vCJD is a prion disease that affects the central nervous system. These prion infections are known as transmissible spongiform encephalopathies. It is linked with exposure to Bovine spongiform encephalopathy (BSE) also known as "Mad Cow Disease" and is transmitted to human after consumption of meat from infected cows.
There is also a risk of transmission through blood transfusions although it has not yet been reported in Australia. However, in the United Kingdom, there have been a small number of reported cases of putative transfusion transmission since 2004.
There are currently no routine available tests to predict or prevent vCJD from transmission by transfusion. As a precaution, people who have spent a cumulative period of 6 months in the UK between 1 January 1980 and 31 December 1996 or had a transfusion in the UK between 1980 and 2001 are not accepted as blood donors in Australia.
When to suspect vCJD?
Patients can present with fatigue, weight loss, headache, unsteadiness, involuntary movements and deficits in higher cortical function.
Clinically assess patients for neurodegenerative signs and symptoms. Immediately consult with experts.
What to do?
Seek expert advice and notify the Blood Service.
1. Johnson RT, Gibbs Jr CJ. Creutzfeld-Jakob disease and related transmissible spongiform encephalopathies. NEJM 1998;339(27):1994-2004.