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Fatal familial insomnia

     

Fatal Familial Insomnia (FFI) is a rarest of the rare genetic sleeping disorder. All across the world, it is diagnosed or known in only less than 40 families. There has been a famous BBC documentary on this illness in which a Chicago based music teacher, Michael Corke was featured.

This disease starts as an unexpected and unexplained insomnia during the middle age years which develops into fatal insomnia. The causes are genetic mutation and it leads to a type of prion disease something like Mad Cow disease or Laughing disease or Alzheimer's disease.

Some of the additional symptoms of FFI include uncontrolled movement or coordination. The disease could  persist for less than a year to a few years based on the symptoms severity. The normal life of person with this disease diagnosed is about 12 to 18 months and mortality is 100%.

What are Prions?

These are the missing genetic link between all these diseases mentioned above. They tend to tell us about the protein molecules and their nature. , They also tell us the ability of these molecues to cause death. In this particular disease, there are examples of an Italian family who has unwittingly passed FII disease through the generations for multiple centuries.

Strangely, the very first symptom of the disease shows itself only after 40 years of age. Gene site dormancy or brain cells vulnerability during middle years can be the reason behind this.

When FII was discovered and what are the symptoms?

Fatal Familial insomnia is a very puzzling disease which was officially announced and discovered in middle of first decade of 21st century.  Since most of the symptoms of this disease match that of dementia, end-stage alcoholism and encephalitis, it took time to classify it as a separate condition. The first symptom of FII is inability to get asleep. This is often accompanied with high blood pressure, sweating, and loss of motor skills. There are many stages of FII with progressive severity:

  1. Sudden insomnia causes panic attack and phobia and lasts for 3-4 months.
  2. Severe panic attacks and hallucinations lasting for another 4-5 months
  3. The nest stage is total insomnia which causes fast weight loss and very limited mental functioning. This goes for another 3-4 months.
  4. The last stage is dementia and unresponsiveness, which can go up to six months.

There are no currently available treatment, cure or symptoms relaxer for this disease.


Sometimes crying or laughing
are the only options left,
and laughing feels better right now.




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