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.Electronic supplementary materialThe online version of this article (doi:10.1186/s40249-016-0143-8) contains supplementary material, which is available to authorized users.

Clinical and laboratory features of 14 young Chinese probable sCJD patients

“…1 About 85% of all CJD cases are sporadic (sporadic CJD, sCJD), 10-15% cases are inherited (genetic CJD, gCJD) and less than 1% is infectious (iatrogenic CJD, iCJD). [2][3][4] The majority of sCJD patients develop clinical symptoms at middle-aged and old-aged period, with the incident peak at the age group of 60-69 year-old worldwide. 4,5 Young sCJD patients under 40 year-old are extremely rare.…”

Current Infection Control Recommendations for Prion Disease; A Difficult Problem for the Deployed Personnel of the Armed Forces

“…Prion diseases, such as Creutzfeldt-Jacob Disease offers a unique infection control problem because of their resistance to conventional physical (radiation), chemical, enzymatic, and thermal decontamination methods and maintain their stability in the environment for years (22). With a rise in interest from the scientific and public health sects, the potential public health concern around CJD is no longer limited to hospital settings (5).…”

“…Long incubation periods make it hard to predict an epidemic curve (5). Figure 1 displays a geographic distribution of human prion cases globally.…”

“…The United States department of defense currently has a program in place, the national prion research project (NPRP), which has established a research effort to accelerate the capabilities to prevent or ameliorate prion disease (5). The global health and economic catastrophes caused by prion disease have preemptively triggered attempts to avoid the widespread dissemination of the disease among animals, especially those ingested by humans.…”

Quality evaluation for the surveillance system of human prion diseases in China based on the data from 2010 to 2016

“…In the last decade of the 20 th century, countries in Europe, North America, as well as Australia and Japan started and restarted national surveillance for human PrDs, 1,[4][5][6][7][8][9][10][11][12] because of the outbreak of bovine spongiform encephalopathy (BSE) in the middle of 1980s and emergence of variant CJD (vCJD) 10 y late. Later, some other countries and regions in South America and Asia began their national or regional CJD surveillance, gradually forming global network for human PrDs.…”