The National Prion Disease Pathology Surveillance Center

Sponsored by the American Association of Neuropathologists (AANP)

Office: Advisory Committee:

Dr. Pierluigi Gambetti, Director Dr. Barbara Crain

Dr. Bernardino Ghetti, Co-Director Dr. Stephen DeArmond

Dr. Jiri Safar, Co-Director Dr. Daniel Perl

Dr. Wenquan Zou, Associate Director

Dr. Clive Hamlin, Director, Clinical Laboratory


July 2012

Dear Representative,

We respectfully ask for consideration of the appropriation of $ 5 M in FY13 for the Centers for Disease Control and Prevention (CDC) to continue the program for national surveillance of human prion diseases.

The Presidentís budget proposal for FY 2013 combines the prion program with that Chronic Fatigue Syndrome (CFS), a non-fatal, non-transmissible condition, and suggests a 25% cut in the appropriation over that of FY12. We ask that the prion program appropriation for FY13 be separated from that of the Chronic Fatigue Syndrome and that it be subject to no more than a 5-10% reduction. Any larger reduction in one FY would make the current surveillance incomplete rendering it essentially useless.

Discontinuing national surveillance on human prion diseases will have at least four major negative consequences: 1. The identification of U.S. cases in which the prion disease has been acquired from eating prion contaminated meat will become unlikely. Possible sources of prion infection are cattle as well as deer and elk affected by BSE (bovine spongiform encephalopathy or Mad Cow disease) and CWD (chronic wasting disease), respectively. Since the US is not conducting significant surveillance for BSE and CWD, the occurrence of a BSE or CWD epidemic may remain completely undetected.

2. Detection of cases of prion disease acquired from the use of prion-contaminated surgical instruments, tissue and organ transplants, blood transfusion and other medical procedures will also be jeopardized allowing potential spread of prion infection to remain unchecked.

3. The occurrences of clusters of cases of prion disease which point to a possible source of prion infection, which are often sensationalized by the media will not be definitively resolved possibly maintaining a state of alarm and eroding confidence in the governmentís public health protection and safety.

4. The U.S. would become the first industrialized country with no prion surveillance of any kind putting the country at a major disadvantage in the export of beef and beef products. Indeed the recent resumption of beef importation by the government of South Korea was largely predicated on the existence of effective human prion disease surveillance (see attachment).

CONCLUSION: Discontinuing or reducing human prion disease surveillance would not only eliminate an important safety net to US public health, but it would also jeopardize the export of US beef.


Pierluigi Gambetti, MD Florence Kranitz Professor and Director President National Prion Disease Pathology Surveillance Center CJD Foundation, Inc. Case Western Reserve University 3632 W. Market St., Ste 105N 2085 Adelbert Road, Room 419, Cleveland, OH 44106 Akron, Ohio 44333 Email: Phone: 330-665-5590

Wednesday, June 27, 2012

First US BSE Case Since 2006 Underscores Need for Vigilance

Neurology Today 21 June 2012


Tuesday, June 26, 2012

Creutzfeldt Jakob Disease Human TSE report update North America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012

type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the rise in Canada and the USA


previous USA PRION UNIT reports ;



Monday, July 23, 2012

The National Prion Disease Pathology Surveillance Center July 2012