Immunization may prove therapeutic for prion diseases

Researchers at NYU School of Medicine have found that immunization prolongs the incubation period for prion diseases such as Creutzfeldt-Jakob disease and may have therapeutic value for other neurodegenerative illness such as Alzheimer’s disease. 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).

From the American Academy of Neurology :
Immunization may prove therapeutic for prion diseases

Embargoed for release until 10 a.m. HT, Monday, March 31, 2003

HONOLULU, HI ? Researchers at NYU School of Medicine have found that immunization prolongs the incubation period for prion diseases such as Creutzfeldt-Jakob disease and may have therapeutic value for other neurodegenerative illness such as Alzheimer’s disease. Results of their study are being presented at the American Academy of Neurology Annual Meeting in Honolulu, March 29-April 5, 2003.

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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This study is supported by the National Institutes of Health and the Alzheimer’s Disease Association.

The American Academy of Neurology, an association of more than 18,000 neurologists and neuroscience professionals, is dedicated to improving patient care through education and research. For more information about the American Academy of Neurology, visit its online press room at http://www.aan.com/press/index.cfm.

Editor’s Notes: Dr. Wisniewski will present the research at the AAN’s 55th Annual Meeting in Honolulu, Hawaii, during a presentation at 2:15 p.m. on Wed., April 2, in Room 316B at the Hawaii Convention Center (HCC). He and Dr. Marcin Sadowski will be available to answer media questions during a briefing on Mon., March 31 at 10 a.m. in the AAN Press Room, Room 327 of the HCC.

All listed times are for Hawaiian-Aleutian Standard Time (HT).

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