NH man who died of human Mad Cow Disease may have infected others

This tissue slide shows sponge-like lesions in the brain tissue of a classic CJD patient. This lesion is typical of many prion diseases.This tissue slide shows sponge-like lesions in the brain tissue of a classic CJD patient. This lesion is typical of many prion diseases.

A case of the human form of mad cow disease —  the always fatal  Creutzfeldt-Jakob disease — has just been confirmed in New Hampshire.

Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is a fatal transmissible neurodegenerative disease in cows that causes a spongy degeneration in the brain and spinal cord. BSE has a long incubation period, about 30 months to 8 years.

Mad cow disease’s infectious agent is neither a bacterium nor a virus. Instead, the infectious agent is a protein called prion that cannot be destroyed even if the beef or material containing the prions is cooked or heat-treated. Prions remain viable even in high temperatures of over 600 degrees Celsius!

BSE is caused by cattle, which are normally herbivores, being fed the remains of other cattle in the form of infected meat and bone meal. Another contributory factor was the feeding of infected protein supplements to very young calves. The origin of the disease itself remains unknown.

When humans ingest BSE-infected meat — especially brain, spinal cord, and digestive tract tissue, although the infectious agent can be found in virtually all tissues, including blood — what results is Creutzfeldt-Jakob disease (CJD).

Before controls on high-risk offal were introduced in 1989, between 460,000 and 482,000 BSE-infected animals had entered the human food chain. By October 2009, CJD had killed 166 people in the United Kingdom — the country most affected — and 44 elsewhere.

mad cow disease

Greg Botelho reports for CNN, Sept. 21, 2013, that health officials have confirmed that a patient who underwent neurosurgery at a New Hampshire hospital earlier this year had Creutzfeldt-Jacob disease — a rare, rapidly progressing and always-fatal degenerative brain disease.

The diagnosis of Creutzfeldt-Jakob disease was confirmed by the National Prion Disease Pathology Surveillance Center, which reported its conclusion to New Hampshire’s health department and Catholic Medical Center on Friday.

The now-deceased patient had undergone neurosurgery at Catholic Medical Center in Manchester. But by the time CJD was suspected, equipment used in the patient’s surgery had been used several other operations. This raised the possibility that the equipment might have been contaminated — especially since normal sterilization procedures are not enough to get rid of the prions — thus potentially exposing the other patients to infection. So authorities in two states issued a warning that as many as 13 patients may have been exposed to surgical equipment used during the patient’s surgery, thus to the same disease.

People with Creutzfeldt-Jakob disease typically show signs of rapidly progressing dementia, impaired vision and personality changes, among other symptoms, according to the National Institutes of Health. Yet while it can be suspected, the only way the disease can be confirmed is through tests conducted after a person’s death.

Creutzfeldt-Jakob disease has a long incubation period before symptoms appear — sometimes up to 50 years — according the National Institutes of Health. There’s no test, so it may take many years before these patients would know if they were infected.

About 300 people a year in the United States come down with Creutzfeldt-Jakob disease, according to the U.S. Centers for Disease Control and Prevention (CDC). The CDC has said that no cases of the disease linked to the use of contaminated medical equipment have been reported in the United States since 1976.

Most medical devices are sterilized by heat, but the World Health Organization recommends the use of a caustic chemical like sodium hydroxide to disinfect equipment that may have come in contact with tissues that could cause Creutzfeldt-Jakob disease.

That’s just one reason — a minor reason — why I don’t eat meat.


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6 years ago

BSE is a prion based disease. Wildlife has an abundance of it. We have had several deer kills in this area due to wasting disease–prion based. I’m afraid there isn’t much you can do, other than buy quality meat. I for one am confident my meat supply is good. We raise our own.

6 years ago

Know your local farmers if you are not in a position to raise your own. The Weston A. Price Foundation has lots of information concerning nutrition, and they have chapters all over the U.S. and some in Europe. Good place to find a farmer who uses good farming practices. I’ve learned so much from them. We’ve been sold a lot of lies concerning nutrition.

6 years ago

Just a thought on this one. If farmers are mixing their own feed or buying it there are protein additives mixed in when grinding this feed. Unless these additives are garanteed not to have animal by-products in them you might not be safe then. But on the other side of the coin we can’t become so scared to purchase meat and become protein starved zombies. Situations liked this have become more nurmous since importing to many of these by-products from outside the country. Look at dog food contamination in the past five years.

6 years ago

Wait a minute! What I’m saying is nature has prions. A bird could eat some left over deer meat, the cat eats the bird, and what do you know, you’ve got prions in your house!

As for feedlots, yes, they do use a lot of byproducts of ethanol, DGEs. That is really good feed. Totally vegetable based.

6 years ago

Thank you Dr. Eowyn for this revealing post. It is certainly frightening and sobering. I will pray for the man who died of this disease. How horrible he must have suffered.

6 years ago

We ate plenty of the European beef in the 80s and 90s during the big Mad Cow scare, while stationed overseas. And to this day, we are not allowed to give blood.


[…] Eowyn is the Editor of Fellowship of the Minds and a regular contributor to The D.C. […]

4 years ago

That explains a lot.
Debbie and Hillary, and Nancy and Michele, and that skanky loudmouth professor at UMN. They all got mad cow disease which is spread when you believe your own bullshit.