Familial Fatal Insomnia

An extremely rare autosomal dominant prion disease characterised by worsening insomnia that begins between age 30 and 60; FFI results in declining health—for lack of rest—progressive difficulties in talking, walking, mental activity; death occurs 6 months to 3 years after onset
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In vivo detection of thalamic gliosis: A pathoradiologic demonstration in familial fatal insomnia. Arch Neurol 2008;65:545-9.
Familial fatal insomnia involves the thalamus, and Heidenhain's variant involves the occipital lobes.
First reported in 1986, familial fatal insomnia (FFI) is a hereditary prion disease.

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