I read an interesting article the other day. It seems this woman (only identified as SM) isn’t afraid of anything. When she’s exposed to things that normally scare people (spiders, threats) she barely feels anything. She has been in violent situations, scary situations (domestic violence, held up at knifepoint), but still isn’t afraid. Doctors suspect the reason is lesions her amygdala. And I want to know more.
Part 1: The Disease
Urbach-Wiethe disease causes physical symptoms like thickening of the skin and mucous membranes, and can cause epilepsy, attacks of rage and mental retardation. There are other neurological symptoms, too, including schizophrenia and mood disorders. So how does this happen?
U-W is caused by (according to this article) “a defect in the metabolism of basement membrane collagen.” For everyone who isn’t a doctor, the basement membrane according to Medterms.com is “A thin membrane upon which is posed a single layer of cells…made up of proteins held together by type IV collagen.” It’s located directly under the epidermis, hence the reason it’s called “basement.” In UW disease, the basement membrane doesn’t regenerate (metabolize) properly, resulting in lipids (fats) being created throughout the basement membrane. This results in a lot of problems in the skin, mucous membranes, eyes and even the brain. The disease is usually detected in children because of reports that their voice is hoarse and cuts and scrapes don’t heal properly. The disease causes both; the hoarseness is caused by the calcification of the vocal cords among other things, while the scars are caused by improper healing. Check here for more.
The calcification builds up and affects other parts of the body: the eyes, scalp, and of course, the brain.
So now we know how the brain is altered. Tune in tomorrow as we take a closer look at the amygdala, the fear center of the brain.