matty
05-30-2007, 04:46 AM
WEIRD.
http://en.wikipedia.org/wiki/Fatal_familial_insomnia
My favorite parts:
...his deterioration was studied and after his death, his brain was flown to the U.S. for further investigation.
In the late 1990s, researchers discovered that the disease is caused by a dual mutation in a protein called a prion protein (PrP): aspartic acid-178 is replaced by asparagine while methionine is present at amino acid 129. These mutations result in the formation of an insoluble prion protein, termed PrPsc. PrPsc has autocatalytic properties that cause normally soluble PrP to be converted into the PrPsc form upon interaction.
say what?
The disease has four stages, taking 7 to 18 months to run its course:
1. The patient suffers increasing insomnia, resulting in panic attacks and phobias. This stage lasts about four months.
2. Hallucinations and panic attacks become noticeable, continuing about five months.
3. Complete inability to sleep is followed by rapid loss of weight. This lasts about three months.
4. Dementia, turning unresponsive or mute over the course of six months. This is the final progression of the disease, and the patient will subsequently die.
There is no cure or treatment for FFI; hopes rest on the so far unsuccessful gene therapy. Sleeping pills have no effect.
http://en.wikipedia.org/wiki/Fatal_familial_insomnia
My favorite parts:
...his deterioration was studied and after his death, his brain was flown to the U.S. for further investigation.
In the late 1990s, researchers discovered that the disease is caused by a dual mutation in a protein called a prion protein (PrP): aspartic acid-178 is replaced by asparagine while methionine is present at amino acid 129. These mutations result in the formation of an insoluble prion protein, termed PrPsc. PrPsc has autocatalytic properties that cause normally soluble PrP to be converted into the PrPsc form upon interaction.
say what?
The disease has four stages, taking 7 to 18 months to run its course:
1. The patient suffers increasing insomnia, resulting in panic attacks and phobias. This stage lasts about four months.
2. Hallucinations and panic attacks become noticeable, continuing about five months.
3. Complete inability to sleep is followed by rapid loss of weight. This lasts about three months.
4. Dementia, turning unresponsive or mute over the course of six months. This is the final progression of the disease, and the patient will subsequently die.
There is no cure or treatment for FFI; hopes rest on the so far unsuccessful gene therapy. Sleeping pills have no effect.