Canada runs one national system for these diseases, so the local hospital team does not have to work out where to send things.
The national system
The Creutzfeldt-Jakob Disease Surveillance System, run by the Public Health Agency of Canada, carries out national surveillance of all human prion diseases. Its stated aims are to improve understanding of these diseases, support faster and more accurate diagnosis, and reduce the risk of transmission.
For doctors and hospitals it offers consultation, laboratory investigation, logistical support and education. With informed consent from the patient or their representative, it also carries out a detailed review of the medical records and an interview with the family. That interview is part of how the national picture is built, and taking part is a choice.
The toll-free number is 1-888-489-2999.
All human prion diseases are provincially reportable and nationally notifiable in Canada, which means a suspected case is reported to public health as a matter of routine.
Spinal fluid testing
The national reference laboratory has tested every submitted spinal fluid sample with an assay called EP-QuIC since 2016, alongside the 14-3-3 and tau tests on most samples.
Published figures for 2016 to 2024 cover 2,677 samples. Among 282 confirmed cases of CJD tested this way, 267 were positive, 10 negative and 5 gave an indeterminate result, which is a positive rate of about 95 percent. Among 35 confirmed cases that turned out not to be CJD, the test was positive once. The authors note that the non-CJD comparison group is very small, so the specificity figure rests on few cases.
Two things follow for a family. A positive result is strong evidence, and a negative result does not rule the diagnosis out by itself. Ten of those 282 confirmed cases tested negative.
The same analysis estimated about 1.51 cases per million people per year in Canada.
Support for families
The Canadian CJD Association is Canada’s member organisation in the CJD International Support Alliance, which keeps a directory of national associations. The Alliance directory is the current list, and support groups by country collects what is known.
The surveillance system’s own pages are written mainly for health professionals, and its toll-free line is a reasonable route when a local team is unfamiliar with these diseases.
Genetic questions
Genetic counselling comes before any decision about testing, and choosing not to test is a valid choice. Routes differ by province and are arranged through the treating team or a clinical genetics service. Counselling first and the right not to know explain what that involves.
Research and trials
Eligibility for any study is set by the people running it, and what is open changes. Trials outside the United States covers how to ask.
Related pages: labs by country, Australia, India and the countries hub.
Sources for this page
- Public Health Agency of Canada: Creutzfeldt-Jakob Disease Surveillance System
- Slota JA and others. Diagnosis of Creutzfeldt-Jakob disease in Canada: an update on cerebrospinal fluid testing from 2016 to 2024. Canadian Journal of Neurological Sciences 2025
- CJD International Support Alliance: directory of patient associations