1) Fatal familial insomnia (FFI) is caused by
a. Prions
b. Virus
c. Bacteria
d. Fungus
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Answer
Explanation
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ANSWER: Prions
Explanation:
- Fatal familial insomnia (FFI) is an extremely rare autosomal dominant inherited prion disease of the brain.
- Prion diseases are a class of ailments that afflict humans as well as animals. ‘Mad cow disease’, for example, is a prion disease that can affect bovines and can be contracted from eating infected meat.
- Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of progressive conditions that affect the brain (encephalopathies) and nervous system of many animals, including humans.
- Prion is composed entirely of protein material, called PrP (short for prion protein)
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2) 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
a. Only 1
b. Only 1
c. Both 1 and 2
d. Neither 1 nor 2
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Answer
Explanation
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ANSWER: Both 1 and 2
Explanation:
- 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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