About the Authors

Holger Hummerich

Roles Formal analysis

Affiliation MRC Prion Unit at University College London (UCL), Institute of Prion Diseases, UCL, London, United Kingdom

Helen Speedy

Roles Investigation

Affiliation MRC Prion Unit at University College London (UCL), Institute of Prion Diseases, UCL, London, United Kingdom

Tracy Campbell

Roles Investigation

Affiliation MRC Prion Unit at University College London (UCL), Institute of Prion Diseases, UCL, London, United Kingdom

Lee Darwent

Roles Investigation

Affiliation MRC Prion Unit at University College London (UCL), Institute of Prion Diseases, UCL, London, United Kingdom

Elizabeth Hill

Roles Investigation

Affiliation MRC Prion Unit at University College London (UCL), Institute of Prion Diseases, UCL, London, United Kingdom

Steven Collins

Roles Resources

Affiliation Australian National Creutzfeldt-Jakob Disease Registry, The Florey, Department of Medicine (RMH), The University of Melbourne, Victoria, Australia

Christiane Stehmann

Roles Resources

Affiliation Australian National Creutzfeldt-Jakob Disease Registry, The Florey, Department of Medicine (RMH), The University of Melbourne, Victoria, Australia

Gabor G. Kovacs

Roles Resources

Affiliations Department of Laboratory Medicine and Pathobiology and Tanz Centre for Research in Neurodegenerative Disease, University of Toronto, Ontario, Toronto, Canada, Laboratory Medicine Program & Krembil Brain Institute, University Health Network, Toronto, Ontario, Canada, Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna and Austrian Reference Center for Human Prion Diseases (ÖRPE), Vienna, Austria

Michael D. Geschwind

Roles Resources

Affiliation UCSF Memory and Aging Center, Department of Neurology, University of California, San Francisco, California, United States of America

Karl Frontzek

Roles Resources

Affiliation Institute of Neuropathology, University of Zürich, Zürich, Switzerland

Herbert Budka

Roles Resources

Affiliation Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna and Austrian Reference Center for Human Prion Diseases (ÖRPE), Vienna, Austria

Ellen Gelpi

Roles Resources

Affiliation Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna and Austrian Reference Center for Human Prion Diseases (ÖRPE), Vienna, Austria

Adriano Aguzzi

Roles Resources

Affiliation Institute of Neuropathology, University of Zürich, Zürich, Switzerland

Sven J. van der Lee

Roles Resources

Affiliations Section Genomics of Neurodegenerative Diseases and Aging, Department of Clinical Genetics, Vrije Universiteit Amsterdam, Amsterdam UMC, Amsterdam, The Netherlands, Delft Bioinformatics Lab, Delft University of Technology, Delft, The Netherlands, Amsterdam Neuroscience, Neurodegeneration, Amsterdam, The Netherlands

Cornelia M. van Duijn

Roles Resources

Affiliations Nuffield Department of Population Health, University of Oxford, Oxford, United Kingdom, Department of Epidemiology, Erasmus Medical Centre, Rotterdam, The Netherlands

Pawel P. Liberski

Roles Resources

Affiliation Department of Molecular Pathology and Neuropathology, Medical University of Lodz, Lodz, Poland

Miguel Calero

Roles Resources

Affiliation Chronic Disease Programme (UFIEC-CROSADIS) and Network Center for Biomedical Research in Neurodegenerative Diseases (CIBERNED), Instituto de Salud Carlos III, Madrid, Spain

Pascual Sanchez-Juan

Roles Resources

Affiliation Alzheimer’s Centre Reina Sofia-CIEN Foundation-ISCIII, Research Platforms, Madrid, Spain

Elodie Bouaziz-Amar

Roles Resources

Affiliation Department of Biochemistry and Molecular Biology, Lariboisière Hospital, GHU AP-HP Nord, University of Paris Cité, Paris, France

Jean-Louis Laplanche

Roles Resources

Affiliation Department of Biochemistry and Molecular Biology, Lariboisière Hospital, GHU AP-HP Nord, University of Paris Cité, Paris, France

Stéphane Haïk

Roles Resources

Affiliations Paris Brain Institute (Institut du Cerveau, ICM), INSERM, CNRS, Assistance Publique-Hôpitaux de Paris (AP-HP), Sorbonne Université, Paris, France, Assistance Publique-Hôpitaux de Paris (AP-HP), Cellule Nationale de Référence des Maladies de Creutzfeldt-Jakob, Groupe Hospitalier Pitié-Salpêtrière, Paris, France

Jean-Phillipe Brandel

Roles Resources

Affiliations Paris Brain Institute (Institut du Cerveau, ICM), INSERM, CNRS, Assistance Publique-Hôpitaux de Paris (AP-HP), Sorbonne Université, Paris, France, Assistance Publique-Hôpitaux de Paris (AP-HP), Cellule Nationale de Référence des Maladies de Creutzfeldt-Jakob, Groupe Hospitalier Pitié-Salpêtrière, Paris, France

Angela Mammana

Roles Resources

Affiliation IRCCS, Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy

Sabina Capellari

Roles Resources

Affiliations IRCCS, Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy, Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy

