Current Infection Control Recommendations for Prion Disease; A Difficult Problem for the Deployed Personnel of the Armed Forces

Abstract

Background: Kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome, and fatal familial insomnia are a group of transmissible, progressive, and ultimately lethal neurodegenerative disorders caused by prion diseases. Prion disease may present in sporadic, inherited, and acquired forms. Fewer new cases of CJD are presenting themselves compared to the 1990’s when a surge of cases was recognized after an extended incubation period following dura mater and growth hormones replacement up to 25 years before. Currently, prion disease affects approximately 1 - 2 persons per million worldwide annually. In recent years, however, variant CJD has been associated with transfusions, making the possibility of transmission through operative procedures, blood, and tissue donations a real concern. Since the emergence of variant CJD, prion disease has become a considerable public health threat, not only because of their mysterious pathogenesis, but the lack of treatment options, limited diagnostic availability, and emerging transmission routes. Active surveillance of human prion diseases is critical for disease control and prevention considering the limited options for therapeutic and prophylactic interventions. Due to the risk to public health, CJD surveillance systems are now established in many countries; especially developing countries and deployed military units where healthcare is poor and infection control measures in the field are difficult to maintain. Several long-term multinational cooperative projects are on-going as well as reporting systems which protect military families from purchasing bovine in endemic countries. Objective: This paper reviews the epidemiological characteristics of various human prion diseases, infection control issues, current disinfection processes as well as recommended processes, and active surveillance systems in different countries. Conclusions: Prion disease represents a continued public health concern due to its insidious nature. Current practice continues to advance finding better ways at diagnosing earlier. Treatment options are still far from being anything but palliative. Prevention remains the primary component to neutralizing the spread of non-inherited forms of prion disease.