Fatal familial insomnia, hereditary, nerve degrading, insomnia

Q.  Which of the following is/are true?

1) Fatal familial insomnia (FFI) is a hereditary disease.
2) It degrades the nerves, causing a relentless insomnia and eventually death.Fatal familial insomnia, hereditary, nerve degrading, insomnia
- Published on 22 Feb 16

a. Only 1
b. Only 1
c. Both 1 and 2
d. Neither 1 nor 2

ANSWER: Both 1 and 2
 
  • Fatal Familial Insomnia (FFI) is a one-in-a-million hereditary disease that results from a malfunctioning protein that degrades the nerves, causing a relentless insomnia and eventually death.
  • 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.

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