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NPDPSC Newsfeed

 

Aside from testing and surveilling prion disease, an additional aim is to provide accurate information regarding this disease through various outreach efforts. To convey information about prion disease in general and how our center operates, our faculty and staff continuously engage with the public through podcasts, virtual talks and seminars, published articles, and research papers.

Below are some publications and talks, grouped by category.

 

Chronic Wasting Disease Spillover Preparedness and Response: Charting an Uncertain Future by CIDRAP CWD -  With the increasing prevalence of chronic wasting disease (CWD) in the cervid population, the risk of prion disease transmission to humans and non-cervid livestock has become a growing concern. This webinar covers some of the key findings and recommendations from CIDRAP's latest report with the same title, in their efforts to address the possibility of spillover.

The complete CIDRAP's latest report can be found here.

What Every Pathologist Needs to Know - Prion Diseases by Mark Cohen, M.D., for the 2020 American Association of Neuropathologists meeting regarding what every neuropathologist should know about prion diseases. In this talk, Dr. Cohen discusses different types of prion testing performed at the National Prion Disease Pathology Surveillance Center (NPDPSC), prion disease in general, and some information regarding our center. 

For more information on:

National Prion Disease Pathology Surveillance Center Update by Brian Appleby, M.D. - Dr. Appleby gives an update on the work of the National Prion Disease Pathology Surveillance Center (NPDPSC). In this update, he briefly talks about research studies at the center, our testing procedures, and the Autopsy program; a brief introduction to our Center's staff; and the NPDPSC center in the environment of COVID-19.

 
  1. Hope in the fight against deadly prion diseases, and side effects of organic agriculture - the most recent podcast interview about prion diseases, where Staff Writer Meredith Wadman from Science.org talks with host Sarah Crespi about new potential treatments for these fatal neurodegenerative diseases.
    The complete transcript of the conversation can be found by clicking on this link.
     
  2. "Mad cow and the history, cause and spread of prion diseases" - a podcast interview of Dr. Appleby with the American Chemical Society's award-winning science podcast Tiny Matters. In this episode, they discuss what are prion diseases, their various causes, and some of the work being done currently to detect them. 
     
  3. Please click here to listen to the Neurology® podcast interview with Brian Appleby, M.D., in which the Center's published research on the Neurology Journal website, titled "Diagnosis of prion diseases by RT-QuIC results in improved surveillance" is discussed.

    If you want to learn more about CSF and RT-QuIC testing, you can visit and read the Cerebrospinal Fluid Diagnostic Tests page on our website. 

 

Meredith Wadman, a Staff Writer from Science, published an article titled "Foiling Deadly Prions - Can the course of fatal prion diseases be changed by removing the protein before it goes bad?". The article gives an overview of prion disease, with a focus on genetic CJD and the new Ionis ION717 clinical trial.

Science.org is a premier global institution dedicated to the advancement and dissemination of scientific knowledge.

Related to this article, Meredith also participated in a podcast episode called "Hope in the fight against deadly prion diseases, and side effects of organic agriculture". The complete transcript of the conversation can be found by clicking this link.

Our Autopsy Coordination team published this informative article in The Director regarding the stigma of prion disease faces with regard to autopsies and/or burial services. The article gives an overview of prion disease, some of its characteristics, and more importantly - provides important information about the requirements of burial and/or cremation services for patients with prion disease. 

If you want to learn more about our Autopsy Program, please visit our Autopsy Coordination Program page, where you can read some of the Frequently Asked Questions, and also contact information, should you have any other questions.

For some more in depth information about recent findings in the world of prion disease, please take a look at the listed publications.

  1. Bayazid, R., Orru’, C., Aslam, R. et al. A novel subtype of sporadic Creutzfeldt–Jakob disease with PRNP codon 129MM genotype and PrP plaques. Acta Neuropathol (2023). https://doi.org/10.1007/s00401-023-02581-1
  2. Venkatraghavan, V, Pascuzzo, R, Bron, EE, et al. A discriminative event-based model for subtype diagnosis of sporadic Creutzfeldt-Jakob disease using brain MRI. Alzheimer's Dement. 2023; 1- 11. https://doi.org/10.1002/alz.12939
  3. Appleby BS, Shetty S, Elkasaby M.  Genetic aspects of human prion diseases.  Front Neurol 2022;13:1003056 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9579322/
  4. Figgie MP Jr, Appleby BS. Clinical Use of Improved Diagnostic Testing for Detection of Prion Disease. Viruses. 2021 Apr 28;13(5):789 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8146465/
  5. Hermann P, Appleby B, Brandel JP, Caughey B, Collins S, Geschwind MD, Green A, Haïk S, Kovacs GG, Ladogana A, Llorens F, Mead S, Nishida N, Pal S, Parchi P, Pocchiari M, Satoh K, Zanusso G, Zerr I. Biomarkers and diagnostic guidelines for sporadic Creutzfeldt-Jakob disease. Lancet Neurol. 2021 Mar;20(3):235-246 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8285036/
  6. Brennecke N, Cali I, Mok TH, Speedy H, Genomics England Research Consortium, Hosszu LLP, Stehmann C, Cracco L, Puoti G, Prior TW, Cohen ML, Collins SJ, Mead S, Appleby BS. Characterization of Prion Disease Associated with a Two-Octapeptide Repeat Insertion. Viruses. 2021 Sep 8;13(9):1794 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8473248/