Watchdog In Kansas City Is Wrong About Prion Diseases
We always get a little discomfort whenever someone in the media gives themselves the title of 
"Watchdog."
Maybe its because we always think of the late Marvin Zindler, the Texas TV consumer reporter who during one ratings period decided to force the shutdown of a rural brothel. It was the story that musical "Best Little Whorehouse in Texas" was based upon. Since he died last August 1st, I won't say more about Marvin. But here's his picture.
At the Kansas City Star, its Watchdog was recently asked about the death of Milton Eugene Rebarchek, specifically whether it was "related to Mad Cow." Here's how the Watchdog replied:
The man’s death was not related to “mad cow.” Instead, it was Creutzfeldt-Jakob disease, says Joe Blubaugh, spokesman for the Kansas Department of Health and Environment.
And Blubaugh says that you can breathe easy because Creutzfeldt-Jakob disease, or CJD, is far different than mad cow.
Blubaugh says about three people die of CJD each year in Kansas.
The Watchdog says, fortunately, you probably have more to fear from mad dogs than mad cows
We do not think that is a very good answer. Bovine Spongiform Encephalopathy (BSE) and Creutzfeldt-Jakob disease (CJD) are both prion diseases. We reported Mr. Rebarchek's death as being a case of confirmed CJD. We said CJD is related to BSE because they are both prion dieases.
Here's what the Centers for Disease Control has to say about Prion Diseases:
Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.
The causative agent of TSEs is believed to be a prion. A prion is an abnormal, transmissible agent that is able to induce abnormal folding of normal cellular prion proteins in the brain, leading to brain damage and the characteristics signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.
As our readers know, we've followed news and developments involving all kinds of Prion Disease and the investments being made in Prion research. So don't make this subject too cute and simple, no matter how many mad dogs there are in Kansas City.
