On season 7 of the hit series 24, a disease spread by “prions” is said to threaten the United States, and even infects Jack Bauer. What are these prions and what can they do?
Danger level: High
What is it?
Creutzfeldt-Jakob Disease (CJD for short) is a rare brain disorder in which the brain undergoes degeneration.
Who gets it?
CJD occurs in 1 person out of a million. In Libyan-born Israelis and in some populations in restricted areas of Slovakia the disease is 60-100 times more common.
Other than those two examples, there is no race in which it is more common. It’s also as common in men as in women. In terms of age, it can occur anywhere between ages 17 and 83, but it’s more common around age 62.
CJD occurs randomly in 90% of cases, but in 10% it’s hereditary (in which case it happens at an earlier age).
What causes it?
Prions cause CJD. Prions are infectious materials, composed of protein. They can cause other diseases, as well, such as “mad cow disease” in cattle.
We normally have prion proteins in our brains (no one knows why). When proteins in our body are formed, they undergo folding. The prions in CJD are misfolded prion proteins. When they enter the brain, they cause the normal prion proteins in it to become misfolded themselves. The body can’t destroy those prions, and so they accumulate in the brain. They form plaques within the brain, called amyloid, which disrupt the normal brain tissue, causing “holes” in it, among other things.
This is how the brain tissue looks under the microscope when prions strike. You can see the holes it caused.
CJD can be transmitted from people infected by it in a few ways – If someone with CJD who died donates their eye’s cornea, for example. In the past, people treated with growth hormones were given a hormone made from human brain tissue. This could also transfer the disease.
How does it feel?
A few things can happen in CJD:
- Rapidly progressive dementia – Dementia is a decline in the ability to think, learn, in the sense of judgment and other thought-related things. In Alzheimer’s disease there is also dementia. But while there it takes years, here it develops rapidly, with people dying within a year.
The early signs of this are concentration, memory and judgment difficulties. - Myoclonus – Myoclonus is a jerky movement of the muscle. It exists here in more than 90% of people, and is usually caused after a startle.
These are the two main things than happen in CJD. Other things can happen as well, and include problems with speech, balance and walking problems, seizures.
The duration of the disease changes, between months (or even weeks) to years in some people.
This video by Dr. Michael Geschwind, of the University of California, explains more about the disease -
This video is from a news show after a case of CJD was discovered in the US -
How is it discovered?
There are a few ways to discover CJD -
- MRI brain imaging – Will show features which are characteristic of the disease.
- EEG – EEG (or electroencephalogram) shows graphically the activity of the brain (like EKG for the heart).
- Spinal fluid – It’s taken from the spinal cord, and will show an abnormal protein in the disease.
Another thing that can be done is brain biopsy.
How is it treated?
There is currently no treatment for CJD, and the disease is fatal.
The bottom line – How do I avoid it?
People in danger of getting CJD are health workers handling fluids and tissues from patients suspected of having CJD. These people must wear gloves and avoid direct contact with the tissue. (But even if they did contact it, there are methods of disinfection).
Regarding other people – As stated above, most of the time the disease occurs randomly, and there is no known way to prevent it.
You can read more in Terry Singeltary’s CJD blog here. She lost her mother to CJD.
What next?
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