Creutzfeldt-Jakob Disease (CJD)

The Florida Department of Health monitors the number of deaths attributed to Creutzfeldt-Jakob disease (CJD), a rare and fatal brain disease that has been increasingly in the news. Based on death certificate reviews, there are approximately 16 deaths per year from this disease in Florida. This is almost exactly the number expected based on the national trend of having approximately one death per million population per year. The death rate in Florida has not been rising in recent years.

In addition to the classical form of the disease, which is found worldwide, there is a new variant of the disease that has been found almost exclusively in the United Kingdom. This new form of the disease called new variant Creutzfeldt-Jakob disease (nv-CJD or v-CJD) has been associated with Bovine Spongiform Encephalopathy (BSE) which is also known as "Mad Cow Disease". Cases of nv-CJD have slightly different clinical signs, symptoms, and laboratory features from those of the classical form of CJD. To date, no case of nv-CJD has been detected in Florida or the United States.

Monitoring functions for these diseases in humans and animals (collectively called transmissible spongiform encephalopathies or TSEs) are shared by federal, state and local health and agricultural officials. The Florida Department of Health regularly reviews death certificates and tracks the numbers of deaths attributed to CJD. We cooperate with our federal partners at the Centers for Disease Control and Prevention (CDC) to closely investigate cases of CJD that occur in people less than 55 years of age. This age group is where experts feel nv-CJD is most likely to occur. We also take reports from medical providers and the public on clusters of disease.

Agricultural officials are responsible for looking for BSE in livestock. Information on these programs can be obtained from the links that follow. To date, there have been no cases of BSE in Florida or the United States.

To learn more about these devastating diseases check out the following links, which provide you with information about CJD, nv-CJD, and BSE.

Information on Human CJD

Additional Information on CJD and BSE

Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease: Background, Evolution, and Current Concerns (CDC)

The UK National CJD Research and Surveillance Unit

National Prion Disease Pathology Surveillance Center at Case Western Reserve University

Chronic Wasting Disease of Deer and Elk and Human Health