title: Human prion diseases: epidemiology and integrated risk assessment.
abstract: Human prion diseases are devastating and incurable, but are very rare. Fears that the bovine spongiform encephalopathy epizootic would lead to a large epidemic of its presumed human counterpart, variant Creutzfeldt-Jakob disease (vCJD), have not been realised. Yet a feeling of uncertainty prevails in the general public and in the biomedical world. The lack of data on the prevalence of asymptomatic carriers of vCJD compounds this uncertainty. In addition to this problem, Switzerland is currently faced with another issue of major public concern: a recent rise in the incidence of CJD. Here we examine the plausibility of several scenarios that may account for the increase in CJD incidence, including ascertainment bias due to improved reporting of CJD, iatrogenic transmission, and transmission of a prion zoonosis. In addition, we present the design and current status of a Swiss population-wide study of subclinical vCJD prevalence.
authors.affiliation: Institute of Neuropathology and National Reference Center for Prion Diseases, Switzerland.
authors.forename: Markus
authors.initials: M
authors.lastname: Glatzel
authors.forename: Peter M
authors.initials: PM
authors.lastname: Ott
authors.forename: Thomas
authors.initials: T
authors.lastname: Linder
authors.forename: Jan O
authors.initials: JO
authors.lastname: Gebbers
authors.forename: Arnold
authors.initials: A
authors.lastname: Gmür
authors.forename: Werner
authors.initials: W
authors.lastname: Wüst
authors.forename: Gerhard
authors.initials: G
authors.lastname: Huber
authors.forename: Holger
authors.initials: H
authors.lastname: Moch
authors.forename: Michael
authors.initials: M
authors.lastname: Podvinec
authors.forename: Bernhard
authors.initials: B
authors.lastname: Stamm
authors.forename: Adriano
authors.initials: A
authors.lastname: Aguzzi
mesh_terms: D007562:Creutzfeldt-Jakob Syndrome / Q000453:epidemiology* / Q000209:etiology; D006801:Humans; D017096:Prion Diseases / Q000453:epidemiology / Q000209:etiology; D018570:Risk Assessment; D013557:Switzerland / Q000453:epidemiology