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A Retrospective Cohort Study of a Newly Proposed Criteria for Sporadic Creutzfeldt-Jakob Disease.
Diagnostics (Basel)
October 2024
Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8523, Japan.
Background/objectives: Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal neurodegenerative disorder traditionally diagnosed based on the World Health Organization (WHO) criteria in 1998. Recently, Hermann et al. proposed updated diagnostic criteria incorporating advanced biomarkers to enhance early detection of sCJD.
View Article and Find Full Text PDFRevision and update of the position paper on the management of notifications of donors with Creutzfeldt-Jakob disease in Italy.
Blood Transfus
September 2024
National Blood Centre, Italian National Institute of Health, Rome (ISS), Rome, Italy.
Updated global epidemiology atlas of human prion diseases.
Front Public Health
June 2024
National Key-Laboratory of Intelligent Tracking and Forecasting for Infectious Disease, National Health Commission Key Laboratory of Medical Virology and Viral Diseases, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing, China.
Article Synopsis
- Human prion diseases (PrD) are fatal, transmissible neurodegenerative conditions, including CJD and vCJD, that have raised global health concerns, especially following the BSE outbreak.
- A comprehensive analysis from 1993 to 2020 of PrD case numbers was conducted using data from various international surveillance programs, revealing a growing trend in reported cases worldwide.
- The findings showed over 27,000 PrD cases, predominantly in higher-income countries, highlighting a lack of surveillance in many low-income nations, suggesting underreporting of these diseases globally.
[Clinical characteristics and diagnostics of human spongiform encephalopathies: an update].
Nervenarzt
April 2024
Klinik für Neurologie, Universitätsmedizin Göttingen, Robert-Koch-Straße 40, 37075, Göttingen, Deutschland.
Human spongiform encephalopathies are rare transmissible neurodegenerative diseases of the brain and the nervous system that are caused by misfolding of the physiological prion protein into a pathological form and its deposition in the central nervous system (CNS). Prion diseases include Creutzfeldt-Jakob disease (CJD, sporadic or familial), Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal familial insomnia (FFI). Prion diseases can be differentiated into three etiological categories: spontaneous (sporadic CJD), inherited (familial CJD, FFI, and GSS) and acquired (variant CJD and iatrogenic CJD).
View Article and Find Full Text PDFThe Role of PET Imaging in Patients with Prion Disease: A Literature Review.
Mol Imaging Biol
April 2024
Dipartimento Di Diagnostica Per Immagini, Radioterapia Oncologica ed Ematologia, UOC Di Medicina Nucleare, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Largo Agostino Gemelli, 8, 00168, Rome, Italy.
Prion diseases are rare, rapidly progressive, and fatal incurable degenerative brain disorders caused by the misfolding of a normal protein called PrPC into an abnormal protein called PrPSc. Their highly variable clinical presentation mimics various degenerative and non-degenerative brain disorders, making diagnosis a significant challenge for neurologists. Currently, definitive diagnosis relies on post-mortem examination of nervous tissue to detect the pathogenic prion protein.
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