Dear Colleagues:

Often our Friday discussions are about Scary Stuff. Sometimes the topics are scary to some people but not others, such as the current Supreme Court. Sometimes they are about stuff that is scary to lots and lots of people, like the war in Ukraine. Sometimes it’s hard to figure out how scared to be, as with Generative AI, because the scary stuff may have some upside.

This week’s discussion is about something that is awfully darn scary, with absolutely no upside – prion diseases. The good news is, prion diseases in humans are quite rare and do not spread easily. The bad news is, they are out there and, as the CDC website describes:

Aside from being invariably fatal, prion diseases are scary because, while rare, they seem to have a range of variants. They were first observed, though only by symptoms and not with understanding of the mechanism, over a hundred years ago with the identification of Creutzfeldt-Jakob disease (CJD) by German neuropathologists. Worldwide there is about one CJD case per million people per year, concentrated at older ages (“mean onset age of 62”). CJD, however, is just one of “a group of transmissible spongiform encephalopathies affecting people worldwide”, and that term also suggests another reason for fear: it involves, in the WHO’s words, “characteristic spongy degeneration of the brain.” As WHO explains, "animal variants include scrapie in sheep and goats, chronic wasting disease in deer, and bovine spongiform encephalopathies in cattle”

Prion diseases had been around, if not much understood, for a long time before they leaped into public attention with the discovery in March of 1996 that BSE had spread to humans in a few cases in the United Kingdom. This then became known as “variant” CJD (vCJD). One effect was to greatly raise the profile of prion diseases as a public health issue. A thorough review of what was known about the diseases and their spread was published in the Annual Review of Public Health in 2005. The 1996 discovery also made identifying cases seem extremely important, in order to track and possibly stop any new spread. That led to the establishment at CWRU in 1997, within the Division of Neuropathology, of the National Prion Disease Pathology Surveillance Center.

The Center’s founding director, Dr. Pierluigi Gambetti, spoke to the “Friday Lunch” about this issue back in 2013. What has changed since then? Is this a policy success story, in which outbreaks in animals have been quickly identified and the herds “culled,” while dangerous practices (such as making feed for some animals from other animals that could have the disease) have been suppressed? Or have we been dodging bullets mainly due to luck? Is there better understanding of the mechanisms of disease, and potential treatments? What might go wrong, or even right?

Joe White
Luxenberg Family Professor of Public Policy and Director, Center for Policy Studies

About Our Guest

Brian Appleby, MD, is a neuropsychiatrist at University Hospitals Cleveland Medical Center who treats and researches cognitive disorders. Dr. Appleby is an associate professor of neurology, psychiatry, and pathology at Case Western Reserve University and University Hospitals Cleveland Medical Center, the National Prion Disease Pathology Surveillance Center Director, and Medical Director of the Creutzfeldt-Jakob (CJD) Foundation. He is also chair of the Cleveland Chapter Alzheimer’s Association Professional Advisory Board.

His clinical and research interests include prion diseases (e.g., Creutzfeldt-Jakob disease), frontotemporal lobar degeneration, and young-onset dementias.