Mad Cow Disease?

Question

So you see, I got this wonderment from Libba Bray's soon-to-be new book and her main character. Her main character has Mad Cow Disease.

My question is: What is Mad Cow Disease?

I'm pretty sure it has to do with your brain some how - but then again, it could have to do with your toe - who knows? I sure don't. So if you would be so kind as to tell me what Mad Cow Disease is, I would be very happy.

Answer 1

Mad Cow Disease (also called bovine spongiform encephalopathy, BSE) is a fatal disease of cattle. It is caused by prions - proteins in the brain that become abnormally-shaped and spread, causing progressive brain damage. There was an epidemic of BSE in the UK in the 1980s-1990s and there were also quite a lot of cases in other countries (not in the USA though). It probably started when one cow developed BSE all by itself, then the remains of that cow were made into food for other cows. They then caught the disease and so it kept spreading. So to be picky, people can't have mad cow disease, only cows can. However, around 160 people (mostly in the UK, none in the USA) have caught BSE from eating beef from cows that had BSE. When people catch BSE it's called variant Creutzfeldt Jakob Disease, or vCJD. People who get this are usually around 15-30. They start off with psychiatric problems, then develop dementia and movement problems. It is fatal in around 2 years.

There are other types of CJD that have nothing to do with catching BSE, but they are often mistakenly called 'mad cow disease', which is confusing. They are also caused by prions and also cause progressive brain damage.

'Classical' CJD occurs randomly in around one out of every million people. People with it are usually 50-80 - they get dementia and coordination/movement problems. It is fatal in around 2 months to 2 years.

Inherited ('familial') CJD is genetic and runs in families. The symptoms are similar but it usually affects people a bit earlier that classical CJD and the disease lasts a bit longer.

All CJD is infectious but it does not spread easily. People have caught it from surgical instruments, cannibalism, blood transfusion and medicines made from human brain extracts.

There are some other similar disease caused by prions, such as Kuru (affected a tribe in Papua New Guinea who ate their dead), GSS (a very rare human disease), Chronic Wasting Disease (CWD - affects elk/deer), Scrapie (affects sheep).

Answer 2

Mad Cow Disease {Bovine Spongiform Encephalopathy} has a human equivalent which is known as Creutzfeld-Jakob Disease. It is caused by a prion {a virus-like protein} that invades the brain and other parts of the central nervous system and slowly destroys it. The brain turns into a sponge-like tissue, full of holes on it. Its first symptoms in humans are: quick deterioration of memory, judgment and thinking; trouble spelling words & doing simple math; visual disturbances; trouble walking & keeping body balance; and behavioral changes. As it progresses, total loss of vision, marked dementia, involuntary movements, loss of bowel and bladder control, coma and death quickly follows. It has a 100% mortality rate, with over 90% of patients dying within a year of appearance of the symptoms. There is no cure, treatment or vaccine for this disease; it's estimated that it happens in about 1 in 1 million people worldwide, but its incidence could be much higher because many cases are misdiagnosed as Alzheimer's Disease.

For more detailed information on Creutzfeld-Jakob Disease, go to the following links:

http://www.ninds.nih.gov/disorders/cjd/d...

http://www.nlm.nih.gov/medlineplus/ency/...

http://www.cdc.gov/ncidod/dvrd/cjd/

Answer 3

Mad Cow Disease is caused by protein like organisms, called prions, multiplying and slowly destroying the brain.  Not only can it be caught from handling a contaminated brain, but it occurs randomly in every so many thousand people(not quite sure of the exact figure) and scientists don't know why.

Answer 4

Mad Cow Disease is also known as Kuru or Creutzfeldt-Jakob disease.

Kuru is a rare and fatal brain disorder that occurred at epidemic levels during the 1950s-60s among the Fore people in the highlands of New Guinea. The disease was the result of the practice of ritualistic cannibalism among the Fore, in which relatives prepared and consumed the tissues (including brain) of deceased family members. Brain tissue from individuals with kuru was highly infectious, and the disease was transmitted either through eating or by contact with open sores or wounds. Government discouragement of the practice of cannibalism led to a continuing decline in the disease, which has now mostly disappeared.

Kuru belongs to a class of infectious diseases called transmissible spongiform encephalopathies (TSEs), also known as prion diseases. The hallmark of a TSE disease is misshapen protein molecules that clump together and accumulate in brain tissue. Scientists believe that misshapen prion proteins have the ability to change their shape and cause other proteins of the same type to also change shape. Other TSEs include Creutzfeldt-Jakob disease and fatal familial insomnia in humans, bovine spongiform encephalopathy in cattle (also known as mad cow disease), scrapie in sheep and goats, and chronic wasting disease in deer and elk.