This Discovery documentary looks at a very rare and unusual prion disease called Fatal Familial Insomnia, or FFI for short.
FFI is a prion disease that targets the thalamus, which among its many functions is the body’s sleep regulator. Prions form aggregates of amyloid plaques that destroy neurons in the area.
The age of onset varies from 30 to 60 years old, with an average of 50 years old. However, the disease usually occurs later in life, mainly after birth.
Death usually occurs between 7 and 36 months after onset. The presentation of the disease varies from person to person, even among patients from the same family.
The disease has four stages that take 7 to 18 months to develop:
The patient’s insomnia became increasingly severe, leading to panic attacks, paranoia, and phobias. This phase lasts about four months.
Hallucinations and panic attacks become apparent and last about five months.
Weight loss can be rapid after complete insomnia. This lasts for about three months.
Dementia in which patients become unresponsive or dumb within six months. This is the final progression of the disease and the patient subsequently dies.
Other symptoms include profuse sweating, pinched pupils, sudden menopause in women and impotence in men, stiff neck, and increased blood pressure and heart rate. Constipation is also common.
