Prion disease is a fatal brain disease manifested through failure of muscle control and dementia. Forms of this disease have been discovered in deer and elk (chronic wasting disease), in cows (bovine spongiform encephalopathy BSE or "mad cow disease") and in sheep (scrapie strain).
Prion disease in humans (Creutzfeldt-Jakob disease) is extremely rare and is most likely transmitted through exposure to BSE-infected beef or through a surgically acquired infection. (From 1995 through June 2002, a total of 124 human cases of CJD were reported in the United Kingdom, six in France, and one case each in Italy, Ireland, and the United States.)
With the recent major outbreak of chronic wasting disease among deer and elk populations in the mid- and Western United States, there may be an increased risk of transmission to hunters. There is no effective form of therapy for prion disease.
Two approaches were used in this study to evaluate the therapeutic potential of vaccination in mice infected with prion disease: passive immunization, where weekly antibody injections were administered immediately after and for one month following exposure to scrapie strain; and active immunization via an oral route prior to scrapie strain exposure.
"Our studies show that stimulation of the immune system is an important therapeutic target for the prion disease, as well as for other neurodegenerative illnesses characterized by abnormal protein conformations, such as Alzheimer's disease," concluded study author Thomas Wisniewski, MD.
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Contact: Marilee Reu
mreu@aan.com
651-695-2789
American Academy of Neurology
31-Mar-2003