Local Man Dies of Creutzfeldt-Jakob Disease

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Doug Coffey, 62, of Glasgow, died from a rare disease known as Creutzfeldt-Jakob, but doctors say there is no need for alarm because the disease is not contagious.

Creutzfeldt Jakob occurs once in every one million people.

Dr. Dennis O'Keefe is a neurologist. Dr. O'Keefe says, "It's caused by an unusual protein called a prion. What this prion does when it gets in the body, it affects other prions and causes them to unfold and become inactive."

Unfortunately there no known cure for Creutzfeldt-Jakob Disease and it is always fatal.

Dr. O'Keefe says, "The illness varies in its presentation. The most common presentation is a progressive loss of intellectual functions. It may be your speech, maybe confusion, maybe difficulties controlling one part of your body or another."

There are several different versions of CJD. The most common is referred to as Classic CJD. There are about four cases of this version each year in the state of Kentucky. The cause of this rare disease is unknown.

Another version of CJD is called Variant Creutzfeldt-Jakob Disease (vCJD for short). This version is a relatively new disease connected to Bovine Spongiform Encephalopathy (Mad Cow Disease). It is suspected to be contracted from eating contaminated beef. There have only been 153 cases of vCJD worldwide; almost all of them in England. There has only been one case of vCJD ever recorded in the U.S. and this was a woman in Florida who had recently moved from England.

The Kentucky Cabinet for Health Services confirms that there has never been a case of vCJD in this state. Representatives from the cabinet tell WBKO that the way the beef industry is regulated in the U.S. there is almost zero chance that vCJD would ever surface here.