{"id":1567,"date":"2024-09-20T13:14:09","date_gmt":"2024-09-20T13:14:09","guid":{"rendered":"https:\/\/artsci.case.edu\/fridaylunch\/?p=1567"},"modified":"2025-10-26T04:46:48","modified_gmt":"2025-10-26T04:46:48","slug":"1567","status":"publish","type":"post","link":"https:\/\/artsci.case.edu\/fridaylunch\/2024\/09\/20\/1567\/","title":{"rendered":"Science, Public Policy, and the Future of Prion Disease"},"content":{"rendered":"<p>\n<title>Center for Policy Studies Public Affairs Discussion Group &#8211; Science, Public Policy, and the Future of Prion Disease. With Brian S. Appleby, MD, Director, National Prion Disease Pathology Surveillance Center and Professor in Departments of Neurology, Psychiatry, and Pathology at Case Western Reserve University School of Medicine and University Hospitals Cleveland Medical Center.<\/title><\/p>\n<table align=\"center\" width=\"640\" style=\"padding:3px\">\n<tbody>\n<tr>\n<td width=\"702\" align=\"center\" valign=\"top\" style=\"margin: -1.9px 2px 2px 2px;padding:5px\">\n<table style=\"padding:px\" align=\"center\" width=\"637\">\n<tbody>\n<tr>\n<td width=\"629\" colspan=\"3\" valign=\"top\" bgcolor=\"#ffffff\" style=\"margin: 2px 2px 2px 2px;text-align: center;font-size: 12px\">\n<p style=\"width: 100%;text-align: left;margin:                      3px 0px 0px;font-size: 24px\"><img loading=\"lazy\" src=\"https:\/\/artscimedia.case.edu\/wp-content\/uploads\/sites\/238\/2024\/06\/07135207\/cas_logo_newsletters2.jpg\" alt=\"CAS\" longdesc=\"http:\/\/policy.case.edu\" height=\"100\" width=\"328\"><br \/><strong><font face=\"Arial, Helvetica, sans-serif\" color=\"#0a304e\">Center for Policy Studies<br \/>Public Affairs Discussion Group<\/font><\/strong><\/p>\n<\/td>\n<\/tr>\n<\/table>\n<table>\n<tr>\n<td colspan=\"3\" valign=\"top\" bgcolor=\"#ffffff\" style=\"margin: -4px -4px -4px -4px;text-align: center;font-size: 0px\"><\/td>\n<\/tr>\n<\/table>\n<table style=\"margin: -1.9px 2px 2px 2px\" align=\"center\" width=\"640\">\n<tr>\n<td width=\"502\" valign=\"top\" style=\"padding: 3px\">\n<table width=\"627\">\n<tr>\n<td width=\"98%\" height=\"33\" align=\"left\" valign=\"top\" style=\"margin: 0px 2px 2px 2px;font-family: Arial, Helvetica, sans-serif;font-size: 24px;padding: 1px\"><strong><font color=\"#0a304e\">Science, Public Policy, and the Future of Prion Disease<\/font><\/strong><\/td>\n<\/tr>\n<\/table>\n<table width=\"627\">\n<tr>\n<td width=\"70%\" align=\"left\" valign=\"top\" style=\"margin: 0px 2px 2px 2px;font-family: Arial, Helvetica, sans-serif;font-size: 14px;padding: 2px\"><img align=\"left\" src=\"https:\/\/artscimedia.case.edu\/wp-content\/uploads\/sites\/238\/2025\/10\/23054036\/appleby_brian.jpg\" hspace=\"3\"><br \/>\n                   <font color=\"#0a304e\"><strong>Brian S. Appleby, MD &#8211; Director, National Prion Disease Pathology Surveillance Center and Professor in Departments of Neurology, Psychiatry, and Pathology at Case Western Reserve University School of Medicine and University Hospitals Cleveland Medical Center<\/strong><\/font><\/td>\n<\/tr>\n<\/table>\n<table>\n<tr>\n<td width=\"70%\" valign=\"top\" bgcolor=\"FFFFFF\" style=\"margin: 2px 2px 2px 2px;font-family: Arial, Helvetica, sans-serif;font-size: 14px;padding: 2px\"><font color=\"#0a304e\"><strong>Friday September 20, 2024<br \/>\n\t12:30-1:30 p.m.<br \/>Meeting Both In-Person and by Zoom<\/strong><\/font><br \/>\n<font color=\"#990000\"><strong>Alternate Room: Mather House 100<\/strong><\/font><br \/>\n<font color=\"#0a304e\"><strong>Case Western Reserve University<\/strong><\/font><\/p>\n<p><font color=\"#0a304e\">Dear Colleagues:<\/font><\/p>\n<p>\t<font color=\"#0a304e\"><strong>Often our Friday discussions are about Scary Stuff.<\/strong> 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\u2019s hard to figure out how scared to be, as with Generative AI, because the scary stuff may have some upside.<\/p>\n<p>    <strong>This week\u2019s discussion is about something that is awfully darn scary, with absolutely no upside \u2013 prion diseases. <\/strong>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<\/font> <a href=\"https:\/\/www.cdc.gov\/prions\/about\/index.html#:~:text=Prion%20diseases%20occur%20when%20proteins,progresses%20and%20leads%20to%20death.