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Fatal
familial insomnia is a rare brain disease which gradually makes it
impossible for a person to fall asleep. This inability to sleep
becomes fatal. Fatal familial insomnia is classified as an autosomal
dominant disorder characterized by degeneration of the thalamus and
progressive insomnia. It is caused by a mutation in the prion protein
(Petersen, R.B., Tabaton, M., at el. "Analysis of the prion
Protein gene in Thalamic
Dementia" Neurology, 42 (10):
1859-1863 (1992).).
Fatal
familial insomnia is classified as a rare disease. It affects less
than 200,000 people in the United States. The age of contraction of
Fatal familial insomnia ranges from 30 to 60, with an average age of
50. After onset of the condition death is inevitable and will occur
within 7 and 36 months. The disease has four stages:
The
onset of chronic insomnia, which increases in severity and is
accompanied by panic attacks and phobias. This stage last for about
four months.
Hallucinations
and panic attacks which go on for some five months.
Complete
insomnia accompanied by rapid weight loss, lasting for about three
months.
Dementia,
after which the sufferer turns mute. This lasts about six months
after which the person will die.
Sleeping
pills have no effect on this condition. At this stage there is no
cure for fatal familial insomnia, although hope does rest with gene
therapy.
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