出典:Wiktionary
出典:Wikipedia
出典:『Wikipedia』 (2011/05/31 18:27 UTC 版)
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 it can also develop spontaneously in patients without the inherited mutation in a variant called sporadic fatal insomnia (SFI). 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 disease's genesis and the patient's progression into complete sleeplessness is untreatable and ultimately fatal. The first recorded victim was an Italian man, deceased in Venice in the year 1765.