Creutzfeldt-Jakob Disease and Other Prion Diseases

Beth Ann Kavanagh, MT(ASCP), MS, MBA, CIC Director, Infection Control and Prevention

University Health System
San Antonio, TX

October 2, 2014


Creutzfeldt-Jakob disease is one of several neurologically degenerative diseases caused by a group of protein particles that are infectious by nature of their ability to replicate in the central nervous system and interrupt crucial neuron functioning. Creutzfeldt-Jakob disease and other prion diseases were originally called slow virus diseases because of their prolonged incubation period and initial designation as a viral condition. In the 1980s, it was proposed that the etiology of these neurodegenerative diseases was not viral in nature but due to an "infectious" protein particle that was transmissible through infected tissue and could be distinguished from any other infectious agent by a crucial lack of nucleic acid material. Creutzfeldt-Jakob disease and other prion diseases with demonstrated transmissibility remain a concern for the healthcare community because of their inherent resistance to traditional disinfection/sterilization methods and devastating clinical outcomes. Prevention measures in hospitals include Standard Precautions for general patient care and adherence to extensive chemical or increased temperature sterilization in the confines of the operating room. Prion diseases represent the frontier for infectious disease research.