assumptions regarding the incubation period, individual susceptibility, and the level of exposure. The incubation period for another human prion disease, kuru, is 4 to 40 years (Huillard d'Aignaux et al., 2002).

The origin of vCJD in prion-infected cattle raises the concern that chronic wasting disease, a prion disease spreading among North American deer and elk (Williams and Miller, 2002), could cause disease in people who consume venison from the affected regions.

The European Commission has poured millions of euros into research to develop better diagnostics for TSEs, especially BSE, with modest success. Some commercial diagnostic tests for postmortem BSE detection have been developed and are used throughout the United Kingdom and Europe. The tests cannot detect prions present at low levels, however. The lack of highly sensitive, accurate, and rapid tests has led to controls such as categorical importation bans and massive culling of herds to ensure the safety of beef products.

To date the U.S. Food and Drug Administration (FDA) has not received a request from any of the European companies that manufacture BSE-screening tests to approve them for human use in the United States, nor has any company based in the United States submitted any TSE screening test to FDA for approval for human use (Personal communication, D.M.Asher, FDA, July 18, 2002). However, the U.S. Department of Agriculture's Center for Veterinary Biologics has approved the use of one test produced by Bio-Rad Laboratories for the detection of chronic wasting disease (CWD) in mule deer.

Congress Creates the National Prion Research Project

The economic and health consequences of BSE and vCJD in Europe and the risk that U.S. military forces stationed abroad and their dependents could contract a TSE through infected beef or contaminated blood products led the U.S. Congress to pass a law establishing the National Prion Research Project (NPRP) in 2002 (Senate Committee on Appropriations, 2001). NPRP will fund research on TSEs, with special emphasis on developing an antemortem diagnostic test.

Congress mandated that the U.S. Department of Defense (DOD) administer the new project, and the department delegated it to the Army's Medical Research and Material Command (MRMC). MRMC administers grants through a two-tiered process of external scientific peer review, followed by programmatic review by a multidisciplinary group of DOD and civilian experts called an integration panel.



The National Academies | 500 Fifth St. N.W. | Washington, D.C. 20001
Copyright © National Academy of Sciences. All rights reserved.
Terms of Use and Privacy Statement