000008346 001__ 8346 000008346 005__ 20240416120306.0 000008346 0247_ $$2DOI$$a10.6083/gb19f633j 000008346 037__ $$aIR 000008346 041__ $$aeng 000008346 245__ $$aSporadic fatal insomnia: a case report 000008346 260__ $$bOregon Health and Science University 000008346 269__ $$a2020 000008346 336__ $$aAbstract 000008346 520__ $$aThe most common prion disease is Creutzfeldt-Jacob disease (CJD), usually sporadic (sCJD), but in up to 15% familial due to mutations in the prion gene. CJD presents with myoclonis, ataxia, and rapidly progressive dementia with an average age of onset of 67 years. Fatal Familial Insomnia (FFI) is a related uniformly fatal prion disease, with mean onset of dysautonomia and insomnia at 56 years and mean duration of 13 months. 000008346 540__ $$fCC BY 000008346 542__ $$fIn copyright - joint owners 000008346 650__ $$aCreutzfeldt-Jakob Syndrome$$021092 000008346 650__ $$aInsomnia, Fatal Familial$$034537 000008346 650__ $$aPrion Diseases$$029683 000008346 650__ $$aNeurodegenerative Diseases$$031750 000008346 6531_ $$afatal familial insomnia 000008346 6531_ $$asporadic fatal insomnia 000008346 6531_ $$asleep disorders 000008346 691__ $$aSchool of Medicine$$041369 000008346 692__ $$aDepartment of Pathology$$041438 000008346 7001_ $$aAlrohaibani, Alaaeddin$$uOregon Health and Science University$$041354 000008346 7001_ $$aWoltjer, Randall$$uOregon Health and Science University$$041354 000008346 711__ $$aResearch Week$$uOregon Health and Science University$$d2020 000008346 8564_ $$99d41c538-ac4d-4692-8e81-fb6162cacc91$$s44497$$uhttps://digitalcollections.ohsu.edu/record/8346/files/ResearchWeek.2020.Alrohaibani.Alaaeddin.pdf 000008346 905__ $$a/rest/prod/gb/19/f6/33/gb19f633j 000008346 980__ $$aResearch Week