Prion disease is an infectious disease of the central nervous system caused by misfolding of the prion protein, mainly due to pathogen infection. Prion disease is a fatal neurodegenerative disease caused by a pathogen, prion protein, which lacks nucleic acid but is infectious, and binds to the normal prion protein, resulting in the development of pathogenic prions and the formation of the disease. There is no effective treatment for prion disease that can slow down the progression of the disease, but after diagnosis, it is still necessary to actively cooperate with doctors to help control symptoms, improve quality of life, prolong survival, and pay attention to regular follow-up during the treatment period, in order to adjust the treatment program.