Fatal Familial Insomnia is an
autosomal dominant inherited, non-infectious
brain disease. It is caused by
prions, similar to
Creutzfeldt-Jakob Disease and
Bovine Spongiform Encephalopathy. A certain
protein is altered (
asparagine-178 is replaced by
aspartic acid), causing
amyloid plaques[?] in the
thalamus, the region of the brain responsible for
sleep. The dysfunction of the thalamus results in
insomnia.
The average age for the disease to take place is 50. There are four stages, taking seven to eighteen month:
- Increasing insomnia, resulting in panic attacks and phobias. It lasts about four month.
- Hallucinations and panic attacks for about five month.
- Total insomnia, rapid loss of weight. Lasts about three month.
- Dementia, turning mute[?]. Lasts about six month. Always results in death.
There is no known cure; hopes rest on the so far unsuccessful gene therapy.