Anna Poleggi

Roles Resources

Affiliation Department of Neuroscience, Istituto Superiore di Sanità, Rome, Italy

Anna Ladogana

Roles Resources

Affiliation Department of Neuroscience, Istituto Superiore di Sanità, Rome, Italy

Maurizio Pocchiari

Roles Resources

Affiliation Department of Neuroscience, Istituto Superiore di Sanità, Rome, Italy

Saima Zafar

Roles Resources

Affiliations Department of Neurology, Clinical Dementia Center and National Reference Center for CJD Surveillance, University Medical School, Göttingen, Germany, Biomedical Engineering and Sciences Department, School of Mechanical and Manufacturing Engineering, National University of Sciences and Technology, Islamabad, Pakistan

Stephanie Booth

Roles Resources

Affiliation Prion Disease Program, National Microbiology Laboratory, Public Health Agency of Canada, Winnipeg, Canada

Gerard H. Jansen

Roles Resources

Affiliation Department of Pathology and Laboratory Medicine, University of Ottawa, Ottawa, Canada

Aušrinė Areškevičiūtė

Roles Resources

Affiliation Danish Reference Center for Prion Diseases, Department of Pathology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark

Eva Løbner Lund

Roles Resources

Affiliations Danish Reference Center for Prion Diseases, Department of Pathology, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark, Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark

Katie Glisic

Roles Resources

Affiliation National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH, United States of America

Piero Parchi

Roles Resources

Affiliations IRCCS, Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy, Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy

Peter Hermann

Roles Resources

Affiliations Department of Neurology, Clinical Dementia Center and National Reference Center for CJD Surveillance, University Medical School, Göttingen, Germany, German Center for Neurodegenerative Diseases (DZNE), Göttingen, Germany

Inga Zerr

Roles Resources

Affiliations Department of Neurology, Clinical Dementia Center and National Reference Center for CJD Surveillance, University Medical School, Göttingen, Germany, German Center for Neurodegenerative Diseases (DZNE), Göttingen, Germany

Brian S. Appleby

Roles Resources

Affiliation National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH, United States of America

Jiri Safar

Roles Resources

Affiliation Departments of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, United States of America

Pierluigi Gambetti

Roles Resources

Affiliation Departments of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, United States of America

John Collinge

Roles Supervision

Affiliation MRC Prion Unit at University College London (UCL), Institute of Prion Diseases, UCL, London, United Kingdom

Simon Mead

Roles Supervision

s.mead@prion.ucl.ac.uk

Affiliation MRC Prion Unit at University College London (UCL), Institute of Prion Diseases, UCL, London, United Kingdom

Competing Interests

Stéphane Haik reports grants from Santé Publique France, during the conduct of the study; grants from LFB Biomedicaments, grants from Institut de Recherche Servier, grants from MedDay Pharmaceuticals, outside the submitted work; In addition, Stéphane Haik has a patent Method for treating prion diseases (PCT/EP2019/070457) pending. Brian Appleby has received funding from CDC, NIH, CJD Foundation, Alector, and Ionis. He has served as a consultant for Ionis, Sangamo, and Gate Biosciences. He has received royalties from Wolter Kluwers. Karl Fronztek reports grants from Ono Pharmaceuticals outside the submitted work. Simon Mead reports grants from Medical Research Council (UK) and grants from National Institute of Health Research’s Biomedical Research Centre at University College London Hospitals NHS Foundation Trust during the conduct of the study. Gabor G Kovacs reports personal fees from Biogen, outside the submitted work. John Collinge reports grants from Medical Research Council, grants from NIHR UCLH Biomedical Research Centre, during the conduct of the study; and is a Director and shareholder of D-Gen Limited, an academic spinout in the field of prion disease diagnostics, decontamination and therapeutics. Inga Zerr reports grants from the Bundesministerium für Gesundheit via Robert Koch institute, JPND and personal fees (not related to the content of the manuscript) from Ferring Pharmaceuticals and IONIS, speaking honoraria for medical lectures from Lilly, Biogen, Medfora, DGLN (German Society for cerebrospinal fluid diagnostics in Neurology). Maurizio Pocchiari reports personal fees from Ferring Pharmaceuticals, personal fees from CNCCS (Collection of National Chemical Compounds and Screening Center), non-financial support from Fondazione Cellule Staminali, outside the submitted work. Michael D Geschwind has consulted for3D Communications, Adept Field Consulting, Advanced Medical Inc., Best Doctors Inc., Second Opinion Inc., Gerson Lehrman Group Inc., Guidepoint Global LLC, InThought Consulting Inc., Market Plus, Trinity Partners LLC, Biohaven Pharmaceuticals, Quest Diagnostics and various medical-legal consulting. He has received speaking honoraria for various medical center lectures and from Oakstone publishing. He has received past research support from Alliance Biosecure, CurePSP, the Tau Consortium, and Quest Diagnostics. Michael D Geschwind serves on the board of directors for San Francisco Bay Area Physicians for Social Responsibility and on the editorial board of Dementia & Neuropsychologia.