Canadian Creutzfeldt-Jakob Disease Surveillance System (factsheet)


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Organization: Public Health Agency of Canada

Date published: 2023-11-10

What are prion diseases?

Prion diseases are rare, neurodegenerative and fatal brain disorders. They occur worldwide in both humans and animals. The prion protein in the brain does not cause disease in its normal form. However, for unknown reasons and very rarely, the prion protein can become abnormal. This causes brain damage and loss of function over time.

Human prion diseases

The most common human prion disease is Creutzfeldt-Jakob disease (CJD).

Animal prion diseases

Prion diseases that affect animals include:

Creutzfeldt-Jakob disease subtypes

Sporadic (around 93%): cause unknown.

Inherited (6%): passed down through genetics from previous generations.

Acquired (extremely rare, less than 1%):

Common signs and symptoms


Medical care for people with Creutzfeldt-Jakob disease is limited to supportive therapy. There is no cure to slow down human prion disease progression.

The Public Health Agency of Canada operates the Creutzfeldt-Jakob Disease Surveillance System with a focus to:

In 2022, 90 people in Canada were diagnosed with Creutzfeldt-Jakob disease.

Creutzfeldt-Jakob Disease Surveillance System offers:


The Creutzfeldt-Jakob Disease Surveillance System provides specialized laboratory tests though the National Microbiology Laboratory and pathology services to investigate the possibility of Creutzfeldt-Jakob disease.

Notifications of suspect cases of Creutzfeldt-Jakob disease

If you are a health care professional in Canada, you must inform your local public health authority. We strongly encourage you to notify the Creutzfeldt-Jakob Disease Surveillance System as well.

Telephone: 1-888-489-2999


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