94.7%)], 1/2 variant CJD samples, and 9/20 samples from various inherited prion diseases; 5/57 non-prion disease control CSFs had positive reactions, yielding an overall specificity of 91.2% (95% CI 80.1–97.1%). Despite limitations of using post-mortem samples and our results’ discrepancy with other studies, we demonstrated for the first time that BV rPrP is susceptible to conversion by human CSF samples containing certain prion strains not previously responsive in conventional rPrPs, thus justifying further optimisation for wider diagnostic and prognostic use.

Dealing with COVID-19: lessons learned from the Italian experience

Coronaviruses ◽  
2020 ◽  
Vol 01 ◽  
Author(s):  
Silvana Mirella Aliberti ◽  
Francesco De Caro ◽  
Giovanni Boccia ◽  
Rosario Caruso ◽  
Mario Capunzo
Keyword(s):  
Public Health ◽  
Risk Assessment ◽  
Lessons Learned ◽  
Coherent System ◽  
Effective Response ◽  
Medical Practitioners ◽  
Italian Experience ◽  
Rapid Spread ◽  
Public Health Officials ◽  
Future Plans

: Italy was the first western nation affected by the pandemic and was observed as a pilot case in the management of the new coronavirus epidemic. The outbreak of COVID-19 disease has been very difficult in Italy, on June 25, 2020 there are 239,821 total cases of which 33,592 deaths nationwide. Three lessons emerged from this experience that can serve as a blueprint to improve future plans for the outbreak of viruses. First, early reports on the spread of COVID-19 can help inform public health officials and medical practitioners in effort to combat its progression; second, inadequate risk assessment related to the urgency of the situation and limited reporting to the virus has led the rapid spread of COVID-19; third, an effective response to the virus had to be undertaken with coherent system of actions and simultaneously.

scholarly journals A Strong Seasonality Pattern for Covid-19 Incidence Rates Modulated by UV Radiation Levels

Viruses ◽  
2021 ◽  
Vol 13 (4) ◽  
pp. 574
Author(s):  
Christos Karapiperis ◽  
Panos Kouklis ◽  
Stelios Papastratos ◽  
Anastasia Chasapi ◽  
Antoine Danchin ◽  
...  
Keyword(s):  
Public Health ◽  
Uv Radiation ◽  
Initial Conditions ◽  
Face Mask ◽  
Incidence Rates ◽  
Personal Hygiene ◽  
Public Health Policies ◽  
Community Transmission ◽  
Nonpharmaceutical Interventions ◽  
Strong Seasonality

The Covid-19 pandemic has required nonpharmaceutical interventions, primarily physical distancing, personal hygiene and face mask use, to limit community transmission, irrespective of seasons. In fact, the seasonality attributes of this pandemic remain one of its biggest unknowns. Early studies based on past experience from respiratory diseases focused on temperature or humidity, with disappointing results. Our hypothesis that ultraviolet (UV) radiation levels might be a factor and a more appropriate parameter has emerged as an alternative to assess seasonality and exploit it for public health policies. Using geographical, socioeconomic and epidemiological criteria, we selected twelve North-equatorial-South countries with similar characteristics. We then obtained UV levels, mobility and Covid-19 daily incidence rates for nearly the entire 2020. Using machine learning, we demonstrated that UV radiation strongly associated with incidence rates, more so than mobility did, indicating that UV is a key seasonality indicator for Covid-19, irrespective of the initial conditions of the epidemic. Our findings can inform the implementation of public health emergency measures, partly based on seasons in the Northern and Southern Hemispheres, as the pandemic unfolds into 2021.

scholarly journals Fueling the Covid-19 pandemic: summer school holidays and incidence rates in German districts

2021 ◽  
Author(s):  
Thomas Plümper ◽  
Eric Neumayer
Keyword(s):  
Public Health ◽  
Fixed Effects ◽  
Summer School ◽  
Incidence Rates ◽  
Contact Tracing ◽  
Robert Koch Institute ◽  
Cross Sectional ◽  
Holiday Period ◽  
Health Authorities ◽  
Public Health Authorities

AbstractBackgroundThe Robert-Koch-Institute reports that during the summer holiday period a foreign country is stated as the most likely place of infection for an average of 27 and a maximum of 49% of new SARS-CoV-2 infections in Germany.MethodsCross-sectional study on observational data. In Germany, summer school holidays are coordinated between states and spread out over 13 weeks. Employing a dynamic model with district fixed effects, we analyze the association between these holidays and weekly incidence rates across 401 German districts.ResultsWe find effects of the holiday period of around 45% of the average district incidence rates in Germany during their respective final week of holidays and the 2 weeks after holidays end. Western states tend to experience stronger effects than Eastern states. We also find statistically significant interaction effects of school holidays with per capita taxable income and the share of foreign residents in a district’s population.ConclusionsOur results suggest that changed behavior during the holiday season accelerated the pandemic and made it considerably more difficult for public health authorities to contain the spread of the virus by means of contact tracing. Germany’s public health authorities did not prepare adequately for this acceleration.

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