Prion Diseases of Human: A Review

Soundarya S, Usha V, Bhuvana Balaji


Prion diseases are unique group of neurodegenerative diseases including the transmissible spongiform encephalopathies associated with a unique class of infectious proteins. In humans, these diseases include Kuru, Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia and in animals, are scrapie and bovine spongiform encephalopathy (mad cow disease). Awareness about the transmission of these diseases during dental care is very low as the knowledge of such existence is indeed rare. Despite the extensive research in this field based on the proposed pathogenesis the diagnosis and management of the disease requires still further studies. Prion diseases have been clouded by mystery since the description of scrapie, human TSEs and elucidation of the prion hypothesis. The risk of transmission of prions through dental procedures draws our attention towards the need to maintain optimal standards of infection control and decontamination procedures for all infectious agents, especially prions. Hence this review is presented here to throw the light on the this rare infection.


Prions; Prion Disease; Mink Encephalopathy Virus; Prion Proteins; PrPSc Proteins; Viral Proteins; Neurodegenerative Disease.

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