Fatal insomnia

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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Fatal insomnia
File:Cranial imaging of a FFI patient.jpg
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Insomnia, hallucinations, delirium, confusion, autonomic dysfunction
Complications	N/A
Onset	Middle adulthood
Duration	Months to a few years
Types	Fatal familial insomnia, Sporadic fatal insomnia
Causes	Prion disease
Risks	Genetic mutation in the PRNP gene
Diagnosis	Polysomnography, Genetic testing, MRI
Differential diagnosis	Other prion diseases, Alzheimer's disease, Lewy body dementia
Prevention	N/A
Treatment	Supportive care
Medication	N/A
Prognosis	Poor, invariably fatal
Frequency	Extremely rare
Deaths	N/A

Fatal Insomnia

File:Cranial imaging of a FFI patient.jpg

File:Location of PRNP-gene in chromosome 20.png

File:FFI Diagrams.jpg

File:FFI timeline.jpg

Fatal insomnia is a rare disorder characterized by difficulty sleeping, which gradually worsens over time. Other symptoms may include speech and coordination problems, as well as dementia. This prion disease of the brain eventually leads to death within months to years.

Signs and Symptoms

Fatal insomnia progresses through four stages: Increasing insomnia, leading to panic attacks, paranoia, and phobias (about 4 months) Hallucinations and panic attacks become noticeable (about 5 months) Complete inability to sleep, accompanied by rapid weight loss (about 3 months) Dementia, unresponsiveness, or muteness, followed by death (about 6 months) Additional symptoms may include profuse sweating, pinpoint pupils, sudden menopause for women or impotence for men, neck stiffness, elevated blood pressure and heart rate, and constipation.

Cause

Fatal insomnia is caused by a mutation in the protein PrPC. It has two forms: Fatal Familial Insomnia (FFI), which is autosomal dominant, and Sporadic Fatal Insomnia (sFI), which is due to a non-inherited mutation.

Diagnosis

Diagnosis is typically based on a sleep study, PET scan, and genetic testing.

Treatments

There is currently no known cure for fatal insomnia. Treatment involves supportive care, and sleeping pills have not been found helpful.

Prognosis

The disorder is invariably fatal, with life expectancy ranging from 7 months to 6 years, and an average of 18 months.

Epidemiology

Cases of fatal insomnia have been reported in various countries, including Italy, the United States, and the Netherlands. The age of onset varies from 18 to 60 years, with an average of 50.

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See also

Prion Alliance

Creutzfeldt-Jakob Disease Prion Diseases Fatal Familial Insomnia Sporadic Fatal Insomnia