Wednesday, September 11, 2013

What is Fatal Familial Insomnia (FFI)?

Fatal Familial Insomnia (FFI) is an extremely rare inherited autosomal dominant disorder of brain — most often caused by a protein mutation passed on through families. But FFI can also develop spontaneously with a non-inherited mutation variant called Sporadic Fatal Insomnia (sFI).

FFI is clinically characterized by insomnia with/without a diurnal dreaming state, hallucinations, delirium and dysautonomia preceding motor and connective deterioration.

The average age of onset in FFI is 50 but can range from 18-60 years.

Worldwide there are only 40 families which have been found to carry the disease, affecting about 100 people altogether.

If the parent has the mutated gene there will be 50% chance that the child will also inherit the disease.

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