Epidemiological characteristics of human prion diseases

Abstract

Human prion diseases are a group of transmissible, progressive, and invariably fatal neurodegenerative disorders, which include Kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome, and fatal familial insomnia. Human prion diseases affect approximately 1-2 persons per million worldwide annually, occurring in sporadic, inherited, and acquired forms. These diseases have attracted both scientific and public attention not only because of their mysterious pathogen, but also due to their considerable threat to public health since the emergence of the variant CJD.There are still no specific therapeutic and prophylactic interventions available for prion diseases, thus active surveillance of human prion diseases is critical for disease control and prevention. Since 1993, CJD surveillance systems have been established in many countries and regions, and several long-term multinational cooperative projects have been conducted.In this paper, the epidemiological characteristics of various human prion diseases and the active surveillance systems pertaining to them in different countries and regions are summarized and reviewed.

Keywords: Creutzfeldt-Jakob disease; Epidemiology; Prion disease; Surveillance.

Fig. 1

The mean mortality rates per million from sCJD according to the data of the CJD International Surveillance Network from 1993 to 2013. a Data from the member states of the CJD International Surveillance Network. The mortality rate of one per million is marked with a dotted line. b The mean mortality rates per million of all member states. Solid line represents the fitting trend

Fig. 2

The distribution ofdura mater graft-associated and hGH-related iCJD casesglobally

Fig. 3

Number of deaths from vCJD in the UK and outside of the UK from 1995 to 2015

Fig. 4

Countries (marked in gray) where active CJD surveillance is officially conducted under the framework of the CJD International Surveillance Network