Fatal familial insomnia (FFI)

Etiology

Mechanism

  • Normal sleep has different stages that together last 90 to 100 minutes:

    FFI patients cannot go past stage 1 and thus their brains are not getting the rest they need to revive, as most reviving and repairing processes of the body are believed to happen during these deeper sleep stages.

Treatment/Management

  • no known cure
  • sleeping pills and barbiturates are unhelpful and have been shown to worsen the clinical manifestations and hasten the course of the disease

Manifestation: Signs and Symptoms

  • progressively worsening insomnia
  • hallucinations
  • delirium
  • dementia-like confusion
  • random muscular jerks

Prognostication:

  • terminal around 18 months following symptom onset 

Epidemiology:

There are 25 reported families in the world that carry the gene for FFI: 5 Italian, 2 French, 4 American, 1 Japanese, 2 Australian, 8 German, 1 Austrian, and 2 British.

Source:

http://en.wikipedia.org/wiki/Fatal_familial_insomnia

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