Self Management of Fatal Familial Insomnia. Part 1: What Is FFI?

Joyce Schenkein, PhD; Pasquale Montagna, MD

In This Article

Model of Death in FFI as a Result of Sleep Deprivation

FFI patients appear to have some "sleep" relief in a quasi-REM state. However, the REM state is associated with a significant rise in sympathetic activity (heart rate and blood pressure) compared with the waking state.[57] This suggests that the waking REM of FFI offers no protection from excessive sympathetic overdrive. A plausible hypothesis regarding early death in the Met-Met patient may be "burnout" of the sympathetic nervous system. Indeed, energy expenditure of FFI patients is very much higher than that of normal controls, which suggests severe metabolic exhaustion as a consequence.[34]


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