Creutzfeldt-Jakob disease (CJD) in Canada: Dashboard

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Types of CJD

There are multiple types of CJD found in humans.

Sporadic CJD (sCJD)

sCJD is the most reported type. Misfolded proteins that cause the disease happen without any known cause. The average mortality rate is 1 to 2 per million people per year globally. sCJD makes up 85% to 95% of all CJD cases investigated though the Canadian Creutzfeldt-Jakob Disease Surveillance System (CJDSS).

Genetic CJD (gCJD)

gCJD diseases are rare neurological disorders inherited within a family. They result from a mutation in the prion protein gene which leads to misfolded proteins. They are associated with over 50 pathogenic gene mutations. gCJD makes up 5% to 15% of diagnosed CJD cases.

Acquired CJD

Acquired types of CJD make up less than 1% of cases. Iatrogenic (iCJD) is caused accidentally through certain medical procedures involving human tissues with prions. Variant CJD (vCJD) is a unique type of acquired CJD linked to eating meat from cows infected with an animal prion disease called Bovine Spongiform Encephalitis (BSE).

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CJD case counts

The data in the tiles changes to reflect the date range you select. You can use the slider to define your date range or select the date range using the From and To boxes.

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Sporadic CJD case trends over time

Trends in sporadic Creutzfeldt-Jakob disease Measure: over time by province or territory, Year:

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Notes

• Numbers represent surveillance data available at the time of analysis.
• The incidence rate uses suspected CJD cases by the date when the case investigation was opened by the CJDSS.
• Mortality rates are aggregated by year of death. This allows the comparison of current trends to historical trends. The mortality rate captures cases classified as definite or probable sCJD. For each year, rates have been calculated using Statistics Canada, table 17-10-0005-01 Population estimates on July 1, by age and gender.
• For 2026, these rates will be revised when updated population estimates for 2026 are released.
• Death attributable to definite and probable sporadic CJD, genetic CJD and variant CJD. Refer to the Diagnostic Criteria for Surveillance of CJD (2017) for more information.
• No definite or probable CJD cases were identified from Northwest Territories and Nunavut during the given period of surveillance. As the CJDSS is prepared to take notifications from these geographical regions, their populations were included in denominators to calculate national rate estimates.
• An increase of sCJD cases from 2015 to present is observed when compared to historical data prior to 2015. This increase is attributed to the introduction of the Ep-QuIC assay by NML in February 2016.

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Sporadic CJD cases by age and sex

An increase in age-specific mortality over time can be seen in all age groups over 50 years old.

Sporadic Creutzfeldt-Jakob disease mortality rates by age group and sex, includes total definite and probable cases, Year:

Notes

• Age represents age at the year of death. Age is grouped into 10-year strata, except for those under 49 due to low case counts.
• Sex is assigned and recorded at the individual’s birth or recorded by a clinician where clinically relevant. CJDSS uses the reporting categories female and male for both gender identity and sex at birth. Data on the sex of the individual are based on clinician report and/or clinical documentation presented to the CJDSS.

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Sources

Surveillance data were directly obtained from the following sources:

• clinical
• epidemiological
• laboratory
• pathology

Due to periodic updates of surveillance data, counts and rates may change over time.

The majority of CJD cases are identified through the cerebrospinal fluid (CSF) specimens submitted to the NML. However, the CJDSS program also responds to notifications and inquiries from:

• healthcare providers
• public health professionals
• patients and families
• members of the public

This is a comprehensive approach to supporting diagnostics and enhances public health surveillance.

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Data limitations

The number of cases per year may fluctuate due to differences in case reporting and data collection methods between different levels of government. Trends should be interpreted with caution.

Calculating incidence rates based on symptom onset is challenging in CJD surveillance. It is difficult to accurately determine onset dates of clinical symptoms. As a result, case-based rates are presented as a proxy for incidence rates and are calculated using the year case investigations are opened. While these rates may not reflect true incidence rates, they are a useful indicator of disease burden in Canada. These rates also offer a more timely signal for public health response.

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