Article
Fatal familial insomnia and sporadic fatal insomnia.
Handbook of clinical neurology - 1 Jan 2018
Cracco Laura, Appleby Brian S, Gambetti Pierluigi
Abstract excerpt
Fatal familial insomnia (FFI) and sporadic fatal insomnia (sFI), or thalamic form of sporadic Creutzfeldt-Jakob disease MM2 (sCJDMM2T), are prion diseases originally named and characterized in 1992 and 1999, respectively. FFI is genetically determined and linked to a D178N mutation coupled with the M129 genotype in the prion protein gene (PRNP) at chromosome 20. sFI is a phenocopy of FFI and likely its sporadic...
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