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  Fatal Familial Insomnia

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Definition of medical terminology for Fatal Familial Insomnia.
A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS.
These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA, ataxia, and a fatal outcome. Diseases in this category include CREUTZFELDT-JAKOB SYNDROME; GERSTMANN-STRAUSSLER-SCHEINKER SYNDROME; KURU; SCRAPIE; fatal familial insomnia; bovine spongiform encephalopathy (see ENCEPHALOPATHY, BOVINE SPONGIFORM

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