Fatal Insomnia - A Terrifying, Little Understood Disease [English]
282Fatal Familial Insomnia - a disease you may have heard of, as it's gained some notoriety on the internet despite its incredible rarity. I'm sure we've all felt insomnia before and know the frustration and the impact that just one or two bad nights of sleep can have on our functioning.
Fatal Insomnia has two forms, sporadic and familial, although the very large majority of cases are familial. The genes for FFI have been identified in fewer than 80 families across the world.
Fatal Insomnia is thought to be the result of a prion disease which results in damage to the thalamus. Over time, a person with the disease develops chronic insomnia and deteriorating health over several months as disease onset progresses.
This video not only covers fatal insomnia, but a detailed case report of one patient - a doctor himself who had lost family to the disease - who embarked on a medical quest to stave off symptoms and maintain quality of life as long as possible. His case is notable today for having greatly exceeded expectations about the progression of disease symptoms, and perhaps having laid out a blueprint for treatments for the disease moving forward.
References
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1781276/ - Case Report
https://rarediseases.org/rare-diseases/fatal-familial-insomnia/#:~:text=Fatal%20familial%20insomnia%20(FFI)%20is,significant%20physical%20and%20mental%20deterioration.
Cureffi.org
Attributions
Thumbnail image of eyes:
https://www.medicalnewstoday.com/articles/325989
Thalamus reference image:
https://library.neura.edu.au/schizophrenia/physical-features/brain-regions/thalamus/
Prion protein:
https://en.wikipedia.org/wiki/File:Prion_protein_immunostaining_(purple)_and_spongiform_change_in_the_brain_of_a_patient_with_Creutzfeldt-Jakob_Disease.jpg#filelinks
Sensory Deprivation Chamber:
https://commons.wikimedia.org/wiki/File:Flotation_Tank_Isolation_Tank.jpg
00:00 Intro
01:10 Basics of Fatal Insomnia
03:52 Report of Living With and Treating FFI
14:24 The Impact of This Experimentation
15:24 Education and Resources About Fatal Insomnia