Disease name: Fatal familial insomnia (FFI)
Affected populations: The disease affects an estimated 1 to 2 people per million every year, according to the National Organization of Rare Disorders. FFI is passed from parent to child, and between 50 and 70 families worldwide are believed to carry the genetic mutation that causes FFI. Males and females are equally likely to develop the condition.
Causes: FFI is a neurodegenerative prion disease that is caused by a mutation in a gene called PRNP, which produces a so-called prion protein. Prions are misfolded versions of normal proteins, and their abnormal shape is toxic to cells in the body, particularly neurons in the brain. One of the tissues that is primarily damaged in patients with FFI is the thalamus, a region of the brain that regulates an array of body functions including sleep, body temperature and appetite.
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