Every person tries to be brave and fights their fears. But in fact, it is impossible to completely get rid of fear, because thanks to it we manage to live in our threatening world. If it were not for fear, we would take too many risks and would hardly be able to live long. But some people are still not afraid of anything – they simply cannot understand what fear is. If they are attacked by an armed robber, they will not even move and will calmly talk to him. If they are offered to jump with a parachute for a couple of thousand rubles, they will easily do it. People who do not know fear have a genetic mutation from birth that affects their brain function. They suffer from a rare disease that has only been found in about 300 people over the last hundred years.
There were about 300 people in the world who were really not afraid of anything
Contents
- 1 Disease that deprives you of fear
- 2 Symptoms of Urbach-Wiethe disease
- 3 Lack of fear in people
- 4 How people with Urbach-Wiethe disease live
- 5 Treatment of Urbach-Wiethe disease
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A disease that deprives you of fear
A disease that deprives people of a sense of fear is called Urbach-Wiethe syndrome. Scientists first suspected its existence in 1908. However, doctors Erich Urbach and Camillo Wiethe gave it a scientific description only in 1929. They called this disease lipoid proteinosis, but in most sources it is called after them, Urbach-Wiethe disease.
It is believed that the cause of Urbach-Wiethe disease is a mutation of the ECM1 gene. It leads to the accumulation of a substance called hyaline in the skin and brain. Because of it, people have serious problems with their skin, mucous membranes and even voice. Due to the accumulation of the substance in a certain part of the brain, a person stops experiencing fear.
The absence of fear in Urbach-Wiethe disease is pathological. Source: fishki.net
Since the official discovery of the disease about 100 years ago, only about 300 people with this diagnosis have been registered in the world. This is a genetic disease, so it can only be congenital; you cannot become infected with it. Sometimes a person has it, but does not cause symptoms. If a carrier of this mutation meets a significant other with the same change in the ECM1 gene, with a high degree of probability, they will have a child with all the symptoms of Urbach-Wiethe disease.
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Symptoms of Urbach-Wiethe disease
Most often, hyaline accumulation causes problems with the skin, respiratory tract and central nervous system. But in general, any internal organ can be damaged. Usually symptoms of lipoid proteinosis appear at the age of 1-3 years and accompany a person throughout his life.
People with Urbach-Wiethe disease often develop thick, rough skin, and their faces become mask-like. They develop thick patches of skin, papules, along their eyelids. With age, alopecia, or hair loss and bald spots, occurs. Due to the thickening of the mucous membranes and throat, people's voices become rough from an early age.
Lack of fear in people
Urbach-Wiethe disease affects a part of the brain called the amygdala. It is responsible for the feeling of fear, therefore, depending on the size of the situation, a person’s fear is either dulled or completely absent. At the same time, people with a rare disease may experience joy, sadness and other emotions. But they simply cannot understand fear – they cannot even recognize the fears of other people.
The location of the amygdala in the human brain. Source: scorcher.ru
There is only one way to scare people with Urbach-Wiethe disease. In one experiment, scientists gave subjects air with an increased content of carbon dioxide to breathe. It turned out that people with a rare diagnosis may have a fear of suffocation. At the same time, they easily touch poisonous animals, are not afraid of heights and are not even afraid of death.
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How people with Urbach-Wiethe disease live
Neuroscientists have been following some people with Urbach-Wiethe disease for decades. For example, in the USA there lives a woman who once did not panic after a robber pointed a gun at her – she did not even call the police. She was also beaten almost to death by her husband, but at that moment the woman also did not experience fear. These cases are known from scientific reports, but her name is kept strictly secret because if she becomes known, other people may start following her and even deliberately endanger her.
Treatment of Urbach-Wiethe disease
It is impossible to completely recover from Urbach-Wiethe disease. But cosmetics help with skin problems, and in order not to take unnecessary risks, people with this diagnosis always carefully weigh their actions from a logical point of view. In general, they are prone to extreme sports. The disease does not shorten their life expectancy, but they may die suddenly if they take unnecessary risks.
Scientists hope that monitoring people with a rare disease will help in developing a cure. Source: kostroma.mk.ru
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Urbach-Wiethe syndrome is very rare, and it can well be added to our article “The rarest and most unusual diseases in the world.” A person can also suffer from other diseases that are rarely mentioned anywhere. For example, did you know about the existence of “face blindness”?