Preferred Label : Dementia due to fatal familial insomnia;
ICD-11 definition : Dementia due to an autosomal dominant hereditary disease linked to mutation in the
prion protein gene. It has a rapidly progressive course. Concomitant symptoms include
insomnia, autonomic dysregulation, and sometimes incoordination and movement disorder.;
ICD-11 synonym : Dementia due to FFI; Dementia due to FFI - [fatal familial insomnia]; fatal familial insomnia;
Origin ID : 467518334;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- See also inter- (CISMeF)
Dementia due to an autosomal dominant hereditary disease linked to mutation in the
prion protein gene. It has a rapidly progressive course. Concomitant symptoms include
insomnia, autonomic dysregulation, and sometimes incoordination and movement disorder.
