Prion Pie

February 16, 2011

I am amazed at the variety and ingenuity of diseases. Human beings are large animals and not easy to kill, but there are illnesses that find remarkable ways to do so.

Fatal insomnia is a disease caused by infectious proteins called prions (normal proteins that have become incorrectly folded), which spread throughout the brain like dominoes and destroy healthy tissue. Prions are responsible for several other diseases, all of which are fatal and leave the brain riddled with tiny holes, like a sponge. Fatal insomnia is passed down genetically or can arise spontaneously through a mutation. It causes a progressive inability to sleep, ultimately leading to death.

(From Wikipedia)
The disease has four stages, taking 7 to 18 months to run its course:

  1. The patient suffers increasing insomnia, resulting in panic attacks, paranoia, and phobias. This stage lasts for about four months.
  2. Hallucinations and panic attacks become noticeable, continuing for about five months.
  3. Complete inability to sleep is followed by rapid loss of weight. This lasts for about three months.
  4. Dementia, during which the patient becomes unresponsive or mute over the course of six months. This is the final progression of the disease, resulting in death.

Other symptoms include profuse sweating, pinprick pupils, the sudden entrance into menopause for women and impotence for men, neck stiffness, and elevation of blood pressure and heart rate. Constipation is common as well.

Prions are also responsible for a disease called kuru, which is most famously spread through cannibalism. It is characterized by headaches, joint pain, shaking of the limbs, difficulty moving, incontinence, and bursts of pathological laughter. From the onset of symptoms, death usually occurs in 3-6 months.

Have a nice day, and watch out for prions!


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