Prospective 25-year surveillance of prion diseases in France, 1992 to 2016: a slow waning of epidemics and an increase in observed sporadic forms.

BackgroundPrion diseases are rare, fatal disorders that have repeatedly raised public health concerns since the early 1990s. An active prion disease surveillance network providing national level data was implemented in France in 1992.AimWe aimed to describe the epidemiology of sporadic, genetic and infectious forms of prion diseases in France since surveillance implementation.MethodsWe included all suspected cases notified from January 1992 to December 2016, and cases who died during the period with a definite or probable prion disease diagnosis according to EuroCJD criteria. Demographic, clinical, genetic, neuropathological and biochemical data were collected.ResultsIn total, 25,676 suspected cases were notified and 2,907 were diagnosed as prion diseases, including 2,510 (86%) with sporadic Creutzfeldt-Jakob disease (sCJD), 240 (8%) genetic and 157 (6%) with infectious prion disease. Suspected cases and sCJD cases increased over time. Younger sCJD patients (≤ 50 years) showed phenotypes related to a distinct molecular subtype distribution vs those above 50 years. Compared to other European countries, France has had a higher number of cases with iatrogenic CJD after growth hormone treatment and variant CJD (vCJD) linked to bovine spongiform encephalopathy (second after the United Kingdom), but numbers slowly decreased over time.ConclusionWe observed a decrease of CJD infectious forms, demonstrating the effectiveness of measures to limit human exposure to exogenous prions. However, active surveillance is needed regarding uncertainties about future occurrences of vCJD, possible zoonotic potential of chronic wasting diseases in cervids and increasing trends of sCJD observed in France and other countries.

Drivers of Routine and Outbreak Vaccination Uptake in the Western Democratic Republic of Congo: An Exploratory Study in Ten Health Zones.

We performed a cross-sectional survey on vaccination-related knowledge, attitudes, and practices (KAP) among randomly selected parents of <5 years-old children, elderly populations (aged ≥ 55 years), and health care workers (HCWs) in 10 health zones from 4 provinces of the Democratic Republic of Congo (DRC). Questionnaires targeted both routine (BCG, measles, polio) and outbreak-related (cholera, Ebola, COVID-19) vaccinations. In total, 2751 participants were included, 1165 parents, 1040 elderly, and 546 HCWs. In general, KAP expressed were supportive of vaccination uptake, although concerns regarding side effects and feelings of being insufficiently informed were more prevalent among parents and the elderly. Vaccine acceptance was lower for outbreak vaccinations (57%) than for routine vaccinations (90%). HCWs expressed the highest vaccine acceptance. Problems with the vaccine supply chain were reported by 20% of respondents. Despite a high level of positive KAP towards vaccination, parents and the elderly expressed a need to be better informed and had concerns regarding vaccine side-effects. A high acceptance for routine vaccinations was reported by participants, but somewhat less for outbreak vaccinations. In conclusion, HCWs in the communities could play a key role in the increased uptake of routine vaccinations and in optimizing uptake during outbreaks, provided that the supply chain is functioning well.