Prion Disease

Abstract

Abstract Since the discovery of Prion Proteins (PrPs) in 1982, a new model for an “infectious agent” has been proposed that is substantially different from our understanding of disease transmission by other microorganisms. Prion proteins have been implicated in many different diseases. Various transmission modes have been discovered, including consumption of infected meat, blood transfusion, medical devices used in neurosurgery and corneal transplantation, environmental routes (such as soil), and airborne routes. Familial and sporadic instances of the disease have also been recognized, such as fatal familial insomnia. Prion disease harbors long incubation periods, and subclinical infectious states have been discovered in asymptomatic individuals owing to lymphoreticular accumulation of PrPs. The uniformly fatal outcome after symptom onset secondary to prion disease has prompted rigorous research and attempts at development of prophylactic immunomodulatory techniques, gene therapy, and clinical trials investigating various medications such as doxycycline, quinacrine, pentosan polysulfate, and flupirtine to improve the survival and outcome in patients. In this article, we extensively review the background of PrPs and Creutzfeldt-Jakob disease, the various transmission methods, and recent developments into discovering a treatment for Creutzfeldt-Jakob disease.