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Fatal Insomnia


Fatal insomnia is a very rare disease of the brain where the patient progressively sleeps less and less until they become completely sleepless. The condition is, as its name suggests, fatal. The illness is due to a genetic condition affecting a very small number of families around the world. The responsible gene is so strong that if one parent has it, their children have a 50% chance of inheriting the condition and developing the disease.

History
Fatal insomnia was discovered in 1974, when an Italian doctor came across two women from the same family who apparently died of insomnia. Their family history showed other related deaths. Another member of the family became unwell in 1984 and his brain was studied after his death.

The genetic mutation affects  the thalamus, an area of the brain responsible for the regulation of sleep.

Symptoms
The age of onset ranges between 30 to 60, often following childbirth. Death usually occurs between 7 and 36 months from onset. The presentation of the disease differs considerably from person to person, even among patients from within the same family.

The condition has four stages, taking 7 to 18 months until the end:

  1. Increased insomnia, resulting in panic attacks, paranoia, and phobias. This stage lasts for about four months.
  2. Hallucinations and panic attacks are more noticeable and continue for about five months.
  3. Total inability to sleep, followed by rapid weigh loss, lasting approximately three months.
  4. Dementia and non-responsiveness over a period of six months. This is the final stage of the disease, and the patient subsequently dies.

Treatment
There is no present cure or treatment for the condition. Sleeping pills are not helpful, and actually make the condition worse. Gene therapy is being investigated for future possibility.


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