The National Prion Disease Surveillance Center will receive funding of up to $20 million in a grant from the Centers for Disease Control and Prevention to continue research into the deadly neurodegenerative prion diseases in the brain.

Newswise – Cleveland – National Prion Disease Surveillance Center (NPDPSC) At Case Western Reserve University, you will receive funding of up to $20 million as part of a grant renewal from the Centers for Disease Control and Prevention (CDC) to understand how and why prion diseases–degenerative brain diseases found in both humans and animals–develop and spread.

The five-year grant period began this month and will allow the center to continue research and diagnostic activities related to prion diseases.

Prion diseases are different from other infectious diseases caused by viruses or bacteria. They develop when normal prion proteins in the brain change shape and clump together, causing damage to nerve cells. The damage results in tiny, sponge-like holes in brain tissue.

There is no known cure for these disorders, which rapidly accelerate death.

The first of its kind in the United States

The NPDPSC is the only center of its kind in the United States that coordinates autopsies and neuropathological examinations of suspected prion disease cases from around the country, said Brian Appleby, director of the NPDPSC and principal investigator at the center. Local autopsies are performed on site and other cases are performed elsewhere through a network of providers coordinated by the NPDPSC. The monitoring center was originally established in 1997 in the Department of Neurology at Case Western Reserve University by Pierluigi Gambetti.

The NPDPSC collects tissue samples and clinical information from cases of prion disease to monitor its occurrence and investigate potential cases in which these diseases have been acquired from humans or other animals, such as chronic wasting disease transmitted from deer to humans. The additional grant money will allow us to continue monitoring for the emergence of new human prion diseases.

The center also operates the nation’s clinical reference laboratory for prion disease and performs cerebrospinal fluid testing. CDC funding is critical to setting up brain tissue testing and using a relatively new diagnostic test called RT-QuIC (Real-Time Vibration-Induced Shift), which has revolutionized the way disease is diagnosed clinically. We are pleased to receive the renewal of this scholarship to continue this important research.”

Prion diseases unlike other brain diseases

Human prion diseases include Creutzfeldt-Jakob disease, Gerstmann-Straussler-Schenker syndrome, and fatal familial insomnia, among others. Animal prion diseases consist of bovine spongiform encephalopathy, known as “mad cow disease,” chronic wasting disease found in deer, and scrapie found in sheep and goats.

Symptoms of people’s prion diseases include memory loss, difficulty speaking and walking, and behavior changes — manifestations very similar to Alzheimer’s disease and other types of dementia, making the condition difficult to diagnose.

Prion diseases in humans can be transmitted through contaminated food or contact with infected medical equipment, inherited through mutations in the PRNP gene that codes for prion proteins, or intermittently when prion proteins fold abnormally without a known cause.

The most common form of prion disease in people, Creutzfeldt-Jakob disease, caused more than 500 deaths in 2020, according to the CDC.


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