\" target=\"_blank\" rel=\"noopener\"><font color=\"#0a304e\"><u>website<\/u><\/font><\/a> <font color=\"#0a304e\">describes:<\/p>\n<p>&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;\u201cPrion diseases are a family of illnesses that affect people and animals&quot;.<br \/>\n&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;\u201cThese diseases are rare but always lead to death in the person or animal within months or years of symptoms beginning.&quot;<br \/>\n&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;\u201cThere is no treatment or vaccine.\u201d<\/p>\n<p>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<\/font> <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC5443459\/\" target=\"_blank\" rel=\"noopener\"><font color=\"#0a304e\"><u>identification of Creutzfeldt-Jakob disease<\/u><\/font><\/a> <font color=\"#0a304e\">(CJD) by German neuropathologists.  Worldwide there is about one CJD case per million people per year, concentrated at older ages<\/font> (<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK507860\/#:~:text=CJD%20affects%20about%201%20individual,form%20of%20human%20prion%20disease.\" target=\"_blank\" rel=\"noopener\"><font color=\"#0a304e\"><u>\u201cmean onset age of 62\u201d<\/u><\/font><\/a>)<font color=\"#0a304e\">. CJD, however, is just one of \u201c<\/font><a href=\"https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK507860\/#:~:text=CJD%20affects%20about%201%20individual,form%20of%20human%20prion%20disease.\" target=\"_blank\" rel=\"noopener\"><font color=\"#0a304e\"><u>a group of transmissible spongiform encephalopathies affecting people worldwide<\/u><\/font><\/a><font color=\"#0a304e\">\u201d, and that term also suggests another reason for fear: it involves, in the WHO\u2019s words, \u201ccharacteristic spongy degeneration of the brain.\u201d As<\/font> <a href=\"https:\/\/www.who.int\/teams\/health-product-policy-and-standards\/standards-and-specifications\/norms-and-standards\/transmissible-spongiform-encephalopathies\" target=\"_blank\" rel=\"noopener\"><font color=\"#0a304e\"><u>WHO explains<\/u><\/font><\/a><font color=\"#0a304e\">, &quot;animal variants include scrapie in sheep and goats, chronic wasting disease in deer, and bovine spongiform encephalopathies in cattle\u201d<\/p>\n<p>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 \u201cvariant\u201d CJD (vCJD).  One effect was to greatly raise the profile of prion diseases as a public health issue. A<\/font> <a href=\"https:\/\/www.cdc.gov\/prions\/pdfs\/public-health-impact.pdf\" target=\"_blank\" rel=\"noopener\"><font color=\"#0a304e\"><u>thorough review<\/u><\/font><\/a> <font color=\"#0a304e\">of what was known about the diseases and their spread was published in the <em>Annual Review of Public Health<\/em> 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.<\/p>\n<p>The Center\u2019s founding director, Dr. Pierluigi Gambetti, spoke to the \u201cFriday Lunch\u201d about this issue back<\/font> <a href=\"https:\/\/artsci.case.edu\/fridaylunch\/2013\/01\/25\/learning-from-mad-cows\/\" target=\"_blank\" rel=\"noopener\"><font color=\"#0a304e\"><u>in 2013<\/u><\/font><\/a><font color=\"#0a304e\">.<strong> What has changed since then? Is this a policy success story, in which outbreaks in animals have been quickly identified and the herds \u201cculled,\u201d 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?<\/strong><\/p>\n<p>Best wishes for safety and security for you and yours,<\/p>\n<p>    Joe White<br \/>\n    Luxenberg Family Professor of Public Policy and Director, Center for Policy Studies<\/font>\n  <\/p>\n<hr width=\"100%\" style=\"padding:0px\" \/>\n<h3><font color=\"#0a304e\">About Our Guest<\/font><\/h3>\n<p><font color=\"#0a304e\"><strong>Brian Appleby, MD<\/strong>, 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&#8217;s Association Professional Advisory Board.<\/p>\n<p>Dr. Appleby received his medical degree from Georgetown University School of Medicine. He completed a psychiatry residency at The Johns Hopkins Hospital, where he also completed a geriatric psychiatry fellowship.