Despite many sensational and intimidating reports in the mass media, transmissible spongiform encephalopathies (prion disease) are not contagious in the usual sense. Successful transmission requires both specific material (an affected individual's tissue, from or adjacent to CNS) and specific modes (mainly penetrating contact with the recipient). Nevertheless, specific safety precautions are mandatory to avoid accidental transmission and to decontaminate any infectivity. Autopsy is essential for definite diagnosis of these disorders. Recommendations are given here for performance of the autopsy, for neuropathology service and appropriate decontamination; they are based on the current literature and on precautions taken in most laboratories with experience in handling tissue from transmissible spongiform encephalopathies. In particular, special care must be taken to avoid penetrating wounds, possible contamination should be kept to a minimum, and potential infectious material must be adequately decontaminated by specific means.
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http://dx.doi.org/10.1111/j.1750-3639.1995.tb00609.x | DOI Listing |
Cureus
December 2024
Medicine, King Saud University Medical School, Riyadh, SAU.
Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and incurable neurodegenerative disorder caused by prions. It is invariably fatal and classified under transmissible spongiform encephalopathies. This case report presents a 66-year-old Saudi female who was admitted to the neurology department due to a rapidly advancing cognitive decline.
View Article and Find Full Text PDFAnimals (Basel)
December 2024
Korea Zoonosis Research Institute, Jeonbuk National University, Iksan 54531, Republic of Korea.
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. One of the candidate genes involved in prion diseases is the shadow of the prion protein () gene. Raccoon dogs, a canid, are considered to be a prion disease-resistant species.
View Article and Find Full Text PDFAnimals (Basel)
December 2024
Department of Epidemiological Sciences, Animal and Plant Health Agency, Addlestone KT15 3NB, UK.
Atypical scrapie (AS) is a transmissible spongiform encephalopathy (TSE) that affects sheep and goats. Low within-flock incidence suggests that AS is not transmissible between animals, and testing of all animals that exit positive flocks for two years following detection (i.e.
View Article and Find Full Text PDFiScience
December 2024
Université Paris-Saclay, INRAe, UVSQ, VIM, 78350 Jouy-en-Josas, France.
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are neurodegenerative disorders caused by the accumulation of misfolded conformers (PrP) of the cellular prion protein (PrP). During the pathogenesis, the PrP seeds disseminate in the central nervous system and convert PrP leading to the formation of insoluble assemblies. As for conventional infectious diseases, variations in the clinical manifestation define a specific prion strain which correspond to different PrP structures.
View Article and Find Full Text PDFRadiol Case Rep
February 2025
Schulich School of Medicine and Dentistry, University of Western Ontario, London, Ontario, Canada.
Creutzfeldt-Jakob Disease (CJD) is a rare, fatal neurodegenerative disorder that is caused by prion proteins. Patients often present with rapidly progressive dementia, ataxia, myoclonus, memory impairment, visual problems, and changes in personality. In this case report, we aimed to address the course of a 62 year old female who presented with progressive decline in cognitive function and died within 6 months of presentation.
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