Saturday, June 15, 2013

Creutzfeldt-Jakob Disease: Background Information

Creutzfeldt-Jakob Disease: Background Information

June 14, 2013

Q: What is CJD and how many cases are there in one year?

Creutzfeldt-Jakob Disease (CJD) is a rare prion disease that affects the central nervous system. The disease is always fatal, and there is no known treatment or prevention. There are two main forms of human prion disease, classical CJD and a newer form, called variant CJD (vCJD).

Classical CJD occurs in the general population at a rate of 1–2 cases per million people per year throughout the world. In Canada, between 30 and 50 cases of classical CJD are diagnosed each year.

Classical CJD is recognized to exist in three forms, sporadic, genetic and iatrogenic. Sporadic has an unknown cause, occurs unpredictably and accounts for over 90 per cent of Canadian CJD cases. Genetic CJD, is associated with a gene mutation and iatrogenic CJD is acquired from the accidental transmission of the disease through medical procedures.

Variant CJD has been linked to consumption of meat or meat products from cattle infected with bovine spongiform encephalopathy (BSE) or "mad-cow disease."

Q. What are the symptoms of CJD?

The first symptoms tend to be psychiatric in nature - anxiety, depression, withdrawal and behavioural changes as well as persistent pain and odd sensations in the face and limbs. After several weeks or months, more clear-cut neurological symptoms may set in, including:
Unsteadiness in walking, sudden jerking movements.
Progressive dementia.
Eventually, the patient loses the ability to move or speak, and will need 24-hour nursing care.

Death occurs around a year after the onset of symptoms.

For more information on CJD, visit