All of us experience the odd bad night of sleep. Some of us have had a dose of insomnia during our lives. Or maybe you’re suffering from chronic insomnia right now. Whatever your sleep problems may be, you can be very glad you’re not suffering from Fatal Familial Insomnia.
What is Fatal Familial Insomnia
Fatal Familial Insomnia, also known as Sporadic Fatal Insomnia, is a very rare genetic disorder recorded in only 50 families worldwide.
It was first detected in 1974 by Dr Ignazio Roiter from Italy. He found two women who had supposedly died of insomnia. Looking in their family records, he noticed that other members of their family had died in the same way. Another member of the family was found to be suffering with the very same symptoms. Scientists monitored their condition and when they died, their brain was flown to America for further investigation.
The condition was found to be caused by a faulty gene. It affects the part of the brain responsible for sleep, the thalamus. The job of the thalamus is to slow down your body’s signal transfers to allow us to reach the unconscious state we know as sleep. The mutated gene prevents this from happening and so sleep cannot occur.
The faulty gene is a dominant. This means if one parent has the gene, their children have a 50% chance of having the disease sometime in their lives. The average age in which people suffer from the disease is 49, so parents may not know they have the disease before passing it to their children.
The same gene is also thought to be responsible for the human version of BSE or Mad Cow Disease and it shows many of the same qualities. Rats fed brain tissue from infected individuals begin showing signs of the disease. The disease has also been found in a man with a family who had no previous record of the disease. This leads scientists to thinking the disease previously thought to be only genetic may also be somewhat infectious.
Symptoms of Fatal Familial Insomnia
Fatal Familial Insomnia is very slow in its progression. At first, people start having problems falling to sleep. This may also be accompanied with muscle spasms, twitches and stiffness. This develops over time until they find themselves unable to fall asleep at all.
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