The Generative Brain

Public Health Agency of Canada and University of Manitoba (national prion laboratory)

This entry covers the Canadian federal public health system for human prion disease, which has two linked parts inside the Public Health Agency of Canada (PHAC), a federal government agency. The first is the Canadian Creutzfeldt-Jakob Disease Surveillance System (CJDSS), which has run prospective national surveillance of all human prion diseases since April 1998, coordinates case investigation from Ottawa, and with consent does chart reviews and family interviews. The second is the Prion Diseases Section of the National Microbiology Laboratory (NML) at 1015 Arlington Street, Winnipeg, Manitoba, which is the national reference laboratory: it runs an accredited endpoint QuIC test (EP-QuIC) on cerebrospinal fluid, PRNP gene sequencing on blood for inherited prion disease, and post-mortem prion protein analysis of brain tissue. The laboratory scientists also hold appointments in the Department of Medical Microbiology and Infectious Diseases at the University of Manitoba, which is why the group is often named as PHAC and University of Manitoba. Human prion diseases are reportable in every province and nationally notifiable. In 2025 and 2026 the Winnipeg group published a retrospective validation of a modified assay, ES-QuIC, that detected inherited prion disease cases the standard tests missed.

Key facts

  • The CJDSS is run by the Public Health Agency of Canada and carries out prospective national surveillance of all types of human prion disease, following the WHO promoted model of comprehensive surveillance; its stated aims are to understand the epidemiology, improve rapid and accurate diagnosis and reduce transmission risk.
  • National prospective surveillance began in April 1998, prompted by the Krever Commission question about blood-borne transmission (R649, to be rechecked; R663 instead names the UK variant CJD outbreak), and the system is reported to have moved into PHAC from Health Canada in 2004 (T3 only).
  • The CJDSS supports clinicians with consultation, laboratory investigation, logistic support and education, and with informed consent from the patient or a representative completes a medical chart review and a family interview; health professionals notify their local public health authority and can contact the CJDSS directly by a toll free line or email.
  • Human prion diseases are reportable in every province and nationally notifiable in Canada.
  • The Prion Diseases Section at the NML in Winnipeg is the national reference laboratory for suspected CJD; it offers an accredited EP-QuIC test on CSF, PRNP sequencing on blood to confirm or exclude mutations linked to inherited prion disease, and post-mortem prion protein analysis of brain tissue, each with its own posted requisition.
  • PRNP sequencing is only done after a consent for genetic testing form is obtained through the CJDSS; Ontario, for example, refers all CJD testing to the Winnipeg section, with stated turnaround of 15 calendar days for the CSF panel and 25 calendar days for PRNP sequencing from receipt.
  • Over 1998 to 2013 the CJDSS recorded 662 deaths, of which 43 were genetic prion disease, 613 sporadic, 4 iatrogenic and 2 variant, backed by 635 genetic analyses and 861 neuropathological examinations; the current public dashboard states that genetic CJD makes up 5 to 15 percent of diagnosed cases.
  • PHAC states that 90 people in Canada were diagnosed with CJD in 2022, and the NML estimates national incidence from CSF testing in 2016 to 2024 at 1.51 cases per million per year.
  • The Winnipeg group has built Canada's QuIC testing in stages: RT-QuIC was introduced in 2015, the faster endpoint variant EP-QuIC was described in 2016 and became the accredited reference test, and in 2026 a deer mouse substrate version, ES-QuIC, reached 96.6 percent sensitivity and 100 percent specificity on 116 cases, including about 40 inherited prion disease cases such as D178N (the paper's own counts are inconsistent, see dx-es-quic and C-106) that earlier assays missed.
  • Scientists of the section hold dual NML and University of Manitoba affiliations; Stephanie Booth joined the NML in 1999 and is the senior author on the 2025 and 2026 diagnostic papers.
  • The group publishes with the main surveillance and research centres: the US National Prion Disease Pathology Surveillance Center and the UK National CJD Research and Surveillance Unit on assays and VPSPr, the Tanz Centre in Toronto on knockin mouse models of genetic prion disease, the University of Alberta prion centre on sporadic subtypes, and an international genetics consortium that includes ISS Rome and Goettingen; it is also a coauthor on the EUROCJD genetic prion disease series.
  • Outside funding found is small and dated: a 2021 CJD Foundation grant to Booth on rare strains of sporadic CJD. The earlier entry text listing PHAC and University of Manitoba among 2025 CJD Foundation grantees is not supported, and the 2026 grant list has no PHAC or Manitoba award.

Timeline

  1. 1998Canadian CJD Surveillance System starts national prospective surveillance.
  2. 1999Stephanie Booth joins the NML in Winnipeg.
  3. 2015PHAC announces RT-QuIC as a new diagnostic test for CJD in Canada.
  4. 2016NML describes EP-QuIC as a sensitive, high-throughput antemortem CJD test.
  5. 2021CJD Foundation grant to Stephanie Booth for detecting rare sporadic CJD strains.
  6. 2024JCI paper with the Tanz Centre on knockin mouse models of genetic prion disease (D178N and E200K).
  7. 2024Paper with the UK NCJDRSU and US NPDPSC on characterising VPSPr by capillary electrophoresis.
  8. 2025Review of national CSF testing from 2016 to 2024 estimates incidence at 1.51 per million per year.
  9. 2025Paper shows Canadian CJD incidence stayed stable through the COVID-19 pandemic, using national EP-QuIC data from 2016 to 2025.
  10. 2026Annals of Neurology paper with the US NPDPSC reports ES-QuIC detects sporadic and inherited prion disease with 96.6 percent sensitivity.
  11. 2026PHAC CJDSS program page and factsheet last updated.
  12. 2026Paper with the University of Alberta prion centre on the variable MV1 subtype of sporadic CJD.
  13. 2026Health Infobase CJD dashboard updated with data to 7 August 2026.
  14. 2026Review from the NML group, now listed under the High Consequence Pathogens Division, argues for ES-QuIC in standard diagnostic workflows for sporadic and inherited prion disease.
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