The Generative Brain

Canadian Creutzfeldt-Jakob Disease Surveillance System (CJDSS), Public Health Agency of Canada, Ottawa

The CJDSS is the national surveillance program for human prion disease in Canada, run from Ottawa by the Public Health Agency of Canada (PHAC), a federal government agency. It was set up in 1998 and does prospective surveillance of all types of human prion disease on a model promoted by the World Health Organization. In practice it is the federal contact point for clinicians with a suspected case: it gives consultation and logistic support, arranges laboratory testing through the National Microbiology Laboratory in Winnipeg, coordinates case investigation and, with consent, reviews the medical chart and interviews the family. It also holds the consent for genetic testing form without which the Winnipeg laboratory will not sequence the PRNP gene. The Winnipeg laboratory is a separate entity in the atlas (org-phac-manitoba). The CJDSS published national mortality data and research with the main international surveillance centres up to 2023; its long-serving head is reported in the media to have retired in 2026, with no successor named publicly.

Key facts

  • PHAC operates the CJDSS, which conducts prospective national surveillance of all types of human prion disease in Canada, aiming to understand the epidemiology, improve rapid and accurate diagnosis and reduce transmission risk.
  • The federal publications catalogue says the system was established in 1998 as a national CJD surveillance system and that it also supports patients, families and health care providers through education, including a family newsletter issued by the PHAC Prion Disease Program (issues held for Winter 2011 and Spring 2013).
  • The CJDSS offers clinicians consultation, laboratory investigation, logistic support and education, and with informed consent from the patient or a representative completes a detailed medical chart review and a family interview.
  • Health care professionals must report a suspected case to their local public health authority and are strongly encouraged to also notify the CJDSS; the factsheet lists phone and email contact, and the program page a toll free line.
  • All human prion diseases are provincially reportable and nationally notifiable in Canada; a secondary list dates national notification of CJD from 2000.
  • PRNP gene sequencing at the Winnipeg Prion Diseases Section does not start until a signed Consent for Genetic Testing form, supplied by the CJDSS in Ottawa, has been received; the full report goes to the authorised physician or genetic counsellor in about 25 calendar days.
  • CJDSS authors reported 662 definite and probable CJD deaths in Canadian residents from 1998 to 2013, of which 43 were genetic, 613 sporadic, 4 iatrogenic and 2 variant, and judged the rising sporadic rate to reflect better case finding rather than more disease.
  • The public dashboard (data to 7 August 2026) states genetic CJD makes up 5 to 15 percent of diagnosed cases; the factsheet says 90 people in Canada were diagnosed with CJD in 2022.
  • In 2016 the CJDSS led an analysis of three variant CJD patients raised in Saudi Arabia, with UCSF, US CDC and Case Western co-authors, that supported BSE exposure in that country.
  • The CJDSS took part in the 2020 international sporadic CJD genome-wide association study led by the MRC Prion Unit, alongside the UK, Italian, German, Australian and US surveillance centres.
  • PHAC carried out a privacy impact assessment of the CJDSS because of the sensitivity of the personal information it holds, covering its personal information bank, sharing and security.
  • No paper indexed in Europe PMC has carried a CJDSS affiliation since January 2023; the two 2025 Canadian national CJD papers name only the Winnipeg laboratory and, for two Ottawa co-authors, the Infectious Diseases and Vaccination Programs Branch.
  • In routine case management in 2019 the CJDSS noticed New Brunswick referrals with shared features, ruled out prion disease in them and alerted the province in December 2020; PHAC later said its CJD lead stopped work on the file once prion disease was excluded. These points rest on media and encyclopedia sources, not official pages.

Timeline

  1. 1998CJDSS established as the national CJD surveillance system.
  2. 2000CJD listed as nationally notifiable (secondary source).
  3. 2007PHAC classic CJD infection control quick reference guide published, now linked from the CJDSS page.
  4. 2011Winter first family newsletter issue held in the federal catalogue; the last held issue is Spring 2013.
  5. 2015CJDSS mortality report for 1998 to 2013 published in the Canada Communicable Disease Report.
  6. 2016Saudi Arabia linked variant CJD cluster paper led from the CJDSS.
  7. 2020International sporadic CJD GWAS published with a CJDSS co-author.
  8. 2020CJDSS reported to have alerted New Brunswick about unexplained neurological referrals (media and encyclopedia sources).
  9. 2022PHAC privacy impact assessment summary for the CJDSS posted.
  10. 2023Last indexed paper with a CJDSS affiliation (capillary immunoassay with the Winnipeg NML).
  11. 2025National CJD incidence paper published with Ottawa co-authors under the Infectious Diseases and Vaccination Programs Branch, not the CJDSS.
  12. 2026CJDSS program pages and factsheet last updated.
  13. 2026Media report says the long-serving CJDSS head had retired from PHAC a few months earlier.
  14. 2026Health Infobase CJD dashboard updated with data to 7 August 2026.
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