Prion Disease Mismanaged

By John Fauber, Reporter, MedPage Today

When Tim Suroviak developed cognitive problems after coming home following heart surgery last year, one of the first questions doctors asked was whether he was a hunter. He was not, though his family did eat venison a few times year.

Suroviak, 63, deteriorated quickly from a rare brain condition known as Creutzfeldt-Jakob disease (CJD). The disease is in the same family as chronic wasting disease, which is endemic in Wisconsin’s deer herd. The Suroviaks live in Wausau, Wisconsin.

After his death, Suroviak’s brain was sent to a lab in Ohio, which led to some shocking news for the family: The type of CJD he had was especially rare, caused by a genetic defect.

prions disease and Alzheimer's disease and Creutzfeldt-Jakob disease

That discovery allowed family members to be tested for the disease. So far, no one, including his two daughters, carries the mutation, said Suroviak’s wife, Monica.

But the search for rare prion diseases, whether genetic, sporadic, or potentially caused by eating meat from infected animals, could be curtailed beginning next year.

The country’s prion disease surveillance center that looks for new brain disorders would lose all of its federal funding and cease operations under President Trump’s proposed fiscal 2018 budget.

For 20 years, the brains of people who died of suspected prion diseases, such as Creutzfeldt-Jakob disease, have been sent to the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland.

The center also is the only licensed lab that can test spinal fluid from living people who have suspected Creutzfeldt-Jakob disease. Such tests can allow families to find out if someone has the disease and what type it is, and then plan how to spend the person’s remaining months. The disease is always fatal.

The loss in funding would come at a time of heightened concern over whether chronic wasting disease can infect people. Recent research showed that it could infect monkeys that were fed venison from infected deer.

At the same time, the number of Creutzfeldt-Jakob cases has jumped substantially nationally and especially in Wisconsin, where chronic wasting disease has now been identified in wild deer in 18 counties. For now, officials attribute the increase to better surveillance and an aging population.

CWD and prions

“I don’t know why we want to stop surveillance at a time like this,” said Debbie Yobs, president of the Creutzfeldt-Jakob Disease Foundation. “It doesn’t seem logical.”

The foundation is a nonprofit organization that supports families affected by prion diseases, as well as raising awareness and supporting medical research.

For the Suroviaks, the lab provided peace of mind. First, it let the family know that Tim had a genetic form of the disease, which occurs in between 5 percent and 15 percent of cases. Overall, Creutzfeldt-Jakob disease occurs in about 1 to 1.5 one million people a year worldwide with the vast majority being the sporadic type, meaning the cause is unknown.

While no family members have tested positive for the disease, it turns out that two first cousins of Suroviak died of the disease, Monica Suroviak said.

“If the testing had not been done, we would have never known,” she said.

In 2002, the year CWD was discovered in Wisconsin, six cases of Creutzfeldt-Jakob disease were recorded in the state, according to the Department of Health Services. In two of the last four years, 13 cases have been recorded in the state. That’s a 117 percent increase.

Nationally, there also has been an increase in Creutzfeldt-Jakob cases. In 2002, there were 260 cases, compared with 481 in 2015, an 85 percent increase, according to data from the CDC.

mad cow disease

Creutzfeldt-Jakob is closely related to the form of mad cow disease that infected people, primarily in Great Britain, in the late 1990s and early 2000s, after they ate beef from infected cows. Indeed, human mad cow disease is known as variant Creutzfeldt-Jakob. Both diseases attack the brain, and death usually occurs within a year.

Chronic wasting disease now is in 21 states, primarily in the Upper Midwest and Rocky Mountain states.

Mark Zabel, a prion researcher at Colorado State University, said chronic wasting disease may be mutating and someday could infect people, if it hasn’t already.

“If you don’t have a prion surveillance center that looks for it, you are going to miss it,” he said. “Then, it probably is going to be too late.”

About 400 brains are sent to the prion surveillance center each year. Since 1997 it has contracted with the CDC to provide prion disease surveillance. It has developed a vast library of tissue samples that help show how prion diseases manifest and mutate in people.

Prion diseases are not static; they evolve, Jiri Safar, MD, director of the prion surveillance center and a professor of pathology and neurology at Case Western, said.

“We do not have evidence of a jump to humans, but that is the concern among many in the public health and research community,” he said. (Editor’s Note: There also is no evidence of a reliable species barrier.)

The federal government spends about $6 million a year supporting prion disease surveillance.

Christine Pearson, a spokesperson for the CDC, would not say whether it supported the budget cut.

“The President’s budget is an important part of the federal appropriation cycle; however, Congress will determine final funding levels for all federal agencies,” she said in an email.

Thanks to the Milwaukee Journal Sentinel and MedPage Today for the great report.

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Alzheimer's disease infectious and CJD infectious

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Gary Chandler is a prion expert. He is the CEO of Crossbow Communications, author of several books and producer of documentaries about health and environmental issues around the world. Chandler is connecting the dots to the global surge in neurodegenerative disease, including Alzheimer’s disease, Parkinson’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and other forms of prion disease. The scientific name for prion disease is transmissible spongiform encephalopathy. The operative word is “transmissible.” Even the global surge in autism appears to be related.