vCJD - Mad Human Disease?

We all know a few, but no, that's not what it's called. vCJD is the human form of Mad Cow. It's named variant Creutzfeldt-Jakob disease, after the researchers who discovered it. vCJD is one of the family of human and animal diseases known as transmissible spongiform "encephalopathies" which in plain English simply means “sick brain.”

Spongiform refers to what happens to an infected brain, which ultimately becomes filled with holes much like a sponge. In cattle, the disease is called Bovine Spongiform Encephalopathy (BSE).

The term Mad Cow originated in the United Kingdom where the disease was first reported. It's a good description of what happens to an infected cow.

The disease effects balance and coordination causing the animal to wobble and stumble. Behavior can become erratic as you might expect from a disease that makes mush of the brain. Eventually the cow collapses, hence the term "downer cow." The downed cow then dies or is euthenized and disposed of in a way that keeps its parts out of all human and animal food chains. Or so we hope.

One way humans become infected with the vCJD is by eating the contaminated meat from cattle that were infected. Other possibilities are products such as marketed drugs made from the tissue and organs of cattle, and encapsulated herbs and vitamins. Think about it: gelatin is made from cattle hooves, bones, and hides – and it is also used in many types of medicine capsules. Gelatin is used in many processed foods as well. To avoid it, check labels carefully.

I know I was never thinking about mad cow while I ate gelatin desserts or popped my daily herbals...until now. Thank goodness for veggie capsules! There are several non-animal derived gelatin substitutes available. The one most like gelatin is called agar-agar. It comes from seaweed and is sold as a powder, in flakes or in bars. It's easy to use when cooking at home because you just substitute equal parts of it for gelatin.

Once infected, the disease progresses slowly for many years in the body without any symptoms. Eventually, a person with vCJD will begin to experience dementia, psychotic behavior, and coordination problems. Early symptoms may include trying to bite others and/or losing the ability to swallow. Usually, a few months after the initial symptoms occur they lose the ability to walk. Coma and death soon follow.

Once symptoms are present, vCJD progresses much faster than other types of dementia. Here's a list of 14 common and 2 possible symptoms:

• Personality changes
• Hallucinations
• Muscle twitching
• Muscle stiffness
• Nervous, jumpy feelings
• Changes in gait (walking, locomotion)
• Lack of coordination -- stumbling, falls
• Speech impairment
• Poor enunciation (hard-to-understand speech or mumbling)
• Sleepiness
• Delirium or dementia develops rapidly
• Deterioration in all aspects of brain function
• Profound confusion, disorientation

Additional symptoms that may be associated with vCJD:

• Memory loss
• Anxiety, stress, and tension

Since the year 1995, 147 cases were reported in Britain. Seven people had the disease in France. Canada, Ireland, Italy, and the United States had one person each with the disease. The resident of Canada and the resident of United States had both resided in Britain when they were infected. Other cases were found in as Bosnia-Herzegovina, Liechtenstein, Macedonia, Norway, Sweden, and Yugoslavia.

There is currently concern in the U.K. that vCJD is being misdiagnosed in children as Alpers’ syndrome.

As of today (July '06) there is no cure for vCJD, and the treatments used only help the infected person to manage the symptoms.

vCJD World Health Organization overview