Information

May 2003

First Canadian Case of Variant Creutzfeldt-Jakob Disease (Variant CJD)

In April 2002, a suspected case of variant CJD was reported to the Public Health Agency of Canada's CJD Surveillance System. Because of the clinical presentation, age of the patient and past multiple stays in the United Kingdom (UK), this was classified as a possible variant CJD case. However, the diagnosis of classical CJD was also considered.

As part of the investigation, a full medical history was obtained. The following information on this case can be shared:

• The patient, a male under the age of 50, had multiple stays in the UK during the outbreak of bovine spongiform encephalopathy (BSE), commonly know as "mad-cow disease."
• While staying in the UK, the patient ate processed meat products. Processed meat products made from cows infected with BSE carried a high risk of transmission of BSE.
• After coming to Canada in the early 1990s, the patient ate very little beef and had not eaten venison from deer or elk.
• The patient has not donated blood in Canada.
• The patient underwent a medical procedure in Canada.

Upon learning of the patient's procedure, Health Canada immediately advised the hospital to remove from service the medical devices used during the procedure, until such time as a diagnosis be confirmed. The hospital immediately complied with Health Canada's recommendation.

Following the death of the patient, an autopsy was performed and variant CJD was confirmed by Canadian experts. However, because this was the first case of variant CJD diagnosed in Canada, final confirmation was sought from an expert on variant CJD in the UK. This expert confirmed variant CJD. The attending physician was notified and spoke to the family of the patient.

The hospital, together with provincial authorities and Health Canada, has identified individuals who were exposed to the medical devices before the diagnosis of variant CJD was suspected in that patient.

As a precautionary measure, the hospital has a plan of action in place to advise those individuals not to donate blood, organs or tissues. However, if any of these individuals have donated blood since their procedure, their blood components that have not been pooled will be retrieved and destroyed. If any blood components donated by those individuals have been pooled, the theoretical and remote risk of CJD transmission does not merit further action.

Although the risk of acquiring variant CJD from the medical devices used on the patient with the disease is considered to be extremely low, Health Canada recommended the medical devices be permanently removed from service now that the diagnosis has been confirmed.

This situation does not represent a threat to those who have been in contact with this person, since variant CJD is not contagious through personal contact. For example, there is no evidence that variant CJD can be spread through touching or kissing a person with the disease. Similarly, health care workers who have provided usual care to a patient with variant CJD do not have an increased risk of acquiring the disease from that patient.

Because the disease has a long incubation period, it was expected that a case of variant CJD would be diagnosed in Canada several years after a person acquired the disease while staying in the UK.