Chapter 93 – Creutzfeldt-Jakob Disease and Other Prion Diseases

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Carlene A. Muto, MD, MS
Medical Director of Infection Control and Hospital Epidemiology
University of Pittsburgh Medical Center (UPMC), Presbyterian
Medical Director for Infection Control
Center for Quality Improvement and Innovation, UPMC Health System
Associate Professor of Epidemiology and Medicine
Division of Infectious Diseases
University of Pittsburgh School of Medicine
Pittsburgh, Pennsylvania

Marian Pokrywka, MS, CIC
Infection Control Practitioner
Division of Infection Control and Hospital Epidemiology
University of Pittsburgh Medical Center
Pittsburgh, Pennsylvania

APIC recognizes and appreciates the contributions made to this chapter by prior authors.

ABSTRACT

Creutzfeldt-Jakob disease (CJD) 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. CJD 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. CJD 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.

Last Revised: 12/13/09 11:28 PM

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