title: Review: Laboratory diagnosis and surveillance of Creutzfeldt-Jakob disease.
abstract: Creutzfeldt-Jakob disease (CJD) is a representative human transmissible spongiform encephalopathy associated with central nervous system degeneration. Prions, the causative agents of CJD, are composed of misfolded prion proteins and are able to self-replicate. While CJD is a rare disease affecting only 1-1.5 people per million worldwide annually, it has attracted both scientific and public attention as a threatening disease since an epidemic of variant CJD (vCJD) cases appeared in the mid-1990s. Due to its unconventional transmission and invariable fatality, CJD poses a serious risk to public health. The hundreds of sporadic, genetic, and iatrogenic CJD cases as well as potential zoonotic transmission suggest that CJD is an ongoing concern for the field of medicine. Nevertheless, treatment aimed at clinical prevention and treatment that reverses the course of disease does not exist currently. Active surveillance and effective laboratory diagnosis of CJD are, therefore, critical. In this report, the surveillance systems and laboratory tests used currently to diagnose CJD in different countries are reviewed. The current efforts to improve surveillance and diagnosis for CJD using molecular and biochemical findings are also described.
authors: Division of Zoonoses, Center for Immunology & Pathology, National Institute of Health, Korea Centers for Diseases Control & Prevention, Chungcheongbuk-do, Republic of Korea.
authors: Jeongmin
authors: J
authors: Lee
authors: Jae Wook
authors: JW
authors: Hyeon
authors: Su Yeon
authors: SY
authors: Kim
authors: Kyu-Jam
authors: KJ
authors: Hwang
authors: Young Ran
authors: YR
authors: Ju
authors: Chongsuk
authors: C
authors: Ryou
mesh_terms: D019411:Clinical Laboratory Techniques / Q000379:methods*; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000453:epidemiology*; D062665:Epidemiological Monitoring; D006801:Humans
keywords: Creutzfeldt-Jakob disease; diagnosis; prion disease; surveillance; transmissible spongiform encephalopathy