Accepted for Publication: December 15, 2015.
Published Online: February 22, 2016. doi:10.1001/jamaneurol.2015.4885.
Study concept and design: Mead, Burnell, Rudge, Walker, Collinge.
Acquisition, analysis, or interpretation of data: All authors.
Drafting of the manuscript: Mead, Burnell, Rudge, Walker, Collinge.
Critical revision of the manuscript for important intellectual content: Mead, Burnell, Lowe, Thompson, Lukic, Porter, Carswell, Kaski, Kenny, Mok, Bjurstrom, Franko, Gorham, Druyeh, Wadsworth, Jaunmuktane, Brandner, Hyare, Rudge.
Statistical analysis: Mead, Burnell, Walker.
Obtained funding: Mead, Collinge.
Administrative, technical, or material support: Mead.
Study supervision: Mead, Rudge, Collinge.
Conflict of Interest Disclosures: Dr Collinge is a director and Drs Wadsworth and Collinge are shareholders of D-Gen Ltd, an academic spinout company working in the field of prion disease diagnosis, decontamination, and therapeutics. No other disclosures were reported.
Funding/Support: The cohort study was funded by the Department of Health (England), Medical Research Council, and University College London Hospitals/University College London National Institutes for Health Research Biomedical Research Centre. This study was funded by the Medical Research Council, Department of Health (England), and the University College London /University College London Hospitals Biomedical Research Centre.
Role of the Funder/Sponsor: The funding sources had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.
Additional Contributions: We thank all the individuals, their caregivers, and families, who took part in the PRION-1 and cohort studies as well as UK neurologists and the National CJD Research and Surveillance Unit for referring patients. We thank officials at the Department of Health, Medical Research Council, co-chairs of the PRION-1 and cohort steering committee, and our colleagues at the National CJD Research and Surveillance Unit for establishing the National CJD referral arrangements, without which these studies would not have been possible. We thank all past and present colleagues at the National Prion Clinic (National Hospital for Neurology and Neurosurgery, Queen Square, London, England). Ray Young and Richard Newton, paid employees of the Medical Research Council Prion Unit, assisted with figure design.