<\/p>\n<p>His clinical and research interests include prion diseases (e.g., Creutzfeldt-Jakob disease), frontotemporal lobar degeneration, and young-onset dementias.<\/font><\/td>\n<\/tr>\n<\/table>\n<\/td>\n<\/tr>\n<\/table>\n<table>\n<tr>\n<td colspan=\"3\" valign=\"top\" bgcolor=\"#ffffff\" style=\"margin: -4px -4px -4px -4px;text-align: center;font-size: 0px\"><\/td>\n<\/tr>\n<\/table>\n<table style=\"margin: -4px 2px 2px 2px;padding: 0px\" align=\"center\" width=\"640\">\n<tr>\n<td style=\"margin-top: 0px;padding: 0px;font-family: Arial, Helvetica, sans-serif;font-size: 14px\">\n<p align=\"center\"> Visit the <a href=\"http:\/\/fridaylunch.case.edu\"><font color=\"#004480\">Public Affairs Discussion Group Web Site.<\/font><\/a><\/p>\n<p align=\"center\"> Center for Policy Studies | Mather House<br \/>\n        111 | 11201 Euclid Avenue |<br \/>\n        Cleveland, Ohio 44106-7109 |&nbsp;Phone: 216.368.6730 |&nbsp;<a href=\"mailto:padg@case.edu\"><font color=\"#004480\"><u>padg@case.edu<\/u><\/font><\/a> |<br \/>Part of the:&nbsp;<a href=\"http:\/\/www.case.edu\/artsci\"><font color=\"#004480\"><u>College of Arts and Sciences<\/u><\/font><\/a><\/p>\n<p align=\"center\">&copy; 2024 Case Western Reserve University |<br \/> Cleveland, Ohio<br \/>\n        44106 | 216.368.2000 |&nbsp;<a href=\"http:\/\/www.case.edu\/legal.htm\"><font color=\"#004480\"><u>legal notice<\/u><\/font><\/a><\/p>\n<\/td>\n<\/tr>\n<\/table>\n<\/td>\n<\/tr>\n<\/table>\n","protected":false},"excerpt":{"rendered":"<p>\nCenter for Policy Studies Public Affairs Discussion Group &#8211; Science, Public Policy, and the Future of Prion Disease. With Brian S. Appleby, MD, Director, National Prion Disease Pathology Surveillance Center and Professor in Departments of Neurology, Psychiatry, and Pathology at Case Western Reserve University School of Medicine and University Hospitals Cleveland Medical Center.<\/p>\n<p style=\"width: 100%;text-align: left;margin:                      3px 0px 0px;font-size: 24px\"><strong>Center for Policy StudiesPublic Affairs Discussion Group<\/strong><\/p>\n<p>                <strong>Science, Public Policy, and the Future of Prion Disease<\/strong><\/p>\n<p>                   <strong>Brian S. Appleby, MD &#8211; Director, National Prion Disease Pathology Surveillance Center and Professor in Departments of Neurology, Psychiatry, and Pathology at Case Western Reserve University School of Medicine and University Hospitals Cleveland Medical Center<\/strong><\/p>\n<p><strong>Friday September 20,<\/strong><\/p>\n<p><a href=\"https:\/\/artsci.case.edu\/fridaylunch\/2024\/09\/20\/1567\/\" class=\"more-link\">Continue reading&#8230; <span class=\"screen-reader-text\">Science, Public Policy, and the Future of Prion Disease<\/span><\/a><\/p>\n","protected":false},"author":101,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"spay_email":""},"categories":[1],"tags":[],"jetpack_featured_media_url":"","_links":{"self":[{"href":"https:\/\/artsci.case.edu\/fridaylunch\/wp-json\/wp\/v2\/posts\/1567"}],"collection":[{"href":"https:\/\/artsci.case.edu\/fridaylunch\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/artsci.case.edu\/fridaylunch\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/artsci.case.edu\/fridaylunch\/wp-json\/wp\/v2\/users\/101"}],"replies":[{"embeddable":true,"href":"https:\/\/artsci.case.edu\/fridaylunch\/wp-json\/wp\/v2\/comments?post=1567"}],"version-history":[{"count":5,"href":"https:\/\/artsci.case.edu\/fridaylunch\/wp-json\/wp\/v2\/posts\/1567\/revisions"}],"predecessor-version":[{"id":1642,"href":"https:\/\/artsci.case.edu\/fridaylunch\/wp-json\/wp\/v2\/posts\/1567\/revisions\/1642"}],"wp:attachment":[{"href":"https:\/\/artsci.case.edu\/fridaylunch\/wp-json\/wp\/v2\/media?parent=1567"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/artsci.case.edu\/fridaylunch\/wp-json\/wp\/v2\/categories?post=1567"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/artsci.case.edu\/fridaylunch\/wp-json\/wp\/v2\/tags?post=1567"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}