Fatal familial insomnia (FFI) is a very rare autosomal dominant inherited prion disease of the brain. It is almost always caused by a mutation to the protein PrPC, but can also develop spontaneously in patients with a non-inherited mutation variant called sporadic Fatal Insomnia (sFI). FFI has no known cure and involves progressively worsening insomnia, which leads to hallucinations, delirium, and confusional states like that of dementia. The average survival span for patients diagnosed with FFI after the onset of symptoms is 18 months.
The mutated protein, called PrPSc, has been found in just 40 families worldwide, affecting about 100 people; if only one parent has the gene, the offspring have a 50% chance of inheriting it and developing the disease. The first recorded victim was an Italian man, deceased in Venice in the year 1765.
Read more about Fatal Familial Insomnia: The Prion, Sporadic Fatal Insomnia, Presentation, Treatment, Normal Sleep and FFI, Related Conditions
Famous quotes containing the words fatal and/or familial:
“Alone he rides, alone,
The fair and fatal king:
Dark night is all his own,
That strange and solemn thing.”
—Lionel Pigot Johnson (1867–1902)
“That, of course, was the thing about the fifties with all their patina of familial bliss: A lot of the memories were not happy, not mine, not my friends’. That’s probably why the myth so endures, because of the dissonance in our lives between what actually went on at home and what went on up there on those TV screens where we were allegedly seeing ourselves reflected back.”
—Anne Taylor Fleming (20th century)