View Prof.John Collinge talking about CJD and Prion Disease

In this short video Professor John Collinge talks about Creutzfeltdt Jakob Disease (CJD) and Prion Disease, explaining the different strains of the disease and the disease progression.

Professor John Collinge

Real stories of CJD

Francesca Certo shares her experience of living with the knowledge that she is at heightened risk of Genetic CJD.  Colin also share his experience of caring for his wife with Sporadic CJD.

Real stories of CJD

Types of CJD

There are four types of CJD: Factsheets on each strain can be downloaded from the links below

• Sporadic (or classical) CJD is the most common form, accounting for around 85 per cent of cases. The cause is unknown. It mainly affects the over 50s and has a sudden onset. The course of the disease is measured in months.

• Genetic CJD  (sometimes known as familial CJD) is an inherited form of the disease, with younger onset and a longer time course than sporadic CJD.

• Iatrogenic CJD occurs through contamination with infected tissue via medical procedures, for example, the use of human growth hormone, blood transfusion or surgical instruments.

• Variant CJD is caused by exposure to bovine spongiform encephalopathy, or BSE, and typically affects younger people. It has a relatively longer time course - an average of 14 months from onset of symptoms to death. There is a combination of psychiatric, neurological and physical symptoms.

Symptoms of CJD

Early symptoms of CJD include minor lapses of memory, mood changes and loss of interest. Within weeks an infected person may complain of clumsiness and feeling muddled, become unsteady in walking and may exhibit slow or slurred speech. Eyesight often becomes blurred.

The disease progresses to jerky movements, shakiness and stiffness of limbs, incontinence and the loss of the ability to move or speak. It is likely that the person is no longer aware of their surroundings or disabilities. Eventually the person will need full nursing care.

People affected by CJD usually die within six months of early symptoms, often from pneumonia. In a minority of patients the disease may take two years to run its course. In rare cases, the disease can last for many years.

What is CJD?

Creutzfeldt-Jakob Disease (or sometimes known as Jakob-Creutzfeldt Disease) is one of a group of rare brain disorders known as prion diseases which occur both in humans and certain animals. The infectious agent, or prion, attacks the brain, killing cells. Gaps in brain tissue develop, giving the brain a characteristic sponge-like appearance under the microscope.

Prions may exist in the body for many years before symptoms begin, but death may then result within a year. CJD usually attacks people over the age of 55.

The number of confirmed cases of CJD has risen steadily in recent years. This may be due to more accurate diagnosis and an increased awareness of the disease, but is also due to the emergence of a new form of the disease - variant CJD, or vCJD. This new form has been linked to a prion disease which affects cattle, bovine spongiform encephalopathy, or BSE. Variant CJD appears to affect younger people.