Found a short reference to Fatal Familial Insomnia in my Ganong
Physiology textbook (Oxford textbook of medicine turned up nothing).
"Fatal Familial insomnia is a progressive disease with autosomal
dominant inheritance that is characterised by worsening insomnia,
impaired autonomic and motor functions, dementia and death. Patients
with the disease have severe neuronal loss and gliosis in the ventral
and mediodorsal nuclei of the thalamus. It has now been shown to be a
prion disease that is transmissible to animals and is a member of the
same family of diseases as Creutzfeldt-Jakob disease and kuru"
- Ganong, Review of Medical Physiology, pg 192
it's quite interesting that there are now quite number of prion
diseases out there ever since the paradigm shift.
