President Franklin D. Roosevelt is quoted to have said, “The only thing we have to fear is fear itself.” Perhaps for some, this quote would prove untrue and a correction should be made: “the only thing we have to fear is no fear.”
Such is the case for patients with Urbach-Wiethe syndrome. This is a recessive genetically inherited disease that causes calcification on the medial temporal lobes of the brain. These calcifications can affect the amygdala (two groups of nuclei located within the temporal lobes of the brain, and are involved with memory, decision making, and emotional responses). The amygdala is particularly associated with the emotional feeling of ‘fear’ and as such, Urbach-Wiethe disease patients may suffer from an impaired ‘fear’ response—and thus may not feel fear at all.
Urbach-Wiethe disease is caused by mutations in chromosome 1 and 1q21, the extracellular matrix protein 1 (ECM1 gene). These mutations cause a thickening of skin and mucous membranes, which subsequently can cause a weak cry or hoarse voice due to a thickening of the vocal cords. Scars and lesions can also be present on the skin as there is a poor ability to heal and scarring can increase with age. Hair loss and dental issues may also be present.
There is currently no cure for this disease; however certain symptoms can be treated, such as the increased mucous in the respiratory tract. Patients can live normal lives assuming these symptoms are treated and taken care of.
Perhaps one may speculate that lacking the ability to experience fear may be the least troublesome symptom for such patients. However, this may in fact prove to be incorrect.
The feeling of fear exists to protect an individual the same way the feeling of pain warns of something wrong in order for corrective measures to be taken. Those who do not feel pain can inflict great damage upon themselves without even realizing.
The same is true for those who do not feel fear, as related from a woman who does suffer from this disorder and who conveyed her experience to YahooNews.
“Years ago, when my three sons were small, I was walking to the store and I saw this man on a park bench. He said, ‘Come here, please.’ So I went over to him. I said, ‘What do you need?’ He grabbed me by the shirt, and he held a knife to my throat and told me he was going to cut me. I told him—I said, ‘Go ahead and cut me.’… I wasn’t afraid. And for some reason, he let me go. And I went home.”
This life without fear can expose a person to dangers that she could otherwise have avoided—in all likelihood, someone who could feel fear would never have even approached such a man in the first place, even upon his request.
This situation illustrates that despite the negative associations with feelings of pain and fear, in reality, they provide us with the truest forms of protection we could ask for. Sometimes, it is only through the absence of certain functions that we take for granted that we realize their importance and significance to our daily survival.