Prevention of transmission among individuals and development of treatment in prion diseases

Abstract

Prion diseases are characterized by spongiform changes and scrapie form of prion protein (PrP^Sc) in the brain, and can transmit among individuals. PrP^Sc is infectious agent of prion diseases, which is difficult to decontaminate with general sterilization methods for bacteria or viruses. Furthermore, because PrP^Sc shows some resistance against treatments with proteolytic enzymes and protein denaturants, it is difficult to completely prevent transmissions of prion diseases among individuals in the clinical situation. So it is important to diagnose the patients as having prion diseases in the early phase of the disease correctly, and we should use single use devices in the high risk medical procedures, such as neurosurgeries, for the patients with prion diseases. In addition, if re–use instruments are used in the high–risk medical procedures for the patients with prion diseases, it is strongly recommended that sterilization methods of the instruments should follow the ‘Guideline for prevention of transmission in prion diseases 2020’. So far, several clinical trials using quinacrine or doxycycline have been conducted, but all of them resulted in failure. Some drugs have been reported to be able to modify the pathogenesis of prion diseases under experimental settings, and we are now preparing for future clinical trials for prion diseases.